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Definitions:
Primordium: is defined as an organ or tissue in its earliest recognizable stage of
development.
Premaxilla: the embryonic bone that later fuse with the maxilla to form the incisive
bone.
Submucous cleft palate: 3 diagnostic signs of submucous cleft palate, 1) bifid uvula
2) notching of the posterior border of the hard palate 3) and muscular separation of the
soft palate with an intact mucosal layer.the development of velopharyngeal
insufficiency is the only indication for surgery.
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Theories of emberiogenesis:
Too many theories exist that describe how emberiologic failure results in craniofacial
cleft malformation.
Facial development largely occurs between the fourth and eighth weeks, and the face
has a clearly human appearance by age of 10 weeks
The branchial arches are largely responsible for the formation of the face, neck, nasal
cavities, mouth, larynx, and pharynx. The rst branchial arch contributes to the
maxillary and mandibular prominences and the anterior portion of the auricle
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A unilateral cleft lip results from failure of fusion of the medial nasal
prominence and maxillary prominence on one side
A bilateral cleft lip results from failure of fusion of the merged medial
nasal prominences with the maxillary prominence on either side. As a
result, the merged medial nasal prominences are often quite prominent,
as they are not restrained by attachment to the maxillary prominences
laterally. This is manifest at birth, as in a patient with a complete
bilateral cleft lip and anterior overprojection of the premaxilla and
prolabium.
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The structures of the secondary palate include the hard palate posterior to
the incisive foramen, the soft palate "velum" the alveolus and associated
dentition posterior to the incisive foramen.
The process of formation of secondary palate is slightly delays in female
embryos that might explain the increase in the incidence of cleft palate in
female.
Epidemiology:
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A survey by Fraser and Calnan in 1961, found cleft lip and palate unilateral in
80% of patients and bilateral in 20%. Left sided cleft were twice as frequent
as right sided clefts.
Fogh-Andersen in 1942 also reported in his thesis that cleft lip and palate
occurs more frequently in males , whereas cleft palate occur more frequently
in females.males predominate in isolated cleft without palate.
In contrast, complete clefts of the secondary palate are twice common in
females as in males, and the incidence of isolated soft palate clefts is
approximately the same.
The mean incidence in Japan is approximately 2.1 in 1000 live birth; in china,
1.7 in 1000 live birth; in Western European whites, 1 in 1000; and in AfricanAmericans, and 0.41 in 10009.
Superficial anatomy:
The upper lip extends from the base of the nose superiorly, to the nasolabial
folds laterally, and to the free edge of the vermilion border inferiorly
The lower lip extends from the superior free vermilion edge superiorly, to the
commissures laterally, and to the mandible inferiorly.
Around the circumferential vermilion-skin border, a fine line of pale skin
accentuates the color difference between the vermilion and normal skin"
white roll"
Along the upper vermilion-skin border, 2 paramedian elevations of the
vermilion form the Cupid bow.
Two raised vertical columns of tissue form a midline depression called the
philtrum.
Muscle groups:
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Group I
o Orbicularis oris
o Buccinator
o Levator anguli oris
Insert Into the modiolous
o Depressor anguli oris
o Zygomaticus major
o Risorius
Group II
o Levator labii superioris
o Levator labii superioris alaeque nasi
Insert into the upper lip
o Zygomaticus minor
Group III
o Depressor labii inferioris
o Mentalis
Insert into the lower lip
o Platysma
I.
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Orbicularis oris:
The normal orbicularis oris muscle is composed of a superficial "pars
superficialis" and deep "pars marginalis" component.
The deep component originate from the modiolus , continue with
horizontal fibers to cross the midline to the opposite commissure just
superficial to labial mucosa.
Its lower border curls upon itself forming the vermilion by everting the
mucos membrane.
The deep component function is to catch food and general sphincter
activity.
The superficial component functions in facial expression and speech.
Consist of lower " nasolabial" and upper"nasal" bundle.
The lower bundle derives its fibers from the depressor anguli oris and
insert in the skin forming the philtral edge.
The upper bundle represents the insertion of too many muscles and
insert into the anterior nasal spine and the septo-premaxillary ligament
deep to the alar base.
CLEFT LIP AND PALATE
The muscle fibers are not distorted by the cleft, but simply interrupted.
Blood Supply:
The facial artery, a branch from the external carotid system is the main blood
supply to the lip.
The facial artery ascends from the neck over the mid body of the
mandible just anterior to the insertion of the masseter muscle.
The facial artery branches into the submental artery that passes under
the mandibular body
The facial artery ascends and branches into an inferior and a superior
labial artery, which course beneath the orbicularis oris and
anastomose with the contralateral vessel.
The superior labial artery usually branches from the facial artery 1.1
cm lateral and 0.9 cm superior to the oral commissure
The inferior labial artery branches from the facial artery 2.6 cm
lateral and 1.5 cm inferior to the oral commissure
Motor innervations:
Sensory innervations:
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The mandibular nerve give the inferior alveolar nerve that give rise to the
mental nerve to supply the lower lip skin down to the labiomental
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Two third of the Cupid's bow , one philtral column, and a dimple hollow are
preserved
The musculature between the philtral midline and the cleft is hypoplastic
The superficial portion of orbicularis oris muscle abnormally inserts on the
cleft margin and base of the columella.
The inferior edge of the septum is dislocated out of the vomer groove and
presents with the nasal spine in the floor of the normal nostril.
The septum is deviated to the non-cleft side and convex on the cleft side,
impinging on the airway.
The base of the columella deviates toward the non-cleft side.
There is unilateral shortness in the vertical height of the columella
The nasal tip is deviated to the non cleft side and the dome is depressed on the
cleft side.
The cleft side lower lateral cartilage is attenuated , its medial crus lower in the
columella and its dome separated and inferior to the opposite alar cartilage.
The lateral crus is caudally displaced on the cleft side.
The alar rim is distorted by a skin curtain that droops over the alar rim like a
web and further reduces the apparent height of the columella.
The alar facial groove on the cleft side is absent
The vestibular lining is deficient on the cleft side
The nostril floor on the cleft side is widened.
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Cleft palate:
The normal hard palate composed of : the premaxilla anterior to the incisive
foramen , the palatine process of the maxilla , the palatine process of the
palatine bone .
The oronasal surface coverd with dense mucoperiosteal layer.
The soft palate "velume" is made up of of 7 paired muscles "that interdigitate
to separate the oropharynx from the nasopharynx for proper phonation,
swallowing and breathing" involved in velopharyngeal closure
The 7 muscles are :
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II.
III.
IV.
V.
VI.
VII.
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The greater palatine artery from the maxillary artery innervates the hard
palate.
The lesser palatine artery from the maxillary artery innervates the soft
palate.
The ascending pharyngeal artery "External Carotide" and a scending palatine
a branch from facial artery supply the lateral velopharyngeal structure.
Innervations:
The great palatine nerve "CN V" via greater palatine foramen innervates the
hard palate.
The nasopalatine nerve "CN V" communicate with the greater palatine nerve
at the incisive foramen to supply the premaxilla.
The lesser palatine nerve "CN V " via the lesser palatine foramen supply the
soft palate.
The muscles of velum are innervated by pharyngeal plexus "CN IX,X,XI"
except TVP which supplied by CN V.
Classification:
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Diagram keys:
o
o
o
o
o
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Unilateral or bilateral
Clefting of the lip with or without the palate, or of the palate in isolation
Etiology:
Syndromic "30%"
Non syndromic"70%"
Probability of
subsequent child with
cleft palate %
2
2-4
7
Probability of
subsequent child with
cleft lip+/-cleft palate %
4
2-4
7
15
14-17
Craniofacial Syndromes:
History:
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Ectrodactyly-Ectodermal-Dysplasia-clefting syndrome:
Velo-cardio-facial syndrome/22q11deletion:
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Blepharo-Cheilo-Dontic syndrome:
Non-Syndromic Clefts:
1. Facial dimension:
Emberyonic face shape predisposes to cleft lip in mice.
Increased transverse facial dimension maybe risk factor for
palatal clefts due to the longer distance between palatal shelves
that must overcome for fusion
This may help explain ethic gender differences in cleft
incidence. For example, Asian typically have wider faces that
may contribute to the more frequent failure of palatal shelf
fusion in this group.
2. Environmental factors:
Maternal alcohol consumption women who consumed 5 or
more drinks per week at increased risk of having children with
isolated cleft lip or palate.
Maternal Cigarette smoking recent metanalysis of 24 studies
revealed a consistent and significant association between cleft
lip or palate and maternal smoking.
Maternal folic acid deficiency
Medication example: retinoid, anticonvulsant, and steroids.
Altitude higher risk of cleft lip in the highlands than in the
lowlands.
Metabolic mother with DM have higher incidence oral clefts
babies than non diabetic mother
Parental age recent metanalysis shows no elevated risk of
NSCLP in older mothers , on the other hand increase maternal
age has been associated with increased risk of SCLP.increased
paternal age maybe more significant than the mother's .
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