Sever Pop

ENT BASICS

Coperta: Patricia Puca Editur acreditat CNCSIS (24)

Sever Pop, 2011

ISBN Descrierea CIP este disponibil la Biblioteca Naional a Romniei

Sever Pop

ENT BASICS

Casa Crii de tiin Cluj-Napoca, 2011

CONTENTS
I. EAR 1. EAR BASICS ............................................................................. 11
1.1. Clinical anatomy ................................................................ 11 1.2. Physiology of the ear.......................................................... 15 1.3. Symptoms of ear diseases .................................................. 18 1.4. Clinical examination .......................................................... 21

2. DISEASES OF THE EXTERNAL EAR .................................................. 31

2.1. Diseases of the auricle ........................................................ 31 2.2. Diseases of the external auditory canal .............................. 34

3. DISEASES OF THE MIDDLE EAR ..................................................... 42

3.1. Trauma ............................................................................... 42 3.2. Bullous myringitis .............................................................. 47 3.3. Acute suppurative otitis media (AOM) ............................. 48 3.4. Otitis media with effusion (OME) .................................... 51 3.5. Chronic suppurative otitis media ....................................... 53 3.6. Otosclerosis........................................................................ 60

4. DISEASES OF THE INNER EAR ....................................................... 63

4.1. Sudden sensorineural hearing loss (SSHL) ....................... 63 4.2. Progressive sensorineural hearing loss ................................ 65 4.3. Inner ear infections ............................................................ 71 4.4. Management of hearing impairment ................................. 74

5. PERIPHERAL VESTIBULAR SYNDROME ............................................. 78

5.1. Vertigo ............................................................................... 78 5.2. Examination of the vestibular function .............................. 79 5.3. Vertigo: clinical syndromes ................................................ 82

6. REFERENCES ............................................................................. 91 II. NOSE 1. NOSE AND PARANASAL SINUSES BASICS......................................... 95

1.1. Clinical anatomy ................................................................ 95 1.2. Physiology of the nose ..................................................... 101 1.3. Symptoms of nasal diseases.............................................. 102 1.4. Clinical examination ........................................................ 104 1.5. Clinical investigations ...................................................... 108

2. DISEASES OF THE EXTERNAL NOSE AND NASAL VESTIBULE ...............112

2.1. Acute nasal infections ...................................................... 112 2.2. Trauma ............................................................................. 114 2.3. Dermatological conditions ............................................... 115 2.4. Tumors ............................................................................. 116

3. RHINITIS ................................................................................120
3.1. Acute rhinitis ................................................................... 120 3.2. Chronic specific rhinitis ................................................... 122 3.3. Atrophic rhinitis .............................................................. 127 3.4. Nasal manifestations in systemic diseases ........................ 128 3.5. Allergic rhinitis ................................................................ 131 3.6. Non-allergic perennial rhinitis ......................................... 134 3.7. Nasal polyposis ................................................................ 137

4. RHINOSINUSITIS ......................................................................140
4.1. Acute rhinosinusitis ......................................................... 140 4.2. Chronic rhinosinusitis...................................................... 143 4.3. Fungal rhinosinusitis ........................................................ 145

5. EPISTAXIS ..............................................................................147
5.1. Definition. Classification ................................................. 147 5.2. Aetiology ......................................................................... 147 5.3. Management .................................................................... 148

6. TUMORS OF THE NOSE AND PARANASAL SINUSES...........................150

6.1. Benign tumors ................................................................. 150 6.2. Malignant tumors ............................................................ 150

7. REFERENCES ...........................................................................154 III. PHARYNX 1. PHARYNX BASICS ....................................................................159

1.1. Clinical anatomy .............................................................. 159 1.2. Phisiology ........................................................................ 164 1.3. Symptoms of pharyngeal diseases .................................... 165 1.4. Clinical examination ........................................................ 166

2. ACUTE AND CHRONIC PHARYNGEAL INFECTION .............................170

2.1. Acute viral pharyngitis ..................................................... 170 2.2. Acute bacterial pharyngitis (Follicular tonsillitis) ............ 171 2.3. Acute adenoiditis ............................................................. 173 2.4. Suppurative complications of acute bacterial pharyngitis. 174 2.5. Specific acute bacterial pharyngitis .................................. 177 2.6. Specific viral pharyngitis .................................................. 180 2.7. Pharyngeal manifestations in blood disorders .................. 182 2.8. Chronic non-specific conditions ...................................... 183 2.9. Chronic specific pharyngitis............................................. 188

3. TUMOURS OF THE PHARYNX ......................................................193 8

3.1. Tumors of the nasopharynx.............................................. 193 3.2. Tumors of the oropharynx................................................ 198 3.3. Tumors of the hypopharynx ............................................. 202

4. NEUROLOGICAL DISORDERS OF THE

PHARYNX ..............................204

4.1. Sensory disorders ............................................................. 204 4.2. Motor disorders ............................................................... 205

5. FOREIGN BODIES IN THE PHARYNX ..............................................207 6. REFERENCES ...........................................................................208 IV. LARYNX 1. LARYNX BASICS.......................................................................211
1.1. Clinical anatomy .............................................................. 211 1.2. Phisiology ........................................................................ 216 1.3. Symptoms of laryngeal diseases ....................................... 217 1.4. Clinical examination ........................................................ 218

2. INFLAMMATORY DISEASES .........................................................223

2.1. Acute laryngitis ................................................................ 223 2.2. Acute edematous subglottic laryngitis.............................. 224 2.3. Acute epiglottitis.............................................................. 225 2.4. Laryngeal diphtheria........................................................ 225 2.5. Acute laryngeal edema ..................................................... 226 2.6. Chronic nonspecific laryngitis.......................................... 227 2.7. Chronic specific laryngitis................................................ 229 2.8. Pseudo-myxomatous laryngitis (Reinkes edema) ............ 232 2.9. Vocal fold polyp ............................................................... 233 2.10. Vocal nodules ................................................................. 234

3. TUMORS OF THE LARYNX ..........................................................236

3.1. Benign Tumors ................................................................ 236 3.2. Malignant tumors ............................................................ 237

4. LARYNGEAL NERVE DISORDERS ..................................................243

4.1. Laryngeal nerve palsy....................................................... 243

5. TRACHEOSTOMY ......................................................................247
5.1. Definition. History .......................................................... 247 5.2. Indications ....................................................................... 247 5.3. Techniques ....................................................................... 248 5.4. Postoperative management .............................................. 249 5.5. Complications .................................................................. 249

6. LARYNGEAL TRAUMA ...............................................................251

6.1. Definition. Aetiology ....................................................... 251 6.2. Symptoms ........................................................................ 252 6.3. Diagnosis ......................................................................... 252 6.4. Treatment......................................................................... 252

7. FOREIGN BODIES IN THE LARYNX ................................................254 9

7.1. Definition. Aetiology ....................................................... 254 7.2. Symptoms ........................................................................ 255 7.3. Diagnosis ......................................................................... 255 7.4. Treatment......................................................................... 255

8. REFERENCES ...........................................................................256 V. SALIVARY GLAND BASICS 1. SALIVARY GLAND BASICS ..........................................................259

1.1. Clinical anatomy .............................................................. 259 1.2. Physiology........................................................................ 262 1.3. Disorders of salivary secretion.......................................... 263 1.4. Clinical examination ........................................................ 264

2. INFLAMMATORY DISORDERS ......................................................267

2.1. Infectious inflammatory disorders.................................... 267 2.2. Noninfectious inflammatory disorders ............................. 271

3. OBSTRUCTIVE DISORDERS .........................................................274

3.1. Sialolithiasis ..................................................................... 274 3.2. Other obstructive disorders .............................................. 275

4. SALIVARY GLANDS TUMORS ......................................................276

4.1. Benign tumors ................................................................. 276 4.2. Malignant tumors ............................................................ 280

5. REFERENCES ...........................................................................284 VI. NECK BASICS 1. NECK BASICS ..........................................................................287

1.1. Surgical anatomy of the neck ........................................... 287 1.2. Clinical examination ........................................................ 293

2. NECK MASSES .........................................................................296

2.1. Congenital neck masses ................................................... 296 2.2. Inflammatory/infectious neck masses .............................. 301 2.3. Benign tumors ................................................................. 305 2.4. Malignant tumors ............................................................ 310

3. REFERENCES ...........................................................................318

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I. EAR

1. EAR BASICS
1.1. Clinical anatomy
1.1.1. External ear The external ear comprises the auricle (pinna) and the external auditory canal (meatus). The auricle is composed of cartilage, covered by perichondrium and skin. The length of the external auditory canal is approximately 2.5 cm. In adults the canal is not straight, there is an italic S-shaped angulation. Thats why a certain amount of upward traction of the auricle is required in order to expose the eardrum during otoscopy. The external auditory canal is covered by skin and in the outer cartilaginous portion contains hair follicles and glands which secrete wax. The epithelium lining the canal is continued onto the surface of the eardrum. The narrowest part of the canal (isthmus) is located between the fibrocartilaginous and the bony canal. In the bony part, the skin is thinner and closely adherent to the periosteum and does not have hair follicles and ceruminous glands. Thats why furuncles occur only at the level of the outer cartilaginous meatus1. The sensory innervation is supplied by the auriculo-temporal branch of the Vth cranial nerve, the vagus nerve, the facial nerve and the cervical plexus.
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1.1.2. Middle ear and mastoid

1.1.2.1. Tympanic membrane: It consists of three layers: an outer epithelial or epidermal layer, a middle layer of elastic fibrous tissue (radial and circular fibers) and an inner layer of mucous membrane. The eardrum is inserted into a bony groove (tympanic sulcus) by the Gerlachs ligament (annulus). It is slightly oval in shape, forming an angle of about 55 with the floor of the external auditory canal2. The most important anatomical landmarks which are visible at otoscopy are: The handle of the malleus and the lateral or short process of the malleus The Gerlachs ligament (annulus) The anterior and posterior malleo-tympanic folds An anteroinferior light reflex: the Politzers triangle (cone of light) The anterior and posterior malleo-tympanic folds divide the eardrum into a superior part called Shrapnells membrane (pars flaccida) and an inferior one called pars tensa. 1.1.2.2. Middle ear ossicles: The ossicular chain consists of three ossicles: malleus, incus and stapes. The malleus is the largest one, measuring up to 9 mm in length. It comprises a head, a neck and a handle or manubrium, which is embedded in the eardrum. The incus has a body and two processes. The stapes footplate articulates with the oval window by the annular ligament. 1.1.2.3. Middle ear cavity: The middle ear cavity is divided into three portions: the attic, the meso-tympanum and the hypo-tympanum. The middle ear extends beyond the limits of the eardrum. At the level of the anterior wall we have the eustachian tube opening. The posterior wall presents the communication with the mastoid antrum. The external wall consists of the eardrum and the outer bony wall. The most important anatomical landmarks 12

Figure 1. External, middle and inner ear: 1 - External auditory canal; 2 - Eardrum; 3 - Middle ear cavity; 4 - Round window; 5 - Eustachian tube; 6 - Malleus; 7 - Incus; 8 Stapes; 9 - Semicircular canals; 10 - Vestibular nerve; 11 - Cochlear nerve; 12 - Cochlea

Figure 2. The tympanic membrane 1 - Handle of the malleus; 2 - Gerlachs ligament (annulus); 3 Pars tensa; 4 - Umbo; 5 - Politzers triangle (cone of light); 6 Short process of the malleus; 7 - Anterior malleo-tympanic fold; 8 - Pars flaccida (Shrapnells membrane); 9 - Posterior malleotympanic fold

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Figure 3. Middle ear cavity 1 - Malleus; 2 - Incus; 3 - Stapes; 4 - Eardrum; 5 - External auditory canal; 6 - Middle ear cavity; 7 - Eustachian tube

of the internal wall are the lateral or horizontal semicircular canal, the facial nerve, the oval window, the promontory (corresponding to the basal turn of the cochlea) and the round window. The facial nerve is dehiscent in its tympanic course (oval window region) in about 30% of individuals1. Under the floor of the middle ear lies the jugular bulb. Above the middle ear is the dura-mater of the middle cranial fossa. The mastoid antrum lies just behind the middle ear cavity and communicates with it via the aditus ad antrum. In most of the population the mastoid air cell system is fairly extensive with air cells extending into the mastoid tip, the zygomatic arch, the petrous apex. Sometimes the mastoid antrum is the only air-filled space in the mastoid. This condition is called sclerotic mastoid and occurs in perhaps 20% of adult temporal bones, mainly in individuals with chronic ear diseases2. The Eustachian tube communicates with the rhino-pharynx and may be opened by pharyngeal movements (swallowing). It has 14

a bony upper part and a cartilaginous lower end. The pharyngeal opening has a typical triangular-shaped appearance.

1.1.3. Internal Ear The inner ear labyrinth consists of a bony capsule within which is the membranous labyrinth. The bony capsule consists of three parts: Posteriorly, the three semicircular canals In the middle, the vestibule Anteriorly, the cochlea The bony labyrinth contains perilymph. Suspended in the perilymph lies the membranous labyrinth. This is a complex series of sacs and tubes containing a different fluid, called the endolymph. The membranous labyrinth consists of three semicircular canals: horizontal, superior and posterior, the superior and the posterior share a common crus. The anterior end of each canal is dilated to form its ampulla, which contains a patch of neuroepithelium called crista ampullaris, the hairs of which are embedded in the overlying gelatinous cupula. The canals open into the utricle, this and the saccule both lie in the vestibule of the bony labyrinth. Each contains a patch of neuroepithelium known as the macula. This resembles the crista ampullaris, except that the overlying membrane is flatter and contains particles of calcium carbonate called otoliths. The membranous cochlear duct is a simple tube situated in the bony cochlea and coiled for two and a half turns around its central bony modiolus. The neuroepithelium of the cochlea is arranged along the entire length of the basilar membrane and is known as the spiral organ of Corti3.

1.2. Physiology of the ear

1.2.1. Middle ear Sound represents a combination of waves that are generated by a vibrating sound source (or sources) and propagated through the air until they reach the ear. The study of hearing is often concerned with measuring the minimum intensity of sound that can be detected by the ear. This is defined as the auditory threshold. The sound intensity is measured in decibels (dB).
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The external and middle ears, which together form the conductive component of the auditory apparatus, transmit sound waves from the external environment to the sensory organ of hearing, the inner ear. As they transmit sound, they also amplify it and modify its frequency spectrum. The ability of a wave to be transferred from one medium to another is dictated by the impedances of the media. When a wave is transferred from a low-impedance medium (ex. air) to one of high impedance (ex. water), a considerable amount of its energy is reflected and fails to enter the liquid. Water has 3470 times the acoustic impedance of air, meaning that only 0.1% of the sound energy present in an acoustic signal traveling through air actually enters a water-filled medium. Moreover, because of the small surface area of the oval window, approximately 3% of the sound energy of an acoustic signal enters the cochlea. For hearing to occur, a system of amplification must be present to address the impedance mismatch that exists between air and water. The external and middle ears provide that amplification system. The anatomic arrangement of the structures within the middle ear, allow it to amplify sound waves delivered to it via the external ear. Sound is transferred from the large surface area of the tympanic membrane to the small surface area of the oval window. Amplification of the wave occurs because of a concentration of sound energy at the level of the oval window. In addition, the long axis of the malleus is longer than that of the incus, creating a lever effect that further augments the amplitude of the wave. The amplification conferred on the sound wave by the area ratio (22:1) and the lever ratio (1.3:1) substantially mitigates the effect of the impedance mismatch. The eustachian tube is responsible for maintaining the middle ear in a condition that optimizes middle ear function. Its functions include the following: to equilibrate air pressure in the middle ear with that of the external environment, to drain middle ear secretions into the nasopharynx and to protect the middle ear from potentially infectious nasopharyngeal secretions1.

1.2.2. Inner Ear The inner ear functions as the sensorineural receptor organ of the auditory system, converting an acoustic waveform into an electrochemical stimulus that can be transmitted to the Central nervous system. The cochlea consists of 3 fluid-filled ducts or scalae. These ducts are functionally divided into 2 spaces. The scala tympani and
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Figure 4. Inner ear 1 - Superior semicircular canal; 2 - Perilymph; 3 - Endolymph; 4 - Horizontal semicircular canal; 5 - Posterior semicircular canal; 6 - Ampullae; 7 - Oval window; 8 - Utricle and saccule; 9 - Cochlear duct; 10 - Cochlea; 12 - Round window

Figure 5. Inner ear 1 - Scala vestibuli; 2 - Cochlear duct; 3 - Scala tympani; 4 - Tectorial membrane; 5 - Stria vascularis; 6 - Spiral ganglion; 7 - Cochlear nerve; 8 - Basilar membrane; 9 - Spiral organ of Corti; 10 - Spiral ligament; 11 - Vestibular membrane (Reissner)

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scala vestibuli communicate with each other and are filled with perilymph. The scala media is isolated from the perilymphatic space and contains endolymph. The difference between the electrolyte composition of the perilymphatic and endolymphatic spaces creates an electrochemical environment that makes sensorineural transduction possible. A sound wave is transmitted to the middle ear, eliciting vibration of the ossicular chain. The vibration of the stapes transmits the sound wave via the oval window to the scala vestibuli, generating fluid waves in the perilymph. The displacement of the perilymph causes a wavelike displacement of the basilar membrane and organ of Corti, ultimately causing distention of the round window membrane. Depolarization of the hair cells generates impulses which passes along the fibers of the cochlear nerve to reach the auditory cortex3.

1.3. Symptoms of ear diseases

1.3.1. Earache (Otalgia) Pain in the ear is usually determined by inflammatory conditions of the external auditory canal and the middle ear, impacted wax etc. In the majority of cases, the pains etiology does not belong to the ear. It is the so-called referred otalgia. In these cases, a complete ENT examination must be performed after we excluded during otoscopy a local cause. 1.3.2. Hearing Loss Hearing loss may be unilateral or bilateral, with sudden onset or long-standing, stable or progressive, fluctuating. The history is extremely important. Ask the patient about the onset, the evolution of the hearing loss Ask the patient about his/her job (noise exposure) Is there a family history of hearing loss? Is there a history of meningitis, parotitis, measles or syphilis, head trauma? Is there a history of ototoxic drugs (e.g. streptomycin, gentamicin, loop diuretics, cisplatin)? Is the hearing loss associated with tinnitus, vertigo, other neurological symptoms?
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OTOSCOPIC EXAMINATION IN EARACHE Pathological External auditory canal Tympanic membrane Normal

Dental problems Acute tonsillitis Trauma Peri-tonsillar abscess Lacerations Traumatic perforation Temporo-mandibular joint Hematoma Haemotympanum syndrome Furuncle Acute suppurative otitis Cervical spine Diffuse external otitis media Mucosal aero-digestive tract Otomycosis Serous otitis media (mild) malignancies Herpes zoster oticus Bullous myringitis Elongated stiloid process Impacted cerumen (Eagles syndrome) Foreign bodies Table 1. Otoscopic examination in earache HEARING LOSS Conductive External ear Middle ear Eardrum perforation Acute suppurative otitis media Serous otitis media Chronic suppurative otitis media Otosclerosis Noise exposure Ototoxic drugs Presbyacusis Menieres disease Sensorineural

Wax Diffuse external otitis Foreign bodies Tumors of the EAC

Table 2. Types of hearing loss

1.3.3. Aural discharge (Otorrhoea) The aural discharge may be unilateral or bilateral, acute or chronic (long-standing), continuous or intermittent. Semiologic characteristics of the discharge may give us a hint for a quick diagnosis.
Muco-purulent Foul-smelling muco-purulent Blood-stained muco-purulent Creamy, fluffy Watery Diffuse external otitis Foreign bodies Chronic suppurative otitis media Cholesteatoma Malignant external otitis Aural polyp Carcinoma Otomycosis

Cerebrospinal fluid leak following temporal bone trauma Table 3. Semiologic characteristics of aural discharge

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1.3.4. Tinnitus Tinnitus is described as an annoying perception of noises or sounds within the head or the ears. It causes a lot of distress, especially at night when the patient is trying to sleep. The term tinnitus derives from the Latin word tinnire, meaning to ring. Sound that only the patient hears is subjective tinnitus, while sound that others can hear as well is called objective tinnitus. Estimates of patients with tinnitus range from 10-15% of the population (30-40 million people). Of patients presenting with ear-related symptoms, 85% report experiencing tinnitus as well. Both adults and children report experiencing tinnitus. Development of tinnitus increases in incidence with age4. Tinnitus is a symptom not a disease, and therefore reflects an underlying abnormality. In most cases is associated with sensorineural hearing loss. History is extremely important: Is it bilateral, unilateral? Time of onset? Is it continuous, intermittent, pulsating, low-pitched or high-pitched? Objective tinnitus is a rare condition and has a muscular or vascular etiology. 1.3.5. Vertigo (Dizziness) Vertigo is a sensation of abnormal movement of the surroundings in relation to the patient, or of the patient in relation to his surroundings. It is often accompanied by vegetative symptoms: nausea, vomiting, sweating, tachycardia, but never loss of consciousness. It has a rotatory or spinning pattern, and is related to head movements. It represents the main symptom in the peripheral vestibular syndrome. In the peripheral vestibular syndrome patients describe episodic attacks, with normal equilibrium between the crises. The duration of the vestibular crisis is very important for the diagnosis. We have vertigo lasting seconds (benign positional paroxysmal vertigo BPPV), hours (Menieres disease) or days (vestibular neuronitis)5. It is essential to collect a detailed clinical history: Differentiate the true vertigo from other pathological movement sensations (dizziness, unsteadiness)!
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 The duration of the attack? Does head motion impel the crises? The presence of associated vegetative symptoms: nausea, vomiting? The association of a hearing loss, tinnitus, aural fullness? The association of other neurological symptoms? Is there a history of head trauma, chronic ear disease, ear surgery?

1.4. Clinical examination

1.4.1. Inspection and palpation Inspection of the pinna will follow the anatomical landmarks determined by the cartilage prominences: the helix, the ante helix, the tragus, the anti tragus, the triangular fossa. The ear lobule does not contain cartilage, only adipose tissue. Gross inspection of the pinna and the retro-auricular region may reveal the presence of congenital malformations, inflammatory processes and neoplastic diseases. Palpation of the mastoid area: Three mastoid trigger points to palpate The antral point: it is located posterosuperiorly to the meatus, it corresponds to the antrum, it is sensitive in acute otomastoiditis The tip of the mastoid: it is sensitive in a particular form of mastoiditis (Betzolds), in which the pus erodes the bony cortical wall of the mastoids tip and accumulates beneath the sternocleidomastoid muscles sheath, at the level of its superior insertion The sinusal point is located at the level of the posteroinferior margin of the mastoid bone, 1-1.5 cm from the mastoids tip, posteriorly; it is sensitive in sigmoid sinus thrombosis, a serious complication of cholesteatoma. 1.4.2. Otoscopy Instruments: Aural speculum Siegles aural speculum: squeezing of the bulb causes the eardrum to move, by applying positive and negative pressures (pneumatic otoscopy)
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Figure 6. The auricle (pinna) 1 - Helix; 2 - Ante helix; 3 - Triangular fossa; 4 - Tragus; 5 - Anti tragus; 6 - Ear lobule; 7 - Concha

Figure 7. The mastoid points 1 - Antral point; 2 - Tip of the mastoid; 3 - Sinusal point

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Figure 8. Aural speculum

Figure 9. Otoscope

Figure 10. Otoscopy

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 Electric otoscope (magnification 2.5) The 0, 4 mm tele-otoscope (ear rigid endoscopy) The binocular microscope: greatest accuracy in ear examination The largest speculum which can be inserted in the meatus without pain should be used. To get a clear view, the pinna and cartilaginous canal must be pulled upwards and backwards. For the examination of the left ear, the ear speculum is held between the thumb and the forefinger of the left hand. For the examination of the right ear the speculum is held in the right hand. The external auditory canal is inspected for foreign bodies, cerumen, secretions, mycosis, exostosis. If secretions are present in the external auditory canal, a careful toilette must be performed using suction. The appearance of a normal tympanic membrane (anatomical landmarks): Gerlachs ligament (annulus): the eardrum is attached to the tympanic ring Handle of the malleus, with the short, lateral process projecting laterally from the upper end of the handle Umbo: the central point of the tympanic membrane (at the tip of the malleus handle) Anterior and posterior tympano-malleolar folds Pars tensa: a pale grey tense thin membrane Pars flaccida (Schrapnells membrane), covering the attic region is loose and much thicker Politzer triangle: a cone of light reflex located in the antero-inferior quadrant of the tympanic membrane

1.4.3. Examination of the Eustachian tube Valsalvas method of inflation: the patient is instructed to take a deep breath, keep the nose tightly pinched and the lips firmly closed, and then forcibly attempt to blow the nose. If succesfull, the drum will be seen to move outward and a click may be audible Politzers method: the olive attachement of a Politzer bag is applied to one nostril, the other nostril is closed with a finger, the patient is told to say jac or cucu, at the moment of the nasopharynx closure the bag is compressed sharply and the intra-nasal pressure thereby
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raised considerably above normal, the result is to force air up to the Eustachian tube Inflation with a catheter placed at the level of the pharyngeal ostium of the Eustachian tube, through the nasal cavities

1.4.4. Examination of hearing Hearing can be assessed by the clinician during the ENT examination. Some subjective tests are available, even if they are not so accurate as a pure-tone audiometry. For example, the freefield speech test will in most cases discover a hearing loss greater than 30 dB with a false-positive rate around 13%6.
1.4.4.1. The whispered voice test (Free-field speech testing) A normal ear is capable of hearing a whispered voice from a distance of nearly 6 meters. So, the examiner will be placed at 6 meters from the patient, opposite the ear to be tested, in a silent environment. It is compulsory to mask the non test ear (pushing the opposite ear tragus in and out by an assistant). Also the eyes must be covered, in order to avoid lip-reading by the patient. The examiner explains to the patient that a combination of numbers will be whispered and spoken in the test ear, and the patient is asked to repeat them. If the patient is capable of recognizing more than 80% of the whispered numbers, we will consider a normal hearing. If the patient doesnt hear anything from 6 meters, we will repeat the test from 3 meters and 1 meter. 1.4.4.2. Tuning fork tests Tuning fork tests are used to differentiate between a conductive and a sensorineural hearing loss. The most frequently used forks are the 256- and 512-Hz, as these give more reliable outcomes than the 1024-Hz fork7. The tuning forks are activated by striking them lightly against a bony prominence (knee, elbow). Its very important the way you are handling the tuning fork, you are not allowed to touch the vibrating parts! 1.4.4.2.1. The Weber test The examiner places the activated tuning fork centrally on the forehead, on the bridge of the nose or over the incisor teeth,

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Figure 11. The Weber test

and ask the patient to tell him if a sound or a vibration is audible. If the answer is positive, the examiner will ask the patient to describe where the sound is perceived: in the midline, in the left ear or in the right ear? We have three clinical possibilities: The patient locates the sound in the midline or in both ears (normal hearing or symmetrical bilateral hearing loss) The sound is perceived in the affected ear (conductive hearing loss) The sound is perceived in the healthy ear (sensorineural hearing loss) Unfortunately, the Weber test has a low sensitivity and specificity, the chance of accurately differentiating conductive and sensorineural hearing loss is 33%8.
1.4.4.2.2. The Rinne test The examiner places the activated fork on the mastoid bone behind the auricle (bone conduction) and asks the patient to confirm if the sound is audible. If the patients answer is positive, the examiner asks the patient to notice when the sound is no longer heard. The average time for bone conduction in a normal

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Figure 12 - The Rinne test 1

Figure 13 - The Rinne test 2

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ear, using the 512-Hz fork, is about 20 seconds. After the sound on the mastoid bone is no longer perceived, the examiner places the fork in front of the tested ear (air conduction), and asks the patient to tell if the sound is audible again, and if the answer is yes, to report when the sound is no longer audible. In normal hearing, the patient should hear the sound other 20 seconds. This is the positive Rinne test. What happens if the patient has a conductive hearing loss in the tested ear? The bone conduction is normal, so the sound will be heard on the mastoid for 20 seconds or even more, but when the examiner places the fork in front of the ear, no sound will be heard. Its the negative Rinne test. If a sensorineural hearing loss is present, the patient will hear the sound on the mastoid less than 20 seconds, and the same period of time when the fork is placed in front of the ear (air conduction is normal). Its a positive shortened Rinne test. Though many ENT doctors use a 516-Hz tuning fork, the 256-Hz fork give superior sensitivities and specificities9.
1.4.4.3. Pure-tone audiometry The purpose of the pure-tone audiometry is to determine hearing threshold levels for pure tones. The hearing threshold is defined as the level of a sound at which, under specified conditions, a person gives 50 per cent

Figure 14. Pure tone audiometry: normal hearing (< : bone conduction right ear; : air conduction right ear; > : bone conduction left ear; : air conduction left ear)

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Figure 15. Pure tone audiometry: conductive hearing loss ( < : bone conduction right ear; : air conduction right ear; > : bone conduction left ear; : air conduction left ear)

Figure 16. Pure tone audiometry: sensorineural hearing loss ( < : bone conduction right ear; : air conduction right ear; > : bone conduction left ear; : air conduction left ear)

of correct detection responses on repeated trials. Specified conditions means the type of sound and ways of presenting the sound. The normal test sound is pure tone pulses at standardized frequencies in the range of 125-8000 Hz, and the normal presentation mode is monaurally by means of a standardized type of earphone10. The necessary equipment consists of a pure tone audiometer. Air conduction and bone conduction is assesed, with the patient placed in a phonic isolated room. Data are recorded on a typical graph. 29

A complete audiogram, using both air- and boneconduction, supplies important information for the diagnosis, in terms of differentiation between conductive and sensorineural disorders. An air- and bone-conduction loss at the same level suggests a sensorineural hearing loss, whereas a larger loss by airconduction than by bone-conduction (the so-called air-bone gap), indicates the presence of a conductive hearing loss. The air-bone gap at a single frequency needs to be at least 15 dB in order to be considered statistically significant10.
1.4.4.4. Speech audiometry Speech audiometry is a considerably more complex procedure. In pure-tone audiometry, the listening task is simply detection of the stimuli. In speech audiometry the usual task is not detection but recognition, which requires both detection and identification of the phonemes and recognition of sets of phonemes as words. Equipment for speech audiometry is usually integrated with a clinical pure-tone audiometer. In conventional clinical speech audiometry, the test signal is presented monaurally by means of earphones. The speech test material may differ widely from nonsense combinations of consonants and vowels, so-called logatoms, to natural connected speech10. In practice, lists of two-syllable words are supplied by airconduction through earphones or in free-field to both ears. The patient is asked to repeat the words and the results are recorded on a special graph 1. 1.4.4.5. Objective assessment of hearing All the methods previously described are subjective, because they require an active participation of the tested subject. A number of objective methods for hearing evaluation are also available: Impedance audiometry: tympanometry and stapedial reflex (acoustic reflex) Electro-cochleography Auditory brainstem responses (ABR) Oto-acoustic emissions (OAE)

30

2. DISEASES OF THE EXTERNAL EAR

2.1. Diseases of the auricle
2.1.1. Congenital abnormalities Congenital malformations of the external ear are, by definition, present at birth. The etiology could be determined by genetic defects (e.g. chromosomal abnormalities) or by acquired factors that disturb the normal embryonic development of the ear (drugs, nutritional deficiencies, viral infections, hormonal, metabolic disorders, autoimmune reactions). Microtia, macrotia, poliotia, anotia, melotia (ear located on the cheek) Auricular appendages

Figure 17. Ear malformations (outstanding ear, melotia, auricular appendages)

31

 Atresia of the external auditory canal Auricular fistulas Bat ears (outstanding ears)

2.1.2. Trauma
2.1.2.1. Sharp trauma, lacerations and bites From history, try to determine the etiology: aggression, accident, human bite, animal bite. Perform photographic documentation for legal purposes. In case of infection, a bacterial culture is required. Treatment involves: Local toilet and disinfection (H2O2, Betadine) Removal of debris and devitelized tissues Antibiotics Rabies prevention in case of animal bites Tetanos prevention for all contaminated wounds Surgical repair if necessary 2.1.2.2. Othematoma Othematoma is a sero-sanguinolent fluid accumulation between the perichondrium and the cartilage of the pinna, located at the level of the triangular fossa. Most frequently it is determined by a blunt trauma to the external ear (boxers, wrestlers, rugby players, assaults). A soft tumefaction is visible at inspection on the outer part of the auricle, at the level of the triangular fossa. The overlying skin is normal in appearance. Palpation of the mass reveals fluctuation. Treatment requires the aseptic drainage of the fluid. Simple aspiration is not recommended, because of the high risk of recurrence. Its compulsory to place a pressure dressing on both sides of the pinna, and fix it into the concha with through-andthrough mattress sutures. To avoid secondary infection, antibiotics are prescribed. Because Pseudomonas aeruginosa has a great propensity to the ear cartilage, Ciprofloxacin is the first-choice antibiotic. Complications: Recurrences

32

Figure 18. Othematoma

Figure 19. Perichondritis

Infection (perichondritis) Chronic organization of the hematoma, with chondronecrosis, perichondritis, chondroneogenesis, leading to the typical aspect of a cauliflower ear

2.1.3. Perichondritis Perichondritis is an infection and inflammation of the ear cartilages perichondrium. It may follow the infection of an othematoma or other traumatic injuries (even after ear surgery). Pseudomonas aeruginosa is the most frequently involved bacterial agent (75-90%)11. The patient complains of severe pain. Local examination reveals a swollen, erythematous and tender pinna. Fluctuation indicates the presence of an abscess and possible chondritis. The lobule, which contains no cartilage, is spared. Treatment: Local disinfection Incisions, drainage Broad-spectrum antipseudomonal antibiotics (oral quinolones), non-steroidal anti-inflammatory drugs (NSAIDs) 33

2.1.4. Skin infections Impetigo: staphylococcal infection, most commonly occurs in young children, frequently secondary to an otorrhoea. Treatment: crusts removal, application of an antibiotic-steroid ointment Erysipelas: streptococcal infection which produces a raised, red edematous eruption with sharply defined margins, malaise, high fever. Treatment: high-doses Penicillin Herpes zoster oticus: Ramsey-Hunt syndrome (painful neuralgia, vesicular eruption in the concha, facial nerve palsy) 2.1.5. Tumors Benign: keloids, epidermal cysts, keratoacanthomas Malignant: basal cell carcinomas, squamous cell carcinomas, malignant melanomas

2.2. Diseases of the external auditory canal

2.2.1. Wax (Cerumen) Wax represents a normal secretion of the ceruminous glands, which are located in the skin covering the outer cartilaginous portion of the canal. In case of impacted ear wax, patient complains of a hearing loss, aural fullness, earache, irritation in the ear. The hearing loss is conductive because it is caused by the obstruction of the external canal (air-conduction). Patients often describe a sudden onset of the hearing loss, mainly after bathing. Water causes the wax to swell and the passage is completely blocked. Wax removal: Aural irrigation (syringing) with warm water after preliminary softening: this maneuver is not allowed if the patient has undergone previous ear surgery, or if a perforation of the eardrum is present. Care must be taken to avoid any trauma to the tympanic membrane and the skin of the auditory canal Under direct microscopic control using a special curette and/or suctioning Ceruminolytics: olive oil, H2O2 4%, sodium bicarbonate 25%12
34

Figure 20. Cerumen (wax)

Figure 21. Guyons syringe for aural irrigation (wax removal)

2.2.2. Foreign bodies Foreign bodies are almost invariably confined to children: vegetables, parts of plastic toys, beads, insects. Removal is performed under direct microscopic control. Sometimes general anesthesia is required (mainly in children). Syringing may be also useful, in case of small plastic or metallic foreign bodies. Dont forget the main damage by a foreign body in the EAC is caused by its careless removal12!
Type of foreign body Living insects Irregular/graspable objects Organic/vegetable Button batteries Round, hard, smooth non-graspable Method of removal First kill the insects with oil Remove with crocodile forceps Do not syringe Do not syringe Syringe/remove with wax hooks

Table 3. Techniques of removal advised for different types of foreign bodies13

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2.2.3. Trauma The canal may be injured while removing wax or a foreign body with a sharp instrument or during syringing. 2.2.4. Localized external otitis (Furuncle)
2.2.4.1. Definition. Aetiology Furuncle is a localized infection of a hair follicle, located at the level of the skin covering the outer cartilaginous portion of the canal, caused by Staphylococcus aureus. 2.2.4.2. Symptoms Patient complains of extremely severe otalgia, augmented by moving or touching the pinna. 2.2.4.3. Diagnosis

Inspection may reveal swelling of the parts around the ear, corresponding to the location of the furuncle within the meatus. Palpation of the tragus is painful. Sometimes palpation may disclose a retro-auricular inflammatory lymphadenopathy. Otoscopic examination enables an accurate diagnosis. A localized swelling of the skin is noticed, with signs of acute inflammation Figure 22. Furuncle of the external and a characteristic white point ear canal (bourbillion) in the middle of the tumefaction.
2.2.4.4. Treatment Topical disinfectants Topical antibiotic ointments Analgetics, non-steroidal antiinflammatory drugs (NSAIDs) Systemic antibiotics (Oxacillin, Amoxicillin with Clavulanate)

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 Surgery (incision, drainage) In case of multiple recurrences, check if the patient doesnt have diabetes!

2.2.5. Diffuse external otitis (Swimmers ear)

2.2.5.1. Definition. Aetiology Diffuse external otitis is an acute infection and inflammation of the skin covering the external auditory canal. It is most frequently encountered during summer season, with patients addressing the ENT doctor after bathing in public swimming pools.The most common bacterial agents involved are Pseudomonas aeruginosa (40% of cases), Staphylococcus epidermidis (9% of cases) and Staphylococcus aureus (8% of cases)14. Predisposing factors: absence of cerumen, high humidity, increased temperature, local trauma. 2.2.5.2. Symptoms Otalgia, augmented by temporomandibular movements or by pressure applied to the tragus Itching, sensation of aural fullness Clear or muco-purulent otorrhoea Hearing loss

joint

2.2.5.3. Diagnosis Palpation of the tragus is painful. Sometimes sensitive periauricular lymph nodes are present. Otoscopy: the entire skin of the canal has a red, swollen, edematous appearance with accumulation of debris and secretions. A careful toilette of the canal must be performed by suctioning. If the edema is pronounced, the tympanic membrane may not be visible. The hearing loss is conductive (Weber lateralization Figure 23. Diffuse external otitis
(swimmers ear)

37

to the affected ear, negative Rinne test, air-bone gap in pure-tone audiometry). In cases resistant to adequate treatment, bacteriologic examination is required.
2.2.5.4. Differential diagnosis Localized external otitis (Furuncle) Otomycosis Acute suppurative otitis media Chronic otitis media Malignant tumors of the external auditory canal (EAC) Referred otalgia 2.2.5.5. Treatment Removal of the debris and secretions from the ear canal is required, under microscopic control, if available. A sterile gauze strip with a topical antibiotic/steroid otic solution is inserted within the canal. Antibiotic drops continue to be the mainstay of treatment for otitis externa. The main characteristics of the ideal drop are exposed here 15: Broad-spectrum coverage for involved bacteria An acidic vehicle No ototoxicity (great care if the patient has a perforation of the eardrum!) No potential for allergic reactions Low cost A steroid to alleviate pain and decrease edema more rapidly Quinolone antibiotics (no ototoxicity, no risk of contact dermatitis) are available in both otic and ophthalmic solutions (Ciplox). Other alternatives: Polymyxin, Neomycin, Gentamycin (Garasone). The aminoglycosides are not recommended if the patient has an eardrum perforation, because of their potential for ototoxicity! Oral antibiotics (Ciprofloxacin) are prescribed only in severe cases with fever, peri-auricular adenopathies, and always in diabetic patients. Analgetics and non-steroidal antiinflammatory drugs (NSAIDs) are also prescribed.

38

It is important to tell the patient to avoid water penetration into the canal during treatment period.
2.2.5.6. Complications Chronic external otitis with stenosis of the ear canal Otomycosis: in case of long-term administration of topical antibiotic/steroid otic solutions Malignant external otitis: a necrotizing evolution with otogenic skull base osteo-myelitis (elderly patients with diabetes mellitus or severe immunodeficiencies)

2.2.6. Otomycosis (Fungal external otitis)

2.2.6.1. Definition. Aetiology The most frequently involved agents are: Aspergillus fumigatus, Aspergillus flavus, Aspergillus niger and Candida albicans. In most cases, otomycosis is not a primary infection, it follows a longterm administration of antibiotic and steroid drops. 2.2.6.2. Symptoms Aural fullness Itching, vague pain in the ear Ear discharge: thick, creamy, curdlike, fluffy 2.2.6.3. Diagnosis Otoscopic examination reveals the presence of masses of material like wet blotting paper, upon which the mycelia can be seen. The color of the mass may be whitish, yellow or black, depending on the involved agent. 2.2.6.4. Treatment Treatment requires repeated and careful cleaning of all debris, under microscopic control, followed by topical application of

Figure 24. Otomycosis

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an anti-fungal agent (Clotrimazole, Miconazole). Local treatment must be continued for at least 3 weeks after apparent cure. Topical disinfectants (Betadine solution) are also useful. In persistent cases of external otitis, otomycosis should always be taken into account!

2.2.7. Tumors
2.2.7.1. Benign tumors 2.2.7.1.1. Osteomas Osteomas are benign bony tumors located at the level of the inner bony portion of the external auditory canal. The main symptom is a unilateral conductive hearing loss. Otoscopy reveals a solitary bony mass covered by normal skin, obstructing the canal. CT scan is useful to evaluate the extension of the mass. Treatment: surgical removal by drilling, under microscopic control. 2.2.7.1.2. Exostoses Exostoses are areas of localized bulges of bone covered by a thin, normal skin. They are located in the osseous portion of the external canal, usually multiple and bilateral. Frequently, the patients describe a history of various aquatic activities (swimming in cold water, surfing, diving)12. Generally asymptomatic, they require surgical treatment only when a subtotal obstruction of the canal, with subsequent wax retention and conductive hearing loss is present. 2.2.7.1.3. Ceruminomas Ceruminomas are benign tumors developed from the ceruminous glands. These glands are located in the skin covering the outer part of the external auditory canal. 2.2.7.2. Malignant tumors 2.2.7.2.1. Squamous cell carcinomas (SCC) Squamous cell carcinoma is the most frequent malignant

40

skin tumor located at the level of the external auditory canal (60%)12. It is frequently associated with chronic muco-purulent otorrhoea. Biopsy is mandatory for an accurate diagnosis. In evolution invades the middle ear and the mastoid bone.
2.2.7.2.2. Basal cell carcinomas (BCC) The external auditory canal (EAC) is rarely the primary site of development. Most frequently BCC of the EAC is the result of a local invasion originating from an auricle lesion. 2.2.7.2.3. Malignant ceruminomas Malignant ceruminomas account for nearly 15% of all malignant tumors of the external canal12. Other rare tumors: adenocarcinomas, rhabdomyosarcomas, melanomas.

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3. DISEASES OF THE MIDDLE EAR

3.1. Trauma
3.1.1. Traumatic perforations of the eardrum
3.1.1.1. Definition. Aetiology Direct trauma: sharp instruments, sharp foreign bodies, iatrogenic (syringing, foreign bodies removal) Indirect trauma: abrupt increase of the pressure in the external canal caused by an explosion, diving, getting slapped on the ear 3.1.1.2. Symptoms Otalgia Aural fullness Tinnitus Conductive hearing loss with sudden onset 3.1.1.3. Diagnosis Otoscopy reveals the traumatic perforation, located in pars tensa. The perforations shape is usually linear, but it may be also triangular, round, ovalar. Blood may be noticed in the canal or at the level of the perforations margins. These margins may be slightly erythematous. Photographic documentation for legal purposes is strongly recommended.

Figure 25. Traumatic perforation of the tympanic membrane

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Evaluation of hearing: Weber lateralization to the damaged ear, negative Rinne test and a light air-bone gap (10-30 dB) in pure-tone audiometry (conductive hearing loss).
3.1.1.4. Treatment Sterile dressings, antibiotics to prevent infection NO water in the ear! Observation: small recent perforations may heal spontaneously Small perforations that do not heal spontaneously can be treated by surgery, under microscopic control, in local or general anesthesia (placement of a steri-strip patch over the perforation, placement of a cigarette paper patch moistened with Ringer solution16, fat graft myringoplasty using a small piece of fat harvested from the ear lobule) Large perforations may require an underlay myringoplasty using perichondrium, cartilage or temporal fascia

3.1.2. Hemotympanum
3.1.2.1. Definition. Aetiology Hemotympanum is an accumulation of blood in the middle ear cavity, behind a non-perforated eardrum. The aetiology is traumatic. 3.1.2.2. Symptoms Aural fullness Conductive hearing loss 3.1.2.3. Diagnosis Otoscopic examination reveals a non-perforated eardrum with a typical dark-bluish color. Pneumatic otoscopy with the Siegles otoscope demonstrates a decreased mobility of the tympanic membrane, caused by the accumulation of fluid in the middle ear.

Figure 26. Hemotympanum

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Evaluation of hearing: Weber lateralization to the affected ear, negative Rinne test and an air-bone gap in pure-tone audiometry (conductive hearing loss).
3.1.2.4. Treatment Oral antibiotics (Amoxicillin, Amoxicillin with Clavulanate): in order to prevent infection Nasal decongestants: in order to facilitate blood evacuation from the middle ear through the Eustachian tube

3.1.3. Otitic barotrauma

3.1.3.1. Definition. Aetiology Barotrauma is defined as an injury produced by mechanical forces caused by a change of pressure in a gas-filled space 17. Therefore, otitic barotrauma is a term that encompasses those pathological conditions of the ear induced by pressure changes. Middle ear barotrauma is the most frequent pressureinduced ear condition. In the majority of cases, barotrauma is the outcome of air pressure changes experienced during commercial flights. Diving is the second important etiological factor. Transient evidence of middle ear barotrauma has been reported in 5% of adults and 25% of young children after flying18. 3.1.3.2. Pathophysiology Middle ear barotrauma is the result of an acute loss of ventilation in the middle ear, caused by a sudden increase of atmospheric pressure which is not compensated by the Eustachian tube function: the external pressure increases rapidly, collapsing the eardrum inwards. 3.1.3.3. Symptoms Aural fullness Otalgia: typically augmented with increased compression and the inability to equalize middle ear air pressure Hearing loss Tinnitus, vertigo in case of associated inner ear barotrauma

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3.1.3.4. Diagnosis Otoscopic examination reveals various aspects: normal tympanic membrane, redness and retraction, intratympanic haemorrhage, hemotympanum, perforation. Evaluation of hearing: tuning fork tests, pure-tone audiometry, tympanometry (conductive hearing loss and decrease of compliance with negative middle ear pressure). It is also recommended to perform an evaluation of the Eustachian tube function and an accurate examination of the nose and nasopharynx. 3.1.3.5. Complications Rupture of the round window membrane involves the labyrinth (inner ear barotrauma) with subsequent fluctuating sensorineural hearing loss, tinnitus and vertigo. If a perforation of the eardrum occurs during diving, the penetration of water within the middle ear cleft may elicit a caloric vertigo, which can be fatal! 3.1.3.6. Treatment Nasal decongestants: sprays/drops (0.1% Xylomethazoline) 3-4 times daily Valsalva maneuvers, air inflations with a Politzer bag or a catheter In cases of persistent negative pressure in the middle ear: myringotomy with ventilation tube insertion In cases of inner ear involvement: high doses of corticosteroids, rheologic treatment In cases of hemotympanum: antibiotics In cases of suspected rupture of the round window membrane: surgical exploration of the middle ear cavity and closure of the fistula 3.1.3.7. Suggestions Avoid air travel during severe upper airway tract infections In case of Eustachian tube dysfunction apply nasal decongestants 20 minutes before take-off and landing19 Use chewing gum during flights

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3.1.4. Temporal bone trauma

3.1.4.1. Definition. Aetiology Temporal bone trauma is a physical insult of the temporal bone induced by impact with a blunt surface or penetrating missile. This may or may not be associated with a temporal bone fracture20. Aetiology: Road traffic accidents (40-50%) Falls Assaults, aggressions Industrial and sporting accidents 3.1.4.2. Symptoms History is very important! Patient may be conscious or in coma Headaches, otalgia Hearing loss Otorrhagia (blood exteriorization from the ear canal), otoliquorea (cerebrospinal fluid CSF exteriorization from the ear canal) Tinnitus, vertigo, nausea, vomiting (labyrinthine involvement) 3.1.4.3. Diagnosis Inspection must evaluate the presence or absence of lower motor neuron facial nerve palsy. Also the presence of bruising over the mastoid process must be noticed (Battles sign). Evidence of wounds, hematomas must be accurately documented for legal purposes. Otoscopic examination may reveal the presence of fresh blood or clots in the external canal, lacerations of the skin covering the canal, tympanic membrane perforations, hemotympanum, a step deformity in the bony wall of the canal. Radiological examinations: CT scans, MRI Hearing assessment: tuning fork tests in emergency, a standard pure-tone audiometry should be performed as soon as possible, tympanometry, electric response audiometry (can be performed in the unconscious patient) Vestibular assessment: nystagmus Evaluation of facial nerve function: observing active and passive facial movements. Evaluation of a cerebrospinal fluid leak.

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A complete neurological and neurosurgical examination must be performed: evaluation of the level of consciousness (Glasgow score) is mandatory.
3.1.4.4. Complications Peripheral facial nerve palsy: which can be immediate, determined by the trauma itself, or delayed (2-12 days) owing to neural edema or to herpes virus reactivation in the geniculate ganglion 19 Perilymphatic fistula CSF fistula: clear, pink ear discharge Meningitis Fracture of the labyrinth: a destructive peripheral vestibular syndrome 3.1.4.5. Treatment 3.1.4.5.1. Conservative treatment: Analgetics, antibiotics Collection and examination of the ear discharge: very important in case of suspicion of otoliquorea Close clinical survey for minimum 3 days Toilette of the external ear canal, calibration of the meatus if needed 3.1.4.5.2. Surgical treatment: Emergency exploratory tympanotomy: in case of suspicion of perilymphatic fistula Surgical treatment of CSF leaks: rarely indicated because they usually heal spontaneously Treatment of facial nerve lesions

3.2. Bullous myringitis

3.2.1. Definition. Aetiology Bullous myringitis is an inflammatory disease of the eardrum, characterized by the formation of sero-haemorrhagic bullae on the outer surface of the tympanic membrane. These bullae may extend to the adjacent skin of the external canal.
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An infection by influenza virus or by Mycoplasma pneumoniae has been suggested as the aetiological agent, but no clear evidence has been presented21.

3.2.2. Symptoms Severe otalgia with sudden onset, usually unilateral Blood-stained otorrhoea, which appears in the same day with otalgia Unilateral hearing impairment The symptoms usually occur during or after an acute viral upper respiratory tract infection 3.2.3. Diagnosis Otoscopic examination reveals the haemorrhagic bullae, formed on the eardrum. They appear as dark-bluish or red swellings. The bullae may spread on the wall of the external auditory canal (EAC). The bullae burst the same day, discharging a sero-sanguinolent fluid. The tympanic membrane is intact, no perforation is seen. Sometimes this condition is associated with serous otitis media and a sensorineural hearing loss (viral infection)22. 3.2.4. Treatment Antibiotics (Amoxicillin, Amoxicillin with Clavulanate) to prevent bacterial infection Analgetics

3.3. Acute suppurative otitis media (AOM)

3.3.1. Definition. Aetiology A common illness in infants and young children, AOM is an acute (in most cases purulent) inflammation of the mucosa of the middle ear cavity. The vast majority of AOM episodes are triggered by an acute viral upper respiratory tract infection, involving the nasopharynx. Pathogenic bacteria colonize the normally sterile middle ear space by direct extension from the nasopharynx, through the Eustachian tube. 48

Another possibility is colonization through eardrum perforations. The bacteria responsible for AOM are: Streptococcus pneumoniae (40-50%) Haemophilus influenzae (30-40%) Moraxella catarrhalis Staphylococcus aureus (10-20%) In children, chronically enlarged and inflamed adenoids are the most important predisposing factor for development of recurrent episodes of AOM. More than 60% of children under the age of 6 years, experience one or more episodes of AOM16.

3.3.2. Symptoms Otalgia is severe, sharp, pulsating, with sudden onset, causing the child to awaken from sleep and scream High fever (38-40C), chills, malaise Hearing loss Nasal obstruction, rhinorrhoea, sore throat, cough After 2-3 days of evolution, a purulent otorrhoea appears bringing instant relief from the earache 3.3.3. Diagnosis Inspection and palpation: mastoid process and retro-auricular area should be examined for tenderness or edema. Otoscopic examination reveals signs of progressive inflammation. The eardrum has a typical appearance: congestion, edema, loss of normal anatomical landmarks. It bulges and finally ruptures after 2-3 days of evolution. Secondary, a mucopurulent discharge is noticeable in the external auditory canal. The eardrum perforation is not visible, because of the massive edema.The discharge may pulsate and reflect light intermittently Figure 27. Acute suppurative otitis (the Lighthouse sign).
media

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Hearing assessment (tuning fork tests, audiogram) reveals a conductive hearing loss. A complete ENT examination should be performed.

3.3.4. Evolution and complications Spontaneous recovery Complications: acute mastoiditis (inflammation of the mastoid cell system), facial nerve palsy, acute labyrinthitis, petrositis, meningitis, encephalitis, sigmoid sinus thrombosis 3.3.5. Treatment
3.3.5.1. Conservative treatment: Antibiotics: AOM is a great example of the efficacy of an appropriate antibiotic treatment. Considering the aetiology, Amoxicillin or Amoxicillin with Clavulanate are the first-choice antibiotics to be prescribed. The duration of treatment is at least one week Analgetics, non-steroidal antiinflammatory drugs (NSAIDs) Nasal decongestants: nose sprays/nose drops (0.1% Xylomethazoline for adults, 0.05% Xylomethazoline for children) Rest in a warm and well humidified room Application of local hyperthermia 3.3.5.2. Surgical treatment: The recommended European standard 16 is: Myringotomy (paracentesis): when bulging of the eardrum persists, for immediate relief of pain! Adenoidectomy: in recurrent AOM, in most cases accompanied by myringotomy and insertion of grommets Antrostomy/mastoidectomy: in case of imminent mastoiditis, labyrinthitis, sigmoid sinus thrombosis

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3.4. Otitis media with effusion (OME)

3.4.1. Definition. Aetiology Otitis media with effusion (serous otitis media, secretory otitis media, glue-ear) is characterized by an accumulation of a non-purulent effusion in the middle ear cavity that may be either serous or mucoid. A persistent Eustachian tube dysfunction (which is the most important factor in OMEs pathophysiology) can be determined by: Recurrent viral infections of the upper respiratory tract, mainly of the adenoids Tumor development in the nasopharynx (especially in adults) Allergy, chronic rhinosinusitis Cleft palate Radiotherapy in the area of the ear/nasopharynx The highest incidence of OME is reported within the age group between 2 and 5 years (20%). Bilateral disease is reported in 84% of cases, mainly in young children16. 3.4.2. Pathophysiology One of the major functions of the Eustachian tube is to equilibrate the air pressure between the middle ear cleft and the external ear. This accounts for an appropriate physiologic vibration of the ear drum and ossicular chain. A chronic dysfunction of the tube will determine the development of a negative pressure within the middle ear cavity which elicits a transudate from the mucosa, leading to the accumulation of a serous effusion. OME may also occur during the resolution of an AOM. 3.4.3. Symptoms In children: reports from the parents concerned with symptoms consistent with decreased hearing (TV volume too loud, child does not respond when called, often asks what?) In adults: aural fullness, conductive hearing loss No pain

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3.4.4. Diagnosis Otoscopic examination reveals a translucent, yellowish tympanic membrane, with decreased mobility, air-fluid levels, serous middle ear fluid, prominence of the lateral process of the malleus, a more horizontal orientation of the malleus. If the patient is asked to perform a Valsalva maneuver, air will be inflated within the middle ear cavity, and air bubbles can bee seen in the fluid behind the eardrum. Evaluation of hearing: tuning fork tests, audiogram (conductive air-bone gap up to Figure 28. Otitis media with effusion 40 dB), tympanometry (typical (air-fluid level) type B flat curve). An accurate and complete ENT examination must be performed. Endoscopy of the nasopharynx is mandatory, mainly in adults with unilateral disease, to rule out a nasopharyngeal malignancy! 3.4.5. Evolution. Complications Spontaneous recovery Chronic otitis media with effusion: the viscosity of the fluid increases (glue ear) Recurrent episodes of AOM caused by viral/bacterial infection of the effusion Retraction pockets, cholesteatoma 3.4.6. Treatment The recommended European standard 16 follows:
3.4.6.1. Conservative treatment: A trial of antibiotic therapy as for AOM is beneficial Nasal decongestants (drops/sprays) to alleviate the dysfunction of the Eustachian tube

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 Antihistamines (10 mg loratadine per day for adults and children older than 12 years) Eustachian tube ventilation exercises: autoinflation (Valsalva maneuver), inflation with a Politzer bag (Politzers method), or inflation with a catheter introduced through the nasal fossae into the nasopharynx at the level of the Eustachian tube opening
3.4.6.2. Surgical treatment: Myringotomy, followed by the insertion of a ventilating tube (grommet, pressure equalization tube PET). This procedure is indicated in cases with an air-bone gap higher than 30 dB, unresponsive to conservative treatment for 3 months, and in cases of development of retraction pockets Adenoidectomy

3.5. Chronic suppurative otitis media

3.5.1. Simple chronic suppurative otitis media (SCSOM)
3.5.1.1. Definition. Aetiology SCSOM is a chronic inflammatory condition of the mucosa covering the middle ear cleft, accompanied by a long-standing central perforation, with permanent or intermittent otorrhoea (active and inactive stages). It differs from chronic OME, which is defined by a middle ear effusion, without perforation, reported to persist more than 1-3 months. The involved micro-organisms 16: Pseudomonas aeruginosa (60-80%) Staphylococcus aureus (10-25%) Proteus (10-20%) Streptococcus viridans Enterobacter 3.5.1.2. Pathophysiology SCSOM may be the result of an AOM which has left a permanent unhealed perforation. In most cases, these long-standing central perforations

53

occur in patients with repeated episodes of infection owing to Eustachian tube dysfunction. This chronic dysfunction is the result of adenoid hypertrophy with repeated adenoid infections, impaired nasal breathing (septal deviations, allergy), chronic rhino-sinusal infections, cleft palate, immune deficiencies. The chronic perforation of the eardrum may be accompanied by destructive lesions of the ossicular chain.
3.5.1.3. Symptoms Chronic otorrhoea: mucoid, muco-purulent in acute stages. The ear discharge may be constant, but it may dry up at times to reappear with the onset of upper respiratory tract infections or accidental entrance of water into the ear Hearing loss Otalgia: only in acute exacerbations 3.5.1.4. Diagnosis Otoscopic examination reveals a typical central perforation, located always in pars tensa.The word central means that the perforation does not involve the eardrum margins! The shape of the perforation may be round, oval, kidney-shaped, sub-total. In active stages a mucopurulent otorrhoea is visible. After suctioning the ear secretions, we may notice the inflamed mucosa of the middle ear cleft through the perforation, granulation tissue around the margins of the perforation. Hearing assessment: tuning fork tests, pure-tone audiometry (conductive hearing loss; a sensorineural component is not common, but is possible Figure 29. Simple chronic otitis during evolution). media (central perforation of the A complete and eardrum) careful ENT examination must be performed, including endoscopic control of the nose and nasopharynx! Other useful diagnostic procedures: X-ray Schuller (may reveal a sclerotic mastoid or a reduction in the number of cells),

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paranasal sinuses X-ray, culture swab from the ear discharge with antibiogram.
3.5.1.5. Evolution. Complications This type of chronic suppurative otitis media is not characterized by development of serious life-threatening complications. Thats why it is called simple CSOM. The main complications are: Acute exacerbations: after accidental entrance of water in the ear, or after upper respiratory tract infections Diffuse external otitis: due to chronic otorrhoea Ossicular resorption or fixation Tympanosclerosis: calcified white plaques confined to the level of pars tensa. These lesions may be limited to the eardrum or may involve the whole middle ear cavity with subsequent blockage of the ossicular chain Mixed hearing loss (inner ear toxic involvement) 3.5.1.6. Treatment: The recommended European standard16 follows: 3.5.1.6.1. Conservative treatment: Periodic aggressive aural toilet: daily suction under microscopic control Topical treatment: quinolone ear drops are routinely prescribed twice or three times daily, for 7 to 10 days Oral or parenteral antibiotics may be occasionally beneficial for treating particularly active and resistant drainage Additional/useful therapeutic options: treatment of the nose, paranasal sinuses or nasopharynx, elimination if possible of allergic factors Patients with SCSOM respond more frequently to topical than to systemic therapy. Successful topical therapy consists of three important components: selection of an appropriate antibiotic drop, regular aggressive aural toilet and control of granulation tissue. Ciprofloxacin remains the most effective of the quinolones against Pseudomonas. Fluoroquinolones are not approved for use in children because they elicit joint injury in juvenile experimental animals. 55

The control of granulation tissue involves the use of topical antibiotic-steroid drops, chemical cautery using silver nitrate, excision using microinstruments.
3.5.1.6.2. Surgical treatment: Myringoplasty and tympanoplasty without mastoidectomy: if the perforation is dry, repair the eardrum perforation (myringoplasty) and eventually the ossicular chain lesions (tympanoplasty) Tympanoplasty with mastoidectomy: if suppurative drainage persists and the mastoid is involved

3.5.2. Cholesteatoma of the middle ear

3.5.2.1. Definition. Aetiology Cholesteatoma is recognized for decades as a destructive lesion of the skull base that can erode bone and damage important structures within the temporal bone. Its potential for causing central nervous system complications makes it a serious lifethreatening disease. A cholesteatoma consists of keratinizing squamous epithelium that is trapped within the temporal bone (skin in the wrong place). It has a matrix which continually desquamates and sheets of exfoliated keratin debris which accumulate and form the bulk of the cholesteatoma. 3.5.2.2. Pathophysiology: Three patterns of cholesteatoma formation are described 16: Congenital cholesteatoma: cholesteatoma is rarely a primary lesion. This type is characterized by the presence of a cholesteatoma behind an intact eardrum Primary acquired cholesteatoma: usually develops in relation with a chronic Eustachian tube dysfunction. The result is a persistent negative pressure in the middle ear cleft and the development of retraction pockets (which may perforate). These retractions are mostly located at the level of pars flaccida or in the postero-superior quadrant of pars tensa. Local inflammation processes (Pseudomonas aeruginosa, Proteus species) stimulate the production of keratin, the exfoliation and thus the cholesteatoma

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growth. Cholesteatoma has a great potential for bone erosion. Therefore, local destructive processes may develop during evolution, leading to serious complications Secondary acquired cholesteatoma: occur as a direct consequence of some type of injury of the tympanic membrane: perforation after AOM (unsafe marginal perforation), trauma or after ear surgery (iatrogenic)
3.5.2.2. Symptoms Chronic foul-smelling muco-purulent otorrhoea Hearing loss Otalgia (acute exacerbations) Tinnitus Dizziness, vertigo (it may suggest the presence of a labyrinthine fistula) Headaches (intracranial complications?) 3.5.2.3. Diagnosis Otoscopy reveals a foul-smelling otorrhoea, which must be removed (suction under microscopic control) before the eardrum can be accurately examined. The tympanic membrane perforation is marginal, located in pars flaccida or at the level of the posterosuperior quadrant of the eardrum. A white mass appears from the perforation, discharging white scales. Sometimes the visibility of the eardrum is obscured by the presence of granulation tissue or aural polyps. Audiometry: Pure-tone and speech audiometry are performed to evaluate the degree of conductive and sensorineural hearing loss. An ear swab culture may be indicated in infected cholesteatomas. Radiologic examinations: Schuller X-ray, High-resolution CT scans, MRI Vestibular testing: in case of suspicion of labyrinthine fistula.
Figure 30. Attic cholesteatoma

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3.5.2.4. Evolution. Complications Sometimes cholesteatoma is first diagnosed based on symptoms and signs determined by a serious complication: facial palsy, headaches, fever, vertigo. Its propensity for causing central nervous system complications makes it a potentially fatal lesion. 3.5.2.4.1. Extracranial complications: Sensorineural hearing loss: it has been suggested that toxins in chronic suppurative otitis media can damage the cochlea23 Labyrinthine fistula, labyrinthitis: fever, dizziness, vertigo, nystagmus, fistula sign Mastoiditis with sub-periostal abscess: fever, fluctuance over the mastoid area, lateral displacement of the pinna. It may present as Betzolds abscess, which represents extension of the abscess from the mastoid tip into the digastric groove (the pus erodes the cortical bone of the mastoid tip and exteriorizes under the superior insertion of the sternocleidomastoid muscle) Petrositis: retro-orbital pain, persistent aural discharge and diplopia caused by cranial nerve VI paralysis (the Gradenigo syndrome) Facial nerve palsy 3.5.2.4.2. Intracranial complications: are usually the result of direct spread of infection from the ear to the middle or posterior cranial fossae. This spread is favoured by bone erosion, congenital dehiscences, thrombosis of the veins between the middle ear and the meninges. Extra-dural abscesses, sub-dural abscesses Meningitis: headaches, fever, photophobia, neck rigidity, Kernigs and Brudzinskis signs, positive lumbar puncture Brain abscess: localized in the temporal lobe or in the cerebellum (headaches and nausea, seizures, focal neurologic signs, signs of intracranial hypertension) Sigmoid sinus thrombosis: rarely seen nowadays, this complication does still occur and is easily overlooked because of the masking effects of antibiotic therapy (septic-

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type fever, headaches, anemia, edema and tenderness over the posterior aspect of the mastoid bone) Otitic hydrocephalus: increased intracranial pressure in the absence of a brain abscess Modern imagistic techniques (CT scans, MRI) are mandatory for an accurate diagnosis!
3.5.2.5. Treatment 3.5.2.5.1. Conservative treatment: Periodic observation may be indicated in limited cases of a dry cholesteatoma in the only functional ear, in elderely patients or in individuals with very poor health 16 Medical therapy is not a viable treatment for cholesteatoma, it doesnt stop further expansion and does not eliminate risk The mainstay of anti-microbial therapy should be topical, but systemic therapy is occasionally a helpful adjunct 3.5.2.5.2. Surgical treatment: Virtually all cholesteatomas should be excised! Surgical therapy consists of cholesteatoma removal. The aims of surgery are eradication of disease, an epithelialized self-cleaning ear and hearing preservation or improvement 24. Two types of surgical procedures are available: Canal wall down mastoidectomies (open techniques): the traditional method for cholesteatoma removal was the modified radical mastoidectomy, in which the mastoid is opened behind the external canal, the cholesteatoma is identified and followed forwards through the aditus ad antrum into the attic, with the removal of the posterior bony wall of the external canal. The final result is a large cavity. Smaller cavities may be obtained using the anterior to posterior endaurally approach (attico-antrostomies) Canal wall up mastoidectomies (closed techniques) have the advantage of leaving an intact external canal and no mastoid cavities Reconstructive procedures for hearing improvement (myringoplasties, tympanoplasties) may be performed during initial surgery or at a later stage. Canal wall down techniques have lower rates of recurrences

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of cholesteatoma (5-15%), and recurrences are easily diagnosed in the outpatient clinic, so second look operations are not required. In canal wall up procedures the rate of recurrences is higher (20-50%). Therefore a second look surgery after 12-18 months is recommended in all cases 24.

3.6. Otosclerosis
3.6.1. Definition. Aetiology Otosclerosis is a localized hereditary disease affecting the endochondral bone of the otic capsule, that is characterized by disordered resorption and deposition of bone. An otosclerotic lesion consists of areas of bone resorption, new bone formation, vascular proliferation and a connective tissue stroma 25. The most common site for otosclerosis lesions is the area anterior to the oval window (80-95% of cases), followed by the round window niche (30% of cases) and the stapes footplate (12% of cases) 26. The final result in evolution is the fixation of the stapes footplate in the stapedio-vestibular joint, with subsequent conductive or mixed hearing impairment. Ankylosis of the stapes was first described by Valsalva in 1704. Politzer described this condition as an otic capsule disorder, characterized by abnormal new bone formation. Factors involved in otosclerosis aetiology: Genetic predisposition: otosclerosis is most common among Caucasians, uncommon among Asian people and extremely rare in black people. Women are two times more likely to develop the clinical disease than men27. Familial aggregation of individuals affected by otosclerosis has been recognized for many ears. New evidences suggest some otosclerosis cases may be related to defects in expression of the COL1A1 gene 28 Measles virus infection: measles RNA has been found in fresh footplate specimens with otosclerosis. Increased levels of anti-measles antibodies have also been reported in perilymph from patients undergoing stapedectomy, as compared to controls 29 Autoimmune disease: autoimmunity to type II collagen 60

or a closely related antigen that is present in the regions of predilection25 Hormonal factors: pregnancy sometimes accelerates the progression of the disease

3.6.2. Symptoms Often a family history is revealed Onset of symptoms: 2-3rd decade of life Slow-progressive unilateral (70%) or bilateral (30%) hearing loss Tinnitus (60%) Vestibular complaints 3.6.3. Diagnosis Otoscopic examination: the main clinical feature of otosclerosis is a progressive conductive/mixed hearing impairment with a normal tympanic membrane! Hearing tests: Tuning fork tests (Weber, Rinne) Pure-tone audiometry: pure conductive hearing loss (30%), mixed hearing loss (70%). In 20% of patients there is an elevation of 20-30 dB of the bone conduction threshold called Carharts notch19 Tympanometry: normal (type A), stapedius reflex is absent because of stapes fixation Speech audiometry Imaging techniques: high-resolution CT scans are capable to demonstrate the otosclerotic foci within the temporal bone. 3.6.4. Differential diagnosis Inflammatory conditions of the middle ear Post-traumatic lesions of the ossicular chain Tympanosclerosis Malleus head fixation Osteogenesis imperfecta (van der Hoeve syndrome): classic triad of hearing loss (conductive, mixed), spontaneous bone fractures, and blue sclera Paget disease of the bone: ear symptoms are identical to otosclerosis
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 Superior semicircular canal dehiscence: a recently described condition resulting in a low frequency inner ear conductive hearing loss19

3.6.5. Treatment
3.6.5.1. Conservative treatment: If the air-bone gap is less than 20 dB, no treatment is required Hearing aids: if the patient refuses surgical treatment or has strong contraindications for surgery Supplementation of fluoride 3.6.5.2. Surgical treatment Surgical treatment is recommended mainly in young patients with an air-bone gap higher than 20 dB. Stapedectomy: after total removal of the stapes footplate and stapes suprastructure, the oval window is covered with a graft and a commercial prosthesis is placed between the incus and the graft Stapedotomy: after removal of the stapes suprastructure, a small perforation of the footplate is created using a micro-drill or the laser, followed by the insertion of the prosthesis into the calibrated hole and fixation of the prosthesis to the long process of the incus

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4. DISEASES OF THE INNER EAR

4.1. Sudden sensorineural hearing loss (SSHL)
4.1.1. Definition. Aetiology SSHL is characterized by a 30 dB hearing loss in 3 contiguous frequencies, with the loss occuring 3 days30. Sometimes, the hearing loss is perceived by the patient on awakening. SSHL is an ENT emergency! Regarding the aetiology, we may refer to SSHL of a specific cause (clearly identified from history, clinical examination and specific investigations), and idiopathic SSHL. In less than 5% of cases a specific cause is found31. Specific causes of SSHL: Infections: viral (mumps, measles, herpes virus type II, herpes zoster, HIV), bacterial (syphilis, Borellia burgdorferi, meningitis, encephalitis) Vascular: haemorrhage, vascular occlusion, thrombosis, spasm Trauma: ear surgery, head injury, blast injury (an explosive blast may produce injury both as a result of the high intensity of the noise and the shock wave), perilymph fistula (abnormal communication between the perilymphatic space and the middle ear, most commonly occurs at the level of the round window membrane, the leak is usually associated with vestibular symptoms, could be determined by a minor trauma like barotraumas, physical exertions such as straining, lifting, coughing, vomiting) Tumors: acoustic neuromas Autoimmune Neurological: multiple sclerosis Haematological: hyperviscosity syndromes, sickle cell disease
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4.1.2. Symptoms: Abrupt unilateral hearing loss occurring in less than three days, or apparent on awakening Tinnitus (85% of cases) Sometimes the hearing loss is associated with vestibular symptoms: vertigo, nausea, vomiting (30% of cases) 4.1.3. Diagnosis: A detailed history is extremely important! Complete ENT examination Hearing and vestibular assessment Imaging techniques: MRI (to rule out an acoustic neuroma, multiple sclerosis) Blood cell count, haemoglobin, erythrocyte sedimentation rate (ESR), lipid profile, creatinine, urea and electrolytes, glucose, auto-antibodies, serologic tests for syphilis, Borellia, herpes viruses, HIV Internal medicine, neurological, endocrinological examinations 4.1.4. Evolution. Prognosis Complete spontaneous recovery is seen in about 50% of the patients31. Following treatment we may have a complete recovery, a partial recovery or no recovery. The presence of associated vestibular symptoms worsens the prognosis. 4.1.5. Treatment
4.1.5.1. Conservative treatment: Empirical treatment is usually initiated. Many regimens have been proposed, but there is no firm evidence to support the effectiveness of any of them. The most frequently used agents in the conservative management of SSHL 31: Short-term glucocorticoid steroids (Hydrocortisone sodium succinate i.v., Prednisolone i.v., intratympanic Dexamethasone, oral steroids) Agents to improve cochlear blood flow: Dextran, hydroxyethyl starch (HES), Pentoxifylline i.v., Procaine i.v.

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 Vasoactive agents: calcium channel blockers, Histamine, Nicergoline (Sermion) Vitamins and antioxidants: vitamin B1, B6, B12, vitamin C, A, E, nicotinic acid, -lipoic acid Others: antivirals (Acyclovir, Interferon), ATP, CO2/O2 inhalation (Carbogen), Ginko extracts, hyperbaric oxygen
4.1.5.2. Surgical treatment: In cases of fluctuating unilateral sensorineural loss associated with vestibular symptoms, a tympanoscopy should be performed in order to rule out a perilymphatic fistula. If a fistula is discovered, it should be sealed with a connective tissue graft (e.g. fat harvested from the ear lobule).

4.2. Progressive sensorineural hearing loss

4.2.1. Presbyacusis
4.2.1.1. Definition. Aetiopathology Presbyacusis or age-related hearing loss is a mid- to lateadult onset, bilateral, progressive sensorineural hearing loss 32. It is more or less symmetric and starts in the higher-frequency range, with or without tinnitus. The hearing impairment is determined by the normal, physiological process of aging, based on an individual genetic predisposition and, on the other hand, by exogenous degeneration of the inner ear structures (supporting cells, basilar membrane, outer hair cells). This degeneration is the result of various environmental influences, nutritional habits, toxicities etc. A hereditary component of prebyacusis has recently been demonstrated33. 4.2.1.2. Symptoms Slow-progressive hearing loss with insidious onset in the 5th-6th decades of life, decreased speech comprehension, mainly in ambient noise, discomfort in noisy environment, during phone calls, the TV is often louder than is comfortable for the others members of the family

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 Tinnitus (30-50% of cases) may be the most annoying symptom The hearing disability, the loss of ability to communicate and the presence of tinnitus may finally lead to behavioral alterations, psychosocial isolation
4.2.1.3. Diagnosis Detailed and accurate history Complete ENT examination Hearing assessment: tuning-fork tests, pure-tone audiogram, speech audiometry, tympanogram, auditory brain-stem responses (ABR) 4.2.1.4. Treatment Unfortunately, theres no therapeutic modality to replace the hearing that has been lost. Medical therapy is indicated only when the patient reports a rapid deterioration of the hearing loss or annoying tinnitus. The best management solution for presbyacusis is the prescription of binaural hearing aids associated with psychological counselling.

4.2.2 Noise-induced hearing loss

4.2.2.1. Definition. Aetiopathology The term noise-induced hearing loss refers to a reduction in auditory acuity associated with noise exposure. The term acoustic trauma has been used to describe the situation where a single exposure to an intense sound leads to an immediate hearing loss34. Chronic exposure to noise causes metabolic and mechanical structural damage at the level of the cochlea, leading subsequently to neuronal degeneration. Other involved factors: Apoptosis and necrosis: there is evidence that apoptosis (programmed cell death) play an important role in noiseinduced hearing loss35 Genetic susceptibility: there is experimental evidence from studies with mice, the Ahl gene being involved36 Smoking, diabetes, cardiovascular diseases The noise-induced hearing loss is a result of working in

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noisy environments (metal workers, mine workers, airports, disc jockeys, military personnel, etc.). Typically, the hearing loss initially occurs as a high-frequency SNHL, and a characteristic sensorineural notch (normally at 4 kHz) is visible on the pure-tone audiogram. Mid-frequencies, and low-frequencies are affected much later.
4.2.2.2. Symptoms Progressive bilateral hearing loss: at the debut the patients description involves a lack of clarity rather than a loss of volume. In time, the patient is confronted with problems of communication in noisy environments, problems during phone conversations, the TV set is louder than comfortable for the rest of the family Tinnitus (70% of cases)33 The hearing disability, the loss of ability to communicate and the presence of tinnitus may finally lead to behavioral alterations, psychosocial isolation 4.2.2.3. Diagnosis Detailed patient history, including professional and recreational sound exposure

Figure 31. Noise-induced hearing loss ( < : bone conduction right ear; : air conduction right ear; > : bone conduction left ear; : air conduction left ear; red arrrow: characteristic notch at 4000 Hz)

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 Otological examination is normal A complete ENT examination should be performed Hearing assessment: tuning fork tests, pure-tone audiometry (the characteristic sensorineural notch at 4000 Hz), speech audiometry, objective tests
4.2.2.4. Treatment A medical therapy to recover the hearing loss is not available. Steroids and vasodilators may be indicated in the management of annoying tinnitus. As the hearing loss becomes more severe, a hearing aid should be recommended. Prevention and counselling are very important: avoid noise exposure if possible, noise surveys for the employers, periodic hearing assessment, personal protection (earplugs, earmuffs).

4.2.3. Ototoxicity
4.2.3.1. Definition. Aetiology Ototoxicity is a chemical injury to the labyrinth occurring as a side effect of pharmacotherapy. An ototoxic insult may affect the hearing, the vestibular function or both 37. The most frequently encountered ototoxic agents are the aminoglycoside antibiotics and the chemotherapeutic agent Cisplatin (table 4).
Drugs Streptomycin Dihydrostreptomycin Neomycin Gentamicin Loop diuretics Salicylate Cisplatin Primarily ototoxic + +++ +++ + + + ++ Primarily vestibulotoxic +++ + + +++ + -

Table 4. Ototoxic compounds in clinical practice 33

There is evidence for a genetic susceptibility to aminoglycoside ototoxicity38. Aminoglycoside toxicity may occur after systemic administration or topical application to the tympanic cavity. 68

Drops containing aminoglycosides should not be administered in the presence of a tympanic membrane perforation. The vestibulotoxic effect of trans-tympanic gentamicin is used as a therapeutic method in Menieres disease.
4.2.3.2. Symptoms Bilateral, symmetric, progressive hearing loss High-pitched tinnitus Vertigo, dizziness, nausea 4.2.3.3. Diagnosis Otologic examination is normal Hearing evaluation: bilateral symmetric sensorineural hearing loss Vestibular function evaluation 4.2.3.4. Prevention. Treatment There are strong evidences in the medical literature about the protective effects of antioxidants and iron-chelators. This efficacy reflects the role of oxidative stress in ototoxicity39. In patients with renal failure, doses should be accurately adjusted. Permanent hearing loss is treated with a hearing aid or a cochlear implant. Vestibular toxicity is best managed by vestibular rehabilitation.

4.2.4. Hearing loss in children

4.2.4.1. Definition. Aetiology Hearing impairment in childhood refers to any unilateral or bilateral hearing loss, occurring from birth to late childhood. The hearing loss may be present at birth (congenital), or may be acquired after birth. Because the hearing impairment affects the acquisition of speech, it is classified in relation to the stages of speech development as prelingual (0-2 years of age), perilingual (2-4 years of age) and postlingual (more than 4 years)33. The hearing loss can be conductive, sensorineural or mixed. The most important causes for hearing loss in childhood are: 69

 Conductive hearing loss determined by otitis media with effusion secondary to repeated episodes of upper respiratory tract infections (see Sect. 3.4.) Other causes for conductive hearing loss: chronic suppurative otitis media, malformations of the external canal and middle ear Congenital sensorineural hearing loss: a genetic cause accounts for more than 50% of these cases. Almost three quarters of these are non-syndromic; one quarter are part of a hereditary syndrome carrying other specific features besides deafness. In most of the cases the hereditary course is autosomal recessive. The commonest findings are mutations in the gene GJB2, coding for connexin 26, a gap junction protein located in the inner ear which is essential for the maintenance of the endo-cochlear potential 40. Other causes for congenital hearing impairment are infections during pregnancy (rubella, cytomegalovirus, toxoplasmosis), inner ear malformations Acquired sensorineural hearing loss: in the perinatal period it may be caused by hypoxemia, severe infections, prolonged newborn icterus. Following the perinatal period the commonest aetiological factors are meningitis, infections (measles, mumps) and ototoxicity Progressive sensorineural hearing loss during childhood may be associated with various genetic syndromes (Pendred, Usher, Alport)
4.2.4.2. Symptoms Unresponsiveness to sound stimuli from the environment Delay or absence of speech acquisition 4.2.4.3. Diagnosis The recommended European standard 33: General neonatal screening is widely recommended (European consensus conference 1998). Methods available for screening are the otoacoustic emissions (OAE) and the auditory brain-stem responses (ABR) History and physical examination: note the presence of associated cranio-facial abnormalities, as part of various hereditary syndromes (Treacher Collins, Waardenburg etc.)

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 Hearing assessment: subjective auditory tests (behavioural response audiometry can be performed from 0-2 years, pure-tone audiometry can be performed starting from 4 years, speech audiometry) and objective measurements (tympanometry, OAE, ABR) Imaging techniques: high-resolution CT scans, MRI Evaluation of speech development Neurological and ophthalmological examination Genetic testing and counselling
4.2.4.4. Treatment 4.2.4.4.1. Conservative treatment: Monitoring the childrens development Fitting of hearing aids: as soon as possible after the hearing impairment has been confirmed 4.2.4.4.2. Surgical treatment: Conductive hearing loss: surgery may be performed in cases of OME unresponsive to conservative treatment (myringotomy with grommets insertion), cholesteatoma, ear malformations In children with severe or profound sensorineural hearing loss, cochlear implantation must be taken into account

4.2.5. Menieres disease See section 5.3.1.

4.3. Inner ear infections

4.3.1. Bacterial labyrinthitis
4.3.1.1. Definition. Aetiology Bacterial labyrinthitis may be the consequence of the dissemination of the infection from the middle ear space (AOM, cholesteatoma with perilymphatic fistula), or from the subarachnoid spaces during the evolution of a purulent meningitis. The most common agents involved: -haemolytic streptococci, pneumococci, staphylococci, Haemophilus influenzae, Proteus vulgaris and Pseudomonas aeruginosa41.

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According to Schuknecht 42, bacterial labyrinthitis can be classified into three stages based on the pathogenesis: Serous labyrinthitis is an irritation of labyrinth induced by the bacterial toxins invasion either from an acute or chronic otitis media, labyrinthine fistula or meningitis, leading to vertigo and sensorineural hearing loss Acute suppurative otogenic labyrinthitis (a rare condition nowadays) is the consequence of bacterial invasion into the inner ear from the middle ear clefts, the final outcome being the development of a destructive peripheral cochleo-vestibular syndrome Suppurative meningogenic labyrinthitis: implies a dissemination of bacteria from the subarachnoid spaces
4.3.1.2. Symptoms Otogenic labyrinthitis: severe or complete hearing loss, vertigo, nausea, vomiting, high fever Meningogenic labyrinthitis: classic symptoms of meningitis, severe vertigo, nausea, vomiting, unilateral or bilateral often fluctuating hearing loss or complete deafness 4.3.1.3. Diagnosis Recommended European standard33: Otoscopic examination: purulent otitis media, cholesteatoma Hearing evaluation Vestibular function evaluation High-resolution emergency CT scan Microbiology: cultures taken from the ear discharge or from the nasopharynx, CSF diagnostic, serology for syphilis 4.3.1.4. Treatment 4.3.1.4.1. Conservative treatment: Wide-spectrum antibiotics in high-doses Vestibular suppressant medications and antiemetics 4.3.1.4.2. Surgical treatment: Myringotomy Mastoidectomy, labyrinthectomy

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4.3.2. Viral labyrinthitis

4.3.2.1. Definition. Aetiology This condition develops during the evolution of a viral upper respiratory tract infection including the middle ear (influenza viruses, parainfluenza viruses, picornaviruses, respiratory syncytial viruses, adenoviruses, coronaviruses etc.). 4.3.2.2. Clinical picture The clinical presentation is of an upper respiratory infection, associated with an acute disturbance of auditory and vestibular function, with vertigo and nystagmus lasting for three to five days43. Varying degrees of permanent hearing loss may be noted. 4.3.2.3. Diagnosis Recommended European standard33: Otoscopy: normal, serous otitis media Hearing and vestibular assessment CT scan Cultures taken from the nasopharynx, CSF diagnostic, serology for rubella virus, paramyxoviruses, influenza virus, adenoviruses, syphilis 4.3.2.4. Treatment Glucocorticoids i.v. Antibiotics to prevent bacterial superinfection Vestibular suppressant medication and antiemetics Treatment of the rhinogenic infection: nasal sprays, mucolytics

4.3.3. Mumps Deafness appears in as much as 4 per cent of adult cases of epidemic parotitis44. It is often severe and usually unilateral. Vertigo has also been reported in mumps45. 4.3.4. Otosyphilis
4.3.4.1. Definition. Aetiology. Pathology The bacterial agent involved is spirocheta Treponema pallidum. Syphilis may determine a meningo/labyrinthitis or an osteitis of

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the temporal bone with secondary changes of the membranous labyrinth, characterized by the development of an endolymphatic hydrops (abnormal increase of endolymph pressure) and degenerative changes of the sensorineural structures41.
4.3.4.2. Symptoms Hearing loss: may be with sudden onset, or slowlyprogressive and fluctuating Tinnitus Vertigo, nausea, vomiting Symptoms are similar to Menieres disease, with attacks of vertigo lasting hours and fluctuating progressive sensorineural hearing loss. 4.3.4.3. Diagnosis Otoscopy: normal Hearing and vestibular assessment Serology for Treponema pallidum CT scans 4.3.4.4. Treatment Penicillin G Vestibular suppressants and antiemetics

4.4. Management of hearing impairment

Management of patients with a hearing loss can be determined by the degree of the hearing impairment, irrespective of whether the impairment is sensorineural, conductive or mixed. For ears with a mild, moderate or severe impairment, hearing aids are the main option. For those with a profound or total impairment, cochlear implants are the more suitable management strategy. For all subjects, some accessory devices can be of benefit46. For the child with a congenital hearing loss, the problem is more complicated because the child has not yet learned the symbols of the language system. The main objective is not to restore a skill that once existed (as in adults), but to help the child develop a new skill, the ability to communicate. 74

4.4.1. Conventional hearing aids Hearing aids are described according to where they are worn. Nowadays, six types of hearing aids are available: body aid, eyeglass (spectacle) aid, behind-the-ear (BTE) aid, in-the-ear (ITE) aid, in-the-canal (ITC) aid, and completely-in-the-canal (CITC) aid47. The hearing aid is referred to as an electroacoustic device. Initially, an ear-level microphone converts the acoustic signal (such as speech sound) into an electrical signal. The electrical signal is amplified and manipulated in various ways by a processor, and finally is reconverted to an acoustic signal by a receiver, which is delivered to the ear canal of the wearer47. Hearing aids can be classified by the way they process the sounds into analogue, digitally programmable analogue and fully digital types. 4.4.2. Bone-anchored hearing aids Some patients with hearing loss are unable to use conventional air conduction hearing aids because of pinna abnormalities, atresia of the external auditory canal or chronic discharging ear diseases. In these conditions, bone conduction hearing aids represent a viable alternative. The traditional bone conduction hearing aids deliver amplified sound to the cochlea through a bone vibrator placed on the mastoid. They were used formerly with body or eyeglasses aids. In recent years, the bone-anchored hearing aid (BAHA) has been developed, and appears to avoid many of the disadvantages of conventional bone conduction hearing aids. BAHAs provide mechanical vibration that is transmitted to the skull by way of a titanium screw embedded in the mastoid. A small titanium implant in the skull, behind the ear, osseointegrates (bonds) with the living bone. An abutment is attached to the implant and a sound processor is clipped on. The processor can be worn or taken off at any time48. 4.4.3. Cochlear implants In the 1980s, the cochlear implant emerged as a reliable alternative to conventional amplification for individuals with profound hearing impairment.
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The cochlear implant is a device that is surgically implanted, with its stimulating electrode array (wire) inserted directly into the cochlea. The implant consists of 1 to 22 channels. The electrode is used to stimulate the auditory nerve directly with electric current, bypassing the damaged cochlear structures47. For older children and adults, the ideal candidates are those who acquired profound bilateral sensorineural hearing loss after acquiring language. Cochlear implants, however, appear to hold even greater promise for profoundly impaired children under 2 or 3 years of age, although research regarding the comparative benefits of rehabilitative devices is still in progress. Cochlear implantation can be performed as early as 8-12 months, if indicated. The presence of viable auditory nerve fibers is fundamental to the success of cochlear implantation49. Cochlear implantation is not possible if the auditory nerve is absent. In these cases, brainstem implants may be an alternative approach33. Preoperative imaging is mandatory.Two modalities are available: high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI). MRI is more accurate at identifying cochlear dysplasia and the presence of the cochlear nerve. The most common abnormalities encountered are the ossified cochlea (most frequently after meningitis) and the cochlear dysplasia (1.5% of the cases)46. A variety of surgical approaches have been developed to access the scala tympani of the inner ear, the preferred placement for the implant. Almost all surgeons use a transmastoid approach. Children with cochlear implants require regular programming and control of the speech processor, which is best ensured in multidisciplinary cochlear implant rehabilitation programmes. Additional speech and language therapy is necessary, together with general support, careful choice of educational settings and parents counselling 33.

4.4.4. Middle ear implants Middle ear implants represent a modern alternative to conventional amplification aids. Current devices are suitable for patients with mild to severe sensorineural hearing loss. The hearing loss should ideally be stable; however, very slowly
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progressive losses can be considered. Classically, a normal middle ear function is required. The indications are expanding and extended applications are being explored. Devices have been applied to stimulate the round window membrane directly, in various types of conductive and mixed hearing loss. The device consists of a microphone, an amplifier and a transducer. The transducer uses either a coil and a magnet (electromagnetic) or a piezoelectric mode of transmission, and is connected to one of the middle ear ossicles, or cochlear windows46. A major problem of these devices has been to produce a device that is small enough to fit within the middle ear cavity, and yet powerful enough to supply the required gain. Current devices available50 are: Vibrant Soundbridge (Symphonix, Med-El): an active semi-implantable device, consisting of an internal, surgically implanted vibrating ossicular prosthesis (VORP), coupled to the long process of the incus, and an external audio processor OtologicsTM middle ear transducer (MET): a fully implantable device, consisting of a subcutaneous microphone and an electronic receiver connected to a transducer, coupled to the body of the incus EsteemR-Hearing ImplantTM: a fully implantable piezoelectric device, comprising a piezoelectric sensor on the incus body and a driver cemented to the stapes head; the implantation requires disarticulation of the ossicular chain

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5. PERIPHERAL VESTIBULAR SYNDROME

5.1. Vertigo
The most important clinical feature in the diagnosis of peripheral vestibular disorders is its pattern of presentation. A detailed and accurate history is of paramount importance! Vertigo is the main symptom of the peripheral vestibular syndrome. It is characterized by an acute sensation of abnormal rotatory movement of the surroundings in relation to the patient or of the patient in relation to his/her surroundings (suddenly everything spins round). It is accompanied by vegetative symptoms: nausea, vomiting, sweating, tachycardia, but never loss of consciousness. It is very important to differentiate the true vertigo from other chronic disequilibrium sensations described by patients in various ways: dizziness, giddiness, light-headedness etc. It has been pointed out that even the most enthusiastic doctor may experience a decline in spirits when faced with such patients. A minimum vertigo history should address the following: Does the sensation of imbalance occur in attacks or is chronic? The duration of the individual attack: seconds, hours or days? The frequency of the crises: daily, monthly? The effect of head movements Is there a specific position that brings on the attack? (e.g. rolling onto the right side in bed) Associated otologic symptoms: hearing loss, tinnitus Associated neurologic symtoms Concomitant ear disease: otorrhoea, prior ear surgery, ear trauma One of the most important features of the pattern of presentation is the duration of the attack. Based on this, the following classification of peripheral vestibular disorders is put forward 5: 78

A. Vertigo lasting minutes to hours: Idiopathic endolymphatic hydrops (Menieres disease) Secondary endolymphatic hydrops (Otosyphilis) B. Vertigo lasting seconds: benign paroxysmal positional vertigo (BPPV) C. Vertigo lasting days: vestibular neuronitis D. Vertigo of variable duration: Inner ear fistula Inner ear trauma Superior semicircular canal dehiscence syndrome

5.2. Examination of the vestibular function

5.2.1. Clinical examination of eye movements (Nystagmus) Nystagmus is characterized by involuntary, rhythmic, oscillatory movements of the eyes. Nystagmus arising from vestibular disorders has two components: a slow labyrinthine phase in one direction, and a fast correcting cerebral or voluntary phase in the other. The fast phase is the one visible during examination and is used to define the nystagmus direction. The main nystagmus characteristics in the peripheral vestibular syndrome are: It is always present during the attack It is nearly always horizontal-rotatory or torsional (the eyes beat like a car windscreen wiper), and it is named after the direction of the fast phase It is unidirectional It is always directed to the irritated labyrinth, away from the paretic labyrinth It is associated with auditory symptoms and vertigo It is visually suppressed: thats why it is better visualized using the Frenzel glasses (20 diopter lenses that magnify the eyes and eliminate eye fixation) Nystagmus of central origin beats in any direction, is often vertical or pure horizontal, is not suppressed by visual fixation, is not accompanied by auditory symptoms, vertigo may or not be present, is associated with other neurological symptoms and signs.
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Figure 32. Nystagmus

5.2.2. Romberg test The Romberg test was originally described as a test for tabes dorsalis (neurosyphilis). Ask the patient to stand erect with feet together and eyes closed. Its very important to stand nearby the patient as a precaution in order to stop the person from falling over and hurting himself or herself ! Watch the bodys deviations in relation to a perpendicular object behind the subject (corner of the room, door, window etc.). If a destructive lesion in the vestibular system is present, the patient tends to fall toward the side of the lesion. So, deviations are always to the paretic labyrinth, opposite to the nystagmus. The sharpened Romberg test is performed asking the patient to stand heel to toe, with one foot in front of the other. This test is required to detect abnormalities mainly in younger patients51. 5.2.3. Unterberger test The patient is asked to step in place, keeping the eyes closed. Deviations will be always directed to the paretic labyrinths side.
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Figure 33. Romberg test

5.2.3. The Dix-Hallpike manoeuvre One of the most common causes of vertigo is the so-called benign positional paroxysmal vertigo (BPPV), accounting for 25% of all patients with dizziness and vertigo. The Dix-Hallpike manoeuvre is one of the most important tests for patients who experience true vertigo. This test involve having the patient seated (with Frenzel glasses, if available) on the examination couch in such a way that when moved into the supine position the head and neck will extend beyond the edge of the couch. The head is held by the examiner and is moved abruptly in the supine position, to the left and to the right side. This procedure will cause a vestibular attack, so the patient must be warned before. Eye movements are recorded. The main characteristics of this nystagmus are1: Fixed direction Torsional (horizontal rotatory) Occurs after a latency period of seconds Lasts less than one minute
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 Fatigability: weaker and shorter responses when repeating the procedure

5.2.4. Caloric test Caloric testing remains the most useful test in determining the responsiveness of a labyrinth. It is one of the few tests that allow one labyrinth to be studied independently of the other 52. The principle of the caloric testing is that changes in the temperature of the external ear canal influence the activity level of the vestibular labyrinth. The ear canal is irrigated with water (syringing) or air. In the conventional procedure two temperatures are used, one above and one below the body temperature. Water irrigation at 30 and 43 C (37 7 C) is the standard original technique, described by Fitzgerald and Hallpike53. Each irrigation lasts 40 seconds. The patient lies down with the head raised 30 above horizontal. This places the horizontal semicircular canal in an approximately vertical position. Irrigation with cold water induces a nystagmus beating in the opposite direction of irrigation. Warm water causes an ipsilateral beating of the nystagmus54. 5.2.5. Other tests for vestibular function evaluation Rotational tests: patient is seated in a rotating chair, both labyrinths being stimulated simultaneously Electronystagmography, videonystagmography: the eye movements can be recorded electrically or using a video camera Posturography: is an objective Romberg test using the Luzern platform Click-evoked vestibular myogenic potentials: loud sounds, brief in duration (clicks) can stimulate the labyrinth

5.3. Vertigo: clinical syndromes

The vestibular syndrome can be classified in two main categories: The peripheral vestibular syndrome: its management belongs to the ENT physicians 82

 The central vestibular syndrome: its management belongs to the neurologists The peripheral vestibular syndrome can be further divided in two categories: Irritative: serous labyrinthitis, perylimph fistula, Menieres disease (first hours of the first crises) Destructive: trauma, purulent bacterial labyrinthitis, Menieres disease, drugs Another classification of peripheral vestibular syndromes focuses on the presence or absence of hearing impairment: With hearing loss: Menieres disease, labyrinthitis, trauma, perylimph fistula, drugs Without hearing loss: BPPV, vestibular neuronitis, drugs
Central Imbalance Neurologic symptoms Nystagmus Hearing loss Nausea Recovery by central compensation Severe Frequent Changes direction in different gaze positions; not influenced by visual fixation Rare Variable, may be absent Slow Peripheral Mild to moderate Rare Unidirectional in all gaze positions; suppressed by visual fixation Frequent Severe Rapid

Table 5. Features differentiating central from peripheral vestibular syndromes55

5.3.1. Menieres disease

5.3.1.1. Definition Menieres disease is a disorder of the inner ear characterized by recurrent spontaneous attacks of vertigo, fluctuating progressive sensorineural hearing loss, tinnitus and often a sensation of aural fullness. It was first described by Prosper Meniere in 1861. 5.3.1.2. Aetiology. Pathology Menieres disease is considered to be idiopathic. The main pathological mechanism involved is an overproduction or malabsorption of endolymph, resulting in endolymphatic hypertension.This hypertension finally results in gross enlargement

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of the membranous labyrinth (endolymphatic hydrops) 56. Periodic ruptures of the membranous labyrinth, which result in leakage of the potassium-rich endolymph into the perilymph, are supposed to be the trigger factor for Menieres attacks 57. Some inner ear diseases (e.g. otosyphilis) may develop during evolution an endolymphatic hydrops (delayed or secondary hydrops) with a clinical picture similar to Menieres disease (socalled Menieres syndrome). The main factors involved in the aethiopathology of Menieres disease are: Obstruction of the endolymphatic duct: the basis for development of hydrops in experimental animals 5 Autoimmune processes: studies of the human endolymphatic sac has suggested it is the primary immunocompetent structure of the inner ear, capable of processing antigens, synthesizing antibodies, and generating a cellular immune response 58 Reactivation of a latent viral infection: Herpes simplex virus type159,60
5.3.1.3. Symptoms Recurring attacks of spontaneous vertigo, associated with nausea and vomiting, lasting from several minutes to hours Fluctuating, progressive sensorineural hearing loss Tinnitus Aural fullness Between attacks patients dont experience vertigo, but the hearing loss is present and is augmented by the next crisis. Generally, the attacks increase in frequency and severity with progression of the disease. 5.3.1.4. Diagnosis History is extremely important Hearing evaluation: tuning fork tests, pure-tone audiometry (fluctuating, progressive sensorineural hearing loss with a flat pattern in later stages), speech audiometry, electrocochleography, auditory brainstem responses (ABR) Vestibular evaluation: between attacks the patient displays normal findings

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 Imagistic techniques: MRI with Gadolinium, to rule out a retro-cochlear lesion (vestibular schwannoma) The American Academy of Otolaryngology-Head and Neck Surgery has published diagnostic guidelines for Menieres disease61. A diagnosis of definite Menieres disease can be made if the patient has two or more spontaneous attacks of vertigo (each lasting 20 minutes or longer), hearing loss documented on pure-tone audiometry on at least one occasion, tinnitus or aural fullness on the affected side, and other possible causes excluded.
5.3.1.5. Treatment The Recommended European Standard 33: 5.3.1.5.1. Conservative treatment: In acute attacks bed rest, vestibular suppressant medication (diazepam) and antiemetics are recommended. To prevent attacks: Diet: low salt intake (less than 3 g per day) and decreased water intake Diuretics: acetazolamide, chlortalidone, hydrochlorothiazide, furosemide Vasoactive drugs (betahistine), to improve blood circulation in the inner ear Steroids, to suppress the inflammatory and/or allergic tissue reactions within the labyrinth (endolymphatic sac) Avoidance of alcohol, caffeine, smoking Antiviral approach: oral acyclovir 62 5.3.1.5.2. Semiconservative treatment: Insertion of a transtympanic ventilating tube, followed by transtympanic unilateral chemical labyrinthectomy with gentamicin In bilateral cases, intramuscular streptomycin Following insertion of a ventilating tube, self-administered treatment with the Meniette device (intermittent lowpressure pulses to stimulate the flow of the endolymph) Intratympanic dexamethasone injections Intratympanic application of antiviral agents (ganciclovir)63

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5.3.1.5.3. Surgical treatment: Endolymphatic sac decompression procedures (saccotomy). Complete resolution of vertigo is reported in about 50-75% of patients. The efficacy of these procedures was seriously doubted by some authors. Foremost in this controversy is the sham study of Thomsen from 1981, in which the authors performed a simple mastoidectomy with or without an endolymphatic decompression procedure, and reported identical results in both groups5,64 Selective vestibular nerve neurectomy: 98% control rates Labyrinthectomy: implies a complete hearing loss Tenotomy: sections of the tendons of tensor tympani and stapedius muscle in the middle ear cavity (a new approach)

5.3.2. Vestibular neuritis

5.3.2.1. Definition. Aetiology Vestibular neuritis is an acute condition characterized by a single severe spontaneous attack of vertigo, lasting days, caused probably by a viral inflammation. A viral infection of the vestibular nerve is considered to be the main aetiological factor. Latent infection with Herpes simplex virus type1 of the superior and inferior vestibular ganglia has been documented65, 66. Due to immunologic deficiencies, the viruses are reactivated and subsequently destroy vestibular sensory fibers. 5.3.2.2. Symptoms Acute spontaneous severe vertigo attack typically lasting days, with gradual improvement during evolution No hearing loss, no tinnitus! No other neurological symptoms 5.3.2.3. Diagnosis History Hearing evaluation: normal Vestibular function evaluation: horizontal rotatory nystagmus beating towards the intact side, Romberg deviations to the affected side, caloric testing proves the hypofunction of the affected side

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5.3.2.4. Evolution The acute clinical manifestations of the disease invariably subside over the following days as a consequence of central vestibular compensation. Nearly 25 per cent of the patients will develop a BPPV on the affected side67. 5.3.2.5. Treatment steroids, vasodilators, intravenous fluid support vestibular suppressants and antiemetics vestibular rehabilitation

5.3.3. Benign paroxysmal positional vertigo (BPPV)

5.3.3.1. Definition. Aetio-pathology BPPV is the most common cause of vertigo seen by ENT doctors, representing 20% to 40% of patients with peripheral vestibular disease5. It is a vestibular disorder characterized by brief attacks of vertigo (lasting less than a minute) precipitated by certain changes in head position, and caused by a cupulolithiasis or a canalolithiasis. Otoconia (otoliths) are calcium carbonate crystals which are normally embedded in the gelatinous otolithic membranes of the utricle and saccule. In some conditions (trauma, old age, idiopathically), otoconia are released from these membranes and start to freely float in the endolymph. In a certain head position the otoliths reach and stimulate the ampula of the semicircular canals (posterior canal is the most frequently involved 98%), causing the typical vertigo. BPPV may occur as a complication in the evolution of a head trauma or a vestibular neuritis 67 5.3.3.2. Symptoms Brief attacks of vertigo, lasting only seconds (typically less than a minute). The attacks are triggered by certain head positions (for example, rolling onto the right side in bed) and may be accompanied by nausea and occasionally vomiting No hearing loss and tinnitus! No other neurological symptoms

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5.3.3.3. Diagnosis History Dix-Hallpike manoeuvre (see section 5.2.3.) 5.3.3.4. Treatment There is no medical treatment for BPPV The most effective current therapy is organized around the so-called repositioning manoeuvres (Epley and Semont) that use gravity to move out the otoliths from the affected canal into the vestibule Only in extremely rare cases (less than 1%) surgical treatment can be indicated: selective neurectomy of the posterior canal nerve or posterior semicircular canal occlusion procedure33

5.3.4. Vestibular schwannoma

5.3.4.1. Definition. Aethio-pathology Vestibular schwannoma is a slowly growing benign tumor which arises from the Schwann cells of the vestibular nerve, within the internal auditory canal. In evolution, it may extend into the cerebellopontine angle 19. The 1992 National Institutes of Health Consensus Conference made vestibular schwannoma the official nomenclature for these lesions, replacing the old term acoustic neuroma 68. The tumors are usually solid, but may present cystic areas. The growth rate is slow, with an average of 0.2 cm per year. If not treated they are potentially lethal, gradual enlargement leads to indentation of the brainstem, increased intracranial pressure and death during a course of 5 to 15 years68. Vestibular schwannomas can present as sporadic tumors (95% of the cases), or as part of the familial disorder neurofibromatosis type 2 (5%). 5.3.4.2. Symptoms Slow-progressive unilateral sensorineural hearing loss with poor speech discrimination (retro-cochlear lesion) In 5-20% of the cases patients may present with a sudden sensorineural hearing loss Unilateral tinnitus

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 Mild balance disturbances: not a true vertigo Facial hypoesthesia due to compression of the Vth cranial nerve Facial palsy due to compression of the VIIth cranial nerve Cerebellar ataxia, symptoms of raised intracranial pressure (headaches, visual disturbances, nausea, vomiting, mental status changed) in late stages of evolution
5.3.4.3. Diagnosis Otoscopy is normal Hearing evaluation: tuning fork tests, pure-tone audiometry (unilateral or asymmetric bilateral sensorineural hearing loss), speech audiometry (poor speech discrimination), auditory brainstem responses (ABR) Vestibular evaluation Imaging techniques: CT scan may detect large tumors, MRI with gadolinium enhancement is the gold-standard method for diagnosing vestibular schwannomas Neurologic and ophtalmologic examinations

Figure 34. MRI - Right vestibular schwannoma

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5.3.4.4. Treatment The Recommended European Standard19: 5.3.4.4.1. Conservative treatment: Wait and see strategy, mainly in elderly patients and individuals with poor health and serious contraindications for surgery Radiotherapy: stereotactic radiosurgery (gamma knife) or fractionated stereotactic radiotherapy (55-60 Gy) Annual imaging is recommended for all patients being managed conservatively 5.3.4.4.2. Surgical treatment: Translabyrinthine approach Middle fossa approach Retrolabyrinthine or retrosigmoid approach A multi-disciplinary team (otologic surgeon, neurosurgeon) is the key for success.

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II. NOSE

1. NOSE AND PARANASAL SINUSES BASICS

1.1. Clinical anatomy
1.1.1. External nose and nasal vestibule External nose (nasal pyramid) is a bony and cartilaginous prominence covered by skin, located at the level of the mid-face. Its shape is very important for facial aesthetics. The bony framework consists of the paired nasal bones and the frontal (ascending) processes of the maxillary bones. The cartilaginous supporting framework consists of the upper lateral cartilages (triangular cartilages), the lower lateral cartilages (alar cartilages with the lateral and medial crurae) and the midline septum. The medial crurae of the alars are loosely attached to each other in the midline and contribute to the columella. Attached to the external surface of the cartilages are the muscles for dilating the nares. The pyriform bony aperture is bounded inferiorly and laterally by the maxilla, and superiorly by the nasal bones. The nasal vestibule is a dilated passageway leading from the external nares (nostrils) into the nasal cavities (fossae). The boundary between nasal vestibule and nasal fossae is called limen nasi and is located at the level of the superior margin of the alar cartilages. The nasal vestibule is covered by skin bearing hair follicles (vibrissae), sebaceous and sweat glands.
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Figure 1. Nasal anatomy

1 - Glabella; 2 - Root of the nose; 3 - Nasion; 4 Nasal bone; 5 - Ascending process of the maxilla; 6 - Upper lateral cartilage (triangular cartilage); 7 - Anterior septal cartilage; 8 - Lateral crus of the alar cartilage; 9 - Medial crus of the alar cartilage; 10 - Columella; 11 - Nasal vestibule

Figure 2. Nasal anatomy

1 - Septal cartilage; 2 - Medial crus of the alar cartilage; 3 - Lateral crus of the alar cartilage; 4 - Nasal vestibule; 5 - Anterior nasal spine

1.1.2. Nasal cavities The nasal cavity extends from the limen nasi to the posterior nasal orifices (choanae). These choanae allow the posterior communication between the nasal fossae and the nasopharynx. Each nasal cavity has a floor, a roof, a medial wall and a lateral wall. The floor separates the nasal cavity from the oral cavity, is nearly horizontal and consists of the palatine process of the maxilla (3/4 anteriorly) and the horizontal process of the palatine bone (1/4 posteriorly). The roof is narrow and may be divided from anteriorly to posteriorly into frontonasal, ethmoidal and sphenoidal parts. The ethmoidal part consists of the cribriform horizontal plate. It is covered by sensory olfactory epithelium. The nasal septum has a posterior bony framework (the perpendicular plate of the ethmoid bone and the vomer), and an anterior cartilaginous part (the quadrilateral cartilage). 96

Deviations may develop at any of the septal level, and spurs may be noticed. They are most likely to be acquired due to trauma than congenital1. The septum is covered by mucoperichondrium, mucoperiosteum and mucosa. The lateral nasal wall displays three pairs of small, thin, shell-like bones: the superior, middle and inferior conchae, which form the bony framework of the turbinates. Each of these curved turbinates overhangs a groove known as a meatus (superior, middle and inferior). The inferior turbinate is an independent bony structure attached to the lateral wall of the nose, while the middle and the superior turbinates belong to the ethmoid bone. The inferior meatus 2 is that part of the lateral wall, lateral to the inferior turbinate. The nasolacrimal duct opens at this level. The middle meatus is located lateral to the middle turbinate and receives drainage from the frontal sinus, maxillary sinus and anterior ethmoidal cells. This meatus is the surgical approach in functional endoscopic sinus surgery (FESS). The middle meatus contains several well-defined anatomical structures that represent significant surgical landmarks: the uncinate process, the ethmoidal bulla, the inferior and superior semilunar hiatuses, the ethmoidal infundibulum, the frontal recess. The so-called ostiomeatal complex cannot be considered an anatomical structure. It is a physiological entity of the anterior ethmoid, where frontal, ethmoidal and maxillary sinuses drain3. The superior meatus is the drainage area for the posterior ethmoidal cells. The opening of the sphenoid sinus lies in the sphenoethmoidal recess, medial to the superior turbinate. The turbinates are covered by respiratory ciliated epithelium. Underlying the mucosa, there is an erectile tissue, mainly at the anterior end of the inferior and middle turbinate.

1.1.3. Paranasal sinuses

1.1.3.1. The frontal sinus The frontal bone forms the forehead and orbital roof and is pneumatized to a variable degree. The size and the shape of the frontal sinuses present a lot of variations. In a small percentage of cases it may be absent. The frontal sinus drains in the middle meatus (frontal recess).

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Figure 3. Nasal septum

Figure 4. Frontal section through the nose and paranasal sinuses

1 - Maxillary sinus; 2 - Inferior turbinate; 3 - Middle turbinate; 4 - Middle meatus; 5 - Nasal septum; 6 - Orbit

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1.1.3.2. The maxillary sinus The maxillary bone contributes to the formation of the greatest part of the roof of the oral cavity, the lateral wall and the floor of the nasal cavity and the floor of the orbit. Within the maxillary bones body lies the maxillary sinus (antrum). The maxillary sinuses are relatively symmetrical and rarely absent. The maxillary sinuses drain into the middle meatus (at the base of the ethmoidal infundibulum). The maxillary sinus is covered by ciliated respiratory epithelium which contains the highest density of secretory goblet cells, compared to the other paranasal sinuses (median: 9700/ mm2)4. Anatomical relations: Superior: the floor of the orbit Inferior: hard palate, upper dentition Anterior: skin of the cheek, fat, muscles Posterior: pterygopalatine and infratemporal fossae 1.1.3.3. The ethmoid sinus The ethmoid bone contributes to the formation of the medial orbital wall (a thin bony structure called lamina papyracea), the superior and lateral wall of the nasal cavity (see Sect. 1.1.3.) and the bony nasal septum (the perpendicular plate). The complex ethmoidal cells system is divided by the insertion of the middle turbinate into an anterior and a posterior group. The anterior group drains within the middle meatus, while the posterior group drains within the superior meatus. Anatomical relations: Lateral: the orbit Medial and inferior: the nasal cavity, middle and superior meatus Superior: the skull base 1.1.3.4. The sphenoid sinus The sphenoid sinus lies within the body of the sphenoid bone, at the midportion of the skull base. The natural drainage ostium is located in the sphenoethmoidal recess, superior and medial to the superior turbinate. The sinuses are divided by a paramedian septum. It is completely absent in nearly 1% of the population4.

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1.1.4. Vascular and nerve supply The vascular supply of the superior part of the nose comes from the anterior and posterior ethmoidal arteries, derived from the ophthalmic artery (which is a terminal branch of the internal carotid artery ICA). The lower part is supplied by branches derived from the maxillary artery (branch of the external carotid artery ECA). These internal and external carotid sources anastomose freely at the level of the nose. The Kiesselbachs area (or Littles area) is located on the anterior part of the nasal septum. It is an aggregation of poorly supported blood vessels, being the most frequent site for the anterior epistaxis. The sensory nerve supply of the nose is derived from the first two branches of the trigeminal nerve: ophthalmic and maxillary nerves. The sphenopalatine ganglion is not a sensory ganglion, it belongs to the parasympathetic system. The motor innervation of the noses muscles is supplied by the facial nerve.

Figure 5. Vascular supply of the nasal septum

1 - Anterior ethmoidal artery; 2 - Branches of posterior ethmoidal artery; 3 - Sphenopalatine artery; 4 - Greater palatine artery; 5 - Kiesselbachs plexus

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1.2. Physiology of the nose

1.2.1. Nasal breathing The nose acts as an air conditioner and performs three major functions: heat transfer, humidification and filtration. Because of its ability to transfer heat, the nose may be more important in temperature regulation than in respiration5. The anterior nasal valve is the narrowest part of the airway, and therefore the greatest resistor, producing the most turbulent airflow6. It is delineated by the inferior margin of the upper lateral cartilages, the head of the inferior turbinate and the adjacent nasal septum. The nasal cycle is a physiological alternate nasal blockage between the two nasal passages. It was known by the Yogis since antiquity, but Kayser gave its first description in 18957. It is produced by vascular activity, particularly the blood volume on the venous sinusoids of the turbinates (capacitance vessels). It can be demonstrated in 80% of the people. 1.2.2. Protection of the lower airways The nose protects the lower airways by fixing and removing particles up to 30 m from the inspired air. The mucus film covering the nasal mucosa is responsible for trapping the particles. The transport of the particles to the nasopharynx and oropharynx depends on the mucociliary clearance, which is determined by the motion of the mucus blanket. This motion is supplied by the coordinated waves of cilia8. In addition to this mechanical filtration, the nose actively participates in the immunological defense of the airways. 1.2.3. Olfaction Olfaction is important in regulation of food intake, in the perception of flavors, it has also a protective function by helping to identify toxic and irritating agents from the inspired air. Humans can detect more than 10,000 different odors and discriminate between 5,000 of them8.

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1.3. Symptoms of nasal diseases

1.3.1. Nasal obstruction Nasal obstruction (stuffy nose, nasal congestion, nasal blockage) is the unpleasant sensation of having difficulty breathing through the nose. It can be unilateral or bilateral, partial or complete, transient or permanent. Nasal obstruction can be the result of two main causes9: Mechanical or structural abnormalities: foreign bodies, deviated nasal septum, nasal polyposis, benign tumors, malignant tumors, turbinate hypertrophy, malformations, choanal atresia Functional: allergic rhinosinusitis, acute and chronic rhinosinusitis, rhinitis medicamentosa, rhinitis of pregnancy, atrophic rhinitis, chemical injury of nasal mucosa (inhaled drugs, occupational) 1.3.2. Nasal discharge (Rhinorrhoea) The main semiologic features of nasal discharge are: Thin and watery: onset of common cold, allergic rhinitis Thick mucus: chronic rhinitis Mucopurulent: if bacterial infection is superimposed (persistent unilateral discharge of yellow pus is usually indicative of sinus disease) A foul unilateral mucopurulent discharge in a child: usually a foreign body Mucopurulent, sanguinolent, blood-stained: tumors Crusts: atrophic rhinitis Cerebrospinal fluid rhinorrhoea: the leakage of CSF from the subarachnoid space into the nasal cavity due to a defect in the dura, bone and mucosa Post-nasal discharge (post-nasal drip): disease located at the posterior group of paranasal sinuses 1.3.3. Headache and facial pain Headaches and facial pain can be related to sinus diseases (inflammatory, neoplasms, barotrauma). Many patients are referred to the ENT doctor with chronic headaches, claiming
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they have sinusitis. But there are a lot of other non-sinus causes which must be ruled out: dental pain, vascular (migraine, cluster headaches, temporal arteritis), neuralgias, cervical spine disorders, temporomandibular joint dysfunction. A good collaboration with a neurologist and a maxillo-facial surgeon is mandatory for a proper management of these patients. Cluster headache typically affects men between 30 and 50 years old. The aetiology is vascular. Pain is located frontal, temporal, extends over the cheek or even into the teeth, and is accompanied by lacrimation, rhinorrhoea and nasal obstruction. These symptoms may lead to misdiagnosis of sinusitis. The pain may last for 15 minutes up to two hours. Clusters of attacks may continue for several weeks10. Trigeminal neuralgia is characterized by severe paroxysms of unilateral lancinating pain in one or more branches of the trigeminal nerve, induced by a specific trigger point (lips, nasolabial folds, gingivae).

1.3.4. Smell disorders Smell disorders may be quantitative (anosmia/hyposmia and hyperosmia) or qualitative (parosmia and phantosmia). Decreased or loss of smell perception can be provoked by rhinologic or neurologic causes. Any nasal obstruction which doesnt allow the odor particles to reach the olfactory area in the upper nose will cause hypo- or anosmia. Neurological causes include viral upper respiratory tract infections, head trauma, neurodegenerative disorders (Alzheimer disease, Parkinson disease, multiple sclerosis), epilepsy and migraine. Hyperosmia should be considered rather a quality feature (cooks, wine tasters, perfumes and fragrances makers) than a pathological condition. Parosmia is a wrong, altered perception of a smell which does exist in the environment (nothing smells normal). It is described mainly by women during pregnancy. Olfactory hallucinations or phantosmias (perception of a smell without an odor present) can occur in psychiatric disorders.

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1.4. Clinical examination

1.4.1. Inspection and palpation Prior to instrumental examination, the nasal pyramid and adjacent facial structures should be carefully inspected and palpated. We should evaluate the overall shape of the nose, the presence of nasal deformities of the dorsum, tip or columellae, scars, abnormal swellings, skin lesions. Crepitations or pain on palpation suggest a fracture of the nasal bones. Palpation of the sinusal trigger points: Frontal sinus: the supraorbital point (at the junction of the lateral 2/3rds of the superior orbital rim with the medial 1/3rd) and supero-internal angle of the orbit (corresponds to the floor of the frontal sinus) Anterior ethmoid cells: the internal angle of the orbit (the Ewings point) Maxillary sinus: the infraorbital point (located 1/2 cm below the middle of the inferior orbital rim) and the canine fossa (located laterally to the bony prominence of the canines root) These points are sensitive in acute rhinosinusitis, acute exacerbations of chronic rhinosinusitis and dental problems (the canine fossa). Palpation of these points should be performed alternatively, not simultaneously, on both sides of the face. 1.4.2. Examination of the nasal vestibule Instruments: Light source Head mirror The examiner is in front of the seated patient, light beam is focused on the tip of the nose, the examiners left hand is placed on the patients forehead, fixing the head. Using the left thumb, the examiner elevates the tip of the nose, allowing the visualization of the nasal vestibule. 1.4.3. Anterior rhinoscopy Instruments: Light source
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Figure 6. Examination of the nasal vestibule

Head mirror Nasal speculum (Hartmann, Killian) Otoscope: recommended for small children Straight and curved suction tips With the patient seated, light beam is focused on the tip of the nose. The left hand of the examiner is placed on the forehead, fixing the patients head, while the other gently opens the nostril with the nasal speculum. Anterior rhinoscopy allows visualization of the septum, inferior turbinate and meatus and

Figure 7. Hartmann nasal speculum

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Figure 8. Anterior rhinoscopy

the floor of the nasal cavity. Tilting the patients head backwards gives a consecutive view of the middle turbinate and meatus. The superior turbinate cant be usually visualized because of the extremely narrow space between the middle turbinate and the septum. Vasoconstrictors may be used in selected patients in order to improve the visibility of the nasal cavities. Nasal secretions should be removed before examination by asking the patient to blow the nose, or by suctioning.

1.4.4. Nasal endoscopy Nasal endoscopy has become the gold standard in nasal examination, for it provides accurate and reliable information on the entire nasal cavity, including traditionally inaccessible areas like the sphenoethmoidal recess or the ostiomeatal complex 11. Instruments: Light source Fiber optic cable Endoscopes: rigid telescopes of 25 cm length, 4 mm diameter and angulations of 0 and 30 are most commonly used. Smaller endoscopes are also available (2.7 mm diameter).
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Figure 9. Nasal endoscopes

More recently, 45 and 70 endoscopes have been developed. Fiber optic flexible endoscopes can also be used Straight and curved suction tips, flexible metal cottontipped applicators, straight and upbiting forceps, anti fog solutions Topical nasal decongestants and anaesthetics Video and digital recording equipment, if available

Figure 10. Nasal endoscopy

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Before starting the examination, explain the procedure to the patient. Apply topical vasoconstrictors and anaesthetics. Remove nasal secretions by suctioning. Nasal endoscopy can be performed with the patient either sitting, or in the supine position. The endoscope should be held with one hand, while the other is placed on the patients forehead in order to fix the head. The endoscope is gently introduced into the nasal vestibule and nasal cavity, allowing an accurate examination of the whole nasal cavity and the nasopharynx12.

1.4.5. Examination of the paranasal sinuses

1.4.5.1. Maxillary sinusoscopy Direct visualization of the maxillary sinus cavity can be obtained by performing an antrostomy or maxillary sinusoscopy, via inferior meatus or canine fossa puncture. It is an invasive procedure. It must be performed in the operating room, under local or general anaesthesia. Rigid endoscopes are used to examine the cavity. 1.4.5.2. Transmeatal puncture Local anaesthesia must be applied at the level of the inferior meatus. The tip of the trocar is placed 1 cm behind the head of the inferior turbinate, in the inferior meatus, being pointed to the external commisure of the orbit. Once the sinus cavity is reached, it can be either aspirated or irrigated. Possible complications of the procedure are penetration of the orbit, and perforation of the posterior wall of the sinus.

1.5. Clinical investigations

1.5.1. Imaging techniques
1.5.1.1. Conventional radiography Plain sinus radiographs: diagnosis of rhinosinusitis (sinus opacification, air-fluid levels, mucosal thickening), tumors of the nose and paranasal sinuses Nasal pyramid radiographs: diagnosis and legal documentation in traumatic lesions of the nasal bones

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Figure 11. Conventional radiography of the paranasal sinuses

1 - Maxillary sinus; 2 - Ethmoid sinus; 3 - Frontal sinus; 4 - Orbit

1.5.1.2. Computed tomography CT The gold standard technique for imagistic evaluation of sino-nasal pathology For screening the sinuses, an axial view is available When endoscopic sinus surgery is anticipated, direct coronal views are mandatory for pre-operative evaluation of the extent of the disease, to detect anatomic variations and for planning the surgical approach13 For evaluation of tumors, both axial and coronal views are indicated, with contrast enhancement 1.5.1.3. Magnetic resonance imaging MRI Is primarily indicated for evaluation of sinus tumors, and occasionally inflammatory diseases, such as mucoceles The main advantage of MRI, comparing to CT scans, is the ability to distinguish between tumoral tissues and obstructed sinus secretions and to predict the true extent of the tumor 13 Sagittal, axial and coronal views are available, with or without contrast enhancement 109

Figure 12. MRI of the nose and paranasal sinuses

1 - Maxillary sinus; 2 - Left ethmoid sinus; 3 - Right ethmoiditis; 4 - Orbit; 5 - Right inferior turbinate; 6 - Left middle turbinate

1.5.2. Rhinomanometry Rhinomanometry allows the simultaneous assessment of the flow and pressure variations to which an air current is subjected, as it passes through the nasal airways. The obtained data are used to calculate the nasal resistance. Two methods are available: active anterior rhinomanometry (most frequently used) and active posterior rhinomanometry. Both are based on the same principle, in which the airflow and nostril pressure are measured inside a mask covering the nose and the mouth 14. Acoustic rhinometry is a recently developed modern technique, based on acoustic scanning, which provides an objective geometric study of the nasal cavity 15. 1.5.3. Allergy testing For the diagnosis of allergic aetiology in rhinosinusal diseases, a number of tests are available: Skin tests: scratch, prick or intradermal injections
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demonstrate immediate hypersensitivity mediated by IgE antibodies RAST (Radio Allergo Sorbent Test) detects specific circulating IgE antibodies

1.5.4. Nasal cytology Nasal cytology can be assessed by taking a smear or a nasal brushing. A swab is wiped over the outer surface of the middle turbinate. Afterwards it is smeared onto glass slides and fixed cytologically. The different epithelial cells and the percentage of each leukocyte type is measured by May-Grunwald-Giemsa staining. The normal nasal mucosa does not contain polynuclear neutrophils or eosinophils 14. 1.5.5. Exploration of mucocilliary function Exploration of mucocilliary function is not a routine clinical examination. The most frequently used test is the saccharine transit time test 16. It is performed by placing some saccharine powder at the level of the head of the inferior turbinate. The time needed by the patient to feel the saccharine taste is normally less than 20 minutes 14. Modern techniques used to evaluate the ciliary beat rate and electron microscope analysis of ciliary structure have been developed for scientific purposes. 1.5.6. Olfactory tests The most widely used olfactory test is the 40-item University of Pennsylvania Smell Identification Test (UPSIT). This test can be self-administered in 10-15 minutes by most patients in the waiting room, and scored in less than a minute 17. Other modern techniques, available only in specialized centers, include the recording of olfactory event-related potentials (OERP) and evaluation of olfactory mucosa biopsies.

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2. DISEASES OF THE EXTERNAL NOSE AND

NASAL VESTIBULE

2.1. Acute nasal infections

2.1.1. Nasal vestibulitis
2.1.1.1. Definition. Aetiology Nasal vestibulitis is characterized by a diffuse inflammation of the skin of the vestibule. The infective process is secondary to local trauma (mainly of the finger variety) or excoriations from rhinitis. The bacterial agents involved are commensal bacteria residing in the upper respiratory tract: Staphylococcus aureus (30%) and Streptococcus pyogenes (10%) 18. 2.1.1.2. Symptoms and signs Mild to moderate local discomfort is frequently reported Local erythema, edema and tenderness Crusting 2.1.1.3. Treatment Topical agents (antibiotics plus corticoids) Oral antibiotics are rarely necessary

2.1.2. Nasal furuncle

2.1.2.1. Definition. Aetiology A furuncle (common boil) is a localized infection of the nasal vestibules skin, centered on a hair follicle, caused by Staphylococcus aureus. 2.1.2.2. Symptoms and signs Local severe pain, accompanied sometimes by fever, malaise

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 It usually starts like a tender, firm nodule with surrounding inflammation which subsequently develops an abscess, with a characteristic white point (bourbillion) in the middle of the tumefaction
2.1.2.3. Complications Persistent or recurrent disease may suggest an underlying diabetes mellitus or an immunocompromised state Retrograde dissemination of the infection through the valveless facial veins can determine an extremely serious complication: cavernous sinus thrombosis (periorbital swelling, chemosis, ophtalmoplegia, pupillary changes) 2.1.2.4. Treatment If no systemic symptoms or extensive cellulitis are present, simple topical care is usually curative: topical antibiotics and steroids, topical disinfectants In severe cases: oral antibiotics (Oxacillin, Doxycycline, Clindamycin, Vancomycin) Analgetics, non-steroidal antiinflammatory drugs (NSAIDs) Larger, collected furuncles should be drained by surgical incision

2.1.3. Erysipelas
2.1.3.1. Definition. Aetiology Erysipelas is a skin infection caused by Streptococcus pyogenes group A 2.1.3.2. Symptoms and signs Sudden onset with fever, chills, malaise Red, tender, indurated skin plaque with well-defined elevated borders 2.1.3.3. Penicillin in high doses Analgetics and non-steroidal antiinflammatory drugs (NSAIDs)

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2.2. Trauma
2.2.1. Nasal fractures
2.2.1.1. Definition. Aetiology A nasal fracture is a disruption of the nasal bone structure, due to trauma. It is the most frequently fractured facial bone, because of its location, prominence and delicate structure. Sports, falls and aggressions are the major involved aetiological factors. Men are affected twice as often as are females. Children usually develop cartilaginous lesions and greenstick fractures 19. 2.2.1.2. Symptoms History is extremely important and must be documented for legal purposes Pain, nasal obstruction, epistaxis 2.2.1.3. Diagnosis Inspection may reveal local edema, deviations and asymmetry of the nasal pyramid, epistaxis. Periorbital ecchymosis suggest associated lesions of the lamina papyracea (medial wall of the orbit). The post traumatic appearance of the nose should be compared with older photos (ID card). Palpation of the nose reveals crepitations, abnormal mobility of the nasal pyramid structures and local pain. A careful palpation of the adjacent facial bony structures must be performed. Anterior rhinoscopy allows removal of blood clots by suction, assessment of the associated septal lesions, identification of the bleeding site. Nasal pyramid radiographs (lateral views) help the diagnosis and provide legal documentation. Three-dimensional CT is recommended in case of extensive injuries involving adjacent structures. 2.2.1.4. Treatment Treatment of bleeding (see Epistaxis) Nondisplaced fractures should be treated with observation alone

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 Closed nasal reduction is the method of choice in the majority of the cases An open approach is rarely indicated (severe trauma, the need for wide surgical exposure)

2.3. Dermatological conditions

2.3.1. Rhinophyma (Rosacea)
2.3.1.1. Definition. Aetiology Rosacea is a dermatological condition involving the central face, characterized by the presence of papules and pustules on a background of erythema, edema and telangectasia 18. The final stage of rosacea, affecting almost exclusively mens nose, is called rhinophyma. The exact cause is unknown. Genetic factors, infection and alcohol consumption have been involved in aetio-pathology. There is hyperplasia and hypertrophy of the sebaceous glands and chronic deep inflammation.

Figure 13. Rhinophyma

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2.3.1.2. Diagnosis Patients address to the doctor mainly for cosmetic reasons Sometimes, due to gross enlargement, nasal obstruction is reported Pseudo-tumoral disfiguring appearances of the tip of the nose and alar region may develop during later stages 2.3.1.3. Treatment 2.3.1.3.1. Conservative treatment: Long-term antibiotics for treatment of secondary infections (topical Metronidazole, oral Tetracycline) Synthetic retinoids (Isotretinoin) Skin care 2.3.1.3.2. Surgical treatment: Full-thickness excision followed by application of free skin grafts Partial-thickness excision or decortication: razor blades, surgical scalpel, electrocoagulation, dermabrasion, carbon dioxide, argon and Erbium-Yag lasers 20

2.4. Tumors
2.4.1. Benign tumors
2.4.1.1. Keratoacanthoma 2.4.1.1.1. Definition. Aetiology Keratoacanthoma, or molluscum sebaceum, is a benign cutaneous tumor that follows a specific growth pattern, terminating in complete resolution18. Aetiological factors: ultraviolet radiation exposure, occupational exposure to tars, mineral oils, viral infection. 2.4.1.1.2. Diagnosis Firm, round lesion which grows rapidly (up to 6 weeks), becomes globular and develops a central horn-filled or keratotic plug

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 Complete resolution occurs in a variable interval of 3 months - one year Differential diagnosis: squamous cell carcinoma Histopathology: proliferating keratinizing cells centered on hair follicles
2.4.1.1.3. Treatment: Simple curettage Excision biopsy

2.4.2. Malignant tumors

2.4.2.1. Squamous cell carcinoma (SCC) 2.4.2.1.1. Definition SCC is a malignant cutaneous tumor developed from epidermal keratinocytes. The most frequently involved aetiological factors are exposure to hydrocarbons (tar and mineral oils), thermal factors and solar radiation. Metastases are found in 10 per cent of the patients18.

Figure 14. SCC of the nose

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2.4.2.1.2. Diagnosis Various clinical aspects: erythematous plaque, nodular, verrucous Firm lesion with induration of the surrounding tissue and fixation to underlying structures in later stages In evolution, crusting, ulceration and bleeding occurs Histhopatology is mandatory for diagnosis 2.4.2.1.3. Treatment Surgical excision in oncological safety limits, followed by reconstruction of the defect with various flaps In case of neck metastases, a neck dissection must be performed Radiotherapy and cryotherapy are satisfactory treatment alternatives 2.4.2.2. Basal cell carcinoma (BCC) 2.4.2.2.1. Definition. Aetiology BCC is the most frequently encountered skin cancer in white races, with the highest incidence reported in Australia (726 cases per 100,000)21. It is a neoplastic proliferation of cells that resembles the epidermis basal cells, although the precise origin is still unclear. The tumor is locally invasive. Metastases are uncommon, being reported in less than 0.1 per cent of the cases22. It is more common in men at older ages. The single most important aetiological factor is ultraviolet (UV) solar exposure. Other predisposing factors: light skin color, poor tanning ability, blonde hair, immunosuppressive therapy, arsenic exposure. 2.4.2.2.2. Diagnosis Various clinical aspects: nodular (erythematous papules or plaques, pedunculated lesions), superficial BCC (scaly plaque with poorly defined margins, making excision difficult), morpheaform BCC (skin plaque which develops telangiectasia) The lesion appears initially innocent and is neglected, but cure failures to various topical treatments refers the patient to the doctor

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 Slow-growing evolution, locally invasive Histopathology is mandatory for diagnosis

2.4.2.2.3. Treatment Shave biopsy, curettage, cautery are indicated for small lesions Surgical excision: a 4 mm safety margin is recommended and excision should involve the dermal layer23, followed by reconstruction of the defect Mohs surgical technique: sequential accurate excision of the tumor under frozen section control Intralesional interferon injection, photodynamic therapy, radiotherapy18 2.4.2.3. Malignant melanoma (MM) 2.4.2.3.1. Definition. Aetiology MM is the leading fatal skin malignancy. It develops from melanocytes, in evolution invades the dermis, the depth of the lesion being the most important prognostic factor 24. The most important aetiological factor involved is intense, intermittent sun exposure in fair-skinned individuals. 2.4.2.3.2. Diagnosis Clinical features suggesting a melanocitic lesions malignancy: rapid growth, irregularity, asymmetry, changes in color, pruritus, bleeding and inflammation Several forms exist: superficial spreading type (brown macular lesion which exhibits a long horizontal growth phase and close inspection reveals a variety of hues within the lesion), nodular type (rare on the nose), lentigo melanoma (flat brown-black patch which extends horizontally for months to years) Lymph nodes metastases may be present Histopathology is mandatory for diagnosis 2.4.2.3.3. Treatment Surgical removal within safety oncological margins (1-3 cm), associated with neck dissections Adjuvant therapeutic procedures in advanced stages: chemotherapy protocols, Interferon alpha-2b (Intron), Interleukin-2 (Proleukin)25 119

3. RHINITIS
3.1. Acute rhinitis
3.1.1. Acute viral rhinitis
3.1.1.1. Definition. Aetiology Acute rhinitis is an inflammatory condition of the nasal mucosa, caused by a viral infection. It is known also as common cold or coryza. The disease is highly contagious and is transmitted by means of airborne droplets. It rarely appears as an isolated form, in most of the cases it is part of an upper respiratory tract infection (rhino-pharyngitis, rhino-pharyngo-laryngitis). It may be followed by secondary bacterial infection. Viruses that cause rhinitis include rhinoviruses, adenoviruses, influenza and parainfluenza viruses, coxsackie virus and respiratory syncytial virus 26. Most important predisposing factors are lack of immunity and fatigue. 3.1.1.2. Symptoms The prodromal stage: local irritation, sneezing, dryness in the nose The acute phase: serous rhinorrhoea, nasal obstruction, fever, headaches, malaise, watery eyes The nasal discharge may become thicker and even purulent if secondary bacterial infection occurs

Figure 15. Acute rhinitis

1 - Middle turbinate; 2 - Nasal septum; 3 - Middle meatus

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 After 6-7 days, the nasal passages reopen and the secretions gradually return to normal
3.1.1.3. Diagnosis Anterior rhinoscopy or endoscopic examination: generalized hyperemia of the nasal mucosa, which is covered with serous secretions A complete ENT examination should be performed: rhinitis is frequently associated with acute pharyngitis, laryngitis Serologic assessment of antiviral antibodies titers is not a routine laboratory procedure; it is used mainly for scientific purposes 3.1.1.4. Treatment As the disease is highly contagious, the patient should be isolated from collectivities, if possible Bed rest, hydration Topical nasal decongestants drops/sprays (ephedrine, oxymethazoline, xylomethazoline) Analgetics and antipyretics Antihistamines are frequently prescribed to help decongest the nose and diminish watery serous secretions Antibiotics are not necessary (viral infection), but may be prescribed in order to prevent secondary bacterial infection in predisposed patients

3.1.2. Acute purulent rhinitis

3.1.2.1. Definition. Aetiology Purulent rhinitis commonly complicates the exanthemas, or may appear in the evolution of a common cold by secondary bacterial infection. Membranous forms may occur in pneumococcal, staphylococcal or streptococcal infection, mainly in young children and debilitated patients. 3.1.2.2. Diagnosis Nasal obstruction, purulent nasal discharge, fever, headaches, malaise Complete ENT examination should be performed 121

 A nasal swab culture may be indicated for accurate antibiotic prescriptions

3.1.2.3. Treatment The same as for the common cold, but requires antibiotics or chemotherapy with bacteriological control

3.1.3. Nasal diphtheria

3.1.3.1. Definition. Aetiology Diphtheria is considered nowadays an eradicated disease. It is caused by Corynebacterium diphteriae (Klebs-Loeffler bacillus). The nose can occasionally be affected, mainly in children. 3.1.3.2. Diagnosis Sero-purulent nasal discharge, which may be bloodstained and is often associated with excoriation of the upper lip, nasal obstruction Anterior rhinoscopy or endoscopic examination reveals grayish-white membranes covering the inferior turbinates and adjacent structures, which are extremely adherent and bleed when removed A culture swab should always be taken whenever membranes are seen in the nasal cavities 3.1.3.3. Treatment Patients must be isolated and declared Early administration of antitoxin is mandatory Antibiotics

3.2. Chronic specific rhinitis

3.2.1. Nasal tuberculosis
3.2.1.1. Definition. Aetiology Nasal tuberculosis is a chronic specific granulomatous inflammatory condition,caused by the infection with Mycobacterium tuberculosis. Nasal infection may be the consequence of direct

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inoculation (nose picking and finger nail trauma), dissemination from an open pulmonary tuberculosis or hematogenous spread.
3.2.1.2. Diagnosis Three clinical patterns are described27: Lupus vulgaris (nodular form) usually develops in the nasal vestibule and then extends to the adjacent skin and mucosa. It is the consequence of direct inoculation. The clinical aspect is characterized by the presence of papules or nodules, which in evolution may coalesce and break down to form typical ulcers with a pale granular base. The disease is very slowly progressive, if not treated scarring and deformities occur. Ulcerative form involves the nasal cavity (nasal septum, inferior turbinate) and presents with nasal obstruction, nasal discharge, crusting, epistaxis. Septal perforation may occur, but septal bone is usually spared Sinus granuloma: isolated sinus involvement have been reported without any signs and symptoms in the nose. The CT and MRI characteristics are not specific and reveal a soft tissue polypoid mass with or without bone destruction28 Diagnosis is usually established by a tissue biopsy (typical epitheloid cell granulomas). Microbiologic diagnosis is ideal and is the only confirmatory test for infection with Mycobacterium tuberculosis. Skin testing for delayed hypersensitivity and pulmonary radiographs should also be performed. Differential diagnosis must rule out cancer and other granulomatous diseases affecting the nose. 3.2.1.3. Treatment Chemotherapy protocols

3.2.2. Syphilis
3.2.2.1. Definition. Aetiology Syphilis is an infectious disease caused by Treponema pallidum. The infection may present in various forms that have been classically described as primary, secondary, tertiary and

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congenital syphilis. Nose involvement can occur at any age, from the neonate to the elderly.
3.2.2.2. Diagnosis Primary syphilis rarely involves the nose. The typical lesion is an ulceration (chancre) at the inoculation site, accompanied by regional lymphadenopathy Secondary stage is the most infectious and manifests 6-10 weeks after inoculation. It is a systemic disease. Nasal manifestations include a simple catarrhal rhinitis with no special features, except in its persistence. Crusting and fissuring of the nasal vestibule may occur. Mucous patches are visible at anterior rhinoscopy. Tertiary syphilis is the stage most commonly encountered in the nose. The lesions are chronic and destructive. The typical lesion is a gumma. It begins as a nodule which finally progresses to involve the overlying skin, and then breaks down to form a destructive ulcer. The bony portion of the nasal septum is the predilection site of involvement. In neglected cases, perforation of the affected nasal walls and collapse of the bony support, accompanied by severe scarring may occur27 Congenital syphilis: any of the lesions of secondary or tertiary may be present. Snuffles is the most frequently encountered lesion in children. It begins as a simple catarrhal rhinitis, which becomes purulent in short time. Examination reveals accompanying fissuring and excoriation of the nasal vestibule and upper lip. Gummatous lesions occur mainly at puberty. Other stigmata may be present: Hutchinsons incisors, interstitial keratitis, corneal opacities and sensorineural hearing loss27 Diagnosis is clinical, serological (Venereal disease reference laboratory VDRL, Treponema pallidum haemagglutination test TPHA, Fluorescent treponemal antibody test FTA-ABS), microbiological and histopathological (biopsy). 3.2.2.3. Treatment Antibiotics: Penicillin Local treatment: cleansing of the crusts, nasal irrigations with saline solutions

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3.2.3. Rhinoscleroma
3.2.3.1. Definition. Aetiology Rhinoscleroma is a progressive granulomatous inflammatory disease, commencing in the nose, with later involvement of the pharynx, larynx and sometimes trachea and bronchi29. Rhinoscleroma is a misnomer, the condition is an infectious disease, not a tumor. The causative agent is the gram-negative bacillus Klebsiella rhinoscleromatis (Frisch bacillus). It occurs sporadically throughout the world and is endemic in Eastern Europe and South America30. 3.2.3.2. Diagnosis Three stages are described in the evolution of rhinoscleroma27: The atrophic stage: nasal obstruction, crust formation and foul-smelling rhinorrhoea The granulomatous, nodular or proliferative stage: bluishred nonulcerative nodules develop The cicatrizing stage: fibrosis, adhesions, stenosis, distortions of normal anatomy Diagnosis is difficult in the first atrophic stage. It is usually done in the proliferative and cicatrizing stages. It requires a biopsy. The histopathological findings typical for scleroma are the presence of granulomatous inflammatory lesions (plasma cells, lymphocytes, eosinophils, foamy Mikulicz cells with cytoplasm containing bacilli and Russell bodies). 3.2.3.3. Treatment Long-term and high-dose antibiotic treatment (minimum 4-6 weeks) until two consecutives cultures from the biopsy material are proven negative 31. The traditional used antibiotics are streptomycin and tetracycline, but recent reports have emphasized the good results obtained with a combination of oral ciprofloxacin, rifampicin and sulphametoxazole-trimethoprim32 In later stages, plastic and reconstructive surgery may be required

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3.2.4. Leprosy
3.2.4.1. Definition. Aetiology Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae (Hansens bacillus), an acid-fast bacillus morphologically similar to Mycobacterium tuberculosis. Five countries (Brazil, India, Myanmar, Indonesia and Nigeria) account for 82 per cent of all leprosy patients worldwide27. 3.2.4.2. Diagnosis The clinical features in the nose depend on the type of leprosy33: In tuberculoid leprosy (strong host resistance, non infective, localized) solitary skin lesions are described causing anaesthetic cutaneous patches with involvement of sensory or motor nerves (V, VII cranial nerves). Nasal mucosa is not involved In lepromatous leprosy (poor host resistance, infective and systemic), diffuse infiltration of skin, nerves and mucosa occurs. Clinical features include nasal obstruction, crusts formation, blood-stained nasal discharge containing infecting bacilli. Late disease is characterized by destructive lesions leading to the classical leonine facies Borderline leprosy is of intermediate severity. Skin lesions resemble the tuberculoid form but are more numerous and irregular Diagnosis is made on clinical, bacteriological and histopathological evidences. 3.2.4.3. Treatment Long term (6-12 months) triple medication regimens: Rifampicine, Dapsone and Clofazimine Direct nasal administration of rifampicin has been reported to be more effective than the oral route34 Local treatment: crust removal, nasal irrigations Surgery for cosmesis improvement

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3.2.5. Rhinosporidiosis
3.2.5.1. Definition. Aetiology Rhinosporidiosis is a chronic granulomatous fungal infection. It has a worldwide distribution, mainly in tropical countries, but is endemic only in India and Sri Lanka 30. The disease is caused by Rhinosporidium seeberi, a fungus-like organism, not yet successfully grown in culture medium or transferred from a human to an animal host35. 3.2.5.2. Diagnosis Symptoms: nasal obstruction, epistaxis, blood-stained mucopurulent rhinorrhoea Nasal examination: fleshy nasal polyps, often with grey specks upon them , which gives a ripe strawberry appearance. The polyps are frequently pedunculated and arise mainly from the septum, nasal floor and inferior turbinate Histological examination is mandatory 3.2.5.3. Treatment Complete surgical excision No medical management have been found to be effective

3.3. Atrophic rhinitis

3.3.1. Definition. Aetiology Atrophic rhinitis is a chronic nonspecific disease of the nasal mucosa and subjacent bones, leading to abnormally wide nasal cavities, dryness, crusting, atrophy and a paradoxical subjective sensation of nasal blockage 19. Changes may be slight (rhinitis sicca) or severe, with massive crusting and an extremely unpleasant fetid odor (ozena). The aetiology is still unclear. Originally it was attributed to colonization by Klebsiella ozenae, and now this form is considered primarily. It is seen mostly in young females, in developing countries with warm climates. Genetic, occupational, endocrine, vascular, autoimmune and nutritional factors have also been involved.
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Nowadays, secondary atrophic rhinitis may follow aggressive surgery for nasal obstruction (nasal turbinectomies), trauma, radiation therapy.

3.3.2. Diagnosis The earliest complaint is a feeling of nasal dryness, accompanied by headaches Figure 16. Atrophic rhinitis Dryness or rawness in 1 - Inferior turbinate; 2 - Nasal the rhinopharynx is not septum; 3 - Crust unusual Paradoxical nasal blockage is often described, when in fact there is no obstruction present and the airways are wide. This feeling is probably caused by the lack of normal airflow sensation in the presence of a dry, insensitive mucosa. However, the characteristic accumulation of crusts in the nasal fossae may finally determine a true obstruction Nasal examination: atrophy, crusting, septal perforations 3.3.3. Treatment Local antibiotics (Bioparox spray) and nasal irrigations with crust removal Sea-water sprays or drops (Sterimar, Nazomer) Vitamin A Topical decongestants must be avoided Surgical treatment (Ozena): various techniques were described for narrowing the nasal airways

3.4. Nasal manifestations in systemic diseases

3.4.1. Wegeners granulomatosis (WG)
3.4.1.1. Definition. Aetiology WG is a systemic chronic vasculitis of small- and mediumsized blood vessels, with an autoimmune component 19. In 1939,

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Friedrich Wegener first described necrotizing granulomas and vasculitis of the upper and lower respiratory tract, occurring either together or as separate components.
3.4.1.2. Symptoms The disease may present with upper respiratory tract, pulmonary or renal involvement in a localized or disseminated form. The localized form (25% of the patients) is confined to the upper respiratory tract. Nasal symptoms are non-specific and include nasal obstruction, severe crusting, purulent, blood-stained rhinorrhoea, pain over the dorsum of the nose In the disseminated form, pulmonary involvement is manifested by cough, dyspnoea, haemoptysis, pleuritic pain. Systemic symptoms are also present (malaise, fever, loss of appetite, migratory arthralgias). 90% of the patients have nasal symptoms, which are usually the first manifestations of the disease 3.4.1.3. Diagnosis Nasal examination: painful mucosal ulcers, crusting, septal perforations Complete ENT examination: pharyngeal, laryngeal, otological and orbital involvement may occur Blood tests: elevated ESR, anemia, evaluation of renal function, cytoplasmic-staining antineutrophil cytoplasmic antibodies (c-ANCA) test, confirmed by proteinase 3 (PR3) ANCA test or myeloperoxidase (MPO) ANCA test19 Pulmonary radiographs Nasal mucosa biopsy: mandatory for the diagnosis 3.4.1.4. Treatment The current standard regimen is oral cyclophosphamide and prednisone, associated with Trimethoprimsulphametoxazole36 In localized nasal forms,Trimethoprim-sulphametoxazole is recommended as single therapy37

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3.4.2. Sarcoidosis
3.4.2.1. Definition. Aetiology Sarcoidosis is a systemic disorder of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may affect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver. Skin lesions were first described by Besnier in 1889 as lupus pernio, but the generalized nature of the disease was pointed by Boeck, ten years later38. 3.4.2.2. Symptoms and signs Sarcoidosis is a multisystem disease, primarily affecting the lower respiratory tract, but which may involve the upper respiratory tract, often more frequently than previously realized. Nasal involvement is reported to occur in 3% of the cases 39. Nasal symptoms: nasal obstruction, crusting, bloodstained rhinorrhoea, facial pain Yellowish lesions occur on the septum and lateral nasal wall, associated with crusting Septal perforations and saddle-nose deformity may be present Lupus pernio can occur on the nasal skin (thickening and discoloration of the skin with granular appearance, strawberry skin) 3.4.2.3. Diagnosis Complete ENT examination Chest X-rays, CT scans Biopsy and histopathology The Kveim intradermoreaction, one of the most reliable diagnostic test was unfortunately withdrawn from many countries over fear of virus and prione transfer though without any clear evidence for this 40 3.4.2.4. Treatment Systemic therapy: combination of oral steroids, methotrexate and hydroxychloroquine 38 Local treatment: saline irrigations, crust removal, intranasal steroids

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3.5. Allergic rhinitis

3.5.1. Definition. Classification Allergic rhinitis is an inflammatory condition of the nasal mucosa as a result of an IgE-mediated hypersensitivity reaction. Allergic rhinitis is a global health problem, an increase in its prevalence was noticed during the last 40 years. In European countries, the prevalence of this disease has been rated from 17 to 29%41. Allergic rhinitis has a great impact on the patients social life, affecting school performance and work productivity, the costs incurred by rhinitis being substantial. The Allergic Rhinitis and its Impact on Asthma (ARIA) Initiative has recently developed a state-of-art document including modern guidelines for the diagnosis and treatment of allergic rhinitis42. Allergic rhinitis has been classically described as seasonal if the allergy is to pollen, and perennial if the allergen is present all year round, like the house dust. The modern classification, according to ARIA guidelines, is based on symptoms and quality-of-life parameters. It divides the disease into intermittent and persistent, while severity of symptoms is sub-divided into mild and moderate-severe.
Duration Intermittent symptoms: < 4 days per week Or < 4 weeks Mild: Normal sleep Normal daily activities Normal work and school No troublesome symptoms Persistent symptoms: > 4 days per week and > 4 weeks Moderate-severe: Abnormal sleep Impairment of daily activities Problems at school or work Troublesome symptoms

Severity

Table 1. Classification of allergic rhinitis according to the Aria guidelines

3.5.2. Aetiology. Pathophysiology

3.5.2.1. Aetiology: Inhalants, seasonal or perennial, are the most common and important group of allergens in nasal atopy (pollens, house dust) Various types of food (wheat, dairy products) Drugs (aspirine, iodine, antibiotics)

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3.5.2.2. Risk factors: Genetics and family history: the best established risk factor for allergic rhinitis is a family history of allergy 43 Environment: living in developed countries, pollution and good hygiene 3.5.2.3. Co-morbidities: Allergic rhinitis is a risk factor for the development of subsequent asthma, is a frequent cause of asthma exacerbations and there is evidence that an effective treatment for rhinitis reduces asthma 44 Others: sinusitis, otitis media, sleep disorders, lower respiratory tract infections 3.5.2.4. Pathophysiology: Allergens such as grass pollens, house dust mite, cat dander are harmless molecules which do not elicit symptoms in nonatopic individuals. In atopics, these molecules are inhaled and reach antigen-presenting cells in the nose, the most important of which are the dendritic Langerhans cells. Antigen presentation is a critical first step in activating local T lymphocytes. Once activated, these Th2 cells secrete cytokines (IL-4, IL-13, IL-5) and contribute to the further activation of B lymphocytes. This leads to antibody production of the IgE class. These specific IgEs coat the surface of the mast cells, which are present in the nasal mucosa. A subsequent exposure to the allergen determines a crosslinkage between the antigen and the IgE attached to the mast cells, leading to immediate and delayed release of a number of pre-formed or newly synthesised mediators (mast cells degranulation)45.
Preformed mediators Histamine Serotonin Eosinophil chemotactic factor ECFA Neutrophil chemotactic factor NCF Newly synthesized mediators Leucotriens LTC4, LTB4 Prostaglandins D2 Kinins Platelet activating factor PAF Lipid chemotactic factor HETE

Table 2. Preformed and newly synthesized mediators released after mast cells degranulation

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The immediate mast cells degranulation is responsible for the acute response, but a more prolonged late-phase reaction, which develops within a few hours, is common. This late response is believed to be associated with the eosinophils.

3.5.3. Symptoms Sneezing Profuse watery rhinorrhoea Nasal obstruction Itching Lacrimation Perennial allergic rhinitis often presents as chronic inflammatory rhinosinusitis without acute allergic symptoms. 3.5.4. Diagnosis A careful and detailed history is mandatory Inspection: allergic salute, allergic crease, allergic shiners Anterior rhinoscopy: pale, bluish, edematous nasal turbinates, coated with a thin, clear, watery secretion Nasal endoscopy A full ENT examination Plain sinus radiographs, CT, MRI Rhinomanometry, Figure 17. Allergic rhinitis Olphactometry 1 - Inferior turbinate; 2 - Nasal septum Nasal smears: presence of eosinophils (their detection in nasal secretions is no longer considered pathognomonic of atopic disease) Allergy tests: skin prick test SPT, radioallergosorbent test RAST ( detects specific circulating IgE antibodies), nasal allergen challenge (allergen is introduced into the nose and any reaction is measured and compared to placebo) Evaluation of the pulmonary function (important comorbidity with asthma)
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3.5.5. Treatment Environmental control: allergen avoidance whenever and wherever possible Pharmacotherapy Immunotherapy (specific hyposensitisation): the only one that offers the hope of cure Surgery is rarely needed Medical treatment: Oral antihistamines: the first generation of H1-blockers (e.g. Chlorpheniramine) had a marked sedative effect; the newer, second-generation antihistamines (e.g. Loratadine, Desloratadine, Cetirizine, Ebastine) are non-sedating Topical antihistamines: Azelastine, Levocabastine Topical steroids: most effective treatment for rhinitis (Beclomethasone dipropionate, Fluticasone propionate, Fluticasone furoate, Mometasone furoate, Budesonide, Flunisolide) Systemic corticosteroids Topical anticholinergic agents: Ipratropium bromide Topical chromones: sodium cromoglycate, Nedocromil Oral decongestants (pseudo-ephedrine, phenylpropanolamine) and topical decongestants (oxymethazoline, xylomethazoline) Antileukotriens: Montelukast, Zafirlukast, Pranlukast

3.6. Non-allergic perennial rhinitis

The term non-allergic rhinitis commonly refers to a diagnosis of any nasal condition in which the symptoms are quite similar to those seen in allergic rhinitis, but an allergic aetiology has been excluded. These conditions can broadly be classified as46: Idiopathic rhinitis: also referred to as vasomotor rhinitis or non-allergic non-infectious perennial rhinitis (NANIPER) Drug-induced rhinitis Hormonal rhinitis Non-allergic occupational rhinitis Other forms: non-allergic rhinitis with eosinophilia syndrome (NARES), food-induced rhinitis, rhinitis 134

due to physical and chemical factors, emotion-induced rhinitis

3.6.1. Idiopathic rhinitis Idiopathic rhinitis is characterized by symptoms of nasal obstruction, rhinorrhoea and sneezing. The aetiology is unknown in most cases and the disease is thought to be triggered mainly by irritants and changes in atmospheric conditions47. 3.6.2. Drug-induced rhinitis
3.6.2.1. Topical vasoconstrictors When used topically, the vasoconstriction induced by the nose drops/sprays is so intense that a semi-ischemic state occurs, during which time products of metabolism accumulates that are strong vasodilators. Subsequently, a rebound vasodilation occurs. The more frequent and prolonged the use of topical vasoconstrictors is, the more profound the rebound, until a loss of vascular tone develops, accompanied by hypertrophy of the nasal mucosa and nasal hyperreactivity (Rhinitis medicamentosa). 3.6.2.2. Other drugs Other drugs involved in drug-induced rhinitis: Cocaine abuse, Aspirin, other non-steroidal antiinflammatory drugs (NSAIDs), beta-blockers, angiotensin-converting enzyme (ACE) inhibitors, oral contraceptives, psychotropic agents48.

3.6.3. Hormonal rhinitis This form is associated mainly with pregnancy and premenstrual colds. Estrogens cause vascular engorgement not only in the female genital tract, but also in the nose, leading to nasal obstruction and/or nasal hypersecretion46. 3.6.4. Nonallergic occupational rhinitis Occupational rhinitis may be defined as rhinitis caused by exposure to airborne agents present in the work place. These agents may act via both immunologic (IgE) and non-immunologic mechanisms. The non-immunologic triggers are often irritant or toxic small molecular weight compounds (aldehydes, isocyanates, solvents), or may be physical (long-term exposure to cold air)46. 135

3.6.5. NARES The term NARES (Non-allergic Rhinitis with Eosinophilia Syndrome) refers to a nasal condition characterized by symptoms similar to allergic rhinitis and the presence of more than 20% eosinophils in the nasal smears. A marked feature of this disease is the lack of evidence of allergy (negative skin tests, absence of serum IgE antibodies) 49. 3.6.6. Other forms of rhinitis Rhinitis due to physical and chemical factors: cold, dry air (skiers nose), air conditioning, air pollutants Emotionally induced rhinitis: stress, sexual arousal (postcoital rhinitis)50 End-stage vascular atony of chronic allergic or inflammatory rhinitis: prolonged parasympathetic stimulation of the nasal vascular system may lead finally to permanent loss of vascular tone. Richardson described this condition in 1948 as nasal turbinate priapism48 Food-induced rhinitis: spicy foods cause watery rhinorrhoea, a phenomenon called gustatory rhinitis51 3.6.7. Treatment of nonallergic perennial rhinitis
3.6.7.1. Non-surgical management: Avoid knowing irritants: tobacco smoke Sleep and work in a cool-air (not cold) environment, keeping the body warm Oral decongestants: pseudo-ephedrine, phenylpropanolamine Oral antihistamines Topical steroids: the most effective treatment 3.6.7.2. Surgical management: In cases when nasal obstruction is non-responsive to medical treatment and if the inferior turbinate is hypertrophic, surgery to reduce the size of the turbinate may be an option. Various procedures48 has been shown to be useful: Inferior turbinate injection with corticosteroids: no more

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recommended Electrical or chemical cauterization of the inferior turbinates Partial turbinectomy (lasers can be used) Total turbinectomy: no more recommended, because atrophic rhinitis may develop afterwards Submucous resection of the turbinate bone Modern approaches include radiofrequency turbinate tissue reduction (Coblation-assisted turbinoplasty) and microdebriderassisted turbinoplasty52.

3.7. Nasal polyposis

3.7.1. Definition Nasal polyposis is a part of an inflammatory condition of unknown aetiology, involving the mucous membranes of the nose, the paranasal sinuses and often the lower airways. A nasal polyp presents in the nasal cavity with a grape-like appearance, having a body and a stalk, a smooth surface and a yellowish color. Nasal polyps originate in the upper part of the nose, around the openings of the ethmoidal cells (middle and superior meatus)53. The prevalence rate of nasal polyposis is about 2%. It increases with age. The male/female ratio is 2/154. 3.7.2. Aetiology and associated diseases The aspirin triad (Samters triad, Widals triad): a medical nonallergic condition consisting of asthma, aspirin sensitivity and nasal polyposis . It represents the most aggressive form of the disease. The intolerance is not confined to aspirin, as the patients react to other non-steroidal antiinflammatory drugs (NSAIDs). The aetiology is unknown, but it is widely believed that the disorder is caused by an anomaly in the arachidonic acid cascade, which causes undue production of leukotrienes. When prostaglandin production is blocked by NSAIDs, the cascade shunts entirely to leukotrienes, determining the severe allergy-like effects55 Allergy (?): as tissue eosinophilia is a characteristic feature of most nasal polyps, it has been the belief for decades that allergy is a significant cause of nasal polyposis. However,
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recent studies suggested that IgE-mediated allergy does not play an aetiological role Allergic fungal sinusitis: nasal polyps occur in almost all patients with allergic fungal rhinosinusitis. In this disease, the tissue inflammation is typically eosinophil-dominated56 Cystic fibrosis: nasal endoscopy demonstrated polyps in 45% of adults with cystic fibrosis 57. In children with cystic fibrosis the prevalence of polyposis is even higher. Any child with nasal polyps should be regarded as having cystic fibrosis until proven otherwise Primary ciliary dyskinesia (Kartageners syndrome)

3.7.3. Pathogenesis The site of polyp formation is mainly the middle meatus, and they originate from the mucous membrane of the outlets (ostia, clefts, recesses) from the paranasal sinuses. This region, so critical for sinus pathology, is also referred as the ostiomeatal complex 53. Contact areas may contribute to the formation of the polyps. Polyps are edematous sacks covered by normal airway epithelium and containing very few nerves, blood vessels and glands with cystic degeneration. Polyps contain degranulated mast cells, very high concentrations of histamine and are characteristically infiltrated with eosinophils, which are upregulated by cytokines (most importantly IL-5). As eosinophils generate IL-5, attracting more eosinophils, nasal polyps can be considered as a selfperpetuating inflammatory process53. Various theories involving fungi and superantigenes were suggested to explain the presence of eosinophils. According to the fungi theory, eosinophils are attracted to a stimulus (fungus) in the mucus in patients who are immunologically sensitive to fungus58. 3.7.4. Symptoms Nasal obstruction is the major symptom, constantly present, but of varying degrees depending on the polyps size Watery rhinorrhoea, post-nasal drip Hyposmia/anosmia Headaches and facial pain may be present Patients may have symptoms of acute, recurrent, or chronic rhinosinusitis if the polyps obstruct the sinus ostia
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Figure 18. Nasal polyposis 1 - Nasal polyp; 2 - Middle turbinate; 3 - Nasal septum

Figure 19. Nasal polyposis

3.7.5. Diagnosis Anterior rhinoscopy: fleshy translucent mass or masses in the nasal cavity, usually originating in the middle meatus Nasal endoscopy Imaging: plain X-ray, CT scans, MRI Allergy testing in general is not indicated but it is often expected by the patient Any child with nasal polyposis must be evaluated for cystic fibrosis! Unilateral polyposis requires a careful examination in order to rule out a malignancy! Antro-choanal polyp: it usually originates from the postero-lateral wall of the maxillary sinus and extends through the ostium towards the posterior choana. It is more common in adolescents and young adults. Endoscopic removal is the primary treatment 3.7.6. Treatment Medical treatment: topical and systemic steroids Surgery is not curative and patients must be counselled that polyps may recur and that repeated procedures may be required Endoscopic intranasal procedures (FESS) can range from a simple polypectomy to a complete sphenoethmoidectomy Following surgery, patients usually require a long-term intranasal steroid administration to prevent recurrences
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4. RHINOSINUSITIS
4.1. Acute rhinosinusitis
4.1.1. Definition. Aetiology Acute sinusitis (acute bacterial sinusitis ABS, acute bacterial rhinosinusitis ABRS) is an infectious condition characterized by an acute inflammation of the paranasal sinuses mucosa. The ostia of the paranasal sinuses are the key to the development of acute sinusitis. Any factor which narrows the ostia will predispose the patient to acute sinusitis. Viral upper respiratory tract infections, allergic inflammation, nasal polyposis are the most frequently encountered causes. The main bacterial agents involved are: Streptococcus pneumoniae and Haemophilus influenzae, while Moraxella catarrhalis, Staphylococcus pyogenes, Staphylococcus aureus and various anaerobes are less frequent59. ABRS is frequently encountered in clinical practice, affecting between 10 and 15% of the population of central Europe annually60. 4.1.2. Symptoms History: biphasic evolution of a common cold Nasal obstruction Purulent nasal discharge Facial pain/pressure Fever, malaise 4.1.3. Diagnosis Palpation of the sinusal trigger points (see section 1.4.1.) Anterior rhinoscopy: congestion, edema of the nasal mucosa, mucopurulent discharge coming out from the middle meatus
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 Nasal endoscopy Imaging: plain X-ray, CT scans, MRI, ultrasound have limited value in the routine diagnosis of uncomplicated acute sinusitis The gold standard for diagnosis of bacterial sinusitis is sinus puncture with aspiration of purulent secretions, but this invasive procedure is seldom performed in primary care61

Figure 20. Acute rhinosinusitis

1 - Middle turbinate; 2 - Inferior turbinate; 3 - Nasal septum 4 - Pus in the middle meatus

Figure 21. Left maxillary sinusitis

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4.1.4. Complications Orbital complications: periorbital cellulitis (most commonly seen in children with acute ethmoiditis, the upper eyelid becomes swollen without evidence of orbital infection), subperiostal abscess (accumulation of pus at the medial aspect of the orbit), orbital abscess (severe proptosis and ophtalmoplegia, visual acuity is impaired), cavernous sinus thrombosis (usually bilateral, sepsis, orbital pain, chemosis, proptosis and ophtalmoplegia) Osteomyelitis: frontal sinus osteomyelitis with erosion of the anterior wall of the sinus produces the classic forehead swelling known as Potts puffy tumor62 Intracranial complications: meningitis, epidural abscess, subdural abscess, brain abscess

Figure 22. Left orbital celullitis

4.1.5. Treatment
4.1.5.1. Medical treatment: Antibiotics: first-line antibiotics (Amoxicillin, Amoxicillin-clavulanate), second-line antibiotics (second generation cephalosporins, macrolides, fluoroquinolones with anti-pneumococcal activity, Doxycycline)63

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 Oral and topical nasal decongestants Nasal irrigations with saline, sea-water drops/sprays Analgetics and antipyretics
4.1.5.2. Surgical treatment: Antral puncture is rarely performed nowadays, since the appropriate use of antibiotics cures the disease

4.2. Chronic rhinosinusitis

4.2.1. Definition. Aetio-pathology The European Position Paper on Rhinosinusitis and Nasal Polyps64 proposed the criteria for diagnosis of chronic rhinosinusitis in adults as 12 or more weeks of persistent symptoms and signs with no complete resolution. Rhinosinusitis (including nasal polyps) is defined as inflammation of the nose and paranasal sinuses, characterized by two or more of the following symptoms: nasal blockage/ congestion, nasal discharge (anterior/posterior discoloured), facial pain/pressure, hypo/anosmia plus either endoscopic signs of polyps, mucopurulent discharge from middle meatus, edema/ mucosal obstruction primarily in the middle meatus and/or CT scan changes (mucosal changes within the ostiomeatal complex and/or sinuses. Predisposing factors: allergic rhinitis, nonallergic rhinitis and immune deficiency. Structural abnormalities (concha bulosa, reversed middle turbinate) are no more common in chronic rhinosinusitis than they are in a control group, with the possible exception of septal deviation65. Systemic diseases as Wegeners granulomatosis and sarcoidosis may present with chronic rhinosinusitis. 4.2.2. Diagnosis A careful clinical history remains the cornerstone of diagnosis! Symptoms: see definition of rhinosinusitis above Complete ENT examination Nasal endoscopy 143

 Radiology: plain sinus X-rays, CT scan (mainly coronal sections), MRI

4.2.3. Treatment
4.2.3.1. Medical treatment: Allergen/irritant avoidance Nasal douching: saline, sea-water drops/sprays Nasal decongestants (brief use) Topical corticosteroids Antibiotics: are needed for acute exacerbations, their place in the chronic form is controversial A recent randomized prospective study 66 has revealed that medical treatment in chronic rhinosinusitis is as effective as endoscopic sinus surgery combined with topical steroids, both in polypoid and nonpolypoid chronic rhinosinusitis. 4.2.3.2. Surgical treatment: In the last decade, many ENT doctors accepted endoscopic sinus surgery as the standard procedure for the surgical management of chronic rhinosinusitis The technique, as proposed by Messerklinger and championed by Stammberger in Europe and Kennedy in USA, is minimally invasive endoscopic sinus surgery

Figure 23. FESS basic concept

1 - Frontal sinus; 2 - Orbit; 3 - Maxillary sinus; 4 - Inferior meatus; 5 - Inferior turbinate; 6 - Middle turbinate; 7 - Nasal septum; 8 - Middle meatus; 9 - Ostiomeatal complex; 10 Ethmoidal bulla

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Figure 24. Right maxillary sinus ostium (endoscopic view during FESS)
1 - Right maxillary sinus ostium; 2 Middle turbinate; 3 - Nasal septum

(MISS), or functional endoscopic sinus surgery (FESS) The basic principle of FESS is to remove only the diseased areas (at the level of the ostiomeatal unit), in order to relieve obstruction of the ostia and so restore natural sinus drainage, ventilation and physiology67 Other surgical procedures: antral puncture with lavage, the Caldwell-Luc radical operation

4.3. Fungal rhinosinusitis

4.3.1. Definition. Aetiology The role of the fungi in the nose and paranasal cavities is still unclear. There is poor understanding of when fungi are present as pathogens or simply as part of normal flora 68. A range of acute and chronic manifestations are described. Several fungal organisms may be involved: Aspergillus, Scedosporium, Alternaria and Mucor 69. Classification 70: Noninvasive: sinus fungus ball (mycetoma), allergic fungal rhinosinusitis Invasive: acute fulminant invasive fungal rhinosinusitis, chronic invasive fungal rhinosinusitis 4.3.2. Fungus ball Fungus ball occurs mainly in adults. It is the main fungal rhinosinusitis in immunocompetent patients Aspergillus is the most common agent involved (Aspergillus fumigatus, Aspergillus flavus)
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 Unilateral post-nasal discharge is the most frequent symptom, but some patients are asymptomatic CT, MRI are helpful in diagnosis (MRI is the most reliable modality to identify fungal sinus disease) 71 Histopathology: no mucosal invasion Surgery (FESS) is the most effective treatment. No antifungal therapy is required.

4.3.3. Allergic fungal rhinosinusitis (AFRS) AFRS is a noninvasive form of fungal rhinosinusitis in immunocompetent patients with allergy to fungus Clinical and biological criteria for diagnosis: nasal polyps, thick mucin, hypersensitivity type 1 for fungus, eosinophilic mucin 69 Treatment: surgical removal of eosinophilic mucin, topical and oral steroids 4.3.4. Chronic invasive fungal rhinosinusitis It is probably one of the less frequent forms, occuring in immunocompetent patients. History of chronic rhinosinusitis In evolution: bone destruction, invasion into the orbit or brain Treatment includes a combination of surgery and antifungal therapy 4.3.5. Acute fulminant fungal rhinosinusitis It occurs in immunocompromised patients (AIDS, hematologic diseases, type 1 diabetes, chemotherapy, neutropenia). It is characterized by a gangrenous necrosis of the soft tissues and bony structures of the midface with invasion of the orbit and the skull base Early diagnosis and control of the primary immunological disorder are mandatory for the prognosis Mucor and Aspergillus are most frequently encountered Nasal endoscopy, CT, MRI are essential for diagnosis Treatment: surgery, anti-fungal therapy, re-establishment of immunity
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5. EPISTAXIS
5.1. Definition. Classification
Epistaxis is defined as bleeding from the nasal fossae. Classification72: Adult (>16 years) or childhood (<16 years) epistaxis Primary (no proven causal factor) or secondary (proven causal factor) epistaxis Anterior (more common in the young adult) and posterior epistaxis (more often seen in the older adult with hypertension and arteriosclerosis) About 90% of recurrent epistaxis occur from the anteroinferior septal region called Littles or Kiessellbachs area, where there is a rich vascular anastomotic supply formed by end arteries (see section 1.1.5.).

5.2. Aetiology
About 70-80% of all cases are idiopathic, without any proven precipitant or causal factor73.

5.2.1. Local causes: Trauma: direct trauma to the nose determining fracture of the nasal bones, habitual nose picking, nasal surgery, barotraumas Inflammation Tumors: benign (juvenile nasopharyngeal angiofibroma JNA), malignant Septal deformities: septal spurs and sharp deviations, septal perforations Foreign bodies Granulomatous diseases: tuberculosis, syphilis, Wegeners granulomatosis, sarcoidosis
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5.2.2. Systemic causes: Blood dyscrasias: leukaemias, haemophilias, idiopathic thrombocytopenic purpura Drugs: aspirin, non-steroidal antiinflammatory drugs (NSAIDs) Hereditary hemorrhagic teleangiectasia (the OslerWeber-Rendu disease) Arteriosclerosis: elderly patients with associated hypertension 5.2.3. Idiopathic

5.3. Management
The patient should be assessed in a semi-recumbent position. Everyone involved should wear protective glasses and clothing as blood aerosol contamination is common, especially when inserting nasal packing74. Four major objectives: Resuscitation, assessment of the blood loss, evaluation of associated cardio-vascular co-morbidities, previous medications Determination of the cause: if possible Determination of the site: if possible Control of bleeding Control of bleeding may be divided into direct (bleeding point-specific therapies under endoscopic control in general anesthesia) or indirect treatments, which do not require the precise identification of the bleeding point72. Treatment modalities: In minimal hemorrhages, pinching the nose with the head leaned forward to avoid blood swallowing is effective Cauterization of an identified small bleeding area, mainly in anterior epistaxis Anterior packing (vaseline gauze or expandable packing with Merocel) and posterior packing. Nasal packing has to be removed after 24-48h. Insertion of nasal balloon catheters 148

Figure 25. Anterior nasal packing

Figure 26. Baloon catheter

In recurrent life-threatening epistaxis, surgical therapy may be required: Ligation techniques: anterior/posterior ethmoidal artery ligation, endonasal sphenopalatine artery ligation, internal maxillary artery ligation, external carotid artery ligation Embolization under angiographic guidance75

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6. TUMORS OF THE NOSE AND PARANASAL

SINUSES

6.1. Benign tumors

Squamous papillomas: in the nasal vestibule Osteomas: arise in the frontal, ethmoid and maxillary sinuses Angiofibromas: see Pharynx, section 3.1.1.1. Inverted papillomas: commonly arise at the level of the lateral nasal wall, present as unilateral polyps, are locally aggressive causing bony erosion of the lateral wall, have a high propensity for recurrence after removal and are associated with malignancy in 15% of cases30

6.2. Malignant tumors

6.2.1. Epidemiology. Aetiology Sinonasal malignancies are uncommon. They account for 0.2-0.8 percent of all malignancies and 3 percent of upper aerodigestive tract neoplasms76. Most develop in the 5th and 6th decades of life. The male/female ratio is 2/177. A large number of sinonasal malignancies are caused by inhaled carcinogens78. Risk factors include: Wood dust Nickel Chrome Polycyclic hidrocarbons Aflatoxin 6.2.2. Pathology Sinonasal malignancies tend to spread by local invasion: orbit, anterior skull base, pterygopalatine fossa, infratemporal fossa, nasopharynx, palate, cheek. Ohngren described a line 150

running from the eyes medial canthus to the mandibles angle. This line divided tumors into two groups depending on the localization above or below the line. Superiorly based cancers have the tendency to be more aggressive and poorly differentiated. Regional spread: only 10 percent of the patients have nodal involvement at the time of presentation78. Depending on the site of origin, maxillary sinus tumors are the most frequent (55 percent), followed by the nasal cavity (35 percent), ethmoid sinuses (9 percent) and rarely frontal and sphenoid sinuses (less than 1 percent)78. Histopathology: Squamous cell carcinoma is the most common malignancy (80%) Adenocarcinoma, adenoid cystic carcinoma, olfactory neuroblastoma (aesthesioneuroblastoma),undifferentiated carcinoma, mucosal melanoma, hemangiopericytoma

6.2.3. Symptoms Unilateral nasal obstruction Muco-purulent/sanguinolent rhinorrhoea Epistaxis Facial pain, facial numbness (infiltration of the infraorbital nerve) Trismus (invasion of the pterygopalatine and infratemporal fossae) Diplopia (invasion of the orbit) Oral symptoms: loose of teeth, inability to wear the denture 6.2.4. Diagnosis Inspection: facial asymmetry, proptosis, epiphora, cranial neuropathies Palpation: neck lymph node metastasis is less frequent Anterior rhinoscopy and nasal endoscopy reveals a nasal mass. Nasal endoscopy allows an accurate biopsy. Buco-pharyngoscopy: swelling/ulcerations of the hard and soft palate Histopathology is mandatory for diagnosis! Imaging: CT, MRI to assess the diseases extension
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Figure 27. Malignant tumor of the left paranasal sinuses

Figure 28. Tumor in the left maxillary sinus

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6.2.5. Treatment Surgery combined with radiotherapy/chemotherapy Orbit exenteration may be required if the tumor invades the orbit Craniofacial resection: for tumors of the ethmoid sinuses with invasion of the anterior skull base The prognosis of these sinonasal malignancies has improved over the past three decades, but remains poor overall and is directly related to the degree of local control. Absolute local control rates for all malignancies are 50 percent at 5 years and 31 percent at 10 years78.

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15. Roithmann R, Cole P, Chapnik J, Shpirer I, Hoffstein V, Zamel N. Acoustic rhinometry in the evaluation of nasal obstruction. The Laryngoscope 1995; 105: 275-281. 16. Stanley P, MacWilliam L, Greenstone M, Mackay I, Cole P. Efficacy of a saccharin test for screening to detect abnormal mucociliary clearance. British Journal of Diseases of the Chest 1984; 78: 62-65. 17. Doty RL, Bromley SM. Abnormalities of smell. In: Lund VJ, ed. ScottBrowns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1660-1676. 18. OConnell M. Conditions of the external nose. In: Lund VJ, ed. ScottBrowns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1699-1717. 19. Martinez-Vidal B, Rusiecka M, Bernal-Sprekelsen M. Diseases of the outer and inner nose. In: Bernal-Sprekelsen M, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 195-203. 20. Har-El G, Shapshay SM, Bohigian RK, Krespi YP, Lucente FE. The treatment of rhinophyma. Cold vs laser techniques. Archives of Otolaryngology - Head & Neck Surgery 1993; 119: 628-631. 21. Marks R, Staples M, Giles GG. Trends in non-melanocytic skin cancer treated in Australia: the second national survey. International Journal of Cancer 1993; 53: 585-590. 22. Cotran RS. Metastasising basal cell carcinomas. Cancer 1961; 14: 10361040. 23. Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinomas. Archives of Dermatology 1987; 15: 340-344. 24. Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous malignant melanoma. Annals of Surgery 1970; 172: 902-908. 25. Winston WT. Malignant melanoma: Treatment & medication. 2010; http://emedicine.medscape.com/article/280245-treatment. 26. Gentile DA, Skoner DP. Viral rhinitis. Current Allergy and Asthma Reports 2001; 1: 227-234. 27. Bahadur S, Thakar A. Specific chronic infections. In: Lund VJ, ed. ScottBrowns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1458-1468. 28. Blanco Aparicio M, Verea-Hernando H, Pombo F. Tuberculosis of the nasal fossa manifested by a polipoid mass. Journal of Otolaryngology 1995; 24: 317-318. 29. Soni NK. Scleroma of the larynx. Journal of Laryngology and Otology 1997; 111: 438-440. 30. Colman BH. Diseases of the nasal cavity (III). ed. Hall & Colmans Diseases of the Nose, Throat and Ear, and Head and Neck. Churchill Livingstone 1992; 35-42. 31. Ssali CLK. The management of rhinoscleroma. Journal of Laryngology and Otology 1975; 89: 91-99. 32. Badia L, Lund VJ. A case of rhinoscleroma treated with ciprofloxacin. Journal of Laryngology and Otology 2001; 115: 220-222. 33. Barton RPE, Davey TF. Early leprosy of nose and throat. The Journal of Laryngology and Otology 1976; 90: 953-961.

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34. Mamidi NV, Prabhakar MC, Krishna DR. Disposition of rifampicin following intranasal and oral administration. Indian Journal of Leprosy 1996; 68: 149-153. 35. Lasser A, Smith HW. Rhinosporidiosis. Archives of Otolaryngology 1976; 102: 308-309. 36. McDonald TJ. Nasal manifestations of systemic diseases. In: Schuller DE, Regan Thomas J, eds. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 934-941. 37. Valeriano-Marcet J, Spiera H. Treatment of Wegeners granulomatosis with sulphamethoxazole-trimethoprim. Archives of Internal Medicine 1991; 151: 1649-1652. 38. Howard DJ, Lund VJ. Granulomatous conditions of the nose. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1645-1659. 39. Zeitlin JF,Tami TA, Baughman R, Winget D. Nasal and Sinus Manifestations of Sarcoidosis. American Journal of Rhinology 2000; 14: 157-161. 40. Du Bois RM, Geddes DM, Mitchell DN. Moratorium on Kveim test. The Lancet 1993; 342: 173. 41. Bauchau V, Durham SR. Prevalence and rate of diagnosis of allergic rhinitis in Europe. Eur Respir J 2004; 24: 758-764. 42. Bousquet J, van Cauwenberge P, Khaltaev N, Group ARIAW. Allergic rhinitis and its impact on asthma. ARIA workshop report. The Journal of Allergy and Clinical Immunology 2001; 108: S147-S334. 43. Bahna SL. Factors Determining Development of Allergy in Infants. Allergy Proceedings 1992; 13: 21-25. 44. Ragab SM, Scadding GK, Lund VJ, Saleh H. Treatment of chronic rhinosinusitis and its effects on asthma. Eur Respir J 2006; 28: 68-74. 45. Scadding GK, Durham SR. Allergic rhinitis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 13861407. 46. Bachert C. Nonallergic perennial rhinitis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1408-1414. 47. Sanico A, Togias A. Noninfectious, nonallergic rhinitis (NINAR): considerations on possible mechanisms. American Journal of Rhinology 1998; 12: 65-72. 48. Fairbanks DNF, Raphael GD. Nonallergic rhinitis and infection. In: Krause CJ, ed. Cummings Otolaryngology-Head & Neck Surgery. Mosby Year Book 1993; 775-786. 49. Settipane RA, Lieberman P. Update on nonallergic rhinitis. Annals of Allergy, Asthma and Immunology 2001; 86: 494-507. 50. Shah A, Sircar M. Postcoital asthma and rhinitis. Chest 1991; 100: 10391041. 51. Raphael G, Raphael MH, Kaliner M. Gustatory rhinitis: a syndrome of food-induced rhinorrhea. Journal of Allergy and Clinical Immunology 1989; 83: 110-115. 52. Lee JY, Lee JD. Comparative study on the long-term effectiveness between coblation- and microdebrider-assisted partial turbinoplasty. The Laryngoscope 2006; 116: 729-734.

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53. Mygind N, Lund VJ. Nasal polyposis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 15491559. 54. Settipane GA. Nasal polyps: pathology, immunology and treatment. American Journal of Rhinology 1987; 1: 119-126. 55. McMains KC, Kountakis SE. Medical and surgical considerations in patients with Samters triad. American Journal of Rhinology 2006; 20: 573576. 56. Cody DT, Neel HB, Ferreiro JA, Roberts GT. Allergic fungal sinusitis: The Mayo experience. The Laryngoscope 1994; 104: 1074-1079. 57. Hadfield PJ, Rowe-Jones JM, Mackay IS. The prevalence of nasal polyps in adults with cystic fibrosis. Clinical Otolaryngology and Allied Sciences 2000; 25: 19-22. 58. Onerci M. Nasal polyposis. In: Bernal-Sprekelsen M, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 241-248. 59. Pentilla M, Savolainen S, Kiukaanniemi H, Forsblom B, Jousimies-Somer H. Bacterial findings in acute maxillary sinusitis - European study. Acta Oto-Laryngologica 1997; 529: 165-168. 60. Grevers G, Klemens A. Rhinosinusitis. Current diagnostic and therapeutic aspects. MMW Fortschritte der Medizin 2002; 144: 31-35. 61. Benninger MS, Payne SC, Ferguson BJ, Hadley JA, Ahmad N. Endoscopically directed middle meatal cultures versus maxillary sinus taps in acute bacterial maxillary rhinosinusitis: a meta-analysis. Otolaryngology - Head and Neck Surgery: Official Journal of American Academy of Otolaryngology - Head and Neck Surgery 2006; 134: 3-9. 62. Llorente Pendas JL, Nieto CS. Complications of sinusitis. In: BernalSprekelsen M, ed. Otorhinolaryngology, Head and Neck Surgery. SpringerVerlag 2010; 253-257. 63. Klossek JM, Dufour X, Serrano E. Acute sinusitis. eds. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 229-235. 64. Immunology EAoAaC. European position paper on rhinosinusitis and nasal polyps. Rhinology Supplement 2005; 18: 1-87. 65. Lloyd GAS, Lund VJ, Scadding GK. CT of the paranasal sinuses and functional endoscopic surgery - A critical analysis of 100 symptomatic patients. Journal of Laryngology and Otology 1991; 105: 181-185. 66. Ragab SM, Lund VJ, Scadding G. Evaluation of the medical and surgical treatment of chronic rhinosinusitis: a prospective, randomized, controlled trial. The Laryngoscope 2004; 114: 923-930. 67. Lund VJ, Rowe-Jones JM. Surgical management of rhinosinusitis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1478-1499. 68. Klossek JM, Kauffmann-Lacroix XD. Sinusites fongiques: classification, methodes diagnostique et prise on charge. Journal de Micologie Medicale 2001; 11: 216-221. 69. Klossek JM. Fungal rhinosinusitis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1449-1457. 70. deShazo RD, Chapin K, Swain RE. Fungal sinusitis. New England Journal of Medicine 1997; 337: 254-259.

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71. Lund VJ, Lloyd G, Savy L, Howard D. Fungal rhinosinusitis. Journal of Laryngology and Otology 2000; 114: 76-80. 72. McGarry GW. Epistaxis. In: Lund VJ, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1596-1608. 73. Stell PM. Epistaxis. Clinical Otolaryngology 1977; 2: 263-273. 74. Carney AS, Weir J, Baldwin DL. Contamination with blood during management of epistaxis. British Medical Journal 1995; 311: 1064. 75. Scaramuzzi N, Walsh RM, Brennan P, Walsh M. Treatment of intractable epistaxis using arterial embolization. Clinical Otolaryngology 2001; 26: 307309. 76. Rice DH, Stanley Jr RB. Surgical therapy of tumors of the nasal cavity, ethmoid sinus and maxillary sinus. In: Panje W, ed. Comprehensive management of head and neck tumors. 1999; 558-581. 77. Goldenberg D, Golz A, Fradis M, Martu D, Netzer A, Joachims HZ. Malignant tumors of the nose and paranasal sinuses: A retrospective review of 291 cases. Ear, Nose and Throat Journal 2001; 80: 272-277. 78. McMonagle BA, Gleeson M. Nasal cavity and paranasal sinus malignancy. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2417-2436.

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III. PHARYNX

1. PHARYNX BASICS
1.1. Clinical anatomy
The pharynx is a barrel-shaped anatomical structure, lying at the crossroads between the respiratory and digestive tracts. The pharynx is divided into three areas: the rhinopharynx (nasopharynx, cavum), the oropharynx and the hypopharynx (laryngopharynx). The boundaries of the three areas are: the cavum is limited superiorly by the skull base (basisphenoid), and inferiorly by a line at the level of the free border of the soft palate; the oropharynx commences at the level of the soft palate and extends to the level of the tip of the epiglottis; the hypopharynx extends from the level of the epiglottis tip to the level of the inferior margin of the cricoid cartilage, where it is continuous with the oesophagus1.

1.1.1. Rhinopharynx The superior wall corresponds to the skull base (basisphenoid) The posterior wall lies onto the prevertebral space. At the junction of the roof and the posterior wall, a mass of lymphoid tissue is noticed (Luschkas pharyngeal tonsil) The anterior wall presents two orifices (the posterior nasal choanae): the communication between the nasal cavities and the nasopharynx The inferior wall is formed by the superior surface of the soft palate and opens into the oropharynx. This 159

communication is closed by the elevation of the soft palate when swallowing At the level of the lateral walls, the openings of the Eustachian tubes (triangular-shaped with a prominent posterior ridge called torus tubarius) are located. Behind and above the torus is the pharyngeal recess (fossa of Rosenmuller), in close proximity to the lower cranial nerves This fossa is an important anatomical landmark because its the site where nasoparyngeal carcinoma arises most commonly

1.1.2. Oropharynx The oropharynx is separated from the rhinopharynx by the mobile, membranous-muscular soft palate. In the midline, the uvula hangs from the soft palate and is occasionally bifid The anterior wall presents the communication between the oropharynx and the oral cavity (the bucco-pharyngeal isthmus or fauces) At the level of the lateral walls, two folds of muscles covered by mucosa stretch down on each side from the palate to the lateral border of the tongue and to the lateral wall of the pharynx. These are the anterior (palatoglossus muscle) and posterior (palatopharyngeus muscle) tonsillar pillars, and between them lie the palatine tonsils The posterior wall consists of the constrictor muscles and overlying mucosa. It corresponds to the prevertebral space At the level of the tongue base lie the lingual tonsil Between the tongue base and the lingual face of the epiglottis are two spaces known as the valleculae. The valleculae are divided in the midline by the glosso-epiglottic fold and laterally by the pharyngo-epiglottic folds The Waldeyers ring consists of a circle of lymphoid tissue surrounding the opening of the aero-digestive tract (the pharyngeal, palatine and lingual tonsils). Aggregations of lymphoid tissue are also located on the posterior wall, which can bee seen mainly in chronic inflammation (the cobble-stoned pharynx)
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Figure 1. Nasopharynx (posterior view)

1 - Inferior turbinate; 2 - Middle turbinate; 3 - Superior turbinate; 4 Nasal septum; 5 - Torus tubarius; 6 - Rosenmuller fossa; 7 - Uvula; 8 Eustachian tube opening ; 9 - Soft palate

Figure 2. Oropharynx (anterior view)

1 - Hard palate; 2 - Anterior tonsillar pillar; 3 - Tongue; 4 - Palatine tonsil; 5 - Posterior tonsillar pillar; 6 - Soft palate (uvula)

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1.1.3. Hypopharynx It is the inferior part of the pharynx, lying behind and to the sides of the inner part of the larynx The oesophagus commences at the level of the inferior margin of the cricoid cartilage, corresponding to the 6th cervical vertebra The anterior wall: laryngeal inlet (superiorly) and the post-cricoid area (inferiorly). The post-cricoid area is the site of the only head and neck squamous cell carcinoma (SCC) that is more common in women! To each side of the larynx lie the piriform fossae or sinuses, triangular-shaped spaces with their apices inferiorly (lateral food channels). These are bounded laterally by the thyroid cartilage and medially by the lateral surface of the aryepiglottic folds, the arytenoid and cricoid cartilages 1.1.4. The palatine tonsil The palatine tonsil lies within the tonsillar fossa located at the level of the lateral wall of the oropharynx. Its a triangular-

Figure 3. Oropharynx and hypopharynx (posterior view)

1- Bucco-pharyngeal isthmus (fauces); 2 - Tongue base; 3 - Epiglottis; 4 Aryepiglottic fold; 5 - Piriform sinus; 6 - Prominence over cricoid cartilage (retrocricoid area)

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shaped space delineated by the anterior tonsillar pillar, the posterior tonsillar pillar and the tongue base, inferiorly. The medial surface of the tonsil is characterized by the presence of numerous tonsillar crypts. The deep surface of the tonsil is covered by a fibrous capsule. The space between this capsule and the lateral wall of the pharynx is filled with loose areolar tissue, which enables a fine tonsillar dissection during tonsillectomy. This is also the site where peri-tonsillar abscesses develop.

1.1.5. The pharyngeal muscles The pharyngeal wall consists of four layers. From the inside out, are the mucous membrane lining, the pharyngobasilar fascia (pharyngeal aponeurosis),the muscular layer and the buccopharyngeal fascia2. The muscular layer consists of three constrictor muscles (superior, middle and inferior constrictors). When contracting in succession, these muscles squeeze food downwards to the oesophagus. The lower fibers of the inferior constrictor muscle are called the cricopharyngeus muscle. This muscle relaxes during swallowing, thus acting as the upper oesophageal sphincter. Because the pharynx must allow air to flow between nose and larynx, and food from mouth to oesophagus, a complex system of muscle coordination is required to close the communication between the rhinopharynx and oropharynx, and the laryngeal inlet at appropriate times, and so keep these two functions separately. The communication between the rhinopharynx and the oropharynx is closed by the elevation of the soft palate during swallowing. The muscles which control the soft palate are the levator veli palati, tensor veli palati, palatoglossus and palatopharyngeus. The tensor veli palati and levator veli palati muscles also control the patency of the eustachian tube. 1.1.6. Nerve supply of the pharynx The motor innervation to the constrictors and the soft palate is supplied by branches from the vagus nerve (X). The sensory nerve supply of the rhinopharynx is from branches of the maxillary nerve (V). Branches from the glossopharyngeal nerve (IX) supply sensory innervation of the oropharynx. Branches from the internal laryngeal nerve (X) supply the hypopharynx.
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1.1.7. Pharyngeal vasculature Arterial blood supply comes from branches of the external carotid artery (ascending pharyngeal artery, ascending palatine artery, facial artery, maxillary artery). Venous drainage is directed to the internal jugular vein. Lymphatics drain to the retropharyngeal and laterocervical lymph nodes.

1.2. Phisiology
1.2.1. Deglutition (Swallowing) The pharyngeal stage of the swallowing is the most physiologically important, because airway protection occurs during this stage in healthy people 3. This second stage of deglutition is a reflex one, following the oral voluntary stage (mastication and food bolus formation) and preceding the 3rd reflex oesophageal stage. The tongue forces the bolus through the oropharyngeal isthmus into the oropharynx. The palatoglossus muscle (anterior tonsillar pillar) contracts in order to prevent reflux in the oral cavity. The larynx is elevated under the tongue base, the soft palate elevates, closing the communication between the rhinopharynx and the oropharynx. Through a combination of tongue thrusts and pharyngeal muscular contractions, the food is propelled through the lateral aspects of the hypopharynx (the piriform fossae) into the oesophagus 4. A concomitant relaxation of the cricopharyngeus muscle (upper oesophageal sphincter) occurs. 1.2.2. Phonation The pharyngeal cavities participate to the subsequent modulation of the fundamental sound generated by the vocal folds vibration. 1.2.3. Upper airways protection Protection against food aspiration during swallowing (see section 1.2.1) Protection against viral and bacterial infections: the
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Waldeyers lymphatic ring (the pharyngeal, palatine and lingual tonsils) is the first-line defense mechanism of the human body

1.2.4. Taste perception The bitter taste is perceived at the level of the tongue base, sensorial innervation being supplied through branches from the glossopharyngeal nerve (IX).

1.3. Symptoms of pharyngeal diseases

1.3.1. Pharyngeal pain Pharyngeal pain (sore throat, pain in the throat, odynophagia) is typically augmented by food swallowing and may irradiate in the ear. 1.3.2. Dysphagia Dysphagia is defined as having difficulty in swallowing, which may affect any part of the swallowing pathway from the mouth to the stomach. Patients complain that food or liquids are no longer being swallowed easily and that there is a sensation of food sticking. Additional symptoms may include early (pharyngeal blockage) or late (oesophageal blockage) regurgitation, odynophagia, referred otalgia, hoarseness, coughing after eating. Causes of dysphagia: congenital, trauma, foreign bodies, infections, inflammatory, oesophageal motility disorders, tumors, neurological 5. 1.3.3. Upper airway obstruction Obstruction at the level of the rhinopharynx (adenoids, tumors) causes nasal blockage, oral breathing, nasonated speech. Obstruction at the level of the oro- and hypopharynx (edema, tumors, foreign bodies) may cause significant upper airway obstruction (dyspnea, stridor) requiring emergengy procedures: intubation, tracheostomy.
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1.3.4. Lump in the throat Lump in the throat (globus pharyngeus, globus hystericus, pharyngeal paresthesias) is an abnormal feeling of something stuck or a sensation of a lump or tightness in the throat 6. Hippocrates first mentioned the term globus, and regarded it as a disease of women being inextricably involved in the uterine axis from which all hysteria was believed to be derived at that time 7. Globus accounts for almost 4 percent of otolaryngological referrals 8. Up to 45 percent of the general population have mild, intermittent symptoms resembling globus at some time in their lives 9. Globus is considered functional when no organic explanation is detected. It must be always a diagnosis of exclusion, only after a complete and accurate ENT examination is performed! The most popular organic aetiological theory of recent years is that globus is an atypical manifestation of silent gastro-oesophageal reflux disease (GERD) or oesophageal dysmotility10.

1.4. Clinical examination

1.4.1. Examination of the rhinopharynx
1.4.1.1. Inspection and palpation Inspection may reveal the characteristic adenoid face, in children with long-term untreated adenoids Palpation of the rhinopharynx: not recommended Palpation of the cervical lymph nodes (inflammatory or metastatic) 1.4.1.2. Posterior rhinoscopy Before endoscopy was available,visualization of the nasopharynx was one of the most difficult clinical examinations in ENT. Instruments: Light source Head mirror Small rhinopharyngeal or dental mirrors Tongue depressors A spirit lamp The examiner is in front of the seated patient, light beam is

166

focused on the oral cavity. Previously, the small mirror is warmed with the spirit lamp, to avoid blurring. The temperature of the mirror is checked on the examiners back of the hand. Ask the patient to open the mouth and keep the tongue within the oral cavity. Press the anterior two thirds of the tongue with the tongue depressor and ask the patient to breath simultaneously through the mouth and nose. This will relax the soft palate. The small mirror is gently introduced through the oral cavity, behind the uvula, and the rhinopharyngeal walls are examined. Touching the posterior wall of the pharynx with the mirror causes gagging reflexes, which may compromise the examination.
1.4.1.3. Endoscopy of the rhinopharynx Endoscopy became in the last years the gold-standard method for an accurate examination of the rhinopharynx. Instruments: Light source Fiber optic cable Endoscopes: nasal rigid telescopes of 25 cm length, 4 mm diameter and angulations of 0 and 30 are most commonly used. Smaller endoscopes are also available (2.7 mm diameter). More recently, 45 and 70 endoscopes have been developed. Fiber optic flexible endoscopes can also be used. Laryngeal rigid telescopes (90) can be used for trans-oral endoscopy Straight and curved suction tips, flexible metal cottontipped applicators, straight and upbiting forceps, anti fog solutions Topical nasal decongestants and anaesthetics Video and digital recording equipment, if available After anesthetizing the nares with topical lidocaine spray, many otolaryngologists will spray the nares with a decongestant. The endoscope (flexible or rigid) is Figure 4. Nasopharynx then gently passed along the floor (endoscopic view) of the nasal cavity, beneath the 1 - Eustachian tube opening: 2 - Torus inferior turbinate. The eustachian tubarius

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tube orifice, torus tubarius and Rosenmuller fossa are carefully inspected on each side. Arguably, the best view of all may be obtained using a 90 rigid scope in the oropharynx. By advancing the rigid scope through the mouth and by placing the beveled edge posterior to the soft palate, the nasopharynx may bee seen in its entirety 11.

1.4.2. Examination of the oropharynx

1.4.2.1. Inspection and palpation Palpation of the tongue base (using a glove) may reveal indurated areas, which may require a further biopsy Palpation of the cervical lymph nodes (inflammatory or metastatic) 1.4.2.2. Oropharyngoscopy Instruments: Light source Head mirror Tongue depressors The examiner is in front of the seated patient, light beam is focused on the oral cavity. Ask the patient to open the mouth and keep the tongue within the oral cavity. Gently apply the tongue depressor over the anterior two thirds of the tongue, in order to avoid gagging reflexes. Ask the patient to say a, to check the mobility of the soft palate, which normally elevates in the midline. The motor innervation to the soft palate is supplied by branches of the vagus nerve (X). A unilateral paralysis will determine a deviated elevation of the palate towards the healthy side. Examine the palatine tonsil, the tonsillar pillars, the posterior wall of the oropharynx. The tongue base should be examined using a laryngeal mirror or endoscopy.

1.4.3. Examination of the hypopharynx

1.4.3.1. Inspection and palpation Palpation of the cervical lymph nodes (inflammatory or metastatic)

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Figure 5. Oropharyngoscopy

1.4.3.2. Indirect laryngoscopy The hypopharynx is visible only during indirect laryngoscopy (see examination of the larynx) 1.4.3.3. Endoscopy Endoscopy (flexible or rigid) provides the most accurate examination of the hypopharynx (see examination of the larynx)

1.4.4. Other investigations Bacteriological examinations Biopsy Imaging: CT scans, MRI Functional investigations of the upper gastrointestinal tract (dysphagia): radiology (barium swallow, videofluoroscopy, CT and MRI), pharyngeal and oesophageal manometry, oesophageal and pharyngeal pH monitoring

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2. ACUTE AND CHRONIC PHARYNGEAL

INFECTION

2.1. Acute viral pharyngitis

2.1.1. Definition. Aetiology Acute pharyngitis is an inflammatory infectious condition affecting the pharyngeal mucosa. Often viral in origin (bacterial superinfection may occur during evolution), it usually involves the entire pharyngeal mucosa, is very contagious and is characterized by a seasonal pattern. Viral causes of acute pharyngitis, in order of frequency, include rhinovirus, adenovirus (5%), parainfluenza virus, Coxsackie virus, corona virus, echovirus, herpes simplex virus (<5%) 12. 2.1.2. Symptoms Sudden onset: fever, malaise, headaches, painful dryness of the throat (hyperesthesia of the pharyngeal mucosa), odynophagia (sore throat) Symptoms of nasal disease may be associated: nasal obstruction, nasal discharge The disease follows a short course, usually resolving spontaneously within 5-6 days 2.1.3. Diagnosis Palpation: there is seldom any cervical adenopathy Oropharyngoscopy: generalized hyperemia of the entire pharyngeal mucosa A complete ENT examination should be performed:pharyngitis is frequently associated with acute rhinitis, laryngitis Serologic assessment of antiviral antibodies titers is not a routine laboratory procedure; it is used mainly for scientific purposes
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Figure 6. Acute viral pharyngitis

2.1.4. Treatment Treatment is symptomatic: analgetics, antipyretics, bed rest, hot drinks Antibiotics are of no value (its a viral infection); may be recommended in immunocompromised patients to prevent bacterial superinfection Local treatment: topical disinfectants (sprays, gargles, tablets)

2.2. Acute bacterial pharyngitis (Follicular tonsillitis)

2.2.1. Definition. Aetiology The disease remains localized to the tonsillar tissue and is only moderately contagious. Group A beta-haemolytic streptococci (GABHS) are the commonest cause of acute bacterial pharyngitis and are spread via respiratory secretions through close contact. More than 120 M-protein types of GABHS have been isolated, with serotypes 1, 3, 5, 6, 18, 19 and 24 associated with rheumatic fever and others, such as serotypes 49, 55 and 57, associated with acute poststreptococcal glomerulonephritis 12.
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Pneumococcus may also be responsible, Gram-negative microorganisms are rarely involved.

2.2.2. Symptoms The initial symptoms, both local and general, do not differ from those of erythematous pharyngitis Severe odynophagia (extremely painful swallowing); it may be associated with referred otalgia High fever, chills 2.2.3. Diagnosis Palpation of the neck: tender anterior jugulo-digastric cervical lymphadenopathy Oropharyngoscopy: the tonsils are enlarged, very red, covered with a grayish-white exudate which can be easily detached with a cotton probe White blood count is usually raised, around 10 - 12,000/ mm3 (leukocytosis) associated with elevated erythrocyte sedimentation rate (ESR) values Throat culture is described as the gold-standard diagnostic test in the North American literature, but recent evaluations recommend that throat swabs should not routinely be carried out in sore throat 13

Figure 7. Follicular tonsillitis

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2.2.4. Complications Complications are rare nowadays, peri-tonsillar abscess being commonest, arthritis, endocarditis, nephritis being much less frequent. Suppurative: peri-tonsillar abscess, parapharyngeal and retropharyngeal abscesses, descending necrotizing mediastinitis, septicemia Toxin-mediated: toxic shock-like syndrome Non-suppurative, immune-mediated: rheumatic fever, poststreptococcal glomerulonephritis, pediatric autoimmune neuropsychiatric disorder and acute disseminated encephalomyelitis (similar to Sydenhams chorea) 12 2.2.5. Treatment Antibiotics are mandatory: best antibiotic to start treatment is still Penicillin! If the patient is allergic to Penicillin, it is recommended to use Erythromycin If the responsible agent is Gram-negative, broad-spectrum antibiotics are indicated Bed rest, hydration, analgetics, antipyretics, topical disinfectants In recurrent cases (> 3-4 episodes per year): tonsillectomy A recent assessment of Cochrane reviews suggests that for streptococcal tonsillitis the use of antibiotics seems to be discretionary rather than prohibited or mandatory. This is because the benefit in terms of symptoms is only about 16 hours compared with placebo, and that serious complications such as rheumatic fever and glomerulonephritis are now rare in developed countries14.

2.3. Acute adenoiditis

2.3.1. Definition. Aetiology Acute adenoiditis is an inflammatory condition of the Luschkas pharyngeal tonsil, determined by a bacterial superinfection during the evolution of a viral upper respiratory tract infection. It is one of the most frequent diseases in childhood.
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The bacterial agents involved are Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis and staphylococci.

2.3.2. Symptoms nasal obstruction, mucopurulent rhinorrhoea, nasonated speech fever, chills, malaise 2.3.3. Diagnosis Palpation: upper cervical inflammatory lymphadenopathies Anterior rhinoscopy: congestion of the nasal mucosa which is covered by mucopurulent secretions Endoscopy of the rhinopharynx: the pharyngeal tonsil is congested, hypertrophied and covered by a yellowish exudate Oropharyngoscopy: congestion of the pharyngeal mucosa, mucopurulent secretions on the posterior pharyngeal wall 2.3.4. Complications Acute suppurative otitis media, otitis media with effusion Retropharyngeal abscesses 2.3.5. Treatment Antibiotics: Amoxicillin, Amoxicillin-clavulanate Nasal decongestants, analgetics, antipyretics Hydration

2.4. Suppurative complications of acute bacterial pharyngitis

2.4.1 Peritonsillar abscess (Quinsy)
2.4.1.1. Definition. Aetiology Peritonsillar abscess is a collection of pus developed between the fibrous capsule of the tonsil, usually at the upper pole, and the lateral pharyngeal wall. It generally arises as a complication of acute tonsillitis. It may happen at any age, but the majority is in young adults between 20 and 39 years of age.

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The aetiology of peritonsillar abscesses involves the usual beta-haemolytic streptococcal infection, associated with the presence of anaerobic organisms. A recent study 15 noted an association between periodontal disease and quinsy, which may represent a source of anaerobic organisms. Infection extends beyond the capsule into the loose peritonsillar space, where produces a cellulitis and then an abscess. The abscess is most commonly sited anteriorly, pushes the tonsil medially, inferiorly and posteriorly.
2.4.1.2. Symptoms Debut: unilateral progressive pharyngeal pain, referred otalgia, moderate fever After 2-3 days of evolution: extremely severe odynophagia with referred otalgia, drooling of saliva, trismus (pathognomonic), rhinolalia, fever, deterioration of the general condition 2.4.1.3. Diagnosis Palpation of the neck: ipsilateral jugulodigastric lymph nodes are enlarged and tender Oropharyngoscopy: unilateral oropharyngeal swelling at the level of anterior tonsillar pillar and the soft palate, the tonsil is displaced medially and covered by soft exudate , uvular edema

Figure 8. Peritonsillar abscess (Quinsy)

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2.4.1.4. Evolution. Complications The abscess takes 5-8 days to accumulate and may rupture spontaneously if it is not incised Deep neck space infections and mediastinitis have been described in 1.8% of cases16. Mediastinitis is a condition with significant mortality (23%) even with aggressive management (combined neck and mediastinal drainage, as well as appropriate antibiotics)17 2.4.1.5. Treatment Admission to hospital and treatment with intravenous antibiotics should be the baseline treatment offered to all patients Surgical management: incision through the anterior pillar with pusevacuation, preceded by needle aspiration to confirm the presence of pus and to guide the site of incision Intravenous antibiotics, steroids (extensive edema), analgetics Tonsillectomy, in case of recurrences

2.4.2. Parapharyngeal abscess

2.4.2.1. Definition. Aetiology The parapharyngeal space lies on either side of the superior pharynx (rhino- and oropharynx), lateral to the superior constrictor muscles. It contains the internal carotid artery, the internal jugular vein, the last four cranial nerves and some lymph nodes. Parapharyngeal abscess is a collection of pus developed at the level of the parapharyngeal space. The most common causes are acute bacterial tonsillitis, peritonsillar abscesses and dental infections. The bacteriology of deep space neck infections appears to change with increasing numbers of cases being due to gramnegative aerobic infections, which will not respond to first-line penicillin treatment and may be contributing to the significant mortality still associated with these conditions18. 2.4.2.2. Diagnosis Clinical features are similar to the peritonsillar abscess, except that maximum swelling in the pharynx is more inferiorly placed and behind the tonsil, with less uvular edema Palpation of the neck: tender, firm but fluctuent swelling Neck ultrasound, CT scans

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 Complications: carotid sheath necrotizing fasciitis, mediastinitis

vessel

thrombosis,

2.4.2.3. Treatment Admission to hospital for intravenous antibiotics is the baseline treatment Failure to respond to conservative therapy within 48 hours should prompt surgical abscess drainage

2.4.3. Retropharyngeal abscess

2.4.3.1. Definition The retropharyngeal space lies behind the pharynx, just anterior to the prevertebral fascia. This space contains two layers of lymph nodes (Gillettes nodes). The retropharyngeal abscess occurs mainly as a complication of acute adenoiditis in children. Adults develop rarely retropharyngeal abscesses, the most frequent causes being tuberculosis and foreign body penetration 4. 2.4.3.2. Diagnosis Neck stiffness, odynophagia, drooling, dyspnoea and dysphagia Fever, chills, malaise Examination reveals a fluctuant mass at the level of the posterior pharyngeal wall X-ray, ultrasound, CT scans 2.4.3.3. Treatment Surgical drainage High-dose intravenous antibiotics, analgetics

2.5. Specific acute bacterial pharyngitis

2.5.1 Vincents angina
2.5.1.1. Definition. Aetiology Vincents angina is an unilateral ulcerative pharyngitis, which evolves in two stages: the first being pseudo-membranous and the second ulcerative. It is caused by an association of fusiform bacilli

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and spirochaete (Treponema vincentii, Spirochaeta denticulata, Fusobacterium necroforum). Infection arises in times of overcrowding and is usually found in young adults with poor oral hygiene, chronic gingivo-dental infections and lowered resistance4.
2.5.1.2. Diagnosis History: slow, insidious onset Sore throat, unilateral odynophagia, mild fever Oropharyngoscopy: examination in early stages reveals a friable, necrotizing, exudative false membrane on one tonsil. Later, in evolution, ulceration appears (irregular, fairly deep, soft margins, covered by a yellowish exudate) Laboratory tests, biopsy (to rule out a malignancy and other ulcerative anginas) 2.5.1.3. Treatment aggressive oral hygiene antibiotics (penicillin disinfectants

and

metronidazole), topical

2.5.2. Diphtheria
2.5.2.1. Definition. Aetiology Diphtheria is a pseudo-membranous pharyngitis, caused by Corynebacterium diphtheriae (the Klebs-Loeffler bacillus), a facultative aerobic gram-positive agent. This condition has now become exceedingly rare. The bacillus, spread by air-borne droplets, remains at the level of the pharynx, causing the local typical manifestations of the disease. In the meantime, it produces a strong neurotropic exotoxin which is spread into the organism, causing serious systemic manifestations. 2.5.2.2. Symptoms The common form is usually seen in children, the early symptoms being insidious Malaise, mild fever, headaches Severe sore throat

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2.5.2.3. Diagnosis Palpation of the neck: extensive cercical lymphadenopathy Oropharyngoscopy: pearly-white, velvety, firm, extremely adherent pseudomembranes covering the tonsils, and then extending progressively onto the tonsillar pillars and the soft palate. When these pseudomembranes are detached, the underlying surface bleeds easily The malignant form of the disease: extension of the pseudomembranes to the entire pharynx, nose and larynx, cardiac disorders, peripheral and cranial nerve palsies. This form is generally fatal within 10-12 days Direct cultures should be obtained for smear and culture (Klebs-Loeffler medium) tests 2.5.2.4. Treatment Early administration of antitoxin is mandatory, based only on clinical suspicion, without confirmation of the result of the throat culture! Antibiotics should be also administered: Penicillin G is required The patients need to be isolated and reported

2.5.3. Scarlet fever

2.5.3.1. Definition. Aetiology Scarlet fever is caused by group A beta-haemolytic streptococci (GABHS) which produce a pathognomonic erythrogenic exotoxin. Some authors consider scarlet fever as a nonsuppurative complication of follicular tonsillitis 4. The pharyngitis constitutes both the portal entry for this exanthematous fever and the first sign of the disease. 2.5.3.2. Diagnosis History: sudden onset with high fever, chills, headaches, nausea, vomiting, tachycardia Intense dysphagia and odynophagia Oropharyngoscopy: a diffuse erythematous pharyngitis contrasting with the white appearance of the dorsum of the tongue. The descuamation of the tongue causes the characteristic strawberry tongue within 5-6 days 179

 The exanthema appears the following day, making the diagnosis easy. It begins on the thorax, spreading all over the body and excluding the perioral region
2.5.3.3. Treatment Antibiotics: Penicillin for 10 days Topical disinfectants, analgetics

2.6. Specific viral pharyngitis

2.6.1. Infectious mononucleosis (IMN)
2.6.1.1. Definition. Aetiology Infectious mononucleosis is an acute, systemic viral infection presenting typically with sore throat and generalized lymphadenopathy (glandular fever). It affects primarily young adults. Transmission is via saliva (the kissing disease). The causative infectious agent is the Epstein-Barr virus (EBV), one of six human herpes viruses isolated from blood, lymph nodes and saliva 12. 2.6.1.2. Symptoms History: there is a prodrome of 4-5 days with malaise, fatigue and headaches Odynophagia, dysphagia 2.6.1.3. Diagnosis Palpation: generalized tender lymphadenopathies (100% of the patients) Oropharyngoscopy: pharyngeal signs range from acute follicular tonsillitis, indistinguishable from classic streptococcal tonsillar infection, to pseudo-membranous forms, even ulcero-necrotic Occasionally, all the lymphoid tissue of the Waldeyers ring may enlarge and become covered with pseudomembranes Splenomegaly (50% of the patients), hepatomegaly (10%) A typical rubelliform skin rash develops almost invariably if ampicillin is prescribed

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 Leukocytosis (10-20,000/mm3, even 40,000/mm3) with an inversion of the ratio of the white cells and presence of abnormal monocytes Paul Bunnell test: evidence of heterophile antibodies (agglutinins to sheep and horse red cells) The gold-standard serologic testing: evidence of EBVspecific antibodies (IgM to the EBV viral capsid antigen)12 Differential diagnosis: Cytomegalovirus (CMV) infection, toxoplasmosis, HIV, acute streptococcal tonsillitis, leukaemia The course of the disease is benign and prolonged for 2-3 weeks, followed by a period of malaise and chronic fatigue
2.6.1.4. Treatment Symptomatic for mild-to-moderate cases If there is super-added infection, antibiotics and steroids can be prescribed. Ampicillin-based antibiotics should be avoided

2.6.2. Cytomegalovirus infection (CMV) CMV infection may occur in non-immunocompromised or immunocompromised patients (HIV). The clinical aspects are similar to IMN. Ragnaud reported pharyngitis is much less common in adult CMV than with adult EBV mononucleosis. In children, CMV pharyngitis is more common than in adults 19. Serological diagnosis is confirmed with ELISA test (anti CMV IgM) or complement fixation test. 2.6.3. Herpangina Herpangina is a self-limiting vesicular eruption that occurs on the oropharyngeal mucosa. A number of enteroviruses (30 and 71) and Coxsackie virus group A are involved in aetiology. Its regular spread to the oropharynx differentiate it from herpes simplex type 1 infection, which affects mainly the oral cavity 12. 2.6.4. Hand, foot and mouth disease This condition is caused by enterovirus 71 or Coxsackie viruses, and is characterized by vesicular eruptions in the oral cavity and oropharynx, accompanied by vesicles on the hands and feet, fever, malaise, vomiting. 181

A fulminant form may lead to severe neurological complications, acute pulmonary embolus and cardiopulmonary decompensation 12.

2.6.5. Herpes viruses Type 1 herpes simplex infection usually affects young children. It causes vesicular and ulcerative lesions of the lips, tongue, gums, buccal mucosa and occasionally oropharyngeal mucosa. Herpes zoster pharyngitis is characterized by the presence of unilateral painful vesicular lesions.

2.7. Pharyngeal manifestations in blood disorders

2.7.1. Agranulocytosis
2.7.1.1. Definition. Aetiology Agranulocytosis is characterized by a considerable decrease in the number of polymorphonuclear leucocytes in the peripheral blood. Pharyngeal manifestations are frequent when their absolute number falls below 1000/mm3 . This condition may present as pure agranulocytosis or as part of a pancytopenia syndrome. The history may reveal the cause: sensitivity to a drug (amidopyrine, phenylbutazone, chloramphenicol, sulphonamides, antithyroid drugs, oncological chemotherapy), exposure to various agents (benzene) 1. 2.7.1.2. Diagnosis Severe dysphagia and odynophagia, foetid breath The general health is profoundly affected Oropharyngoscopy: pharynx and later the mouth are covered first by false membranes and then by necrotic ulcers which bleed (in pancytopenia), and extend rapidly Hematologic tests 2.7.1.3. Treatment Massive doses of antibiotics, steroids, blood transfusions

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2.7.2. Acute leukemia

2.7.2.1. Definition Leukemia is a generalized neoplastic proliferation of leukocytes and their precursors. Patients with leukemia are more susceptible to severe infections because of the ineffectiveness of the leukocytic and antibody response. Chemotherapy may further depress the leukocytic response. Hemorrhages are frequent because of the decreased number of platelets, and are quite difficult to control 20. 2.7.2.2. Diagnosis Inspection and palpation: severe paleness (anemia), generalized lymphadenopathy, hepato/splenomegaly Oropharyngoscopy: ulcero-necrotic lesions of the pharyngeal mucosa, which may bleed; leukemic infiltrations of the gingiva and oral cavity mucosa (hypertrophic gingivo-stomatitis), of the tonsillar tissue (tonsillar enlargement) Hematologic tests 2.7.2.3. Treatment Is conducted by the hematologist Pharyngeal infections are treated with massive doses of antibiotics, steroids

2.8. Chronic non-specific conditions

2.8.1. Chronic non-specific pharyngitis
2.8.1.1. Definition. Aetiology Chronic inflammation of the pharynx occurs when the mucosa is irritated over a long period of time. Chronic inflammation of the pharyngeal mucosa may present in two different forms: Chronic catarrhal hyperplastic pharyngitis Chronic atrophic pharyngitis Extrinsic and intrinsic factors are involved in the aetiology4:

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 Extrinsic factors: nicotine, alcohol, chemical inhalational irritants, allergens, radiotherapy Intrinsic factors: acute and chronic infections (tonsillitis, rhinosinusitis, bronchitis), gastroesophageal reflux disease (GERD)
2.8.1.2. Diagnosis Persistent sore throat, foreign body sensations, coughing and hawking In the catarrhal form pharyngeal mucosa is red, dotted with hypertrophic lymphoid follicles, mainly posterior to the posterior pillar, even granulations (the cobblestoned pharynx) Atrophic pharyngitis is often the final stage of the catarrhal form and manifests itself by dryness of the mucosa and sometimes crusting; atrophic pharyngitis can also develop secondary to radiotherapy for head and neck tumors Investigations for gastroesophageal reflux disease (GERD)

Figure 9. Chronic nonspecific pharyngitis

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2.8.1.3. Treatment Elimination of predisposing factors and mucosal care Vitamin A, topical disinfectants, non-steroidal antiinflammatory drugs (NSAIDs)

2.8.2. Chronic tonsillitis

2.8.2.1. Definition Chronic infection can often be found in large cryptic tonsils, in small sclerosed tonsils and in tonsillar remnants left behind by incomplete tonsillectomy. These recurrent infections lead to permanent inflammation in the tonsillar crypts and scarring of the tonsillar tissue. The organisms responsible for chronic tonsillitis are similar to those encountered in acute infections. Chronic infected tonsils are considered to be a focus which may activate other chronic inflammatory diseases in the body by spreading bacteria and toxins4. 2.8.2.2. Diagnosis History: recurrent or persistent sore throat, dysphagia, malaise, fetid breath Palpation: inflammatory upper laterocervical adenopathies

Figure 10. Chronic hypertrophic tonsillitis

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 Oropharyngoscopy: tonsils may appear as hypertrophic, normal or scarred/atrophic, often with surrounding peritonsillar erythematous tissue Presence of pus when the tonsils are squeezed Blood tests: elevated ESR, white blood cells as well as the concentrations of antistreptolysin may increase The most frequent complications: peri-tonsillar abscesses Chronic suppurative tonsillitis must be differentiated by the chronic caseous tonsillitis which is characterized by the presence of crypts full of white concretions, the product of maceration of food debris (caseum), it is not accompanied by suppuration, no complications occur, no need for tonsillectomy
2.8.2.3. Treatment Conservative: analgetics, topical disinfectants, antibiotics when indicated Surgery: tonsillectomy is generally suggested when patient complains of more than five to seven episodes of recurrent infections per year or several infections in two or more subsequent years4

2.8.3. Adenoids
2.8.3.1. Definition Hyperplasia of the pharyngeal tonsillar tissue (Luschkas pharyngeal tonsil), owing to chronic inflammation or allergy of the upper airways. Hyperplastic adenoids are found almost exclusively in children. Enlarged adenoids after the age of puberty and in adults is a very rare condition, various alternative diagnoses must be taken into account (hysthopathology). 2.8.3.2. Symptoms Nasal obstruction, mucopurulent rhinorrhoea, nasonated speech Chronic oral breathing, sleep disorders, snoring, obstructive sleep apnoea syndrome Conductive hearing loss Delay in development

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2.8.3.3. Diagnosis Inspection: severe long-standing obstruction can produce the dull expression known as the adenoid face (narrow, pale, dumb facial expression with open mouth and sunken eyes) Palpation: cervical lymphadenopathies Anterior rhinoscopy: congestion of the nasal mucosa, mucous secretions on the walls of the nasal cavities Posterior rhinoscopy/endoscopy of the nose and rhinopharynx reveals the hypertrophied lymphoid tissue, filling the cavum O ro p h a r y n g o s c o py : dental malocclusion, gothic palate, tonsillar hyperplasia, secretions on the posterior oropharyngeal wall Otoscopy/microscopy of the ear: retracted eardrum with or without middle ear effusion Hearing tests: Figure 11. Adenoids tympanometry,pure tone (endoscopic view) audiometry (conductive hearing loss) Biopsy: if there is a suspicion of neoplasm 2.8.3.4. Complications Recurring nasal, sinus and tonsillar infections Persistent dysfunction of the Eustachian tube, followed by otitis media with effusion Recurrent acute suppurative otitis media 2.8.3.5. Treatment

2.8.3.5.1. Conservative treatment: is only appropriate preoperatively (e.g. acute rhinosinusitis) or when surgery is contraindicated (e.g. cleft palate): antibiotics, nasal decongestants, immunostimulants (Broncho-Vaxom) 187

2.8.3.5.2. Surgical treatment: The recommended European standard21 is: Surgical removal of the adenoids is the first-line treatment! Adenoidectomy: any adenoid hyperplasia with chronic nasal obstruction, recurrent or persistent rhinosinusal infections, chronic purulent rhinorhoea, persistent or recurrent dysfunction of the Eustachian tube, otitis media, chronic bronchitis or in sleep apnoea syndrome Sleep apnoea syndrome: as a rule with simultaneous tonsillectomy Additional useful procedures: myringotomy with grommets insertion

Figure 12. The Beckmann curette

Adenoids are removed with a special curette (Beckmanns adenotome) in general or local anesthesia. The child leaves hospital the day after operation.

2.9. Chronic specific pharyngitis

2.9.1. Tuberculosis of the pharynx
2.9.1.1. Definition. Aetiology The pharynx is not a common site for clinically manifest tuberculosis. It is the site of primary infection almost always in children, resulting in an asymptomatic primary focus usually located in tonsils and adenoids, with accompanying cervical adenopathies. Secondary tuberculosis involves the pharynx, but only in patients with massive, positive and usually cavitating pulmonary lesions. Lupus vulgaris is a low-grade fibro-sclerotic cutaneous form and has been described in the nasal cavities and in the pharynx. 188

The aetiology of tuberculosis is represented by the Mycobacterium tuberculosis agent. The resurgence of tuberculosis has paralleled the HIV epidemic and it will inevitably increase in the next years 12.
2.9.1.2. Diagnosis The primary form in children: usually asymptomatic, adenoid and tonsillar hyperplasia, cervical adenopathies The secondary form: multiple, painful shallow ulcers, pseudo-tumor formation (tuberculoma) Chest X-rays, microscopic examination of stained smears for acid-fast bacilli, new ELISA tests to detect specific antigens and PCR testing for genetic elements 12 Biopsy, to rule out a malignancy! 2.9.1.3. Treatment Treatment is with triple therapy, but drug resistance is an increasing problem worldwide

2.9.2. Syphilis
2.9.2.1. Definition. Aetiology Syphilis is an infectious disease caused by Treponema pallidum. The infection may present in various forms that have been classically described as primary, secondary, tertiary and congenital syphilis. In primary syphilis the typical lesion is the chancre, which appears after an incubation period of 2-3 weeks. The most frequent extragenital sites for the chancre are lips, buccal mucosa, tongue and tonsils. The lesion is characterized by a painless ulcer with indurated margins, accompanied by enlarged upper cervical adenopathies22. Secondary form usually occurs several weeks (four to six) after the primary lesion, and is characterized by malaise, headaches, fever, generalized lymphadenopathy, mucocutaneous rash and sore throat. The pharyngeal mucosa displays congestion, inflammation and is covered by typical mucous patches and superficial ulcerations. Tertiary syphilis develops 3-10 years after the initial infection. The typical lesion is the gumma, located on the hard palate, tonsil or

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the posterior pharyngeal wall. It is a granulomatous, necrotic painless lesion. In evolution there may be perforation of the soft or hard palate, and considerable destructions of the surrounding structures.
2.9.2.2. Diagnosis In the primary and secondary forms, spirochetes can be identified by darkfield illumination microscopy in smears taken directly from the lesion Spirochetes can also be identified in biopsy specimens using silver stains or fluorescent-labelled antibodies. The biopsy of a tertiary lesion is mandatory for the differential diagnosis with malignancies and other granulomatous diseases! Serological tests: those used to identify nonspecific antibodies to cardiolipin Venereal Disease Research Laboratory (VDRL), and those to detect specific treponemal antibodies (TPI, FTA tests) Two rapid enzyme immune assay techniques, the Enzywell TP and the Syphilis ICE enzyme immunoassay (EIA), have been recently developed 12 2.9.2.3. Treatment High-doses of Penicillin Ceftriaxone and Azithromicin are studied as alternative antibiotics 23

2.9.3. Other chronic specific granulomatous diseases

2.9.3.1. Leprosy Chronic granulomatous infection caused by Mycobacterium leprae Isolated leprosy of the pharynx does not occur; it spreads to the pharynx from the nasal cavities Diagnosis: biopsy is mandatory Treatment: multi-drug therapy regimens (see Nose, section 3.2.4.) 2.9.3.2. Rhinoscleroma Progressive granulomatous inflammatory disease, commencing in the nose, with later involvement of the pharynx, larynx and sometimes trachea and bronchi

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 The disease begins in the nose, but may secondarily affect the pharynx: granulomatous lesions and scarring The causative agent is the gram-negative bacillus Klebsiella rhinoscleromatis (Frisch bacillus) (see Nose, section 3.2.3.)

2.9.4. Candidiasis
2.9.4.1. Definition. Aetiology Candida albicans is found in 15-25% of oral cavity and pharyngeal mucosal surfaces, being considered a normally saprophytic agent22. In order to become pathogenic and symptomatic, some predisposing factors must coexist (Table 1 ).
Local Local disease: lichen planus, leukoplakia Systemic antibiotics may change local oral flora to allow overgrowth of candida Radiotherapy Systemic Diabetes mellitus Lymphoma Immunosuppressive drugs AIDS

Table 1. Predisposing factors for candidiasis

2.9.4.2. Diagnosis It may be asymptomatic or accompanied by dysphagia, sore throat, painful swallowing

Figure 13. Oral candidiasis

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 The mucosa shows red, irregular and flat lesions, may be covered by small white patches, which after removal leave an erythematous lesion Diagnosis is confirmed by a Gram stain or a culture
2.9.4.3. Treatment Candidiasis with local predisposing factors responds well at topical anti-fungal therapy (nystatin, clotrimazole) When systemic factors are present, systemic anti-fungal therapy (fluconazole) may be required

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3. TUMOURS OF THE PHARYNX

3.1. Tumors of the nasopharynx
3.1.1. Benign tumors
3.1.1.1. Juvenile nasopharyngeal angiofibroma (JNA) 3.1.1.1.1. Definition JNA is an uncommon, benign and highly vascular tumor that arises at the level of the tissues within the sphenopalatine foramen. Rarely, it is found at other sites in the nasal cavity or paranasal sinuses24. It develops almost exclusively in male adolescents. As it grows, the tumor invades the nasal cavities, the rhinopharynx, the paranasal sinuses, pterygopalatine and infratemporal fossae, the orbit and even the skull base25. 3.1.1.1.2. Pathology JNAs are well-defined, circumscribed, lobulated tumors, covered by normal nasopharyngeal mucosa. They are highly vascular and locally destructive, but non-infiltrating. The tumor consists of a network of proliferating and irregular vascular channels, embedded within a fibrous stroma. Because this tumor occurs exclusively in adolescent boys, there has always been speculations that sex-hormone receptors could play a significant role in its development. Recent studies demonstrated that androgen receptors are present in at least 75% of tumors. In contrast, estrogen receptors have not been demonstrated26. Other factors involved: the angiogenic vascular endothelial growth factor (VEGF)27, overexpression of insulin-like growth factor II (IGFII)28.

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3.1.1.1.3. Symptoms Recurrent episodes of severe epistaxis in young male adolescents (the highest incidence is between the age of 13 and 17) Nasal obstruction, mucopurulent rhinorrhea Hypo/anosmia Nasal speech (rhinolalia clausa) 3.1.1.1.4. Diagnosis Inspection: oral breathing, protrusion of the eye-ball, facial deformities, cranial nerve deficits Posterior rhinoscopy, endoscopy of the nose and nasopharynx Biopsy should not be performed, risk of important haemmorhage (!) Ear microscopy: retraction of the eardrum, middle ear effusion CT scans, MRI: evaluation of tumor Figure 14. Juvenile nasopharyngeal extent angiofibroma (endoscopic view) Angio-MRI/digital substraction angiography: identification of the blood vessels to the tumor and mostly embolization of the afferent arteries at the same time Ophthalmological and neurological examination 3.1.1.1.5. Differential diagnosis Adenoids Craniopharyngioma: benign epithelial tumor, originating from remnants of epithelium of the hypophyseal duct and/or Rathkes pouch Thornwaldts cysts (pharyngeal bursitis): benign cystic formation from remnants of Rathkes pouch Choanal polyp Malignant tumors of the nasopharynx

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3.1.1.1.6. Treatment 3.1.1.1.6.1. Surgical treatment: The recommended European standard 21 is: Complete resection of the tumor via a transnasal or transmaxillary approach, or endoscopic access. If necessary, a midfacial degloving is performed Preoperative embolization is appropriate in most cases, if available Preoperative external carotid ligation is also recommended 3.1.1.1.6.2. Conservative treatment: Radiotherapy Long-term estrogen therapy

3.1.2. Malignant tumors

3.1.2.1. Nasopharyngeal carcinoma (NPC) 3.1.2.1.1. Epidemiology Incidence (new cases/inhabitants/year): in central Europe 0.5-1:100,000, in southern Europe 1:100,000, in southern China, South Asia up to 20:100,000 21 The highest incidence rate occurs in southern China, among the Cantonese population of the Guangdong province. In this province, the NPC is referred as the Guangdong tumor 29. In Hong Kong, where 98% of the population is Cantonese, the incidence rate for men was once reported as 30:100,000, the highest in the world 30 Emigrants from southern China retain a significantly higher risk of developing a NPC, although it decreases in subsequent generations 31 NPC shows a predilection for middle-aged patients; however, it was also observed in children 3.1.2.1.2. Aetiology Genetic factors: HLA class 1 genotypes (HLA-A, B and DR) Epstein-Barr virus (EBV) involvement: mainly

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in undifferentiated or nonkeratinizing forms. The discovery of EBV receptors at the level of human pharyngeal epithelia firmly revealed the close association between EBV and NPC 32. To date, circumstantial evidences has proved that the virus plays a critical role in the pathogenesis of NPC 33 Environmental carcinogens: nitrosamines from salted fish (southern China)

3.1.2.1.3. Pathology The WHO (1978) classification of NPC was revised in 1991. It divides the NPC into two grades: grade 1 (keratinizing squamous cell carcinomas) and grade 2 (nonkeratinizing squamous cell carcinomas and undifferentiated carcinomas)34. The older classification (1978) divided the NPC into three types: Type I: Squamous cell carcinoma (keratinizing): welldifferentiated, moderately differentiated and poorly differentiated Type II: Nonkeratinizing carcinoma Type III: Undifferentiated carcinoma 3.1.2.1.4. Clinical features Several different clinical presenting patterns have been described 35: An upper neck mass (enlarged metastatic laterocervical lymph nodes) is the commonest complaint at first presentation (50% of the patients) Otological symptoms: unilateral conductive hearing loss due to the impairment of eustachian tubes function, secondary to the presence of the nasopharyngeal mass (20% of the patients) Rhinological symptoms: nasal obstruction, blood-stained nasal discharge, post-nasal drip, epistaxis (mainly in large tumors) Neurological complaints: headaches, cranial nerve symptoms secondary to invasion of the skull base (foramen lacerum: III, IV, ophthalmic division of V and VI, foramen jugulare: IX, X, XI)

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3.1.2.1.5. Diagnosis Palpation of the neck: to evaluate the metastatic lymphadenopathies Posterior rhinoscopy Endoscopy: the site of origin is the Rosenmuller fossa, behind the eustachian tubes opening, on the lateral nasopharyngeal wall; this site must be extremely carefully examined Biopsy is considered the first necessary investigation for NPC if a suspicious lesion is found! A complete ENT examination should be performed; care should be taken to look for middle ear effusions and cranial nerves deficits Imaging: CT, MRI to evaluate the extent of the disease Serologic tests for EBV: the detection of IgA antibodies to EBV (anti-VCA, anti-EA titres)36 3.1.2.1.6. Treatment Curative radiotherapy: NPC is an extremely radiosensitive tumor and the mainstay of treatment for primary local and regional disease is radiotherapy, almost irrespective of the diseases stage37 For patients with advanced disease, the addition of chemotherapy appears to enhance the overall treatment outcomes Surgery only plays a limited role in the management of NPC: neck dissections for metastatic disease in the neck, if the nasopharynx is free of tumor; salvage surgery in local recurrences Prognosis: In undifferentiated carcinoma, the 5-year survival is about 60% for stages 1 and 2, and about 20% for advanced stages; in keratinizing and non-keratinizing forms, the 5-year survival is 20%21 3.1.2.2. Other nasopharyngeal malignancies Epithelial tumors: adenocarcinomas, mucoepidermoid carcinomas, adenoid cystic carcinomas Soft tissue tumors: rhabdomyosarcomas, fibrosarcomas Malignant lymphomas

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3.2. Tumors of the oropharynx

3.2.1. Benign tumors Benign tumors occur mainly in the oral cavity than in the oropharynx Squamous papillomas are most frequently reported, they may arise on the tonsil, tonsillar pillars or uvula; treatment consists of surgical removal in local anesthesia Lingual thyroid: mass of ectopic thyroid tissue located at the level of the base of tongue Minor salivary gland adenomas: soft palate

Figure 15. Papilloma of the uvula

3.2.2. Malignant tumors The great majority of oropharyngeal malignant tumors are squamous cell carcinomas (70%)38. Other malignancies include lymphomas, minor salivary gland tumors, sarcomas and metastatic lesions. Lymphomas occur most frequently in the tonsils, where they represent 16% of the tonsilar malignancies. They are almost
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always non-Hodgkin lymphomas and can occur anywhere at the level of the Waldeyers ring.
3.2.2.1 Tonsillar and lateral pharyngeal wall cancers In 85-90% of the cases we have squamous cell carcinomas. Non-Hodgkin lymphomas are on the second place (formerly known as lymphosarcomas and reticulosarcomas). 3.2.2.1.1. Squamous cell carcinomas Males over 50 years of age, smokers, heavy alcohol drinkers Enhanced expression of the human papilloma virus type 2, 11 and 16 has been reported39 Initial symptoms are often vague and nonspecific, leading to a delay in diagnosis: sore throat, globus sensations The first presenting symptom may be the presence of a lump in the neck In advanced disease: dysphagia, odynophagia with referred ear-pain, fetid breath, haemorrhages Neck palpation for evaluation of cervical metastatic adenopathies Careful palpation of the tonsillar region, the floor of the mouth and the tongue Oropharyngoscopy: the tonsillar tumor is usually ulcerative and infiltrating Biopsy is mandatory for the diagnosis! Pan-endoscopy: to eliminate the presence of a possible synchronous second primary malignancy40 Imaging: CT scans, MRI, to evaluate the extent of the disease Treatment: radiotherapy (in early stages), surgery followed by radiotherapy (in advanced disease) Prognosis: in early tonsillar cancer, the expected 5-year survival is 50-90%; in advanced cancer is 20-55%38 3.2.2.1.2. Non-Hodgkin lymphomas Early symptoms are similar The appearance of the tonsillar tumor is different: the entire tonsil is hypertrophied, bulky, grossly enlarged For histologic diagnosis, an adequate amount of tissue is taken for biopsy 199

Figure 16. Squamous cell carcinoma of the left palatine tonsil

Figure 17. Non-Hodgkin lymphoma of the left palatine tonsil

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 Treatment is non-surgical: chemo/radiotherapy protocols 5-year survival rates have been reported at 58-77% 41
3.2.2.2. Base of tongue cancers Squamous cell carcinoma is the most frequently encountered histopathological type. Other malignancies involved are non-Hodgkin lymphomas and malignant glandular tumors Considerable delay in diagnosis, because of the lack of specificity of initial symptoms: sore throat, foreign body sensations, lump in the neck In advanced disease: lump in the neck, dysphagia, odynophagia with referred ear-pain, fetid breath, haemorrhages Palpation of the neck: to evaluate the cervical metastatic adenopathies Careful palpation of the tongue base! Indirect mirror examination, pan-endoscopy, biopsy! Imaging: CT scans, MRI Treatment: surgical resection with or without reconstructive procedures, followed by postoperative radiotherapy; chemoradiotherapy protocols in advanced disease Prognosis: because most of the patients present with advanced disease, the overall 5-year survival for stages 3 and 4 is around 21% 42; in early stages the expected 5-year survival rate is 45-70% 38 3.2.2.3. Soft palate Squamous cell carcinomas (75-95%); other malignancies: minor salivary gland tumors Treatment: radiotherapy, chemotherapy, surgery The functional outcomes may be critically different, with significant hyponasality of speech and nasal food regurgitation which may be intolerable to patients 3.2.2.4. Posterior pharyngeal wall Very rare Disastrous prognosis Treatment: radiotherapy, chemotherapy

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3.3. Tumors of the hypopharynx

3.3.1. Aetiology. Pathology Nearly all tumors of the hypopharynx are malignant and virtually all are squamous cell carcinomas Lymphomas, undifferentiated carcinomas, adenocarcinomas, benign tumors were reported in less than 3.5% of the cases43 Piriform sinus cancer: more common in males, heavy smokers, heavy alcohol drinkers Postcricoid cancer: the only head and neck cancer site which is characterized by a higher incidence in women, classically associated with a history of the Patterson-Kelly-Brown syndrome (glossitis, sideropenic anaemia, dysphagia) 3.3.2. Symptoms Unilateral sore throat, odynophagia (pain on swallowing), referred ipsilateral otalgia, dysphagia, weight loss These tumors invade the adjacent larynx, so hoarseness appears during evolution A lump in the neck is the presenting symptom in about one in five patients with hypopharyngeal cancer 43 3.3.3. Diagnosis: Inspection and palpation: neck palpation will demonstrate metastatic lymph nodes in 75% patients with piriform sinus cancers and 20% patients with postcricoid cancer Indirect mirror examination (indirect hypopharyngolaryngoscopy): allows the evaluation of the larynx invasion, vocal cord mobility Pan-endoscopy (flexible or rigid): provides the most accurate examination of the hypopharynx, allows an appropriate evaluation of the tumors relationship with the larynx, cervical oesophagus and superiorly with the oropharynx Biopsy! Imaging: CT scans, MRI
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Figure 18. Squamous cell carcinoma of the left piriform sinus (surgical specimen)

3.3.4. Treatment. Prognosis Surgery (partial hypopharyngectomies in small tumors, partial hypopharyngectomy associated with total laryngectomy and neck dissection, total pharyngolaryngectomies associated with reconstructive procedures in advanced tumors) Post-operative radiotherapy, chemotherapy Prognosis: Patients with small lesions and no lymph node metastases have a median 5-year survival rate of 70%. Patients with advanced disease have a median survival of 20-30%44

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4. NEUROLOGICAL DISORDERS
OF THE PHARYNX

4.1. Sensory disorders

4.1.1. Anaesthesia/Hyperestesia Anaesthesia of the pharyngeal mucosa Hyperestesia of the pharyngeal mucosa is a common finding mainly in acute inflammatory conditions 4.1.2. Pharyngeal paresthesias Lump in the throat is a common complaint Middle-aged women with persistent unpleasant sensations in the throat The sensations are augmented by saliva swallowing, but do not interfere with the swallowing of food No other symptoms, no loss of weight Cancerophobia A very accurate local examination should be performed in order to rule out serious diseases (malignancies of the upper aerodigestive tract)! Gastroesophageal reflux disease (GERD) investigation Treatment: tranquilisers, anxiolytics, treatment of GERD 4.1.3. Glossopharyngeal neuralgia A very rare condition, similar to that of trigeminal neuralgia Presentation is with a stabbing pain related to the area of the palatine tonsil and base of tongue A careful ENT examination must be performed to rule out local structural causes (malignancies)
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 In some cases, an elongated stiloid process may be the cause of the symptoms (Eagles syndrome); resection of the process via a tonsillar fossa approach relieves the symptoms Treatment is similar to trigeminal neuralgia: carbamazepine, symptomatics Some authors described these neuralgias as a vascular compression syndrome and reported surgical management by intracranial microvascular decompression45

4.2. Motor disorders

Motor disorders of the pharynx usually present as swallowing problems. Almost 75% of oropharyngeal dysphagia has connection with an underlying neurological etiology46. It is extremely important to eliminate a structural cause, therefore an accurate ENT clinical examination and further imaging evaluation are required in all cases. A detailed history provides valuable informations for the diagnosis. Symptoms associated with neurological dysphagia include: drooling, nasal regurgitation, coughing/choking, throat clearing and wet voice 47.

4.2.1. Myogenic disorders Myotonia: is characterized by continued muscle contraction after cessation of voluntary effort Myasthenia gravis: acquired autoimmune disorder determined by the presence of IgG autoantibodies against acethylcoline receptors at the level of the neuromuscular junction. Pharyngo-laryngeal involvement is suggested by the presence of laryngeal weakness, dysphonia and dysphagia 4.2.2. Neurogenic disorders Stroke: probably the most common neurological disorder affecting the pharynx (after a stroke almost 50% of the patients suffer at least temporarily with aspiration and dysphagia)47 Parkinsons disease
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 Demyelinating disorders: multiple sclerosis, GuillainBarre syndrome Systemic infections: poliomyelitis, diphtheria, herpes zoster, rabies, botulism Peripheral nerve lesions: the lower cranial nerves may be affected by local disease involving the brainstem, skull base, neck or thorax (nasopharyngeal carcinoma, temporal bone tumours, metastases, jugular bulb thrombosis, glomus tumours, parotid tumors, lymph node metastases) (Table 2)
Syndrome Vernet Collett-Sicard Villaret Tapia Cranial nerves involved IX, X, XI IX, X, XI, XII IX, X, XI, XII, cervical sympathetic chain (Horners) X, XII Site of the lesions jugular foramen posterior laterocondylar space posterior retroparotid space retromastoid space

Table 2. Eponyms for extra-nevraxial cranial nerve palsies

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5. FOREIGN BODIES IN THE PHARYNX

In most of the cases, fish bones are involved. Fish bones lodge in the palatine tonsil, the lingual tonsil, or even in the piriform fossae. Other foreign bodies: chicken bones, toy parts, needles, pins, metal wires. In locating a foreign body, patients sensations are sometimes a valuable guide 1: If the patient points to one side of the throat, then the foreign body is, or has been there If the patient points to the midline, then the foreign body is probably below the level of the pharynx Diagnosis is based on an accurate history, oropharyngoscopy, indirect laryngoscopy, endoscopy. Sometimes we dont find any foreign body during pharyngeal examination, although the patient complains of its presence due to the local irritation determined by the passage of the foreign body to the oesophagus. Treatment is removal, using a special forceps. To aid the foreign body removal, topical anaesthesia is needed.

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6. REFERENCES
1. Colman BH. The Pharynx. In: Colman BH, ed. Hall & Colmans Diseases of the Nose, Throat and Ear, and Head and Neck. Churchill Livingstone 1992; 93-120. 2. Beasley N. Anatomy of the pharynx and oesophagus. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1942-1953. 3. Logemann JA. Mechanisms of normal and abnormal swallowing. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1437-1447. 4. Bonkowsky V, Gerdemann P. Oropharynx and hypopharynx. In: Bonkowsky V, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 425-442. 5. Chevretton EB. Causes of dysphagia. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 20252036. 6. Karkos PD, Wilson JA. Globus pharyngeus. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2037-2042. 7. Malcomson KJ. Globus Hystericus Vel Pharyngis. Journal of Laryngology and Otology 1968; 82: 219-230. 8. Moloy PJ, Charter R. The globus symptom. Archives of Otolaryngology 1982; 108: 740-744. 9. Thompson WG, Heaton KW. Heartburn and globus in healthy people. Canadian Medical Association Journal 1982; 126: 46-48. 10. Hill J, Stuart RC, Fung HK, Ng EKW, Cheung FM, Chung SCS. Gastroesophageal reflux, motility disorders and psychological profiles in the etiology of globus pharyngis. The Laryngoscope 1997; 107: 1373-1377. 11. Couch ME, Blaugrund J, Kunar D. History, physical examination and the preoperative evaluation. In: Cummings CW, Robbins TK, eds. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 3-24. 12. Macnamara M. Acute and chronic pharyngeal infection. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 1981-2024. 13. Del Mar C. Managing sore throat: a literature review. I: Making the diagnosis. Medical Journal of Australia 1992; 156: 572-575. 14. Arroll B. Antibiotics for upper respiratory tract infections: an overview of Cochrane reviews. Respiratory Medicine 2005; 99: 255-261. 15. Georgalas C, Kanagalingam J, Zainal A, Ahmed H, Singh A, Patel KS. The association between periodontal disease and peritonsillar infection: a prospective study. Otolaryngology and Head and Neck Surgery 2002; 126: 91-94.

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16. Schraff S, McGinn JD, Derkay CS. Peritonsillar abscess in children: a 10year review of diagnosis and management. International Journal of Pediatric Otorhinolaryngology 2001; 57: 213-218. 17. Papalia E, Rena O, Oliaro A, Cavallo A, Giobbe R, Casadio C. Descending necrotizing mediastinitis: surgical management. European Journal of Cardiothoracic Surgery 2001; 20: 739-742. 18. Sethi DS, Stanley RE. Deep neck abscesses - changing trends. Journal of Laryngology and Otology 1994; 108: 138-143. 19. Ragnaud JM, Morlat P, Gin H et al. Clinical, biological and developmental aspects of cytomegalovirus infection in immunocompetent patients. La Revue de Medicine Interne 1994; 15: 13-18. 20. Fierstein JT, Thawley SE. Otolaryngologic manifestations of acute leukemia and lymphoma. The Southern Medical Journal 1978; 71: 277-280. 21. Ganzer U, Arnold A. Diseases of the nasopharynx. In: Ganzer U, Arnold A, eds. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 319-329. 22. Bonkowsky V, Gerdemann P. Oral cavity. In: Bonkowsky V, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 405424. 23. Augenbraun MH. Treatment of syphilis 2001: non-pregnant adults. Clinical Infectious Disease 2002; 35: 5187-5190. 24. Windfuhr JP, Remmert S. Extranasopharyngeal angiofibromas: etiology, incidence and management. Acta Oto-Laryngologica 2004; 124: 880-889. 25. Gleeson M. Juvenile angiofibroma. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 24372444. 26. Hwang HC, Mills SE, Patterson K, Gown AM. Expression of androgen receptors in nasopharyngeal angiofibroma: an immunohistochemical study of 24 cases. Modern Pathology 1998; 11: 1122-1126. 27. Schiff M, Gonzalez AM, Ong M, Baird A. Juvenile nasopharyngeal angiofibromas contain an angiogenic growth factor: basic FGF. The Laryngoscope 1992; 102: 940-945. 28. Coutinho-Camillo CM, Brentani MM, Butugan O, Torloni H, Nagai MA. Relaxation of imprinting of IGFII gene in juvenile nasopharyngeal angiofibromas. Diagnostic Molecular Pathology 2003; 12: 57-62. 29. Ho JH. Epidemiology of nasopharyngeal carcinoma. Gann Monograph on Cancer Research 1976; 18: 49-61. 30. Ho JH. Incidence of nasopharyngeal carcinoma in Hong Kong. UICC Bulletin 1971; 9: 5-10. 31. Buell P. The effect of migration on the risk of nasopharyngeal cancer among Chinese. Cancer Research 1974; 34: 1189-1191. 32. Young LS, Clark D, Sixbey JV, Richinson AB. Epstein-Barr virus receptors on human pharyngeal epithelium. Lancet 1986; 1: 240-242. 33. Niedobitek G. Epstein-Barr virus infection in the pathogenesis of nasopharyngeal carcinoma. Molecular Pathology 2000; 53: 248-252. 34. Shanmugaratnam K, Sobin L. Histological typing of tumors of the upper respiratory tract and ear. WHO International Histological Classification of Tumors. 1991; 7-9, 32-33.

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35. Skinner DW, van Hasselt CA, Tsao SY. Nasopharyngeal carcinoma: a study of the modes presentation. Annals of Otology, Rhinology and Laryngology 1991; 100: 544-551. 36. Tam JS, Murray HGS. Nasopharyngeal carcinoma and Epstein-Barr virusassociated serological markers. Ear Nose and Throat Journal 1990; 69: 261266. 37. Woo JKS, van Hasselt CA. Nasopharyngeal carcinoma. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2445-2474. 38. Bradley PJ. Oropharyngeal tumours. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 25772597. 39. Hammarstedt L, Lindquist D, Dahlstrand H et al. Human papillomavirus as a risk factor for the increase in incidence of tonsillar cancer. Int J Cancer 2006; 119: 2620-2623. 40. Braakhuis BJ, Tabor MP, Leemans CR, van der Waal I, Snow GB, Brakenhoff RH. Second primary tumors and field cancerization in oral and oropharyngeal cancer: molecular techniques provide new insights and definitions. Head and Neck 2002; 24: 198-206. 41. Gassner HG, Sabri AN, Olsen KD. Oropharyngeal malignancy. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1717-1757. 42. Sessions DG, Lenox J, Spector GJ, Chao C, Chaudry OA. Analysis of treatment results for base of tongue cancer. The Laryngoscope 2003; 113: 1252-1261. 43. Jones AS. Tumours of the hypopharynx and oesophagus. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2633-2662. 44. Blomquist E. Head and neck tumours. In: Anniko M, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 671-685. 45. Resnick DK, Jannetta PJ, Bissonnette D, Jho HD, Lanzino G. Microvascular decompression for glossopharyngeal neuralgia. Neurosurgery 1995; 36: 6469. 46. Trate D, Parkman H, Fisher R. Dysphagia: Evaluation, diagnosis and treatment. Primary Care 1996; 23: 417-432. 47. Sanderson RJ, Tierney P, Adamson R. Neurological disease of the pharynx. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2074-2083.

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IV. LARYNX

1. LARYNX BASICS
1.1. Clinical anatomy
The larynx is part of the respiratory system and is located at the upper level of the aerodigestive tract. The laryngeal cavity extends from the level of the epiglottis tip to the inferior margin of the cricoid cartilage, from where it is continued with the trachea.

1.1.1. Skeletal framework The larynx consists of a cartilaginous framework bound together by ligaments and fibrous membranes, and moved by a number of muscles. It is attached superiorly to the U-shaped hyoid bone by the thyrohyoid membrane and ligaments and thyrohyoid muscles. The cartilages of the larynx are paired and unpaired.
1.1.1.1. Unpaired cartilages The epiglottis is a leaf-shaped fibroelastic cartilage which is attached superiorly to the base of tongue by the glossoepiglottic folds, and inferiorly to the inner aspect of the thyroid cartilage, immediately above the anterior insertion of the vocal cords. Laterally, its sides are connected to the arytenoids by the aryepiglottic folds

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Figure 1. Larynx - anterior view

1 - Epiglottis; 2 - Hyoid bone; 3 - Thyrohyoid membrane; 4 - Thyroid cartilage; 5 - Cricothyroid muscle; 6 - Cricothyroid membrane; 7 - Cricoid cartilage; 8 - Tracheal rings

The thyroid cartilage makes the necks midline prominence known as the Adams apple. It consists of two quadrilateral wings (or alae), joined together in the midline anteriorly. The thyroid notch, located at the junction of the alae on the superior margin, can be readily palpated The cricoid cartilage is located below the thyroid. It is a ring-shaped structure which articulates with the thyroid and the arytenoids by synovial joints. Its anterior part (cricoid arch) is connected to the inferior border of the thyroid cartilage by the cricothyroid membrane. At the level of this membrane, a coniotomy (cricothyroidotomy, inferior laryngotomy) is performed in cases of acute airway obstruction
1.1.1.2. Paired cartilages The arytenoid cartilages are located on the superior border of the posterior cricoid plate. They are pyramidshaped cartilages that can rotate and slide on the cricoid (crico-arytenoid joint) and thus play a very important role in the movement of the vocal cords. They have an anterior projection, known as the vocal process, and a posterolateral one, known as the muscular process. The

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vocal process gives insertion to the true vocal cords. The muscular process form the main attachment for those muscles activating the vocal cords in phonation and respiration The aryepiglottic folds connect the arytenoids with the epiglottis and forms the upper edge of the laryngeal inlet

1.1.2. The endolarynx The endolarynx is subdivided into the supraglottis, the glottis and the subglottis.
1.1.2.1. Supraglottis The clinical term supraglottis refers to that part of the endolarynx which lies above the horizontal plane of the true vocal cords. It consists of the laryngeal inlet, or aditus (the aperture between the larynx and the pharynx), the laryngeal ventricle of

Figure 3. Laryngeal levels Figure 2. Endolarynx - frontal view

1 - Epiglottis; 2 - Hyoid bone; 3 - False vocal cord (ventricular band); 4 - Laryngeal ventricle (Morgagni); 5 - True vocal cord; 6 - Vocal muscle; 7 - Cricoid cartilage; 8 Tracheal ring I - Supraglottis; II - Glottis; III - Subglottis 1 - Epiglottis; 2 - Pre-epiglottic space; 3 - Hyoid bone; 4 - Thyrohyoid membrane; 5 - Thyroid cartilage; 6 - Arytenoid cartilage; 7 - Cricothyroid membrane; 8 - Cricoid cartilage

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Morgagni (the space between the true and the false vocal cords), the false vocal cords (the ventricular bands), the laryngeal surface of the epiglottis, the arytenoids and the medial aspects of the aryepiglottic folds.
1.1.2.2. Glottis The glottis include the two paired true vocal cords, the anterior and the posterior comissure. Rima glottidis is the horizontal space between the left and the right vocal cord, triangular-shaped, which narrows during phonation (adduction) and widens during breathing (abduction). Rima glottidis is divided into two parts: the anterior two-thirds (the intermembranous part) which participates actively in the phonation, and the posterior one-third (the intercartilaginous part) between the vocal arytenoid processes. The true cords (mucosa, vocal ligament, vocal thyroarytenoid muscle), pearly white-coloured, are attached anteriorly in the midline to the posterior surface of the thyroid cartilage, and posteriorly to the vocal process of the arytenoids. Their superior aspect corresponds to the floor of the Morgagnis laryngeal ventricle. The potential space between the mucosa and the underlying vocal ligament is known as the Reinkes space. Accumulation of fluid within this space may lead to abnormal swelling of the true vocal cords (Reinkes edema). 1.1.2.3. Subglottis The subglottis extends from 1 cm below the free margin of the true vocal cords to the inferior border of the cricoid cartilage.

1.1.3. Muscles of the larynx The muscles of the larynx are divided into two main categories: the extrinsic and the intrinsic muscles. The extrinsic muscles connect the larynx to adjacent structures and are responsible for its vertical movements during phonation and swallowing (infrahyoid strap muscles and the inferior pharyngeal constrictor. The intrinsic muscles are responsible for altering the length, tension, shape and spatial position of the vocal cords. These
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muscles may be divided in three groups, according to their main actions1: Vocal cords adductors: thyroarytenoid, lateral cricoarytenoid and interarytenoid muscles Vocal cords abductor: posterior cricoarytenoid muscle Tensor of the vocal cord: cricothyroid muscle

1.1.4. Vascular supply and lymphatic drainage The blood supply is derived mainly from the superior laryngeal artery, branch of the superior thyroid pedicle (first collateral branch of the external carotid) and inferior laryngeal arteries, which come from the thyrocervical trunk, branch of the subclavian artery. The venous drainage passes to the internal jugular veins. The lymphatic drainage of the supraglottis and even the subglottis is much more abundant than the glottis, which has implications for the management of cancer of these sites. The incidence of metastatic lymph nodes is higher in supraglottic than in glottic cancer, thus making the prognosis worse. 1.1.5. Nerve supply The nerve supply of the larynx is of great practical importance and comprises the superior and inferior recurrent laryngeal nerves, both being branches of the vagus nerve. The superior laryngeal nerve takes origin from the inferior ganglion of the vagus nerve and divides into the internal and external laryngeal nerves, supplying sensitive innervation to the supraglottis and motor innervation to the cricothyroid muscle. The recurrent laryngeal nerve is also a branch of the vagus nerve, that has a different course on each side: On the left side it passes under the aortic arch and ascends through the mediastinum in the tracheo-oesophageal groove to reach the neck, entering the larynx underneath the inferior constrictor muscle On the right side it does not descend into the thorax The recurrent laryngeal nerve is motor to all muscles of the larynx, apart from the cricothyroid.

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Figure 4. Innervation of the larynx

1 - Hyoid bone; 2 - Internal carotid artery; 3 - External carotid artery; 4 - Right vagus nerve (X); 5 - Common carotid artery; 6 - Thyroid cartilage; 7 - Thyroid gland; 8 - Right recurrent laryngeal nerve; 9 - Trachea; 10 - Right subclavian artery; 11 - Aorta; 12 - Left recurrent laryngeal nerve; 13 - Left subclavian artery; 14 - Left vagus nerve (X); 15 - External branch of the superior laryngeal nerve; 16 - Internal branch of the superior laryngeal nerve

1.2. Phisiology
1.2.1. Protection of the airway The most important laryngeal function during deglutition is to prevent food and liquid entering the airway. This is performed by means of the sphincteric (closed valve) action of the aryepiglottic folds and the true and false vocal cords, which occurs simultaneously with laryngeals elevation under the base of tongue. Epiglottis also protects the airway by covering the laryngeal inlet. Rapid laryngeal elevation, which occurs during the second pharyngeal phase of deglutition, appears to be essential for normal swallowing2. Other important protective reflexes are the coughing reflex and the effort laryngeal closure to increase intrathoracic pressure (Valsalva manoeuvre). The last one is essential during different physiologic conditions: defecation, urination, weight lifting 1. 1.2.2. Respiration During normal breathing at rest, the glottis is open (abduction) and the air passes in and out without an active participation of the larynx in the process itself.
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During high-volume breathing, the glottis performs an active abduction and is also tensed by the contraction of the posterior cricoarytenoid and cricothyroid muscles. The rima glottidis is considerably widened, so the resistance to the airflow is decresed1.

1.2.3. Phonation The vocal folds, vibrated by the pulmonary air stream, are the source of the voice. Each vibratory cycle of the vocal folds consists of three phases: adduction, aerodynamic separation and recoil. Each cycle is the manifestation of a mucosal wave travelling from the inferior to the superior aspect of each vocal fold (the cover/body theory)2. The vocal folds have to be structurally and functionally symmetrical, at the same level and close rapidly in order that a clear vocal note can be initiated and maintained 3. Insufficient adduction (glottic insufficiency) leads to air wastage and a subsequent breathy voice.

1.3. Symptoms of laryngeal diseases

1.3.1. Dysphonia Dysphonia may be defined as any impairment of the voice or difficulty in speaking. Hoarseness is characterized by a perceived rough, harsh or breathy quality of the voice. A disordered voice may have one or more of these following features4: It is not audible, clear or stable It is not appropriate for the age and gender of the patient It fatigues easily It is associated with discomfort or pain during phonation 1.3.2. Upper airway obstruction The main symptoms of upper airway obstruction are: Dyspnea, or difficulty in breathing, is the cardinal symptom of upper airway obstruction. It is accompanied by inspiratory stridor, a high-pitched audible sound resulting
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from the turbulent air passage through a narrowed larynx or trachea. Expiratory stridor is heard in association with obstruction of the lower intrathoracic airway Hoarseness is the effect of abnormal vibration of the vocal folds Suprasternal retraction is secondary to the maximal respiratory effort performed during inspiration Restlessness may be the result of anxiety or hypoxia Other symptoms and signs may be associated: drooling, bleeding, subcutaneous emphysema

1.3.3. Other laryngeal symptoms Laryngeal pain Irritative cough

1.4. Clinical examination

1.4.1. Inspection and palpation Inspection may reveal important signs of acute upper airway obstruction: stridor, suprasternal and supraclavicular inspiratory retraction, associated neck masses. Palpation of the anatomical landmarks of the larynx is followed by the evaluation of its passive mobility, by moving it laterally, over the anterior vertebral bodies, producing the normal laryngeal crepitus. Palpation during swallowing evaluates the active mobility (elevation of laryngeal structures in deglutition). Finally, palpation of the entire neck must be performed, for an accurate assessment of the cervical lymph nodes and the thyroid gland lobes. 1.4.2. Laryngoscopy
1.4.2.1. Mirror examination (Indirect laryngoscopy) Mirror examination is an indirect laryngoscopy performed as an office examination. Since its presentation by Manuel Garcia in 1855, this procedure has changed little5 Instruments: Light source

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Figure 5. Instruments for indirect laryngoscopy

Head mirror Headlight Laryngeal mirrors Spirit lamp The examiner is in front of the seated patient, light beam is focused on the oral cavity. Previously, the laryngeal mirror is warmed with the spirit lamp, to avoid blurring. The temperature of the mirror is checked on the examiners back of the hand. The patient is asked to lean slightly forward at the waist and to open the mouth. The tip of the tongue is grasped by the examiner using a gauze, and the mirror is gently introduced through the oral cavity, with the back of the mirror used to elevate the uvula. An indirect image of the endolarynx, superior aspects of the Figure 6. Endolarynx - superior view pyriform fossae and 1 - Glossoepiglottic fold; 2 - Epiglottis; 3 - Vallecula; tongue base can be seen. 4 - Pharyngoepiglottic fold; 5 - Aryepiglottic fold; 6 The patient is asked to say Pyriform fossa (sinus); 7 - Arytenoid cartilage; 8 - True eeeeeee, followed by a vocal cord; 9 - False vocal cord (ventricular band) 219

deep breath, in order to evaluate the vocal folds mobility in phonation and respiration. Touching the posterior aspects of the pharynx with the mirror causes gagging reflexes, which may compromise the examination. Topical anaesthetics may be applied.
1.4.2.2. Endoscopic examination Indirect laryngoscopy using rigid or flexible endoscopes is nowadays the routine gold-standard method for an accurate visualisation of the endolarynx and the hypopharynx. Instruments: Light source Fiber optic cable Rigid Hopkins laryngeal endoscopes with 90 and 70 angled lenses Flexible fiber optic endoscopes (naso-pharyngolaryngoscopes) Topical anaesthetics and nasal decongestants Photo and video equipment, if available The technique of transorally rigid endoscopy is quite similar to that of mirror examination. Although is easier to perform, it still requires patient cooperation. Rigid endoscopy supplies the best quality images for documentation purposes.

Figure 7. Endoscopy of the larynx

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Flexible endoscopy offers the advantage of being feasible in non-compliant patients such as children, seriously ill, demented or psychiatric patients. Also, it can be used in patients with gagging reflexes, who are not capable to tolerate the rigid technique. After topical decongestants and anaesthetics are applied, the endoscope is introduced through the nasal cavity into the rhinopharynx and then is guided downwards, over the posterior pharyngeal wall, to the laryngeal inlet.
1.4.2.3. Direct laryngoscopy Direct laryngoscopy is not an office examination. This procedure is performed in the operating theater, with patient under general anesthesia in most cases. The main indications6 are: Precise location and diagnosis of laryngeal lesions Biopsy of laryngeal lesions Removal of mass lesions by standard microsurgical techniques or by the use of laser As an adjunct to other therapeutic procedures The patient is placed in supine position, with head extension and neck flexed, eyes covered and dental protection. The rigid scope is passed transorally, in order to visualize the tongue base

Figure 8. Direct laryngoscopy

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and the tip of the epiglottis. The epiglottis is then elevated and the endolarynx is properly visualized. Suspension of the laryngoscope allows the surgeon to use both hands during procedures. For an accurate assessment, microscopes or rigid Hopkins endoscopes may be additionally used.

1.4.3. Voice evaluation

1.4.3.1. Videolaryngostroboscopy Videolaryngostroboscopy is the main clinical tool in diagnosing the voice disorders; it may be used to assess the quality of vocal folds vibrations and to evaluate the treatment effectiveness. Stroboscopy gives additional information about the vibratory pattern of the vocal folds mucosa and improves the accuracy of diagnosis. It has led to changes in diagnosis in nearly 30 percent of the cases, when compared to examination with continuous light alone7. 1.4.3.2. Other methods The protocol recommended by the European Laryngological Society for functional assessment of voice disorders includes, besides the gold-standard stroboscopic examination, the following investigations8: Perceptual analysis of voice by an expert/trained listener Acoustic objective measurements Aerodynamic objective analysis of voice production: airflow, air pressure Subjective rating by patient Other adjuvant procedures: laryngeal electromyography, oesophageal and pharyngeal 24 hours pH monitoring.

1.4.4. Other investigations Imaging techniques: neck ultrasound, conventional radiography, CT scans, MRI, conventional swallows with barium or water-soluble contrast media Biopsy Bacteriological examinations
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2. INFLAMMATORY DISEASES
2.1. Acute laryngitis
2.1.1. Definition. Aetiology Acute laryngitis is a common inflammatory condition of the laryngeal mucosa. In most of the cases the aetiology is viral, being part of an upper respiratory tract infection. Bacterial superinfection may occur. 2.1.2. Symptoms Hoarseness, which may progress to aphonia Dry, irritative cough (the cough becomes productive when an infection of the lower airways is present) Throat discomfort, even pain, due to irritation by coughing Fever, malaise Symptoms of associated rhinitis and/or pharyngitis 2.1.3. Diagnosis History Local examination: congestion/edema of the entire laryngeal mucosa, the vocal cords loose their normal light-coloured appearance; mucopurulent secretions are signs of bacterial superinfection A complete ENT examination should be performed 2.1.4. Treatment Acute viral laryngitis is usually a self-limiting infection that resolves within one week Vocal rest, avoidance of irritants, adequate hydration Symptomatics: analgetics, antipyretics, antitussive agents/ expectorants 223

 Antibiotics should be reserved for bacterial superinfections, persistent laryngeal inflammation, or in specific patients who rely on their voice, professionally9

2.2. Acute edematous subglottic laryngitis

2.2.1. Definition. Aetiology This is a particular form of acute laryngitis, affecting young children aged 6 months to three years, in which the edema of the narrow subglottic region leads to early respiratory distress. The aetiology is viral10: myxoviruses, para-influenza viruses 1 and 3, rhinoviruses, adenoviruses, echoviruses. Bacterial involvement is rare. 2.2.2. Diagnosis Diagnosis is clinical and is based on: History: sometimes the initial context of febrile rhinopharyngitis is missing Dyspnea often occurs during night: inspiratory bradypnea Stridor Inspiratory retraction of the suprasternal and even supraclavicular soft tissues of the neck Barking cough Quite normal voice, normal swallowing Endoscopic examination, if performed, shows a normal epiglottis, inflamed vocal folds and edema of the subglottic region 2.2.3. Treatment Treatment should be started as soon as possible: Hospitalization is recommended Oxygen, steroids and nebulized epinephrine should be administered9 Wide-spectrum antibiotics to prevent bacterial superinfection Sometimes intubation or even tracheostomy are required
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2.3. Acute epiglottitis

2.3.1. Definition. Aetiology Epiglottitis, or supraglottitis, is an acute infection of the supraglottis that involves primarily the epiglottis. It affects both children (mainly between 3 and 6 years old) and adults. The main difference in children, compared to adults, is that acute epiglottitis progresses more rapidly to severe upper airway obstruction. Aetiology is bacterial, Haemophilus influenzae type B being involved in the majority of cases. Thats why a significant decrease in children incidence has been noticed since the introduction of vaccination against Haemophilus (Hib vaccine) in 1985, to prevent childhood meningitis11. 2.3.2. Diagnosis In children: a rapid evolution to acute upper airway obstruction is characteristic, accompanied by fever, drooling (inability to swallow own saliva because of severe pain), a muffled voice, anxiety In adults: an upper airway obstruction may not be present, main symptoms being odynophagia, dysphagia, drooling, fever Laryngeal examination (flexible nasendoscopy in children, mirror examination and/or rigid endoscopy in adults) reveals a cherry-red swollen epiglottis, like a thumb sign10 2.3.3. Treatment Hospitalization and airway monitoring Intravenous antibiotics (second and third generation cephalosporins, active against Haemophilus) Humidified oxygen, steroids, analgetics and antipyretics Intubation or tracheostomy may be required

2.4. Laryngeal diphtheria

Although considered an eradicated disease, after mass immunization was applied, isolated cases of diphtheria may still occur nowadays. 225

2.4.1. Definition. Aetiology The disease is caused by Corynebacterium diphtheriae (the Klebs-Loeffler bacillus), a facultative anaerobic gram-positive agent, which is spread by air-borne droplets. The usual site of infection is the tonsil and fauces, but it can also occur within the nasal cavities or spread into the larynx. The bacillus remains at the level of the pharynx, causing the local typical manifestations of the disease. In the meantime, it produces a strong neurotropic exotoxin, which is spread into the organism, causing serious systemic manifestations (cardiac and neurological complications), which may be fatal. 2.4.2. Diagnosis Symptoms: severe sore throat, malaise, fever, nasal discharge; extension of the pseudo-membranes within the larynx causes an acute upper airway obstruction Palpation of the neck: multiple cervical adenopathies Local examination: characteristic pearly, white-greyish, adherent pseudomembranes in the pharynx, nasal cavities and endolarynx Throat swab cultures 2.4.2. Treatment Early administration of antitoxin is mandatory, based only on clinical suspicion, without confirmation of the result of the throat culture! Antibiotics should be also administered: Penicillin G is required The patients need to be isolated and reported Acute airway obstruction requires intubation and/or tracheostomy

2.5. Acute laryngeal edema

2.5.1. Allergy The vasodilation and increase in capillary permeability caused by abrupt histamine release (type I anaphylactic reaction) causes an acute edematous inflammation of the laryngeal mucosa.
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The most frequently involved factors are10: Exposure to inhaled allergens Foodstuffs Medications: angiotensin conversion enzyme inhibitors, non-steroidal antiinflammatory drugs (NSAIDs), Penicillin Insect bites and stings

2.5.2. Angioneurotic edema (Quinckes edema) This condition corresponds to a sudden swelling of the face and neck, which may lead to severe upper airway obstruction. A hereditary form exists, caused by a genetic deficit of C1esterase inhibitor. 2.5.3. Management Oxygen, epinephrine, steroids and antihistamines Androgenic derivatives (Danazol) augment the levels of C1-esterase inhibitor, and may be considered as a prophylactic treatment in angioneurotic edema10 Sometimes intubation and/or tracheostomy are required

2.6. Chronic nonspecific laryngitis

2.6.1. Definition. Aetiology Chronic nonspecific laryngitis is defined as a chronic inflammation of laryngeal structures, most commonly the laryngeal mucosa. The most important aetiological factors involved are: Tobacco smoke Voice abuse Gastroesophageal reflux disease (GERD) Chronic suppurative infections of the upper respiratory tract Chronic exposure to irritating fumes Classically, distinction is made between the red types, where inflammatory phenomena predominate, and the white types, otherwise known as keratosis10.
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2.6.2. Symptoms Progressive hoarseness is the cardinal symptom Voice is usually worst in the morning (the mucus present in the larynx has dried during the night), becomes better after throat clearing, and deteriorates again as the day progresses Foreign body sensations, persistent cough, throat clearing 2.6.3. Diagnosis Local examination: mirror examination, flexible nasendoscopy, rigid endoscopy with or without stroboscopy The red types: mucosal erythema, mild diffuse edema and mucosal swelling, stasis of secretions, granulations The white types: leukoplakia (flat lesion, pearly greyishcoloured, single or multiple, slightly vague contours, localized on the vocal folds), white pachydermia (the lesion has a warty tumor-like appearance, clearly defined margins, irregular surface); in both forms the whitegreyish color is due to hyperkeratinization Particular forms: posterior laryngitis (red inflammatory lesions on the posterior aspects of the endolarynx; GERD plays an important role in aetiology), Chevalier Jacksons contact ulcers (granulative and ulcerative lesions on both arytenoids) If there is any suspicion with regard to malignancy (mainly in keratotic lesions), direct microlaryngoscopy with biopsy is mandatory12 Keratotic lesions are considered to be pre-malignant. Table 1 shows the most recognized classification of chronic laryngitis epithelial dysplasia (an alteration of cellular maturation) based only on histological criteria10
Grade Grade I Grade II Grade III

Hyperplasia and/or keratosis with or without mild dysplasia Moderate dysplasia Severe dysplasia, carcinoma in situ without infiltration of the basement membrane Table 1 Histological grading in chronic laryngitis

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2.6.4. Treatment
2.6.4.1. Conservative treatment: Smoking cessation, lifestyle modifications Empirical treatment with proton pump inhibitors for the GERD is advised13 Voice therapy 2.6.4.2. Surgical treatment: All degrees of epithelial hyperplastic/keratotic lesions should be treated by microlaryngoscopic removal and histopathological assessment of the specimen Regular follow-up is necessary (pre-malignant lesions)

2.7. Chronic specific laryngitis

2.7.1. Chronic infectious granulomatous diseases
2.7.1.1. Tuberculosis Laryngeal tuberculosis is almost always secondary to pulmonary tuberculosis. Symptoms are nonspecific: dysphonia, pain on speaking and swallowing, referred otalgia. Local examination reveals a diffusely erythematous and edematous larynx, predominantly involving the posterior onethird of the glottis. Granulation tissue is also present nearby the arytenoids. Infected sputum causes, later in evolution, ulcerative lesions mainly in the inter-arytenoid space, the arytenoids and ary-epiglottic folds. This clinical picture may resemble a malignant tumor! An accurate diagnosis requires a biopsy from the laryngeal lesions. Histopathological assessment reveals the typical granulomas with caseating necrotic center, Langhans giant cells and acid-fast bacilli. Treatment is primarily focused on the disease in the lung: rest and tuberculostatic drug regimens. Laryngeal lesions may heal leaving scars and sometimes cicatricial stenosis14.

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2.7.1.2. Syphilis Syphilis is an infectious disease caused by Treponema pallidum. The infection may present in various forms that have been classically described as primary, secondary, tertiary and congenital syphilis. Laryngeal involvement is rare, supraglottis is primarily affected, the most common form is gummatous laryngitis (tertiary syphilis), which mimics a laryngeal malignancy and needs biopsy for diagnosis! Additional serological testing must be performed. Treatment is with high-doses penicilin. 2.7.1.3. Leprosy Leprosy (Hansens disease) is a chronic granulomatous infection caused by Mycobacterium leprae. Laryngeal involvement is rare, supraglottis is primarily affected (edematous and nodular lesions with ulcerations), biopsy is mandatory for diagnosis to rule out malignancy and other granulomatous diseases. Treatment: multi-drug therapy regimens (see Nose, section 3.2.4.) 2.7.1.4. Rhinoscleroma Rhinoscleroma is primarily a chronic granulomatous infection of the nasal cavities. In evolution, lesions may progress downwards, with subsequent pharyngeal and laryngeal involvement (glottis and subglottis). The causative agent is the gram-negative bacillus Klebsiella rhinoscleromatis (Frisch bacillus) (see Nose, section 3.2.3.). Histopathological examination of the biopsy specimens reveals the typical Mikulicz cells (foamy vacuolated histiocytes) and Russell bodies. Bacteriological examinations are also required. Treatment is with aminoglicosides, cephalosporins or tetracycline15. 2.7.1.5. Candidiasis Laryngeal candidiasis (opportunistic infection caused by the oropharyngeal Candida albicans) may occur in immunocompromised patients (chemotherapy, AIDS), prolonged inhaled or systemic corticosteroid use, long-term wide-spectrum antibiotics, radiotherapy for head and neck cancers13.

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Local clinical appearance: diffuse erythema of the mucosa, which is covered by irregular, friable, superficial, white exudates. In immunocompromised hosts, lesions tend to be more severe and may extend into the oesophagus. Treatment is with nystatin, fluconazole. In severe forms, amphotericin B may be required16.
2.7.1.6. Other fungal granulomatous infections Histoplasmosis (Histoplasma capsulatum) Blastomycosis (Blastomyces dermatididis) 2.7.1.7. Laryngeal actinomycosis Actinomycosis is a chronic suppurative granulomatous infection, caused by the anaerobic bacteria Actinomycoses bovis and Actinomycoses Israeli. The larynx is affected following involvement of the necks soft tissues and adenopathies. Diagnosis is performed by identification of the typical sulphur granules in the biopsy specimen and by bacteriological examinations. Treatment requires penicillin or tetracycline17.

2.7.2. Laryngeal involvement in systemic diseases

2.7.2.1. Wegeners granulomatosis Wegeners granulomatosis is a systemic disease with involvement of the upper and lower respiratory tract and the kidneys, characterized by the presence of necrotizing noncaseating granulomas and vasculitis. Larynx is affected in up to 25% of the case (local inflammatory reactions, granulomatous ulcers located mainly in the subglottic region)13. Diagnosis requires a biopsy specimen and the specific anticytoplasmic autoantibody C-ANCA test. Treatment is with corticosteroids or cyclophosphamide. 2.7.2.2. Sarcoidosis Sarcoidosis is a systemic disease of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may affect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver. 231

Laryngeal involvement is reported in less than 5% of all cases, with clinical appearances quite similar to tuberculosis and fungal infections, lesions being located primarily in the supraglottis13. Diagnosis requires a biopsy, chest X-rays, CT Scans, Kveim intradermoreaction Treatment: securing a safe airway, systemic corticosteroids.
2.7.2.3. Amyloidosis Primary or secondary systemic amyloidosis is characterized by diffuse submucosal or small subepithelial masses (deposits of amyloid, with a high fluid content). In laryngeal involvement, patients main complaint is dysphonia, due to the presence of these submucosal deposits in the various subsites of the larynx, and their subsequent effect on the vocal folds mobility. Diagnosis is confirmed by biopsy (the affinity of the amyloid for Congo Red). Treatment requires microsurgical removal of the deposits. The use of CO2 laser is beneficial.

2.8. Pseudo-myxomatous laryngitis (Reinkes edema)

2.8.1. Definition. Aetiology Pseudo-myxomatous laryngitis (Reinkes edema, smokers polyps) is a chronic condition characterized by fluid accumulation in the subepithelial space of the vocal fold mucosa (Reinkes space). This pathological entity is the result of a chronic irritation of the mucosa due to heavy smoking and voice abuse. It is associated typically with middle-aged women, who have been long-term smokers. 2.8.2. Diagnosis Dysphonia is the main symptom (low-pitched voice, often in the masculine range when the condition is seen in female patients) A female patient may complain of being called sir on the phone, or may have problems with increasing hoarseness during the day18
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 Local examination: pale, watery bags of fluid attached to the superior aspect and margins of the vocal folds; an up-and-down motion of the polypoid edematous tissue is often noticed with respiration

2.8.3. Treatment Quit smoking! Microlaryngoscopic surgery procedures: the mucosa is incised parallel to the vocal ligament, fluid is suctioned and the redundant mucosa is carefully removed (GouldHirano technique)10 Speech therapy

2.9. Vocal fold polyp

2.9.1. Definition. Aetiology Vocal fold polyp is a benign unilateral lesion, located mainly at the level of the free margin of the vocal folds or the anterior comissure. It is more common in men, particularly men who engage in intermittent severe voice abuse, or who work in noisy environments. Sometimes, a history of aspirin or other anticoagulant use is noticed18. Polyps are the result of a violent voice abuse that leads to capillary ruptures in the submucosa, with subsequent localized haemorrhage. In evolution, the initial haemorrhagic polyp evolves to a fibrotic form19. 2.9.2. Diagnosis Dysphonia is usually with sudden, abrupt onset, coinciding with the extreme vocal effort In evolution, polyps may become large enough to determine an upper airway obstruction syndrome

Figure 9. Vocal cord polyp

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 Local examination: in early stages, a unilateral redbluish lesion is noticed at the level of the membranous part of the vocal folds; long-standing polyps loose their haemorrhagic appearance and become pedunculated, moving in-and-out of the glottis with respiration

2.9.3. Treatment Microsurgical removal of the lesion, without disruption of the subjacent vocal ligament; CO2 lasers may be used Surgery must be followed by speech therapy, to avoid recurrences

2.10. Vocal nodules

2.10.1. Definition. Aetiology Vocal nodules are bilateral chronic localized swellings of the vocal fold epithelium, usually placed at the junction of the anterior third with the middle third. It is one of the commonest laryngeal disorders determined by voice abuse and misuse. Children (usually boys), who scream and shout, and adults (mainly females) with professions demanding much of their voice (teachers, lawyers, singers, stock traders) are often afflicted19. 2.10.2. Diagnosis Permanent or intermittent dysphonia is the main symptom Local examination: vocal nodules are always bilateral, symmetrical, with a wide base; they appear in the middle of the membranous part of the vocal fold, which corresponds with the junction between the anterior 1/3 and posterior 2/3 of the glottis (the point of maximum vibration Figure 10. Vocal cord nodules during phonation)
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 Early nodules are usually edematous and translucent, whereas chronic lesions are white and fibrotic Stroboscopic examination

2.10.3. Treatment Conservative treatment: speech therapy should play a primary role, initially Surgical treatment: only in long-standing cases with large nodules, which do not respond to conservative measures (microsurgical removal)

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3. TUMORS OF THE LARYNX

3.1. Benign Tumors
3.1.1. Papillomas
3.1.1.1. Definition. Aetiology Squamous papillomas are the commonest benign laryngeal tumors. They are classified in two main histological categories20: Keratinized papillomas: solitary lesions which occur mainly in adults and arise at the level of the true vocal cords; the majority of these solitary adult lesions are not related to viral infection, but are connected to smoking and can develop further malignant transformation Non-keratinized papillomas (recurrent respiratory papillomatosis, RRP), most frequently encountered in children (4 per 100,000 population in children) are multiple benign tumoral lesions, caused by a primary infection with human papilloma viruses (HPV-6 and HPV-11 most commonly found in laryngeal papillomatosis) 3.1.1.2. Diagnosis Main presenting symptom in the adult form is hoarseness The juvenile form is more aggressive: besides hoarseness, progressive dyspnoea, stridor and even acute lifethreatening airway compromise may occur Local examination reveals a solitary exophytic lesion at the level of the true vocal folds (adult form) or multiple pink or white, sessile or exophytic, pedunculated or widebased lesions, resembling a cluster of grapes (juvenile form RRP) In the juvenile form lesions usually regress after puberty, occur mainly on the true and false vocal cords, anterior

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commissure, epiglottis, often extend in the subglottis, trachea and bronchi; they have a high propensity to recur after treatment
3.1.1.3. Treatment Surgical options for papillomas removal include cold microsurgical techniques, CO2 laser (mostly recommended), microdebridement and office-based angyolitic laser21 Adjuvant therapies: -Interferon, intralesional administration of antiviral agent Cidofovir22

3.1.2. Other benign laryngeal tumours Hemangiomas occur in children (10%) and adults (90%); in the paediatric type they are typically subglottic and may lead to severe upper airway obstruction21 Chondromas, leiomyomas, rhabdomyomas, neurofibromas, neurilemmomas or benign schwannomas, paragangliomas, benign minor salivary gland tumours

3.2. Malignant tumors

3.2.1. Squamous cell carcinoma (SCC)
3.2.1.1. Definition. Aetiology. Epidemiology Laryngeal cancer accounts for 3% of male cancers in Europe23. It represents also the most common head and neck cancer worldwide. The incidence is higher in men (6:1 sex ratio), although an increase in women incidence is noticed in the last decades. The peak age incidence is in the 5th and 6th decades of life. Over 70 large case-control studies have investigated the aetiology of laryngeal carcinoma. Tobacco and alcohol are so dominant in these studies that the assessment of risk due to other possible factors (dietary deficiencies, environmental exposures, HPV infection, GERD) is quite difficult24.

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3.2.1.2. Pathology SCC represents 95% of all laryngeal malignancies. It has characteristic patterns of spread, depending on the site of origin (supraglottis, glottis or subglottis). Tumors of the supraglottis tend to remain locally confined (even with pre-epiglottic space or nodal spread) to their subsite until relatively late. They may extend antero-superiorly to the pre-epiglottic space and the base of tongue, or laterally to the hypopharynx (medial aspects of the pyriform fossae). They involve vocal cords later in evolution, hoarseness is not the first symptom, they tend to be diagnosed in a later stage, also they occur in areas of rich lymphatic drainage, so many patients have palpable neck lymph nodes at the moment of diagnosis (60% in advanced disease). Tumors of the glottis are diagnosed earlier because progressive hoarseness is the first alarm symptom. The vocal cord has a poor lymphatic drainage, so these patients have a lower incidence of neck lymph node metastases (20% in advanced disease). Tumors of the subglottis tends to extend caudally and circumferentially, with early cricoid invasion, possible thyroid gland invasion. Upper airway obstruction develops rapidly in evolution. Clinically detectable lymph node metastases are surprisingly uncommon, paratracheal and mediastinal nodes may be involved. Transglottic cancer is defined by involvement of all three laryngeal subsites. True primary transglottic cancer originates from the laryngeal ventricle, and subsequently extends upwards and downwards through the paraglottic space24. 3.2.1.3. Symptoms In glottic cancer, progressive hoarseness is the earliest cardinal symptom. Any patient with hoarseness persisting for three weeks or more should be referred to an otolaryngologist for examination. Advanced disease may lead to upper airway obstruction, haemoptysis, weight loss. Neck nodes are rarely the presenting complaint. In supraglottic cancer early symptoms are nonspecific: foreign body sensations, paresthesias. First presentation with a neck mass (lymph node metastasis) is quite common. As tumour 238

bulk increases, voice alterations appear (muffled, hot-potato voice). Advanced disease may lead to dysphagia, odynophagia with referred otalgia (invasion of the pyriform fossae or tongue base), upper airway obstruction, haemoptysis, weight loss. In subglottic lesions early symptoms are also nonspecific: foreign body sensations, paresthesias. Upper airway obstruction develops rapidly in evolution, due to the presence of a bulky tumour within the narrowed spaces of the subglottic region. Any involvement of the glottis or recurrent laryngeal nerves leads to hoarseness.
3.2.1.4. Diagnosis Office examination: mirror examination, flexible nasendoscopy, rigid endoscopy, stroboscopy; tumor site/ size and vocal fold mobility must be carefully assessed Palpation of the neck: to evaluate the presence of lymph node metastases and the status of the thyroid gland Direct pharyngolaryngoscopy: allows for multiple accurate biopsies for histological examination, provides a precise evaluation of tumor location, size and superficial extent; the systematic examination of the entire upper aerodigestive tract (panendoscopy) allows the detection of synchronous Figure 11. Carcinoma of the left cancers23 hemilarynx Biopsy is mandatory for the diagnosis CT is currently the most useful modality to evaluate the tumor extension and the presence of neck lymph nodes metastases; it is recommended to perform this examination prior to endoscopy and biopsies, to avoid inflammation, which may lead to overestimation of cancer extent24 MRI, neck ultrasound examination

239

Figure 12. CT scan: tumor located at the level of the left hemilarynx

3.2.1.4. Treatment Two possible curative treatment modalities are available: surgery and radiotherapy. The therapeutic decision depends on the location of the tumor (supraglottis, glottis, subglottis), tumor extent and TNM staging, hysthology of the tumor. All recent guidelines and standards recommend that laryngeal cancer patients should be managed by a multidisciplinary team: a head and neck surgeon, a radiotherapist/oncologist, speech therapist and a dietician25. 3.2.1.4.1. Surgical treatment: Early-stage glottic and supraglottic carcinoma (T1/T2) may be treated by partial laryngectomies. The following partial procedures are indicated for surgical treatment of early glottic cancer: Endoscopic cordectomies, using cold microsurgical techniques or CO2 lasers26 Open approach cordectomies (laryngofissure/external thyrotomy) Frontolateral laryngectomies: when the vocal fold tumor involves the anterior commissure 240

 Anterior frontal laryngectomies: for tumors located in the anterior comissure For early supraglottic cancer, these partial procedures are recommended: Horizontal supraglottic laryngectomies Supracricoid partial laryngectomies with cricohyoidopexy, if the tumor extends downwards to the ventricle or true vocal folds, but without arytenoid involvement Near-total/Sub-total laryngectomies combine a supraglottic laryngectomy with a vertical hemilaryngectomy on the site of the tumor27 and may be used as a partial procedure in more advanced disease with vocal fold fixation (T3). In advanced disease (T3/T4), total laryngectomies are advocated. Partial and total laryngectomies are associated with the surgical treatment of the neck: selective, modified or radical neck dissections. Subglottic lesions may require a wide thyroid gland isthmectomy or even partial or total thyroidectomies.
3.2.1.4.2. Radiotherapy: Curative radiotherapy (conventional or hyperfractionated

Figure 13. Laryngeal carcinoma (surgical specimen)

241

techniques): in early glottic cancer, outcomes are similar with surgical treatment28 Postoperative radiotherapy is indicated when tumor extends beyond the larynx or there is metastatic neck lymph nodes involvement
3.2.1.4.3. Chemotherapy: The most frequently used drugs are cisplatin/carboplatin, 5-fluorouracil or, in palliation, methotrexate Chemotherapy is used mainly in larynx preservation protocols (induction therapy, concurrent chemo-radiotherapy, induction followed by concurrent chemoirradiation)29 New agents are under trials: taxanes and targeted therapies (in particular cetuximab)23 3.2.1.4.4. Voice rehabilitation after total laryngectomy: Air-swallowing techniques (erigmophonation) External devices (laryngophones) Tracheoesophageal voice prosthesis In 1998 Strome performed the first true successful laryngeal transplant, in Cleveland, USA30. Further research is required into how this technique might eventually be brought into routine clinical practice. 3.2.1.5. Prognosis 5-year survival rates23 for laryngeal cancer: T1-T2: 70% for supraglottis and 80% for glottis T3: 60% for supraglottis and 70% for glottis T4 resectable: 40% for supraglottis and 50% for glottis T4 non-resectable: below 30%

3.2.2. Other laryngeal malignancies Non-Hodgkin lymphomas are the second most common malignancy of the larynx Epithelial: verrucous carcinomas, adenocarcinomas, adenoid cystic carcinomas, mucoepidermoid carcinomas, malignant melanomas, carcinoids Mesenchymal: chondrosarcomas, leiomyosarcomas, rhabdomyosarcomas, fibrosarcomas, Kaposi sarcomas
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4. LARYNGEAL NERVE DISORDERS

4.1. Laryngeal nerve palsy
The larynx has three major functions: protection of the upper airway (acting like a sphincter to close the airway during deglutition, thus preventing aspiration of food and saliva), voice production and breathing. A functional larynx requires an adequate sensory and motor innervation of its anatomical structures. Both afferent and efferent innervations are supplied by branches of the vagus nerve (10th cranial nerve). The vagus nerve exits the skull base through the jugular foramen, and gives rise to some important branches: The pharyngeal nerve divides into multiple branches to form the pharyngeal plexus in order to supply motor and sensory innervation to the pharynx The superior laryngeal nerve takes origin from the inferior ganglion of the vagus nerve and divides into the internal and external laryngeal nerves, supplying sensitive innervation to the supraglottis and motor innervation to the cricothyroid muscle The recurrent laryngeal nerve supplies motor innervation to all intrinsic laryngeal muscles, except the cricothyroid muscle. On the right side, it arises at the level of the neck base, loops the subclavian artery and ascends to the larynx. On the left side it arises in the upper mediastinum, loops the aortic arch, has a short intrathoracic course and then ascends through the tracheoesophageal groove to the larynx Impairment of laryngeal nerve function is followed by a variety of phonatory, respiratory and swallowing disorders.

4.1.1. Aetiology Laryngeal nerves paralysis may be caused by lesions located at the level of the central nervous system (nucleus ambiguous and
243

its supranuclear tracts) or of the main trunk of the vagus nerve and its two laryngeal collateral branches. Surgical procedures in the neck, skull base and upper mediastinum are the main causes of peripheral lesions. Thyroid gland surgery is frequently followed by recurrent laryngeal paresis or palsy. Paresis after primary thyroid surgery for benign disease has been reported to range from 2-7%, permanent palsy from 0.5-4%; after revision surgery and surgery for malignant thyroid disease, laryngeal nerve lesions rates increase to 10-20%31. Malignant tumors of the larynx, hypopharynx, oesophagus, thyroid gland, trachea, upper left mediastinum, lymph node metastases may invade during evolution the vagus nerve or its collateral laryngeal branches. Viral neuronitis probably accounts for most cases of the idiopathic forms. Other conditions involved: Central nervous system lesions: neurovascular (stroke), tumors, degenerative disorders (e.g. amyotrophic lateral sclerosis) Lateral skull-base lesions: see Pharynx, chapter 4, table 2 Infectious diseases of the peripheral nerve system: poliomyelitis, diphtheria, herpes zoster, rabies, botulism Degenerative lesions of the peripheral nerve system: Guillain-Barres syndrome Primary neurogenic tumors: schwannomas, neurilemmomas Left aortic aneurysms

4.1.2. Symptoms Unilateral paresis/paralysis of the vagus or recurrent laryngeal nerve is characterized by impaired abduction/adduction of the vocal fold or immobility in a paramedian position. The constant glottic gap, determined by the inappropriate adduction during phonation, results in a typical hoarseness and breathiness of the voice. Bilateral recurrent laryngeal nerve lesions may present with an acute or a chronic insidious onset: The acute form (typically after thyroid gland surgery) results in a life-threatening upper airway obstruction, which requires emergency measures (intubation, tracheostomy) 244

 The chronic form is characterized by mild dyspnoea with inspiratory stridor; superimposed acute episodes of upper airway infections may lead to local inflammation and oedema with acute upper respiratory distress Voice quality is only mildly affected (vocal folds are paralyzed in adduction) In rare cases, when vocal folds are fixed in abduction, symptoms of chronic aspiration are the main complaints

4.1.3. Diagnosis The recommended European standard diagnostic steps31

are: Detailed history Inspection and palpation of the neck Indirect endoscopy with rigid telescopes or flexible nasendoscopes Thyroid gland assessment Ultrasound of the neck Additional diagnostic procedures include: Stroboscopy Direct pan-endoscopy Laryngeal electromyography Barium-swallow examination, pulmonary evaluation CT/MRI of the thorax, neck, skull base and brain Laboratory tests

4.1.4. Treatment
4.1.4.1. Conservative treatment: In unilateral vocal fold paresis/paralysis: initial voice rest, oral steroids or non-steroidal antiinflammatory drugs (NSAIDs), antiviral/antibiotics (if an infectious aetiology is suspected), speech therapy In chronic bilateral paralysis: acute episodes of upper airway infections must be promptly and adequately treated (antivirals/antibiotics, intravenous steroids, NSAIDs) to avoid the development of an acute upper airway obstruction syndrome

245

4.1.4.2. Surgical treatment: The recommended European standard for surgical procedures31: For voice improvement in unilateral paralysis: medial vocal fold augmentation using fat, collagen or disperse silicone; medialization thyreoplasty using autologous cartilage or laryngeal titanium implants For airway improvement in bilateral paralysis: temporary intubation/tracheostomy (in acute upper airway obstruction), posterior endoscopic cordectomy, temporary or permanent laterofixation of one vocal fold, endoscopic arytenoidectomy For deglutition disorders:laryngeal elevation,medialization thyroplasty to reduce the glottic gap, myotomy of the crycopharyngeal muscle (upper oesophageal sphincter)

246

5. TRACHEOSTOMY
5.1. Definition. History
Tracheotomy is the term used to describe the surgical opening of the trachea. Tracheostomy is used to describe the creation of a stoma at the skin surface which leads into the trachea. It may be temporary or permanent32. Tracheostomy is one of the oldest surgical procedures33. It was first depicted on Egyptian tablets around 3600 BC. It was described in the Rigveda, a Sanskrit text in 2000 BC. It is believed that a tracheotomy was performed by Asclepeiades of Bithynia, who lived in Rome around 100 BC. Antonio Brassavola (1490-1554) of Ferrara performed a tracheotomy to a patient with peritonsillar abscess,previously refused by barber surgeons. The patient made a complete recovery, and Brassavola published his account in 1546. It is considered the first recorded successful tracheostomy, despite the many ancient references. Sanctorius (1561-1636) was the first to use a trocar and cannula, left in place for 3 days. The currently used technique was described by Chevalier Jackson in 190934. He emphasized the importance of postoperative care, and warned of the complications after high tracheostomy (cricothyrotomy).

5.2. Indications
The main indications for tracheostomy35 are: Upper airway obstruction Prolonged intubation Facilitation of ventilation support Excessive bronchopulmonary secretions Inability to intubate Adjunct to major head and neck surgery procedures Adjunct to management of major head and neck trauma 247

5.3. Techniques
Cricothyroidotomy/minitracheostomy (coniotomy, inferior laryngotomy): in extreme life-threatening situations the upper airway is opened at the level of the cricothyroid membrane and the lumen is maintained by the insertion of a mini tracheostomy tube; patient should be referred in the next 24 hours to an ENT department to undergo a classic tracheostomy; if the coniotomy is maintained for a longer time, subsequent subglottic stenosis may occur Percutaneous tracheostomy: minimally invasive alternative to open tracheostomy; commercial kits based on welldefined techniques are available. The most commonly used is the dilatational technique36 Open surgical tracheostomy Open surgical tracheostomy should be performed, wherever possible, in the operating theater under local or general anesthesia. The patient is placed in the supine position on the table, with neck extension (a sandbag placed under the shoulders). A horizontal skin incision is performed two-fingers above the suprasternal notch. The superficial anterior jugular veins sometimes must be ligated. Strap muscles and cervical fascias are dissected on the midline, the thyroid gland isthmus is identified, divided and ligated. The anterior tracheal wall is completely exposed. Accurate hemostasis and a local intra-tracheal anesthetic injection must be performed before opening the trachea. The incision is done at the level of the second/third tracheal rings. An inferiorly based anterior tracheal wall flap may be designed and sutured to the skin to ease the change of the tracheostomy tube in the

Figur 14. Cuffed tracheostomy tube

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Figure 15. Tracheostomy

postoperative care. The tracheostomy tube, whose cuff integrity has been previously tested by the surgeon, is inserted through the stoma into the tracheal lumen.

5.4. Postoperative management

Humidification and removal of secretions by frequent suctioning Local toilet of the surgical wound; changing of the tube should be performed 3-4 days after the surgery, by the surgeon himself The cuff of the tube must be kept inflated for the shortest time possible, to avoid further damage of the tracheal mucosa and the development of subsequent cicatricial stenosis Antibiotics, analgesics, mucolytics

5.5. Complications
Complications following tracheostomy can be divided into these three categories6: 249

 Immediate (within 24 hours): haemorrhage (usually venous), air embolism, apnea, cardiac arrest, local damage of the cricoid cartilage, recurrent laryngeal nerve, oesophagus Intermediate (within 4 weeks): displacement of the tube, obstruction of the tube with secretions/crusts, surgical subcutaneous emphysema, pneumothorax/ pneumomediastinum, infection, necrosis, tracheooesophageal fistula Late: tracheal stenosis, decannulation problems

250

6. LARYNGEAL TRAUMA
6.1. Definition. Aetiology
Laryngeal traumas are characterized by heterogenous lesions secondary to blunt, perforating or endoluminal forces affecting the head and neck. Injuries to the larynx are relatively rare, less of 1% of all trauma involves the larynx. There is a risk of other concurrent lesions: intracranial (13%), cervical spine (8%) and oesophagus (3%)37. Laryngeal traumas can be classified in internal and external: Internal injuries are secondary to endotracheal anaesthesia procedures, endolaryngeal surgical procedures, ingestion/ inhalation of various toxic agents (caustics, acids, gases) External injuries are secondary to blunt or perforating forces, surgical procedures Laryngeal trauma may be secondary to accidents, aggressions, suicide or iatrogenic. Lesions are heterogenous in terms of their mechanics and consequences. Internal traumas may lead to soft tissue hematomas and edemas, lacerations, ruptures of the vocal folds, dislocations of the arytenoid cartilages. External blunt traumas may lead to endolaryngeal soft tissue lesions, fractures of the cartilaginous framework, ruptures of the laryngeal membranes and ligaments. External penetrating traumas are associated with lacerations of the larynx, trachea and soft tissues of the neck, laryngeal nerve injuries, bleeding of major vessels, aspiration, air embolism, tracheo-arterial and tracheo-oesophageal fistulas38. In evolution, besides the acute respiratory and circulatory complications, chronic sequelae must be carefully monitored. The major long-term, chronic complication is represented by the development of subsequent laryngotracheal stenosis. 251

6.2. Symptoms
Dyspnoea and stridor Hoarseness Hemoptysis and irritative coughing, aspiration External bleedings Pain, fear, shock Cardiopulmonary arrest

6.3. Diagnosis
The recommended European standard diagnostic steps38 include: Documented and accurate history (onset of symptoms, mechanism, previous surgery) Inspection: hematomas and ecchymosis, subcutaneous emphysema, penetrating lesions, bleeding, strangulation marks, cyanosis Palpation: crepitations Indirect endoscopy with rigid or flexible scopes Microlaryngoscopy and tracheobronchoscopy Additional diagnostic procedures: X-ray of the thorax and neck CT/MRI of the thorax and neck Pulmonary functional assessment Blood oxygen saturation monitoring

6.4. Treatment
6.4.1. Conservative treatment: Close clinical observation for up to 48 hours after injury Bed rest, voice rest, Humidified air, oxygen, hydration Steroids, antibiotics, analgesics 6.4.2. Surgical treatment: The recommended European standard surgical procedures38
are: Endolaryngeal microsurgery (repairment of soft tissue lesions, resolution of scars) 252

 Endoscopic surgery of the trachea Endo/extraluminal repositioning and fixation of fragments Additional procedures include: Temporary tracheostomy Laryngeal and tracheal stents Laryngeal and tracheal enlargement procedures

253

7. FOREIGN BODIES IN THE LARYNX

7.1. Definition. Aetiology
Lodging of a foreign body into the larynx causing hoarseness, croupy cough and acute airway obstruction. Most airway foreign bodies aspiration occurs in children younger than 15 years; children aged 1-3 years are the most susceptible39. Children are at risk for putting small toys, candies, or small vegetables (nuts, peanuts, bean, seeds) into their mouth. These objects may be propelled posteriorly, triggering a reflex inhalation. Among adults, some conditions may facilitate foreign body aspiration: impaired swallow and cough reflex, mental retardation, alcohol or sedative use, poor dentition, loss of consciousness. After aspiration, the foreign body can settle into 3 anatomic sites: the larynx, trachea or bronchus. 80-90% are lodged in the bronchi. The right main bronchus is most frequently encountered, because of its lesser angle of convergence compared with the left one39. Larger foreign bodies tend to locate within the larynx or trachea. Laryngeal foreign bodies occur less frequently than bronchial foreign bodies (8% of aspirations) and tend to fall into two categories based on size and shape40: sharp, thin and aerodynamic objects are at risk of embedding in the laryngeal mucosa or of becoming caught between the vocal folds soft, large, conforming objects may become lodged in the narrow, funnel shaped glottic or subglottic airway Acute upper airway obstruction may be due to the foreign body itself, or may be the result of the severe oedema and inflammatory reaction of the surrounding tissues41.

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7.2. Symptoms
Hoarseness, aphonia Barking, croupy cough Inspiratory stridor, cyanosis, acute airway obstruction Dysphagia, dyspnea, hemoptysis, wheezing, chronic cough In the caf coronary syndrome, a large foreign body (often, poorly chewed meat) lodges within the larynx or trachea, causing nearly complete upper airway obstruction. Respiratory distress, aphonia, cyanosis, loss of consciousness and sudden death occur in quick succession, unless the foreign body is dislodged42.

7.3. Diagnosis
A history of an acute choking episode, followed by a period of respiratory manifestations, is extremely important to be identified Imaging studies: neck X-Rays (lateral and AP views); foreign bodies are frequently radiolucent, making diagnosis with standard imaging difficult Endoscopy: diagnostic and therapeutic

7.4. Treatment
Heimlichs maneuver for acute upper airway obstruction (abdominal thrusts) Removal by direct laryngoscopy, bronchoscopy or surgery Respiratory support (intubation, tracheostomy) Abdominal thrusts, also known as the Heimlichs maneuver (after Henry Heimlich, who first described the procedure in 1974), involves a rescuer standing behind the patient and using their hands to exert pressure on the bottom of the diaphragm. This compresses the lungs and exerts pressure on any foreign body lodged within the larynx or trachea, hopefully expelling it43.

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8. REFERENCES
1. Piazza C, Ribeiro JC, Bernal-Sprekelsen M, Paiva A, Peretti G. Anatomy and phisiology of the larynx and hypopharynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 461-471. 2. Mathieson L, Carding P. Phisiology of the larynx. In: Hibbert J, ed. ScottBrowns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2155-2163. 3. Woo P, Colton R, Casper J, Brewer D. Diagnostic value of stroboscopic examination in hoarse patients. Journal of Voice 1991; 5: 231-238. 4. McGlashan J. Disorders of the voice. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 21922210. 5. Sittel C. Office examination of the larynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 473474. 6. Bradley PJ. Basic surgical procedures on the respiratory tract: laryngoscopy, tracheoscopy and including tracheostomy. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 527535. 7. Sataloff RT, Spiegel JR, Hawkshaw MJ. Strobovideolaryngoscopy: Results and clinical value. Annals of Otology, Rhinology and Laryngology 1991; 100: 725-727. 8. Dejonckere PH. Voice evaluation and respiratory function assessment. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 563-574. 9. Swift AC. Acute infections of the larynx. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 22482257. 10. Remacle M. Inflammatory diseases and lasers. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 475481. 11. Wurtele P. Acute epiglottitis in children and adults: a large-scale incidence study. Otolaryngology - Head and Neck Surgery: Official Journal of American Academy of Otolaryngology - Head and Neck Surgery 1990; 103: 902-908. 12. Cupic H, Kruslin B, Belicza M. Epithelial hyperplastic of the larynx in biopsy specimens. Acta Oto-Laryngologica Supplementum 1997; 527: 103104. 13. MacKenzie K. Chronic laryngitis. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 22582270. 14. Travis LW, Hybels RL, Newman MH. Tuberculosis of the larynx. The Laryngoscope 1976; 86: 549-558.

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15. Holinger PH, Gelman HK, Wolfe Jr CK. Rhinoscleroma of the lower respiratory tract. The Laryngoscope 1977; 87: 1-9. 16. Vrabec DP. Fungal infections of the larynx. Otolaryngologic Clinics of North America 1993; 26: 1091-1114. 17. Tsuji DH, Fukuda H, Kawasaki Y, Kavaida M, Ohira T. Actinomycosis of the larynx. Auris, Nasus, Larynx 1991; 18: 79-85. 18. Bastian RW. Benign vocal fold mucosal disorders. In: Flint PW, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 2150-2186. 19. Herranz J, Bouzas JG, Barro CV, Lourdes MM. Mucosal disease of the glottis. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 483-486. 20. Barnes I. Diseases of the larynx, hypopharynx and oesophagus. In: Barnes I, ed. Surgical pathology of the head and neck. New York: Dekker 2001; 151154. 21. Bradley PJ. Benign neoplasms and other tumours of the larynx. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 487-497. 22. Dikkers FG. Treatment of recurrent respiratory papillomatosis with microsurgery in combination with intralesional cidofovir - a prospective study. European Archives of Oto-Rhino-Laryngology 2006; 263: 440-443. 23. Chevalier D, Lefebvre J-L. Laryngeal cancer. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 499502. 24. Birchall M, Pope L. Tumours of the larynx. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 25982622. 25. British Association of Otorhinolaryngologists. Role of the multidisciplinary team. Effective head and neck cancer management. Third consensus document. 2003; 63-65. 26. Eckel HE, Schneider C, Jungehulsing M, Damm M, Schroder U, Vossing M. Potential role of transoral laser surgery for larynx carcinoma. Lasers in Surgery and Medicine 1998; 23: 79-86. 27. Biller HF, Lawson W. Partial laryngectomy for vocal cord cancer with marked limitation or fixation of the vocal cord. The Laryngoscope 1986; 96: 61-64. 28. Dey P, Arnold D, Wight R, MacKenzie K, Kelly C, Wilson J. Radiotherapy versus open surgery versus endolaryngeal surgery (with or without laser) for early laryngeal squamous cell cancer. Cochrane Database of Systematic Reviews 2002; CD002027: 29. Forastiere AA, Goepfert H, Maor M et al. Concurrent chemotherapy and radiotherapy for organ preservation in advanced laryngeal cancer. New England Journal of Medicine 2003; 349: 2091-2098. 30. Strome M, Stein J, Esclamado R, Hicks D, Lorenz RR, Braun W. Laryngeal transplantation and 40-months follow-up. New England Journal of Medicine 2001; 344: 1676-1679. 31. Eckel HE.Laryngeal nerve disorderds.In: Bradley PJ,ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 519-525.

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32. Pracy P. Tracheostomy. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2292-2304. 33. Pratt LW, Ferlito A, Rinaldo A. Tracheotomy: historical review. The Laryngoscope 2008; 118: 1597-1606. 34. Jackson C. Tracheostomy. The Laryngoscope 1909; 19: 285-290. 35. Goldenberg D, Bhatty N. Management of the impaired airway in adult. In: Flint PW, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 2441-2453. 36. Ciaglia P, Firsching R, Syniec C. Elective percutaneous dilatational tracheostomy. A new simple bedside procedure; preliminary report. Chest 1985; 87: 715-719. 37. Jewett BS, Shockley WW, Rutledge R. External laryngeal trauma analysis of 392 patients. Archives of Otolaryngology - Head & Neck Surgery 1999; 125: 877-880. 38. Kleinsasser N. Trauma to the laryngeal and tracheal. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 503506. 39. Bloom DC, Christenson TE, Manning SC et al. Plastic laryngeal foreign bodies in children: a diagnostic challenge. International Journal of Pediatric Otorhinolaryngology 2005; 69: 657-662. 40. Lima JA. Laryngeal foreign bodies in children: a persistent, life-threatening problem. The Laryngoscope 1989; 99: 415-420. 41. Brama I, Fearon B. Laryngeal foreign bodies in children. International Journal of Pediatric Otorhinolaryngology 1982; 4: 259-265. 42. Mittleman RE, Wetli CV. The fatal cafe coronary. Foreign body airway obstruction. JAMA 1982; 247: 1285-1288. 43. Heimlich HJ. A life-saving maneuver to prevent food-choking. JAMA 1975; 234: 398-401.

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V. SALIVARY GLAND BASICS

1. SALIVARY GLAND BASICS

1.1. Clinical anatomy
1.1.1. The parotid gland The parotids are the largest of the major salivary glands. They are compound, tubuloacinar, mepocrine and exocrine glands, composed entirely of serous output-producing acini1. Each gland is located in the parotid space, which lies between the posterior border of the ascending mandibular ramus and the anterior border of the mastoid process of the temporal bone. The superior border is closely related to the temporomandibular joint and the external auditory meatus. The inferior border reaches the anterior margin of the sternocleidomastoid muscle and the posterior belly of the digastric muscle, at the level of the angle of the mandible. The parotid is a unilobular gland through which the facial nerve passes. The intraparotid course of the facial nerve classically divides the gland into a superficial and a deep lobe. Anatomically, no true superficial and deep lobe exists. The term superficial parotidectomy refers only to the surgically created boundary from facial nerve dissection. The deep portion of the gland is located within the anterior prestyloid compartment of the parapharyngeal space. Thats why 259

tumors located at this level may present as intraoral masses, pushing the palatine tonsils medially and inferiorly. The Stensen duct drains the parotid gland. Each duct emerges from the anterior border of the gland and runs horizontally across the masseter muscle, 1 cm below the zygomatic arch. As it crosses the anterior masseter border, it penetrates the buccal fat pad and the buccinator muscle and opens intraorally at the level of the second molar teeth. The facial nerve exits the skull base via the stylomastoid foramen and enters the parotid gland. Within the gland, the nerve divides into two major branches: the upper temporofacial and lower cervicofacial divisions. This division point is known as pes anserinus. Subsequent branching is variable, but the nerve generally forms 5 branches: temporal and zygomatic (from the upper division), buccal, mandibular and cervical (from the lower division). Arterial supply is provided by branches of the external carotid artery. Venous drainage is provided by the posterior facial

Figure 1. The parotid gland

1 - Facial nerve (VII); 2 - Parotid gland; 3 - Marginal branch; 4 - Cervical branch; 5 - Posterior auricular vein; 6 - Retromandibular vein; 7 - External jugular vein; 8 - External carotid artery; 9 - Facial artery; 10 - Submandibular gland; 11 - Submandibular duct (Wharton); 12 - Buccal branch; 13 - Parotid duct (Stensen); 14 - Transverse facial artery; 15 - Zygomatic branch; 16 - Temporal branch; 17 - Superficial temporal artery; 18 - Auriculotemporal nerve

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vein to the internal jugular vein. The parotid is the only major salivary gland which contains lymph nodes, embedded within the glandular parenchymal tissue. The gland receives parasympathetic secretomotor innervation from the inferior salivatory nucleus. Preganglionic fibers synapse in the otic ganglion. The postganglionic fibers travel with the auriculotemporal nerve (branch of the trigeminal nerve) to the parotid tissues.

1.1.2. The submandibular gland The submandibular gland lies within the submandibular triangle or space. This triangle is delineated by the horizontal ramus of the mandible, the posterior and the anterior belly of the digastric muscle. The gland is partially enclosed between the two layers of the deep cervical fascia and is in close proximity to the facial vein and artery. The fascias superficial layer is attached to the inferior border of the mandible and covers the inferior surface of the gland. The deep layer is connected also to the mandible and covers the medial aspect of the gland. The inferior superficial surface of the gland is covered by skin, subcutaneous fat, platysma muscle and cervical fascia and is crossed by the facial vein. The mandibular branch of the facial nerve loops 1 cm below the horizontal ramus of the mandible to supply motor innervation to the lower lip. Besides the gland, the submandibular space hosts the submandibular lymph nodes. The deep lobe of each gland arises at the posterior free edge of the mylohyoid muscle and extends to the back of the sublingual gland. It is in close relationship superiorly with the lingual nerve. The Wharton duct opens into the floor of the oral cavity at the side of the lingual frenulum. The arterial blood supply comes from branches of facial and lingual arteries, the venous drainage is supplied by the deep lingual veins. The parasympathetic secretomotor innervation comes from the superior salivatory nucleus. Preganglionic fibers travel with the facial nerve, the chorda tympani nerve and the lingual nerve to the submandibular ganglion. Postganglionic fibers innervate both submandibular and sublingual glands.
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Figure 2. The submandibular gland

1 - Hypoglossal nerve (XII); 2 - Glossopharyngeal nerve (IX); 3 - Lingual artery; 4 - Hyoid bone; 5 - Hyoglossus muscle; 6 - Mylohyoid muscle; 7 - Deep lobe of the submandibular gland; 8 - Submandibular duct (Wharton); 9 - Stylohyoid muscle; 10 - Lingual nerve

1.1.3. The sublingual gland The sublingual gland lies over the mylohyoid muscle and is covered by the mucosa of the floor of the mouth. The lingual nerve and the Wharton duct lie medially to the sublingual gland. 1.1.4. The minor salivary glands The minor salivary glands are widely dispersed in the submucosa of the head and neck region. The highest density is found in the roof of the oral cavity and oropharynx. They are grouped according to their anatomic location: labial, buccal, palatal, tonsillar, nasal cavity, nasopharynx, larynx, etc.

1.2. Physiology
The basic unit of a salivary gland consists of an acinus, a secretory duct and a collecting, excretory duct. Acini are classified as serous (parotid gland), mucous (sublingual and minor salivary glands) and mixed (submandibular glands). Saliva is a complex mixture of organic (enzymes, immunoglobulins and mucines) and inorganic compounds (electrolytes)2. 262

The main functions of the saliva are3, 4: Lubrication and protection of the structures of the oral cavity and the oropharynx Buffering and clearance: saliva has a slightly alkaline pH and contains some buffer systems, the most important being the bicarbonate Maintenance of tooth integrity Antibacterial activity: saliva contains both immunologic mediators (IgA) and nonimmunologic compounds (various proteins, mucins, enzymes) Taste and digestion: saliva aids in the mastication and deglutition process through its lubricating actions; in addition, salivary amylase initiates the digestion of carbohydrates The secretion of saliva is tightly regulated by various factors, including the autonomous nervous system, humoral factors and disease states.

1.3. Disorders of salivary secretion

1.3.1. Hyposecretion Salivary glands hypofunction is often associated with a sensation of dry mouth (xerostomia). The main causes for hyposalivation5 are: Iatrogenic: medications (antidepressants, anxiolytics, diuretics, anti-hypertensive agents, antihistamines), radiotherapy, chemotherapy, surgical trauma Chronic inflammatory/autoimmune disorders: Sjogrens syndrome Infections: HIV/AIDS, mumps, EBV, tuberculosis Neurological disorders: depression, anxiety, Parkinsons disease Endocrine disorders: diabetes, hyper- and hypothyroidism Genetic abnormalities: cystic fibrosis Malnutrition, anorexia, bulimia, alcohol abuse Others: hypertension, burning mouth syndrome 1.3.2. Hypersecretion The overproduction of saliva is referred to as hypersalivation, sialorrhoea, ptyalism. The symptom is uncommon, as any
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excessive saliva is usually swallowed. Causes are usually local, due to increased reflexes (pain, infections, dental procedures, new dentures) or systemic (nausea, gastroesophageal reflux disease)

1.4. Clinical examination

1.4.1. History Symptoms related to salivary gland diseases are quite limited in number and often nonspecific. The main complaints are local swellings, pain, xerostomia, foul taste, sometimes sialorrhoea. The most common presenting symptom of benign or malignant disease of the major salivary glands is an asymptomatic chronic swelling. A major salivary gland swelling may be secondary to a duct obstruction, inflammation or neoplasia. Intermittent episodes of acute painful swelling, related to salivary stimulation during meals, are typical for duct obstruction with calculi. Pain is reported in 2.5-4% of patients with benign parotid neoplasms and 10-29% of patients with parotid malignancies. The same symptom is associated with malignant submandibular neoplasms in up to 50% of cases6. A detailed history related to the presence of concurrent systemic diseases, drugs being taken, previous radiotherapy/ chemotherapy, dietary and nutrition must be taken into account. 1.4.2. Physical examination Inspection: symmetry, skin color, possible pulsations, unilateral or bilateral swellings, signs of facial nerve palsy External palpation of the parotid and submandibular region: assessment of major salivary gland masses (location, size, mobility, fixation to adjacent structures), the presence of enlarged lymph nodes Combined intraoral and external palpation: may reveal the presence of a calculus in the draining duct Examination of the oral cavity: dental hygiene, inspection of the draining orifices of the Stensen and Wharton ducts, inspection and palpation of the tonsillar region (tumors located in the deep lobe of the parotid may present as
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oropharyngeal swellings, displacing the palatine tonsil medially and inferiorly) A complete ENT examination should be performed: evaluation of minor salivary glands tissues

1.4.3. Other investigations

1.4.3.1. Imaging techniques Plain radiographs: may detect a radio opaque calculus Sialography: lithiasis is the most common indication; contrast media is injected through the intraoral draining orifice of the gland, after a previous topical anaesthesia and a gentle dilation have been performed Ultrasound examination Nuclear medicine: technetium-99m pertechnetate scans are useful in the assessment of Whartin tumors and oncocytomas, showing high radionuclide uptake; all other tumors are generally radionegative6 CT scans and MRI

Figure 3. Ultrasound examination (Whartin tumor in the parotid gland)

1.4.3.2. Sialoendoscopy Sialendoscopy is a modern minimally invasive procedure, which allows direct inspection of the efferent salivary duct system, by means of a narrow-diameter microendoscope. This new technique is performed under local anesthesia on an outpatient basis. A lacrimal probe is used to gently dilate the intraoral orifice, then the scope is introduced under direct visualisation7.

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Sialendoscopy has opened up a new frontier for both diagnostic evaluation and treatment, mainly in obstructive salivary gland disease (lithiasis and stenosis of the glandular ducts)8.
1.4.3.3. Fine-needle aspiration (FNA) cytology FNA provides valuable information about the histopathology of a salivary gland tumor, before surgical treatment. It also helps to differentiate a reactive lymph node adjacent to a salivary gland or within the parenchyma of the gland (parotid gland), from a glandular tumor. It supplies accurate data in nearly 90% of the cases6.

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2. INFLAMMATORY DISORDERS
2.1. Infectious inflammatory disorders
2.1.1. Acute suppurative sialadenitis
2.1.1.1. Definition. Aetiology Acute suppurative sialadenitis is an acute bacterial inflammation of the salivary gland parenchyma, the parotid gland being most commonly involved. This condition is secondary to retrograde bacterial contamination of the salivary ducts from the oral cavity. It is strongly associated with medically debilitated and post surgical patients9. Mechanical impairment of the salivary flow is another factor involved in the development of acute infections. The most commonly isolated germs are penicillin-resistant Staphylococcus aureus, but Streptococcus species, Haemophilus influenzae, gram-negative and anaerobic bacteria are also involved10. 2.1.1.2. Diagnosis Rapid onset of local pain and swelling, fever, chills, malaise Signs of systemic dehydration, with dry mucous membranes (mainly in post surgical patients), signs of local inflammation (rubor, calor, dolor) Bimanual palpation of the gland elicits purulent discharge from the ductal orifice Cultures from the suppurative drainage should be obtained Ultrasound examination, CT scans 2.1.1.3. Treatment Wide-spectrum antibiotic therapy, according to the cultures results

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 Analgetics, antipyretics, non-steroidal antiinflammatory drugs (NSAIDs), hydration Rarely, conservative measures fail to eradicate the infection, and surgical drainage of localized, collected abscesses must be performed

2.1.2. Chronic sialadenitis

2.1.2.1. Definition. Aetiology Chronic sialadenitis is a localized disease of the salivary glands, characterized by repeated episodes of pain and inflammation. The parotid is the commonest site. Salivary stasis following obstructing disease (sialolithiasis, stenosis and strictures of the ducts, extrinsic compression) predisposes to recurrent episodes of infection and inflammation. This leads to subsequent acinar destruction, fibrous replacement and sialectasis10. 2.1.2.2. Diagnosis History of recurrent swelling and tenderness of the glandular tissue Palpation reveals enlargement of the gland Ultrasound examination Sialendoscopy 2.1.2.3. Treatment No specific successful treatment exists Non-steroidal antiinflammatory drugs (NSAIDs), sialogogues (pilocarpine) Sialendoscopy for obstructive problems Surgical removal of the gland, when conservative measures failed, is proved to be safe and effective11

2.1.3. Recurrent parotitis of childhood (RPC)

2.1.3.1. Definition. Aetiology Recurrent parotitis of childhood (RPC) is the most common non-viral infectious condition of salivary glands in children. It usually resolves around puberty12. The aetiology is unclear, no specific treatment exists. Some authors advocated the role of congenital ductal ectasia in the 268

development of the disease13. Staphylococcus aureus and Streptococcus viridans are the most frequently cultured agents10.
2.1.3.2. Diagnosis Recurrent episodes of acute or subacute, unilateral or bilateral parotid swellings, associated with fever and pain Palpation: mucopurulent discharge may be expressed from the erythematous ductal orifices Ultrasound examination Sialendoscopy 2.1.3.3. Treatment Adequate hydration, gland massage, sialogogues (pilocarpine) Antibiotics according to culture results, non-steroidal antiinflammatory drugs (NSAIDs)

2.1.4. Mumps
2.1.4.1. Definition. Aetiology Mumps is an acute nonsuppurative viral parotitis caused by the paramyxovirus, a RNA virus related to influenza and parainfluenza viruses. It is the most common viral infection of the salivary glands, 85% of the cases occur in children below the age of 1510. The disease is highly contagious, spreading by airborne droplets from saliva and nasopharyngeal secretions. 2.1.4.2. Diagnosis History: a viral prodromal period of 1-3 days characterized by malaise, anorexia, headaches, low-grade fever Painful swelling of the parotid gland, associated with otalgia and trismus; pain is augmented by eating or chewing In 75% of the cases, there will be bilateral involvement causing lateral displacement of the pinna14; commonly, the parotid on one side will enlarge first, followed by the opposite gland in 1-5 days The ductal orifice may be oedematous and congested, but no pus is coming out from the duct Laboratory tests: viral serology, leukopenia 269

The most frequent complications are: orchitis (25% in young males), pancreatitis, sensorineural hearing loss (1/20,000 in children, being the first cause of acquired sensorineural hearing loss in children) and meningoencephalitis12.
2.1.4.3. Treatment Bed rest, oral hygiene, adequate hydration, dietary modifications to decrease secretory activity Analgetics, sialogogues (pilocarpine)

2.1.5. HIV HIV-associated salivary gland disease (HIV-SGD) is a term used to describe salivary glands involvement in AIDS. It is characterized by a diffuse swelling of the salivary glands. These diffuse enlargements are secondary to various pathological entities: Kaposis sarcomas, lymphomas, lymphoproliferative and cystic enlargements accompanied by salivary dysfunction (xerostomia)10. The parotid is most commonly involved. As the parotid parenchyma contains lymph nodes, they also may be enlarged. 2.1.6. Granulomatous infections
2.1.6.1. Tuberculosis Mycobacterium tuberculosis and atypical mycobacteria may affect lymph nodes adjacent to the salivary glands or intraparotid lymph nodes Positive PPD skin test, FNA cytology Chest X-ray, ultrasound, CT scans Treatment: specific tuberculostatic chemotherapy regimens 2.1.6.2. Actinomycosis Cervicofacial actinomycosis is a chronic granulomatous infection caused by Actinomyces israelii, a gram-positive, anaerobic bacillus Painless, indurated enlargement, mimicking a neoplasm, associated with chronic purulent drainage (sulfur granules); development of multiple cutaneous draining fistulas is typical15

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 Anaerobic cultures, FNA cytology, ultrasound, CT scans Treatment: long-term high-doses antibiotic treatment (penicillin, clindamycin); surgical drainage of the collected pus
2.1.6.3. Cat-scratch disease (CSD) CSD is a granulomatous infectious lymphadenitis, caused by Bartonella henselae, a gram-negative bacillus; most commonly is the result of cutaneous inoculation through a scratch trauma from a domestic cat10 Typical history is of a papule or pustule at the scratch site, followed in one or two weeks by the development of enlarged lymph nodes in the regions adjacent to the inoculation site Diagnosis is based on serologic testing and a specific polymerase chain reaction (PCR) In most cases, no active therapy is required; the disease is self-limited and usually will resolve spontaneously within few months Although generally prescribed, antibiotics do not shorten the evolution of disease12 2.1.6.4. Toxoplasmosis Toxoplasmosis is caused by the organism Toxoplasma gondii, its usual host being the domestic cat10 A localized, lymphadenopathic and a systemic, disseminated form are classically described Diagnosis: lymph node biopsy, serologic testing Treatment: chemotherapy regimens

2.2. Noninfectious inflammatory disorders

2.2.1. Sjogrens syndrome (SS)
2.2.1.1. Definition. Aetiology SS is a chronic autoimmune disease of the exocrine glands. The most commonly involved sites are the salivary and lacrimal glands. The disease is characterized by a diffuse lymphocytic infiltration, with subsequent glandular hypofunction, leading to

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dryness of the mouth and eyes. The disorder has a quite broad clinical presentation, ranging from glandular disease to systemic extraglandular involvement and to the development of lymphoid malignancies16. SS is classified as primary (previously healthy person) or secondary, in association with other autoimmune disorders (rheumatoid arthritis, systemic lupus erythematosus)
2.2.1.2. Diagnosis Dryness of the mouth (xerostomia), difficulties in chewing and swallowing food, voice problems, dryness of the nose Dryness of the eyes (xerophtalmia), foreign body sensations, keratoconjunctivitis sicca Local examination: dry mucosal surfaces, multiple dental caries, smooth tongue with fissures, Candida albicans infections, cheilitis Salivary gland enlargement, mainly in the parotid gland, is reported in 10-20% of the cases17 Systemic involvement: malaise, low-grade fever, myalgias, arthralgias, vasculitis, pulmonary, hepatic, renal involvement Assessment of salivary flow rate: sialochemistry Assessment of tear secretion rate: Schirmers test Minor salivary gland biopsy remains a highly specific test for diagnosis of SS, although it is an invasive technique17 Assessment of immunological markers 2.2.1.3. Treatment Is mainly symptomatic Saliva substitutes, chewing sugarless gums or candies, treatment and prevention of dental caries and fungal infections, sialogogues (pilocarpine) Commercially available eye lubricants The use of systemic steroids or cytotoxic drugs is reserved for patients with severe systemic extraglandular involvement16

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2.2.2. Sarcoidosis Sarcoidosis is a systemic disease of unknown aetiology, characterized by the presence of non-caseating epitheloid granulomas. It may affect any part of the body, but most frequently involves the lymph nodes, the skin, the lungs, the eyes and the liver. Salivary glands may also be involved, specifically the parotids. Symptoms include swellings and xerostomia. Diagnosis requires a biopsy, Kweim intradermoreaction test, pulmonary evaluation (chest X-rays, CT scans). Treatment: systemic corticosteroids. Heerfordts syndrome or uveoparotid fever (parotid swelling, uveitis and peripheral facial nerve palsy) is a rare form of sarcoidosis, occurring in young patients12.

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3. OBSTRUCTIVE DISORDERS
3.1. Sialolithiasis
3.1.1. Definition. Aetiology Sialolithiasis is the formation of stones (calculi) at the level of the salivary glands ductal system. Submandibular gland is most commonly affected in 60% of the cases, followed by the parotid gland in 40%12. Calculi are composed mainly of calcium phosphate and carbonate, in combination with an organic matrix of glycoproteins and mucopolysaccharides18. No connection have been proven between calculi formation and blood levels of calcium or phosphate19. The exact pathogenesis remains uncertain. Salivary stasis and ductal inflammation and injury contribute to calculi formation. Several factors might account for the higher incidence of calculi formation in the Whartons duct: this duct is longer than Stensens, has a larger diameter, is angulated against gravity as it crosses the mylohyoid muscle and submandibular saliva is more viscous and has a higher concentration of calcium20.
3.1.2. Diagnosis Recurrent episodes of postprandial salivary colics (painful swellings related to meals), which can remain transitory or be complicated by bacterial infection Bimanual palpation may reveal a palpable stone, mainly in submandibular lithiasis Plain radiographs, conventional and digital sialography, ultrasound, CT, MRI Sialendoscopy

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3.1.3. Treatment
3.1.3.1. Conservative treatment: Local heat, adequate hydration, massage of the involved gland Sialogogues (pilocarpine) Antibiotics and non-steroidal antiinflammatory drugs (NSAIDs), if salivary gland infection is suspected 3.1.3.2. Surgical treatment: Palpable stones in the Whartons duct, located no more than 2 cm from the orifice, can be removed using an intraoral approach, in local anaesthesia10 Submandibulectomy/parotidectomy Modern approaches: interventional sialendoscopy, external lithotripsy12

3.2. Other obstructive disorders

Strictures, stenosis: cause the same symptoms as salivary stones Tumors: intraductal tumors are very rare; compressions from adjacent extraductal tumors

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4. SALIVARY GLANDS TUMORS

4.1. Benign tumors
4.1.1. Definition. Aetiology. Epidemiology The incidence of salivary glands tumors has been evaluated at 3-4/100,000 population, nearly 75% of these are benign tumors. Most of all develop in the parotid gland, and the vast majority are pleomorphic adenomas. There is a slight female preponderance. All age groups are involved, with a higher incidence in the fourth decade of life21. 4.1.2. Symptoms and signs Insidious onset, development of a slow-growing, painless mass, with well-defined margins, in the glandular parenchyma Superficial parotid tumors: painless, firm swelling in the preauricular or retromandibular region, mobile over superficial and deep adjacent planes Deep lobe parotid tumors: are not accessible to external palpation; they determine a medial displacement of the lateral oropharyngeal wall (oropharyngoscopy) Submandibular tumors: slow-growing, painless, mobile, firm swellings within the submandibular triangle Sublingual tumors: submucosal mass in the anterior floor of the mouth, lateral to the lingual frenulum Minor salivary glands tumors: firm, slow-growing submucosal masses, most commonly located at the level of the palate; ulcerations of the mucosa are rarely seen Some benign tumors (pleomorphic adenomas) have the potential to transform into a malignant lesion, during evolution. Symptoms and signs suggesting the development of a malignancy are:
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Figure 4 - Left parotid gland tumor

Rapid growth, sudden changes in mass size Local pain Loss of mobility Facial nerve palsy Skin invasion Metastatic neck lymph nodes

4.1.3. Diagnosis History, inspection, palpation, complete ENT examination Ultrasound, CT scans, MRI FNA cytology: allows the preoperative differentiation between a benign and a malignant lesion with 80-90% accuracy22 Frozen intraoperative sections 4.1.4. Histopathology
4.1.4.1. Pleomorphic adenomas: 80% of pleomorphic adenomas are found in the parotid gland, 11% in the submandibular gland and a similar proportion in minor salivary glands (mainly in the palate and lips)21.

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On histologic examination, the tumor consists of an epithelial component, embedded in a fibrocollagenous, myxochondroid or chondroid background.The tumor has a thin capsule, and in one forth of the cases, satellite tumoral nodules and pseudopodia (extensions of tumoral tissue through the incomplete capsule, within the healthy adjacent parenchyma) have been reported23. This is the reason why the recommended surgical treatment is excision with a surrounding cuff of normal tissue, to avoid local recurrences24. The risk of malignant transformation is reported to vary between 1.5% (in the first 5 years of evolution) and 10 % (after 15 years of evolution)25.
4.1.4.2. Whartins tumor: It is the second most common benign salivary gland tumor; they account for nearly 10% of parotid tumors. It develops almost exclusively at the level of the parotid region (most commonly at the inferior pole of the superficial lobe), it has a slightly male predominance, bilateral lesions occur in 10% of the cases25. They are usually ovoid encapsulated masses; papillary cysts are found on sectioning, filled with mucoid brown fluid. It is suggested that these tumors are the result of a neoplastic proliferation of ectopic salivary duct gland ducts, within intraparotid lymph nodes. The absence of lymphoid tissue from other salivary glands parenchyma explains why these tumors develop only at the level of the parotid glands21. 4.1.4.3. Other benign tumors: Oncocytoma Myoepithelioma

4.1.5. Treatment The recommended European standard for surgical treatment26 is:
4.1.5.1. Parotid gland: Lesion limited to the superficial lobe: superficial parotidectomy, with facial nerve dissection and preservation

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Figure 5 - Facial nerve through the parotid gland

Lesion within the deep lobe: total conservative parotidectomy, with facial nerve dissection and preservation
4.1.5.2. Submandibular gland and sublingual gland: Submandibular gland excision Sublingual gland excision

Figure 6 - Submandibular gland tumor (surgical specimen)

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4.1.5.3. Minor salivary glands: Wide excision of the lesion according to the location site All patients must be previously informed about the main complications of this surgery: Parotid gland surgery: facial nerve paresis/palsy, Freys syndrome (gustatory sweating or flushing), salivary cutaneous fistulas Submandibular gland surgery: damage to the mandibular branch of the facial nerve, damage to the lingual or hypoglossal nerve

4.2. Malignant tumors

4.2.1. Definition. Aetiology. Epidemiology Salivary glands malignancies account for 0.3-0.9% of all cancers and 1-3% of all head and neck cancers. The mean age is 55-60 years. Nearly 80% of these neoplasms develop in the parotid gland, 10% in the submandibular gland and 10% in the sublingual and minor salivary glands27. There is some evidence that environmental factors such as radiation exposure, diet (polyunsaturated fatty acids seem to exert a beneficial effect), viruses (EBV), certain occupational exposures (livestock feed processing) may elevate the risk for development of salivary gland cancer28. 4.2.2. Symptoms and signs
4.2.2.1. The parotid gland: One in four parotid tumors are reported to be malignant29 A rapid enlargement of a previously slow-growing, painless, mobile swelling in the parotid region Facial nerve palsy as a presenting symptom appears in 1/3 of the patients; it is considered to be an independent prognostic indicator, its presence decreasing dramatically the survival rates30 Pain, skin infiltration, metastatic intraparotid or neck

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lymph nodes indicate advanced disease and a poorer prognosis

4.2.2.2. The submandibular gland: 40-66% of the submandibular tumors are reported to be malignant28 A history of a painless, slow-growing, mobile mass in the submandibular triangle Pain, loss of mobility, skin invasion, metastatic lymph nodes are suggesting malignancy Weakness or numbness of the tongue reveals invasion of the hypoglossal and lingual nerves 4.2.2.3. Minor salivary glands: Malignancies developed in minor salivary glands are reported to vary between 10-50% of all salivary glands cancers28 Submucosal lumps and ulcerations Rapid enlargement, pain, nerve palsies The most common site is the palate, followed by the lips and sinonasal tract

4.2.3. Diagnosis History, inspection, palpation, complete ENT examination Fine-needle aspiration cytology (ultrasound guided) is the mainstay of early diagnosis, with a sensitivity of 90% and a specificity of 100%31 Frozen sections, in cases where FNA is not available Ultrasound examination CT, MRI of the head and neck Chest X-ray, CT, MRI of the chest and upper abdomen, for distant metastasis assessment 4.2.4. Histopathology Mucoepidermoid carcinoma: the most common malignant tumor of the major salivary glands, 80% developing in the parotid27; they are subdivided in low-grade and highgrade tumors Adenoid cystic carcinoma (cilindroma): nearly one third
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of adenoid cystic carcinomas occur in major salivary glands, 11% have distant metastasis (lung, bone, liver) at presentation, they are subdivided in three subtypes (solidpoorer prognosis, cribriform and tubular), the disease has a very long natural history and a typical propensity for perineural invasion and spread28 Carcinoma ex pleomorphic adenoma: malignant transformation of a pleomorphic adenoma is quoted in 3-12% of the cases, 75% occurring in the parotid gland27 Squamous cell carcinoma: primary (0.3-1.5%) or secondary (invasion from adjacent sites) Acinic cell carcinoma (low-grade adenocarcinoma): best survival rates Undifferentiated carcinoma Non-Hodgkin lymphoma Metastasis: skin cancers of the head and neck, lungs, kidneys and breasts

4.2.5. Treatment
4.2.5.1. Conservative treatment: Adjuvant postoperative radiotherapy after surgery offers superior outcomes than surgery alone. Clear indications for postoperative radiotherapy are: residual tumor, positive resection margins, high-grade tumors (high-grade mucoepidermoid carcinoma, squamous cell carcinoma, carcinoma ex pleomorphic adenoma, undifferentiated carcinoma), perineural spread, neck node metastasis28. Radiotherapy alone or in association with chemotherapy is reserved for non-resectable advanced lesions. 4.2.5.2. Surgical treatment: Primary surgery, followed by postoperative radiotherapy in appropriate cases, is the best treatment modality. Safe, clear oncological margins are mandatory. In advanced disease, wide en-bloc resections are required, followed by various reconstructive techniques. Facial nerve involvement in the parotid malignancies requires the sacrifice of only the involved branches, followed by facial nerve reconstruction.

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Surgical treatment of the neck (selective, modified, radical neck dissections) should be performed as a one-step procedure, together with the primary tumor ablation.

4.2.6. Prognosis 5-year survival range varies from 50-70% 10-year survival range varies from 45-65% Negative prognostic factors: stadialization, older age, positive surgical margins27

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1. Bradley PJ. Salivary gland anatomy. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 333-337. 2. Elluru RG, Kumar M. Physiology of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1293-1312. 3. Edgar WM. Saliva: its secretion, composition and functions. British Dental Journal 1992; 172: 305-312. 4. Humphrey SP, Williamson RT. A review of saliva: normal composition, flow and function. The Journal of Prosthetic Dentistry 2001; 85: 162-169. 5. Bradley PJ. Saliva, salivation and functional testing. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 339342. 6. Carlos J. Clinical examination and limited investigations of salivary gland diseases. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 343-347. 7. Gundlach P, Hopf J, Linnarz M. Introduction of a new diagnostic procedure: salivary duct endoscopy (sialendoscopy) clinical evaluation of sialendoscopy, sialography and X-ray imaging. Endoscopic Surgery and Allied Technologies 1994; 2: 294-296. 8. Koch M, Zenk J, Iro H. Algorithms for treatment of salivary gland obstructions. Otolaryngologic Clinics of North America 2009; 42: 1173-1192. 9. Perry RS. Recognition and management of acute suppurative parotitis. Clinical Pharmacy 1985; 4: 566-571. 10. Arrieta AJ, McCaffrey TV. Inflammatory disorders of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1323-1338. 11. OBrien CJ, Murrant NJ. Surgical management of chronic parotitis. Head & Neck 1993; 15: 445-449. 12. Marchal F. Inflammatory and non-inflammatory affections of the salivary glands. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 351-367. 13. Leake D, Leake R. Neonatal suppurative parotitis. Pediatrics 1970; 46: 203. 14. McAnally T. Parotitis: clinical presentation and management. Postgraduate Medicine 1982; 71: 87-93. 15. Burns BV, al-Ayoubi A, Ray J, Schofield JB, Shotton JC. Actinomycosis of the posterior triangle: a case report and review of the literature. The Journal of Laryngology and Otology 1997; 111: 1082-1085. 16. Manoussakis M, Moutsopoulos M. Sjogrens syndrome. Otolaryngologic Clinics of North America 1999; 32: 843-860.

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17. Caballero M. Sjogrens syndrome and sialosis. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 369373. 18. Hiraide F, Nomura Y. The fine surface structure and composition of salivary calculi. The Laryngoscope 1980; 90: 152-158. 19. Stanley MW, Bardales RH, Beneke J, Korourian S, Stern SJ. Sialolithiasis: differential diagnostic problems in fine-needle aspiration cytology. American Journal of Clinical Pathology 1996; 106: 229-233. 20. Williams MF. Sialolithiasis. Otolaryngologic Clinics of North America 1999; 32: 819-834. 21. Gleeson M, Cawson R. Benign salivary gland tumors. In: Hibbert J, ed. Scott-Browns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2475-2492. 22. Cohen EG, Patel SG, Lin O et al. Fine-needle aspiration biopsy of salivary glands lesions in a selected patient population. Archives of Otolaryngology Head & Neck Surgery 2004; 130: 773-778. 23. Stennert E, Guntinas-Lichius O, Klussmann JP, Arnold G. Histopathology of pleomorphic adenoma in the parotid gland: a prospective unselected series of 100 cases. The Laryngoscope 2001; 111: 2195-2200. 24. Witt RL. The significance of the margin in parotid surgery for pleomorphic adenoma. The Laryngoscope 2002; 112: 2141-2154. 25. Hanna EY, Lee S, Fan CY, Suen JY. Benign neoplasms of the salivary glands. In: Haughey BH, ed. Cummings Otolaryngology-Head & Neck Surgery. Elsevier Mosby 2005; 1348-1377. 26. Lombardi D. Salivary glands: benign tumors. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. Springer-Verlag 2010; 375385. 27. Guntinas-Lichius O. Primary and secondary malignant salivary neoplasms. In: Bradley PJ, ed. Otorhinolaryngology, Head and Neck Surgery. SpringerVerlag 2010; 387-395. 28. Jones AS. Malignant tumors of the salivary glands. In: Hibbert J, ed. ScottBrowns Otorhinolaryngology, Head and Neck Surgery. Hodder Arnold 2008; 2493-2521. 29. Gallia LJ, Johnson JT. The incidence of neoplastic versus inflammatory disease in major salivary gland masses diagnosed by surgery. The Laryngoscope 1981; 91: 512-516. 30. Sullivan MJ, Breslin K, McClatchey KD, Ho L, Farrior EH, Krause KJ. Malignant parotid gland tumor: a retrospective study. Otolaryngology - Head and Neck Surgery: Official Journal of American Academy of Otolaryngology Head and Neck Surgery 1987; 97: 529-533. 31. Roland NJ, Caslin AW, Smith PA, Turnbull LS, Panarese A, Jones AS. Fine needle aspiration cytology of salivary gland lesions reported immediately in a head and neck clinic. The Journal of Laryngology and Otology 1993; 107: 1025-1028.

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VI. NECK BASICS

1. NECK BASICS
1.1. Surgical anatomy of the neck
1.1.1. Fascial layers The neck has a superficial and a deep fascia1. The superficial fascia is very thin, adjacent to the overlying skin, and is of less importance. It invests the superficial platysma muscle. The deep fascia is divided into three layers, extends from the skull base to the upper mediastinum and forms important compartments in the neck. The deep fascia layers are: Superficial or investing layer: invests the entire neck, enveloping the trapezius, the omohyoid, the sternocleidomastoid, the anterior strap muscles and the parotid gland Middle or visceral layer: encircles the trachea, the oesophagus and the thyroid gland Deep or prevertebral layer: covers the deep muscles of the neck and the prevertebral space Carotid sheath: is derived from the superficial layer of the deep fascia, medial to the sternocleidomastoid muscle; it contains the carotid artery, the internal jugular vein, the vagus nerve and lymph nodes
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Figure 1. Neck fascial layers

I - External layer (investing, superficial); II - Middle layer (visceral); III - Carotid sheath; IV - Internal layer (prevertebral); 1 - Trapezius muscle; 2 - Splenius muscle; 3 - Erector spinae muscle; 4 - Levator scapulae muscle; 5 - Spinal nerve (XI); 6 - Medial scalene muscle; 7 - Medial scalene muscle; 8 - Longus capitus muscle; 9 - Sternocleidomastoid muscle; 10 - Thyroid gland; 11 - Cervical oesophagus; 12 - Trachea; 13 - Common carotid artery; 14 Vagus nerve (X); 15 - Internal jugular vein; 16 - Phrenic nerve; 17 - Cervical plexus (anterior division); 18 - Spinal nerve (XI); 19 - Posterior ramus of the spinal nerve

1.1.2. Triangles of the neck The neck is classically divided into an anterior and a posterior triangle, separated by the sternocleidomastoid muscle2.

Figure 2. Neck triangles

1 - Submental triangle; 2 - Submandibular triangle; 3 - Supraomohyoid (carotid) triangle; 4 - Infraomohyoid triangle; 5 - Lateral neck triangle; 6 - Subclavian triangle

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1.1.2.1. The anterior triangle The anterior triangle is delineated by the anterior border of the sternocleidomastoid muscle, the inferior margin of the horizontal ramus of the mandible and the midline. It is further subdivided into the submental, submandibular, supraomohyoid (carotid) and infraomohyoid triangles. The submental triangles boundaries are the anterior belly of the digastric muscle, the hyoid bone and the midline. It contains lymph nodes. The submandibular triangles boundaries are the inferior margin of the horizontal ramus of the mandible and the two bellies of the digastric muscle. The submandibular gland and the adjacent lymph nodes lie within this space. Other important structures are the facial vein, the facial artery, the mandibular branch of the facial nerve (VII), the lingual and the hypoglossal nerve (XII). The triangle bordered by the anterior margin of the sternocleidomastoid muscle, the superior belly of the omohyoid muscle and the posterior belly of the digastric muscle is called the supraomohyoid or carotid triangle. The carotid sheath, incorporating the carotid artery, the internal jugular vein and the vagus nerve (X), runs through this space. The vagus nerve (X) runs within the carotid sheath, between the carotid artery and the internal jugular vein, on their posterior aspect. It gives an auricular branch (Arnolds nerve), carotid body branches, pharyngeal branches, superior laryngeal branches and recurrent laryngeal branches. The accessory nerve (XI) crosses the upper part of the carotid triangle, as it enters the medial aspect of the sternocleidomastoid muscle. It supplies motor innervation to the sternocleidomastoid and the trapezius muscles. The hypoglossal nerve (XII) runs anteriorly to the carotid and passes medially below the posterior belly of the digastric muscle to supply motor innervation to the tongue muscles. The bifurcation of the common carotid artery lies also within this triangle. It is located nearly at the level of the greater cornu of the hyoid bone. The internal carotid ascends posteriorly to the external carotid, and has no collateral branches. The external carotid has 3 main anterior collateral branches: the superior thyroid, the facial and the lingual arteries. The surgical ligation

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Figure 3. The carotid artery

1 - Posterior auricular artery; 2 - Superficial temporal artery; 3 - Sternocleidomastoid muscle; 4 - Internal carotid artery; 5 - Ascending pharyngeal artery; 6 - External carotid artery; 7 - Common carotid artery; 8 - Omohyoid muscle; 9 - Superior thyroid artery; 10 - Hyoid bone; 11 - Lingual artery; 12 - Facial artery; 13 - Posterior belly of the digastric muscle; 14 - Maxillary artery;

of the external carotid should always be performed above the emergence of the superior thyroid artery. The lack of collateral branches of the internal carotid artery is a major surgical and anatomical criteria, in order to help the surgeon to differentiate the two carotids, during this procedure.

Anterior Posterior Deep Terminal

Superior thyroid Lingual Facial Occipital Posterior auricular Ascending pharyngeal Superficial temporal Maxillary

Table 1. Branches of the external carotid artery

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The upper and middle jugular lymph nodes lie within this space, embedded in a fibrofatty tissue. The infraomohyoid triangle is delineated by the anterior border of the lower portion of the sternocleidomastoid muscle, the inferior border of the superior belly of the omohyoid muscle and the midline. It encompasses the lower carotid sheath, lower jugular lymph nodes, infrahyoid strap muscles, the upper aerodigestive tract and the thyroid gland.
1.1.2.2. The posterior triangle The posterior triangle is delineated by the posterior border of the sternocleidomastoid muscle, the anterior border of the trapezius muscle and the clavicle. It is crossed by the omohyoid muscle, which further subdivides this triangle in the lateral neck and the subclavian triangles. The lateral neck triangles boundaries are the posterior border of the sternocleidomastoid muscle, the anterior border of the trapezius muscle and the superior border of the inferior belly of the omohyoid muscle. It contains the cervical plexus, fibrofatty tissue, lymph nodes and the spinal accessory nerve (XI). The Erbs point is located on the posterior border of the sternocleidomastoid muscle, at the junction between the superior and the middle one third. It is the point where the cervical plexus (C2 and C3) emerges superficially and give rise to four cutaneous branches, supplying sensory innervation to the front and side of the neck, the external ear and the parotid region. The subclavian triangles boundaries are the lower border of the inferior belly of the omohyoid muscle, the posterior border of the lower portion of the sternocleidomastoid muscle and the clavicle. It contains fibrofatty tissue, lymph nodes, the scalene muscles, the brachial plexus and the subclavian vessels, including the thyrocervical trunk. The phrenic nerve is the most important motor branch of the cervical plexus (C4), which runs on the anterior aspect of the scalene muscles and supplies motor innervation to the ipsilateral diaphragm. 1.1.3. Cervical lymph nodes 1.1.3.1. Lymph node groups The cervical lymphatics are divided into superficial and deep. The superficial ones perforate the cervical fascia and drain 291

into the deep nodes. The deep lymphatics drain the mucosa of the upper aerodigestive tract. The deep nodes are divided into four major groups2: Submental group: lies within the submental triangle/ space and drain the anterior floor of the mouth Submandibular group: lies within the submandibular triangle/space, related to the submandibular gland and facial vessels; cancers of the floor of the mouth, oral cavity and mobile tongue metastasize frequently in these nodes Jugular chain: 80% of the neck lymph nodes are closely related to the internal jugular vein; they are further divided into the superior jugular or jugulodigastric nodes (the first echelon nodes for the drainage of the oropharynx, mainly the tonsillar region), the middle and the inferior jugular nodes Posterior group: lies within the posterior triangle and are further classified into two chains, one accompanying the spinal accessory nerve (XI), and a horizontal supraclavicular chain, related to the thyrocervical vessels; the spinal nodes are the first echelon for the nasopharynx and the second echelon for the anterior neck nodes
1.1.3.2. Lymph node levels The most widely accepted classification of the neck lymph nodes was developed at Memorial Sloan Kettering Hospital, New York, and was adopted by the American Academy of

Figure 4. Neck lymph nodes levels

IA - Submental; IB - Submandibular; II Upper jugular (IIA - inferior to the spinal nerve, IIB - superior to the spinal nerve); III - Middle jugular; IV - Lower jugular; V - Posterior triangle group (VA - superior to the inferior belly of the omohyoid muscle, VB - inferior to the inferior body of the omohyoid muscle); VI - Anterior, previsceral.

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Otolaryngology Head and Neck Surgery (AAOHNS) in 1991. Further improvements, including division in subzones, have been subsequently added2.

I (submental and submandibular nodes)

Ia (submental nodes) Ib (submandibular nodes)

anterior floor of the mouth, lower lip, ventral tongue oral cavity, anterior nasal cavities

II (upper jugular nodes)

IIa (anteroinferior to the course of the spinal accessory nerve) oropharynx, larynx, nasopharynx, IIb (posterosuperior to the course hypopharynx and parotid gland of the spinal accessory nerve) larynx, pharynx larynx, hypopharynx Va (superior to the inferior belly of the omohyoid muscle) Vb (inferior to the omohyoid muscle) prelaryngeal (Delphian nodes) paratracheal perithyroidal (along the recurrent laryngeal nerve) nasopharynx thyroid gland

III (middle jugular nodes) IV (lower jugular nodes)

V (posterior triangle group)

VI (anterior, previsceral group)

larynx thyroid gland

Table 2. Lymph node levels of the neck

1.2. Clinical examination

1.2.1. History Examination begins with a detailed history taking. In patients with a primary complaint of a lump in the neck, a history of associated symptoms such as hoarseness, odynophagia, dysphagia, referred otalgia, nasal obstruction must be carefully evaluated. It is important to determine whether the lump is tender, before palpating the region. 1.2.2. Physical examination Inspection of the neck from the front will notice the presence of any swelling, skin lesions, signs of local inflammation, wounds, fistulas, scars. 293

Palpation of the neck is recommended to be performed while standing behind the patient, so that both sides can be examined simultaneously and compared. If the patient has an obvious neck lump, it is logical to start palpation at that site, but the rest of the neck should always be carefully palpated as well. When palpating the larynx, trachea, thyroid gland or midline lumps, it is recommended to ask the patient to swallow, in order to evaluate the active mobility of these midline structures. Palpation of the neck nodes will follow all the groups and levels described previously. The number, location, tenderness and mobility of the nodes will be assessed. Palpation of the neck is extremely important in the followup of the patients with head and neck malignancies. Normal anatomical structures in the neck which may be misdiagnosed during palpation include the carotid bifurcation, the transverse process of the atlas, the greater cornu of the hyoid bone and the submandibular gland3. A complete ENT examination must be performed. Panendoscopy of the upper aerodigestive tract is mandatory to rule out malignancies in patients suspected of lymph node metastases in the neck.

1.2.3. Imaging techniques

1.2.3.1. Ultrasound High-resolution B-mode ultrasound is an inexpensive, reliable and rapid examination used to evaluate palpable abnormalities and guide biopsies4. It allows to differentiate solid from cystic masses, it is especially useful in congenital and developmental cysts, also in vascular lesions. 1.2.3.2. Computed tomography CT Computed tomography is a quick, widely available and relatively cheap method of imaging the head and neck. It is excellent for evaluating bone destruction. 1.2.3.3. Magnetic resonance imaging MRI MRI has superior soft tissue resolution with improved demonstration of tumor-muscle interfaces, compared to CT. Direct axial, coronal and sagittal sections are available. It is

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superior to CT for imaging the skull base, oral cavity, salivary glands and nasopharynx. It is probably no better than CT in evaluation of lymph nodes and the larynx. No radiation is involved. Claustrophobia, the presence of cochlear implants, cardiac pace-makers, metal clips are contraindications to this technique3.
1.2.3.4. Positron emission tomography PET PET scanning is a modern imaging technique that can map functional and metabolic activity before any structural changes occur. It is capable to differentiate malignant from normal tissue based on increased glycolysis or amino acid metabolism by tumor cells, therefore it can identify tumors in normal-sized structures. It appears to be the best method to assess recurrent tumors5. Another advantage is the possibility to perform whole-body scanning, to identify the presence of synchronous tumors, distant metastases and to evaluate the site of the primary tumor in neck metastasis of unknown origin6. A major limitation of this technique is the low-availability and the high expensive costs. 1.2.3.5. Arteriography It is used to evaluate vascular lesions and masses fixed to the carotid artery.

1.2.4. Other investigations Fine needle aspiration (FNA) cytology (ultrasound guided): the gold standard in diagnosis of neck masses Endoscopy and guided biopsies: to identify primary tumor as source of a metastatic lymph node Open biopsy: use only after work-up is complete and if diagnosis is not evident Skin tests: used when chronic or granulomatous inflammatory disorders are suspected Thyroid gland assessment Major salivary glands assessment

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2. NECK MASSES
2.1. Congenital neck masses
2.1.1. Thyroglossal cysts
2.1.1.1. Definition. Aetiology Thyroglossal cysts are the most common cystic lesions of the neck. Up to 50% will present in young adults, 30% are diagnosed during the first decade of life7. Embryologically, the thyroid gland starts to develop at the level of the tongue base, then descends caudally to its final location. The tract that is left usually disappears. Failure of the tract to involute may leave epithelial remnants, which may expand into a cystic lesion. The hyoid bone will entrap a portion of this remnant tract8. 2.1.1.2. Diagnosis Midline,recurrent,painless,supralaryngeal cystic swellings, adjacent to the hyoid bone, 1-3 cm in diameter The cyst ascends during deglutition or with tongue protrusion Cysts may become infected (enlargement, redness, tenderness); fistula formation with pus drainage may occur after infection FNA cytology Ultrasound examination Scintigraphy, if an ectopic thyroid is suspected 2.1.1.3. Treatment Complete surgical excision is the treatment of choice. It is important to follow the cysts tract cranially, and to excise it together with the midportion of the hyoid bone, in order to avoid recurrences. The technique was firstly described by Sistrunk in 19209.

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Figure 5 - Thyroglossal cyst

There have been some reports of thyroid carcinoma presenting within a thyroglossal tract cyst10.

2.1.2. Branchial cysts

2.1.2.1. Definition. Aetiology Branchial cysts are lateral cervical cystic lesions. They occur most commonly in young adults. They are classified as first, second, third and fourth branchial cleft abnormalities. The second branchial cleft cysts are the most frequently encountered11. Six branchial or pharyngeal arches develop during the fourth week of intrauterine life. By the end of the fifth week, the second arch grows over the third and the fourth to form the cervical sinus. This cervical sinus normally closes during the 6th week, leaving a cervical vesicle. This trapped ectoderm is considered to be the origin of branchial cysts development7. 2.1.2.2. Diagnosis Cystic, oval mass, located anterior to the upper third of the sternocleidomastoid muscle A recent upper airway tract infection is often reported Swelling may be intermittent and occasionally local

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Figure 6 - Branchial cyst

Figure 7 - Branchial cyst ultrasonography

infection occurs (pain, enlargement, erythema, fistula formation) FNA citology Ultrasound examination CT/MRI 298

Figure 8 - Branchial cyst (intraoperative view)

2.1.2.3. Treatment Lateral cervical cysts should be surgically removed. The presence of local infection requires appropriate antibiotic treatment. Some authors estimated an incidence of malignant transformation (the so-called branchiogenic carcinoma) around 0.3% of all head and neck carcinomas12.

2.1.3. Dermoid cysts Dermoid cysts develop from ectoderm and mesoderm, along lines of embryonic fusion of the facial processes, mainly in the midline or lateral to the submandibular gland. They grow subcutaneously and contain skin appendages: hair follicles, sebaceous and sweat glands. They must be differentiated from epidermoid cysts (epidermal inclusion cysts), which develop out of ectodermal tissue and do not include skin appendages. Surgical excision is the treatment of choice.
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2.1.4. Lymphangiomas (cystic hygromas)

2.1.4.1. Definition. Aetiology Lymphangiomas are benign congenital lymphatic lesions (abnormalities of the jugular lymphatic sac). One third of cases are found in the oral cavity and cheek and about a quarter in the neck. They are divided in three categories7: Capillary lymphangiomas: are comprised of capillary-like, thin-walled lymphatic vessels, are usually asymptomatic, presenting as pale, small vesicle-like lesions confined to the superficial skin or oral cavity Cavernous lymphangiomas: dilated lymphatic channels typically occurring in the tongue, cheeks and lips, presenting as diffuse swellings Cystic hygromas: are histologically similar to cavernous lymphangiomas, presenting as larger cystic masses, which may communicate or be isolated; they develop mainly at the level of the posterior neck 2.1.4.2. Diagnosis Soft, fluctuant, cystic, diffuse masses found most commonly within the posterior triangle of the neck; as they grow they may involve the parotid area, the cheek, oral cavity, mediastinum, axilla Almost always they are present at birth; they can be diagnosed even prenatally Usually asymptomatic (only cosmetic complaints) Bleeding or infection may lead to rapid enlargement, pain, even life-threatening upper airway obstruction13 FNA cytology Ultrasound examination MRI, CT scans 2.1.4.3. Treatment Surgery is the treatment of choice. In large lesions, resection is often limited to the neck. Great care must be taken to avoid damaging important vessels and nerves. Another therapeutic option is intralesional injection of sclerosing agents: bleomycin, tetracycline, alcohol, picinabil (OK432)7. 300

2.1.5. Hemangiomas
2.1.5.1. Definition. Aetiology Like lymphangiomas, hemangiomas are usually considered congenital vascular abnormalities because they are present at birth or they appear within the first months of life14. Histopathologically, these lesions are benign, but show proliferation of endothelial cells and mitoses. Three phases are described during their life cycle: proliferative (rapid growth from 2 weeks to 1 year), involuting (slow regression from 1 to 7 years) and involuted (complete regression after 8 years of age)11. 2.1.5.2. Diagnosis Skin lesion or cystic mass with a bluish-purple appearance, compressibility followed by refilling A bruit and a thrill may be noticed A change in size is seen when the child strains or cries FNA, ultrasound, MRA (magnetic resonance angiography) 2.1.5.3. Treatment Observation: most of these lesions resolve spontaneously Treatment is indicated if rapid growth occurs, with pressure on adjacent structures; cosmetic considerations are also important Lasers (pulse-dye, argon, Nd-YAG)14 Systemic steroid therapy has been tried with various rates of success

2.2. Inflammatory/infectious neck masses

2.2.1. Acute and subacute infections
2.2.1.1. Acute viral and bacterial lymphadenitis Acute lymphadenitis occurs in nearly every person at some point in life, especially during the first decades15. Viral and bacterial infections of the upper aerodigestive tract cause a reaction in Waldeyers ring, followed by the involvement of the upper jugular lymph nodes. If the infection is restricted to the

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teeth and oral cavity, submental and submandibular nodes are affected. Acute inflammatory nodes are soft, mobile and tender. Infectious mononucleosis is typically associated with bilateral upper jugular and posterior triangle lymphadenopathies (100% of the cases).
2.2.1.2. Brucellosis Brucellosis is a zoonotic infection transmitted from animals to humans by ingestion of infected food products, direct contact with an infected animal, or inhalation of aerosols. Brucellae are aerobic gram-negative coccobacilli that produce urease. An undulating fever, malaise and development of cervical inflammatory lymphadenopathies in 20% of the patients is typical Diagnosis is confirmed by serologic tests. Treatment is with doxycycline and rifampicin for six weeks7. 2.2.1.3. Kikuchi-Fujimoto disease Kikuchis disease (subacute necrotizing lymphadenitis) is a histiocytic necrotizing adenitis without granular cell infiltration, most commonly found in young women. The main complaints are fever, malaise and painless lymphadenopathies especially in the posterior triangle. Evolution is benign and resolves in some weeks or months. Excisional biopsy is required to rule out tuberculosis or lymphomas7. 2.2.1.4. Deep neck infections The great majority are secondary to dental (40%) and oropharyngeal infections, foreign body ingestion, trauma with mandibular fractures. In up to 20% of the cases, no evident cause is revealed16. Most cultures are polymicrobial: Streptococcus viridans, Staphylococcus aureus, Staphylococcus epidermidis, anaerobes. Deep neck infections present as parapharyngeal, lateropharyngeal, retropharyngeal or submandibular space abscesses. The commonest complaints are pain, high fever, malaise and the presence of a diffuse, tender, indurated swelling, most frequently located along the anterior border of the 302

sternocleidomastoid muscle (latero- or parapharyngeal abscesses). If the infection is localized within the submandibular space, a significant oedema of the mouth floor is associated (Ludwigs angina). Deep neck infections are life-threatening conditions. Major complications include airway compromise, internal jugular vein thrombosis, mediastinitis. Evaluation is based on blood tests, ultrasound examination, CT/MRI, needle aspiration, cultures. High doses of wide-spectrum antibiotics must be administered immediately. Surgical drainage of the pus is recommended. Sometimes, in cases of airway compromise, intubation or tracheostomy is required.
2.2.1.5. Cervical necrotizing fasciitis Cervical necrotizing fasciitis is a rare but serious lifethreatening infection, characterized by a rapid, progressive necrosis of the subcutaneous fat and fasciae, and secondary necrosis of the overlying skin. It may develop primarily from an odontogenic or tonsillar infection, or during the evolution of a deep neck abscess. Streptococcus milleri, Streptococcus viridans and anaerobes are most frequently cultured7. Untreated, this condition may be rapidly fatal. High doses wide-spectrum antibiotics and surgical drainage and debridement are the recommended therapies.

2.2.2. HIV infection Cervical lymph nodes hyperplasia is ubiquitous in patients with HIV infection, and nodal masses in these patients infrequently need to be considered for biopsy. Nearly 70% of AIDS cases develop this persistent generalized lymphadenopathy (PGL) within the first months after seroconversion. If the excision is performed, histology will reveal a reactive follicular hyperplasia17. Enlarging or tender nodes are not typical for PGL. In these cases, nodal involvement may be the result of an opportunistic infection (tuberculosis, Nocardia species) or of a malignancy (nonHodgkin lymphoma, Kaposis sarcoma, metastatic carcinoma)14.
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2.2.3. Chronic granulomatous disorders 2.2.3.1. Tuberculosis Cervical lymphadenopathy is the most common head and neck manifestation of tuberculosis; only 10-20 per cent of the patients have associated lung disease7 Nodes tend to be tender and sometimes may develop an overlying erythema or a discharging sinus; they are found mainly in the upper jugular and posterior triangle groups PPD skin tests, chest X-ray Ultrasound examination FNA cytology Total excision with biopsy is recommended, associated with specific chemotherapy regimens 2.2.3.2. Cat-scratch disease (CSD) CSD is a granulomatous infectious lymphadenitis, caused by Bartonella henselae, a gram-negative bacillus; most commonly is the result of cutaneous inoculation through a scratch trauma from a domestic cat Diagnosis is based on serologic testing and a specific polymerase chain reaction (PCR) In most cases, no active therapy is required; the disease is self-limited and usually will resolve spontaneously within few months There is some evidence that azithromycin is related with a rapid resolution of the disease, but in most cases infection will settle without the need for antibiotic therapy18 2.2.3.3. Actinomycosis Cervicofacial actinomycosis is a chronic granulomatous infection caused by Actinomyces israelii, a facultative gram-positive, anaerobic bacillus, whose normal habitat is the oral cavity Painless, slow-growing, indurated masses, mimicking a neoplasm, associated with chronic purulent drainage (sulphur granules); development of multiple cutaneous draining fistulas is typical19 Anaerobic cultures, FNA, ultrasound, CT scans

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 Treatment: long-term high-doses antibiotic treatment (penicillin, clindamycin); surgical drainage of the collected pus
2.2.3.4. Toxoplasmosis Toxoplasmosis is a parasitic infection with Toxoplasma gondii, the main reservoir being domestic cats; transmission may also occur from cysts in undercooked meat7 A localized, lymphadenopathic and a systemic, disseminated form are classically described Diagnosis: lymph node biopsy, serologic testing Treatment: chemotherapy regimens

2.3. Benign tumors

2.3.1. Paragangliomas Paragangliomas are the most common vascular benign neoplasms of the neck. They develop from extraadrenal paraganglionic cells derived from the neural crest (neuroectodermal origin). Historically, they were referred as glomus tumors, chemodectomas and non-chromaffin tumors. The correct terminology is based on their location: carotid paragangliomas, jugulotympanic paragangliomas and vagal paragangliomas20. Most paragangliomas are solitary lesions. In 10% of the cases a syndrome of familial paragangliomas, characterized by multiple locations in the head and neck area, was reported21. Multiple paragangliomas associated with pheochromocytomas are also part of familial multiple endocrine neoplasia (MEN) syndromes. Paragangliomas of the head and neck are rarely vasoactive (1-3%), although they all contain neurosecretory granules22.
2.3.1.1. Carotid paragangliomas 2.3.1.1.1. Definition. Aetiology The carotid body is a paraganglion located in the adventitia of the posteromedial aspect of the bifurcation of the carotid artery. It has a chemoreceptor role, modulating the respiratory and cardiovascular functions in response to changes in arterial pH, O2 and CO2.

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A higher incidence is reported in individuals living at altitudes greater than 2000 meters23. Malignant paragangliomas have been reported in 6% of all carotid body tumors24.
2.3.1.1.2. Diagnosis The most common presenting sign is a slow-growing, painless, elastic or firm neck mass located at the bifurcation of the carotid artery Palpation: the mass is mobile laterally, but not in vertical plane; carotid pulsations may be transmitted through the mass Sometimes a bruit is audible by auscultation Very large tumors are associated with dysphagia, hoarseness and other cranial nerve deficits Carotid sinus syndrome syncope (loss of consciousness, bradycardia, hypertension) has been described in carotid body tumors25 Imaging techniques: ultrasound, Doppler ultrasound, CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography 2.3.1.1.3. Treatment Surgical excision is the treatment of choice. Tumor removal requires subadventitial dissection. To minimize complications, or when plans of vascular reconstruction are taken into account, the assistance of an experienced vascular surgeon might be strongly recommended. The external carotid artery need to be ligated occasionally to gain adequate exposure. Every effort should be made to identify and preserve cranial nerves X, XI and XII. Radiation therapy is an alternative when surgery has major contraindications. 2.3.1.2. Vagal paragangliomas 2.3.1.2.1. Definition. Aetiology Vagal paragangliomas are tumors developed from paraganglionic tissue associated with one of the ganglia of the vagus nerve26. Vagal paragangliomas most commonly arise from the inferior ganglion (nodose ganglion). Tumors developed from

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the superior ganglion (jugular ganglion) may extend intracranially through the jugular foramen.
2.3.1.2.2. Diagnosis Palpable neck mass with lateral but no vertical mobility Ipsilateral vocal fold paralysis with hoarseness and aspiration of liquids secondary to inadequate glottic closure Tumors of the jugular ganglion, at the skull base, may extend intracranially and may be associated with other cranial nerves involvement Imaging techniques: ultrasound, Doppler ultrasound, CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography 2.3.1.2.3. Treatment Most are treated by surgical excision Tumors arising from inferior ganglion are removed by a transcervical approach, while the superior ganglion lesions require a combined transcervical-transmastoid approach Patients must be warned that complete surgical removal may necessitate the sacrifice of the vagus nerve; the subsequent vocal fold paralysis will be managed postoperatively Radiotherapy is reserved to major surgical contraindications 2.3.1.3. Jugular paragangliomas 2.3.1.3.1. Definition. Aetiology Jugular paragangliomas are classically divided in two categories: the glomus tympanicum tumor and the glomus jugulare tumor. Glomus tympanicum typically develops within the middle ear cavity from a branch of the glossopharyngeal nerve (tympanic branch of Jacobson) or the vagus nerve (posterior auricular branch of Arnold). Glomus jugulare tumors originate at the level of the jugular bulb20. 2.3.1.3.2. Diagnosis Pulsatile tinnitus, hearing loss, cranial neuropathies involving cranial nerves IX, X, XI, XII

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 Otoscopic examination may reveal a vascular mass behind the eardrum Imaging techniques: ultrasound, Doppler ultrasound, CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography
2.3.1.3.3. Treatment Surgical management requires a multidisciplinary team including head and neck surgeons, neurosurgeons, neurootologists Radiation therapy, when surgical removal is not feasible

2.3.2. Peripheral nerves tumors

2.3.2.1. Definition. Aetiology Benign tumors of the head and neck arising from peripheral nerves include schwannomas and neurofibromas. Schwannomas (neurilemmomas) are typically slow-growing, well-encapsulated tumors that arise from the Schwann cells of peripheral nerves. Most commonly, these are solitary lesions, but may be multiple and in 50% of the cases occur in the head and neck area27. Schwannomas may develop from cranial nerves, the sympathetic chain, the cervical and the brachial plexus. Neurofibromas are benign nerve sheath tumors that may present as solitary lesions or as multiple tumor nodules as part of the autosomal dominant disorder von Recklinghausen disease (caf au lait spots, multiple neurofibromas, gliomas, spina bifida, acoustic neuromas, pedunculated skin lesions). Unlike schwannomas, these are non-encapsulated tumors; cystic and degenerative changes are uncommon20. Malignant transformation of peripheral nerve tumors is rare. 2.3.2.2. Diagnosis Most commonly appears as slow-growing lateral neck mass; pain and neurologic dysfunction are unusual Lesions arising from cranial nerves or sympathetic chain may present as parapharyngeal space tumors, displacing the lateral pharyngeal wall medially Imaging techniques: ultrasound, Doppler ultrasound,

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CT scans, MRI, carotid angiography, MR (magnetic resonance) angiography

2.3.2.3. Treatment Surgical excision is the treatment of choice.

2.3.3. Lipomas Lipomas are benign tumors derived from adipose tissue. They present as superficial, subcutaneous, soft, encapsulated, painless neck masses. Palpation gives the clinical diagnosis. Ultrasound and FNA cytology may be useful. Lipomas are best treated by complete surgical resection. Madelungs disease (Lanois-Bensaude syndrome, benign symmetric lipomatosis, fat neck) is characterized by the excessive formation of multiple non-encapsulated lipomas with a symmetrical distribution at the level of the neck, supraclavicular fossa, shoulder and even upper mediastinum, with sparing of distal arms and leggs. The disease is largely more prevalent in middle-aged Mediterranean male subjects. A history of high ethanol intake (red wine) is frequently associated28.

Figure 9 - Madelung disease

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2.4. Malignant tumors

2.4.1. Primary malignant neck tumors Most neck malignant lesions in adults are lymph node metastases from a carcinoma of the upper aerodigestive tract. Primary tumors are encountered less frequently. They include: Salivary gland carcinoma

Figure 10 - Neck metastases

Figure 11 - Neck metastases

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 Thyroid gland carcinoma: papillary carcinoma, follicular carcinoma, Hurthle cell carcinoma, medullary carcinoma, anaplastic carcinoma Parathyroid carcinoma Branchial cleft cyst carcinoma/thyroglossal duct cyst carcinoma Sarcomas Cutaneous malignancies: basal cell carcinoma, squamous cell carcinoma, malignant melanoma

2.4.2. Lymphomas
2.4.2.1. Definition. Classification Malignant lymphomas originate from the lymphatic system, which consists of a widespread tissue with complex maturation. The precursor cells are located in the bone marrow. The lymphoid stem cells differentiate into two distinct lineages of T- and B-cell types, respectively29. Malignant lymphomas were classically divided into Hodgkins and non-Hodgkin lymphomas. The latest 2008 World Health Organization (WHO) classification30, separates the malignant lymphomas into immature and mature lymphoid neoplasms. Immature, precursor lymphoid neoplasms of T- and B-cell type (pre-acute B-/T-cell lymphoblastic leukaemia or lymphoma) frequently present with nodal involvement in children, but the clinical picture is dominated by the leukaemia. Mature lymphomas are divided into B-cell and T-/NKcell neoplasms, and further subdivided into 30 and 18 subtypes, respectively. The most common B-cell lymphomas in Western countries are diffuse large B-cell lymphoma (DLBCL) and follicular lymphoma (FL), followed by mucosa-associated lymphoma (MALT). Hodgkins lymphoma is considered a distinct entity, with B-cell origin. It arises almost exclusively in nodal tissue. The most frequent T-cell lymphomas are the peripheral T-cell lymphoma (PTCL), angioimmunoblastic lymphoma (AILT) and anaplastic large-cell lymphoma (ALCL)29.

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2.4.2.2. Diagnosis The ENT area is a common site of presentation in malignant lymphomas. This region has a dense and well-developed lymphatic tissue. Extranodal involvement may be found in the Waldeyers ring, salivary glands, oral cavity, thyroid gland, nasal cavities and paranasal sinuses, larynx. Neck nodes involvement can occur as a part of a generalized lymphadenopathy or isolated in almost all cases of Hodgkins and non-Hodgkin lymphomas (painless, firm, multiple nodes). The gold standard diagnostic procedure is an open biopsy. This method should supply enough material for an extensive pathological work-up with morphology, immunohistochemistry, flow cytometry and ancillary techniques (cytogenetics, molecular genetics and gene-expression arrays)31. Stage of the disease is assessed according to the Ann Arbor staging system. For an accurate stadialization, these routine procedures must be performed32: Imaging techniques: CT, MRI, PET of the head and neck, thorax and abdomen Bone marrow biopsy and aspiration Hematology, blood chemistry The presence (B) or absence (A) of general symptoms (drenching night sweats, fever, 10% or more weight loss within three months) must be evaluated. 2.4.2.3. Treatment Surgery has its role only as a diagnostic procedure. Chemotherapy is the treatment of choice in all non-Hodgkin lymphomas. In B-cell subtypes addition of antibodies against B-cells (anti-CD20, rituximab) to chemotherapy regimens increased cure rates with 10-15%29. In Hodgkins lymphomas stage I and II, radiotherapy is still mandatory as consolidation after chemotherapy. The site of disease has little importance for choice of treatment, the decisive factors are the histology and the stage of the disease.

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2.4.3. Neck metastases

2.4.3.1. Neck metastases with known primary tumor In the greatest majority of cases, metastatic neck disease is secondary to a carcinoma of the upper aerodigestive tract. It is important not to overlook the possibility of metastatic disease from other head and neck sites (the skin of the scalp, ear, face and neck, the major and minor salivary glands, the thyroid gland) or from distant sites (lungs, stomach, kidneys, prostate, ovaries). The probability of lymphatic spread of carcinomas in the head and neck area is related to the abundance of capillary lymphatics at the level of the primary site. The greatest density of lymphatic capillary networks belongs to the nasopharynx, tongue, tonsils and hypopharynx. Thus, the rate of metastases from these sites is higher when compared to other sites: larynx or paranasal sinuses33. Tumors located in midline areas (base of tongue, posterior pharyngeal wall, supraglottis) may present with bilateral nodal involvement. The neck mass in a patient with known primary tumor should be managed according to the principles described for each primary site. 2.4.3.2. Neck metastases with unknown primary tumor 2.4.3.2.1. Definition The presence of neck lymph nodes metastases without clinical evidence of a primary tumor is an unusual, but not rare, condition. It accounts for nearly 2-3% of head and neck malignancies. Metastases develop mainly at nodal levels II and III, with less frequent involvement of levels I, IV, V and VI. Squamous cell carcinoma is the most common histological finding34. 2.4.3.2.2. Diagnosis When a patient presents with an isolated neck mass, a detailed and accurate clinical history and examination is essential. A complete and repeated full ENT examination, including office endoscopy (flexible or rigid) of the upper aerodigestive

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tract, with particular attention to the nasopharynx, tongue base and tonsils, must be performed. Additionally, the skin in the head and neck region, including the scalp should be carefully assessed, together with the external ear canal, pre- and retroauricular areas. Fine needle aspiration (FNA) cytology from the abnormal neck lymph node offers clear indications of the pathological nature of the lesion. The only tumor marker of clinical value is Epstein-Barr virus (EBV) serology. Microamounts of tissue obtained by FNA have also been studied to detect the EBV in neck metastases, using PCR (polymerase chain reaction)35. Positive outcomes should recommend multiple biopsies from the nasopharynx. Imaging techniques (CT scans, MRI, PET scans of the head and neck) should be performed before biopsy, in order to assist in directing subsequent biopsies and to avoid false-positive results determined by local swelling and inflammation induced after the tissue specimen is excised34. Thyroid gland and major salivary glands must be carefully assessed. Chest and abdominal images are helpful, mainly if the neck mass is located in the supraclavicular region. Direct panendoscopy of the upper aerodigestive tract, under general anaesthesia, is the next step. If no evident primary tumor is visible, guided biopsies should be performed from the most logical sites for the occult primary lesion, based on known lymphatic drainage.These areas are the nasopharynx (Rosenmuller fossa), the base of tongue, the tonsil and the pyriform sinus14. Ipsilateral or even bilateral tonsillectomy, instead of a simple incisional biopsy, is recommended in these cases, because up to 25% of occult tumors are found at this site36, 37. Open excisional biopsy of the metastatic lymph node is performed only after workup is complete and no primary tumor is found. If the specimen for histologic frozen section reveals a metastatic squamous cell carcinoma, a definitive neck dissection should be performed during the same intervention. Therefore, the patient must be counselled before undertaking the open biopsy about the possibility of a concomitant neck dissection14. There is no clear evidence in the literature that an open biopsy alters the prognosis, as long as appropriate treatment is initiated within 6 weeks38. 314

2.4.3.2.3. Treatment Despite extensive and accurate investigations, in nearly 60% of the cases, the primary tumor is not revealed. Controversies in the management of these patients range from types of neck dissections to be performed, fields of radiotherapy (ipsilateral neck only, whole neck, panmucosal), the role of chemotherapy. A definitive modified radical neck dissection should be performed in all patients with positive metastatic carcinoma after FNA or open excisional biopsy34. Radiotherapy is recommended after surgery.There is evidence to support the use of radiotherapy alone for management of the neck following excisional biopsy, without neck dissection, with 88-100% control rates39,40. Panmucosal radiotherapy has demonstrated a decrease in primary site occurrence, with no improvement in overall survival34. Benefit of chemotherapy and chemoradiotherapy is still under investigation. Nodal stage is the most important risk factor for local control.

2.4.4. Neck dissection This surgical procedure dates back to 1906, when George Crile described for the first time the classical radical neck dissection. Subsequently, Hayes Martin was a strong proponent of the radical en bloc technique, which was similar to the radical surgery that had evolved for breast cancer. During that period, radiotherapy had not been developed as an effective adjuvant therapy, and radical surgery was the only hope for survival. The significant morbidity of the radical procedure and the further development of radiotherapy led to an interest in more conservative approaches. The following neck dissections are now defined41: Classic radical neck dissection Modified radical neck dissection (types 1-3) Selective neck dissection Extended radical neck dissection
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Figure 12 - Radical neck dissection

1 - Common carotid artery; 2 - Vagus nerve; 3 - Internal carotid artery; 4 - Parotid gland; 5 - Horizontal ramus of the mandible

2.4.4.1. Radical neck dissection This procedure removes lymph nodes from level I-V, and all three non-lymphatic structures: the internal jugular vein, the spinal accessory nerve and the sternocleidomastoid muscle. 2.4.4.2. Modified radical neck dissection The operation consists of lymph node removal from level I-V, with preservation of one or more non-lymphatic structures: Type 1: preservation of the spinal accessory nerve Type 2: preservation of the spinal accessory nerve and the internal jugular vein Type 3: preservation of the spinal accessory nerve, the internal jugular vein and the sternocleidomastoid muscle When all 3 non-lymphatic structures are preserved, the neck dissection is also called functional or comprehensive. The principles of this functional procedure were presented by Suarez in 1963, and subsequently popularized by Ettore Bocca42. 2.4.4.3. Selective neck dissection Selective neck dissection involves the removal of selected

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lymph node levels (see Table 2), with preservation of the 3 nonlymphatic structures1: Selective neck dissection levels I-III (formerly called supraomohyoid neck dissection): most commonly used in cancers of the mobile tongue, floor of the mouth Selective neck dissection levels I-IV (formerly called anterolateral neck dissection): mobile tongue, floor of the mouth Selective neck dissection levels II-IV (formerly called lateral neck dissection): oropharyngeal, hypopharyngeal and laryngeal cancer Selective neck dissection levels II-V (formerly called posterolateral neck dissection): nasopharyngeal cancer, melanoma of the scalp, sometimes oral and hypopharingeal cancer Selective neck dissection level VI (formerly called anterior or central neck dissection): thyroid cancer, subglottic carcinoma Selective neck dissections are only indicated in the N0 neck, when the natural history of the disease is predictable. Its not recommended to be performed following previous surgery or radiotherapy41.
2.4.4.4. Extended radical neck dissection Besides the removal of all the structures described in a radical neck dissection, this procedure involves resection of additional lymph node groups or non-lymphatic structures (retropharyngeal, parapharyngeal, occipital, paratracheal or parotid nodes, parotid gland, mandible, mastoid tip, carotid artery, skin).

Nx N0 N1 N2a N2b N2c N3

Regional lymph nodes cannot be assessed No regional lymph nodes metastases Single ipsilateral lymph node, 3 cm or less in greatest dimension Single ipsilateral lymph node, 3-6 cm in greatest dimension Multiple ipsilateral lymph node, none more than 6 cm in greatest dimension Bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension Lymph node more than 6 cm in greatest dimension Table 3. TNM classification of regional neck lymph nodes

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Casa Crii de tiin Director: Mircea Trifu Fondator: dr. T.A. Codreanu Tehnoredactare computerizat: Czgely Erika Tiparul executat la Casa Crii de tiin 400129 Cluj-Napoca; B-dul Eroilor nr. 6-8 Tel./fax: 0264-431920 www.casacartii.ro; e-mail: editura@casacartii.ro