Table of Contents

1. Internal Medicine

A. Dermatology 1

B. Diabetes Mellitus : ~ 16

C. Infectious Disease

1. Work-up : 23

2. Osteomyelitis 31

3. Septic Arthritis 34

4. mv 47

D. Neurology 49

E. Oncology

1. Bone Tumors 58

2. Soft Tissue Tumors 67

F. Peripheral Vascular Disease .

1. Deep Venous Thrombosis and Pulmonary Embolism 69

2. Arterial Disease ; 71

G. Radiology

1. MRI 74

2. Bone Scans 79

3. CT 83

H. Rheumatic Diseases

1. Gout and CPPD 83

2. Charcot Disease : 89

3. Lyme Diesease ~ 92

4. Rheumatoid Arthritis 94

5. Osteoarthritis 100

6. Paget's Disease of Bone ~~_u~~~_~ 1-f}-1---

----------=t~ronegative Spondyloarthropathies (General) 103

2. Pharmacology

A. Antibiotics 114

B. Antifungals 120

C. Local Anesthetics 126

D. Steroids 128

E. General Medications

1. SedativeslFlypnotics 133

2. Antipruritics 134

3. Antiemetics 135

4. Laxatives 136

5. Antidiarrheals 137

F. Analgesics

1. Narcotics ~ 138

2. NSAIDS 142

G. Tables and Formulas 151

3. Perioperative Management

A. Hospital Charting '" 159

B. Perioperative Complications 163

C. Anemias 167

D. Blood Transfusions ; 169

E. Pre-and Post-Operative Management 171

4. Principles of Surgery

A. Regional Nerve Blocks 180

B. Bone Healing 183

C. Wound Healing 189

D. Principles and Techniques ofFixation : 192

E. Biodegradable Fixation '" 202

F. Suture Materials 203

5. Forefoot Surgery

A. Accessory Bones 209

B. Ossification ofBones ; 211

C. Digital Surgery

1. Anatomy 211

2. Nail Surgery 213

3. Hammertoes/ClawtoeslFloating Toes/Overlapping Fifth Toes 217

4. Polydactyly 221

5. Predislocation Syndrome (Lesser MTPJ Instability) ; 223

D. Lesser Metatarsal Surgery

1. Brachymetatarsia 224

2. Lesser Metatarsal Surgery 226

---- __ ______..}~lor~s_B_unij)..n_ 228

4. Joint Implants 230

E. Amputations 233

F. lntermetatarsal Neuroma 235

G. First Ray Surgery

1. Anatomy of the First Ray 239

2. Radiology of the First Ray : 241

3. Surgery of the First Ray

a. Soft Tissue Procedures 245

b. Proximal Phalangeal Osteotomies 246

c. Distal Metatarsal Osteotomies 246

d. Proximal Metatarsal Osteotomies 249

e. Joint Destructive Procedures 251

f. Hallux LimituslRigidus 258

g. Hallux Varus 262

.--,J

6. Rearfoot and Ankle Surgery

A. Bone Grafting 264

B. Inferior Calcaneal Spur and Plantar Fasciitis 266

C. Endoscopic Plantar Fasciotomy 268

D. Metatarsal-Cuneiform Exostosis/Haglund's Deformity 269

E. Equinus 272

F. Flatfoot 274

G. Cavus Deformity 279

H. Tarsal Tunnel Syndrome ~ ~ 282

I. Triple Arthrodesis 283

J. Tendon Transfers 284

K. Ankle Surgery

1. Medial Ankle Instability 287

2. Lateral Ankle Instability 287

3. Dislocating Peroneal Tendons 289

4. Arthroscopy 290

5. Ankle Fusions 292

7. Plastic Surgery

A. Wound Healing 294

B. Split Thickness Skin Grafts 295

C. Skin Flaps 298

D. Skin Plasties 299

8. Pediatrics

A. Metatarsus Adductus 302

B. Clubfoot 310

C. Calcaneovalgus .....3J._5'---~

_~---D..--V-ertieal--'f-alus 317

E. Torsional Abnormalities 320

9. Emergency Medicine

A. Anaphylaxis 325

B. Chest Pain 326

C. Electrocardiogram 327

D. Comatose Patient 329

E. Shock 330

10. Trauma

A. General Fracture Management 333

B. Digital and Sesamoidal Fractures .336

C. Metatarsal Fractures 337

D. Fifth Metatarsal Base Fractures 338

E. MidfootFractures

1. LisFranc's Fractures 340

10. Rearfoot Fractures (Contined)

F. Rearfoot Fractures

1. CuneiformlCuboidlNavicular Fractures .342

2. Talar Fractures ..................................................................................•.............................. 343

3. Calcaneal Fractures , ~ 347

G. Syndesmosis Sprains 351

H. Ankle Fractures 353

I. Tibial Plafond Fractures (pilon Fractures) .356

J. Physeal Fractures 357

K. Open Fractures 362

L. General Trauma

1. Avascular Necrosis 364

2. Compart:In.ent Syndrome 364

3. Osteochndritides 366

M. Gunshot Wounds : 367

N. Soft Tissue Trauma

1. General Guidelines 368

2. Chronic Regional Pain Syndrome 370

3. Nerve Injury 370

O. Tendon and Ligament Trauma

1. Achilles Tendon Ruptures ; .. 374

2. Ligamentours Ankle Injuries 375

P. Vascular Traurna 377

Q. Nail Bed Injuries ; 377

R. Puncture Wounds '" 378

S. Bite Wounds 379

T. Bums 379

U. Cold Injuries 380

INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE INTERNAL MEDICINE

__________ ~~-_JNT~AL-MEDI~--INTERNAL MEDICINE INTE:RNAL MEDICINE INTERN~AL 1\1EDICINE ·INTTE··RNAT ME·DIC·INE

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IN·TERN~AL MEDICINE INTERNAL M.EDICINE INTERNAL MEDICINE INTERNAL M.EDICINE

DERMATOLOGY

1. SKIN LAYERS

A Epidermis

1. Stratum. Corneum: horny, keratin layer

2. Stratum Granulosum: granular layer

3. Stratum Lucidum: seen with thick. horny layer (palms & soles)

4. Stratum Sninosum: prickle, spiny layer

Langerbans Cells: function as macrophage of the epidermis and process contact

antigens

5. Stratwn Germinativum: basal cell layer

Basal Cell Keratinocyte: only cell of the epidermis capable of division. They divide

and migrate upward Total tumovertime is28 days (14 days ofmigrntion and 14

days to be discarded).

Melanocyte: produces melanin which is transferred to keratinocytes. Melanin protects the skin from uv radiation. In whites, found mostly in basal cell layer. In blacks, found throughout the epidermis.

Melanocyte

Stratum malpighii

Langerhans cell Keratinocyte

Basal layer

. ~

~ ... - .. •• r .. :. ' ..

Layers ofthe Epidermis

B. Dermal-Epidermal Junction (Basement Membrane Zone)

1. Lamina Lucida

2. Lamina Densa

3. Sublamina Densa

c. Dermis

1. Papillary Dermis: immediately beneath epidermis. Thin bapbazardly arranged collagen fibers, abundant ground substance, and delicate elastic :fibers.

2. Reticular Dermis: extends to the subcutaneous fat. Coarse elastic fibers and thick collagen bundles arranged mostly parallel to skin surface.

1

Diagramatic Anatomyofa Hair Follicle

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n. SKIN STRUCTURES

A Hair Follicle: hair growth proceeds through distinct phases Anagen: prolonged growth

Catagen: short lived interphase

Telogen: final resting phase

Phases of the growth cycle of a hair

ANAOEN

CATAOEN

TELOGEN

Cells of hair bulb produce hair shaft which is completely keratinized and has no living cells. Hair color is due to the amount and type of melanin.

B. Eccrine Glands: found in highest concentration on palms, soles, and axillae. Secretory coil located in dermis transmits sweat directly to skin surface for cooling of body .

2

C. Apocrine Glands: found primarily in axillae and anogenital regions and serves as a scent gland Derived from hair germ and opens directly into pilosebaceous follicle rather than skin surface. Become active at puberty.

D. Sebaceous Glands: found on alI body parts except palms and soles. Produces oil (sebum) that is emptied into the hair follicle. This lubricates and protects the hair and skin.

E. Arrectores Pilorum.: smooth muscle attached to the base of the hair follicle. Contract in response to cold or fright "Goose Bumps".

Apocrine unit

nerve

OERMS [paPillary

reticular Sebaceous Hair SnH"IT.......,=

~pi~det~~~~~~~ Pacili ~-f~~-.--q~J

.... -

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Diagramatic cross section of the skin and panniculus

ill. SKIN LESIONS ------------~~ons

1. Macule: circumscribed change in color of normal skin without elevation or depression < lem in

diameter. (Freckle)

2. Patch: similar to macule but > 1 ern.

3. Papule: circumscribed solid elevation, 1 ern in diameter.

4. Wheal: an edematous papule.

5. Nodule: similar to papule but > 1 em

6. Tumor: similar to nodule but > 2 em,

7. Plague: an elevation above the skin with a plateau-like surface.

8. Vesicle: elevated lesion < 1 em, containing clear fluid

9. Bulla: similar to vesicle but > 1 em.

10. Pustule: similar to vesicle but filled with neutrophils and dead bacteria Fluid is white or yellow

(pus). .

3

_'

Macula

Papule

Nodule

Tumor

Pustule

Vesicle

Bulla

B. Secondary Lesions

I. Scale: an excess ofhomy material on the skin

2. Crust: scab consisting of dned blood, serum. or p1S.

3. Erosion: scooped out and shallow break. No damage to the dermis.

4. Fissure: linear form of an erosion.

5. IDcer: deep lesion involving the dermis.

I. Eschar: daIk colored, hard to remove crust on ulcer.

Wheal

Secondary Lesions

Crust

Excoriation

Erosion

Ulcer

Fissure

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C. Shapes and Arrangements of Lesions

I. Annular: round lesion, ring-like. The rim is different from the center.

2. Linear: long, thin lesion or smaller lesions in a long, thin line.

3. Target or Iris: concentric rings like an archers target.

4. Imbricated: target lesions with normal skin between the abnormal zones. Very tare.

5. Serpiginous: snake-like. Partially circular and undulating

6. Geographic: outline of a continent on a map. .

7. Vegetating: lesion has a surface that grows outward in uneven, fleshy tufts that feel soft.

8. Verrucous: wart-like. Tufts of protruding lesion are hyperkeratotic, not soft.

9. Zosteriform: conforming to the distribution of a nerve root

10. Polycyclic or Circinate: annular lesions grow together, parts of their circles form larger lesion. II. Grouped: several similar lesions located in close proximity surrounded by a large area of normal

skin.

D. Morphology of Lesions

The following aspects should be included in every description:

1. Size

2. Color

3. Consistency: soft, medium, fum

4. Configuration: shape or outline oflesion.

5. Margination: sharp or diffuse.

6. Surface Characteristics: smooth or rough.

IV. BACTERIAL INFECTIONS OF THE SKIN

A. Impetigo

1. A superficial skin infection due to S. pyogenes & S. anreus, alone or together. Especially common in children in hot, humid climates.

2. Small, thin-walled vesicles or pustules on an erythematous base ruptme to form characteristic yellow-brown (honey-colored) crusts. Removal of the crusts reveal a superficial, moist base.

Lesions do not ulcerate. .

3. Commonly found on the face and extremities.

4. Involved areas may be pruritic. Regional lymph node involvement is common but other systemic manifestations are tare.

5. Treatment: Topical antibiotics (Mupirocin, Bacitracin, Neomycin)

Systemic antIbiotics (Dicloxici11in, Clindamycin)~ _

B. Ecthyma

1. A superficial skin infection caused by Group A Strep. And or S. aureus.

2. Begins as vesicles or bullae that rupture to form crusts. Removal of crust reveals an ulceration.

Heals with scarring.

3. Lesions are typically erythematous, circular, and multiple. Most commonly involve the lower extremities.

4. Treatment: Oral antibiotics (Dicloxacillin)

C. Erysipelas and Cellulitis

I. When a Strep. infection spreads into the dermal lymphatics. erysipelas occurs and when it involves the deeper dermis and subcutaneous fat, cellulitis occurs.

2. S. Pyogenes releases enzymes to facilitate rapid spread of the infection through tissue planes and prevent abscess formation. Edema, erythema, and heat develop. The enzymes also produce systemic manifestations (fever, tachycardia, confusion, and hypotension).

3. Predisposing factors: edema, tinea pedis, previous trauma to skin- bums, surgery, or radiation.

4. Most common sites are the face and lower extremities.

5. Erysipelas unlike cellulitis has a sharply demarcated and elevated border.

6. Both needle aspiration and skin biopsy of the lesion usually fail to yield organisms.

5

7. Causative agents: Usually Strep.

S. aureus- around abscess or open wound

H. influenzae- facial cellulitis in young children (6-36 months). P. muhocida - cat and dog bites.

P. aeruginosa & other gram (-) organisms- common in immunocompromised hosts.

8. Treatment: Mild- Oral antibiotics Severe- Intravenous antibiotics

Signs and symptoms may worsen after therapy is initiated because the antimicrobial rapidly kills the bacteria causing the release of potent enzymes.

D. Funmcles and Carbwlcles

1. A furuncle is an infection of the hair follicle that produces an inflammatory nodule with a pustule center through which the hair emerges.

2. A carbuncle affects severallKljacent hair follicles and begins as a nodule which enlarges to create an intlammatory mass that discharges pus from multiple follicular orifices. They occur predominantly on the back of the neck and is more common in diabetics.

3. S. aureus is the most common cause of both.

4. Treatment: Small funmcles- moist heat to promote drainage.

Carbuncles & Large funmcles- 1&D

E. Follicolitis

1. Inflammation at the opening of the hair follicle. Pathogenesis involves occlusion of the follicular ostium.

2. Skin lesions are e:rythematous papules or pustules surrounding the individual hairs most commonly on the scalp and exttemities.

3. Predisposing factors: chemicals, trauma, tight clothing, excessive sweating, occlusive dressings, prolonged immersion in water.

4. S. aureus is the most common cause. P. aeruginosa is common with inadequate disinfection of swimming pools, hot tubs, and whirlpools.

5. Treatment: Systemic antibiotics.

F. Erythrasma

I. A superficial infection, usually asymptomatic involving intertriginous areas (groin, axillae, toe webs). Scaling. fissuring, and maceration oftoe webs (particularly the fourth).

2. Causative organism is Corynebacterium minutissimum (gram positive bacilli). This organism produces porphyrins so all lesions fluoresce with a red or pink "coral" color with a Wood's lamp.

3. In other areas the lesions are scaly, brown or red, sharply demarcated patches,

4. Treatment: Wash with soap and water

Whitfield's ointment or topical miconazole Topical antibiotics

Erythromycin

G. Necrotizing Fasciitis (Hemolytic Streptococcus Gangrene)

I. A fulminating infection of the superficial and deep fascia. 1brombosis of subcutaneous vessels occur with gangrene of underlying tissues.

2. Disorder usually follows a cutaneous injury (puncture wound or laceration) and most commonly involves the extremities.

3. Early in course, affected area becomes red, hot, and edematous. Between days 2 and 4, skin assumes a blue, dusky tinge. Blisters may be present Process advances to areas of frank cutaneous gangrene with eventual sloughing.

4. Radiographs usually reveal gas in the soft tissues.

5. S. pyogenes alone may be the cause. but more commonly a mixture of aerobic and anaerobic bacteria is responsible.

6. Treatment: Immediate 1&0 Intravenous antibiotics

6

V. CUTANEOUS SIGNS OF BACI'ERIAL INFECTION A Bacterial Endocarditis

1. An infection of native or prosthetic cardiac valves most commonly due to various Strep. & Staph.

2. Infection causes vegetations offibrin, platelets, blood cells, and organisms to form on the valve.

These vegetations can break off and obstruct small and medium sized vessels,

3. Most patients have a fever and a heart murmur.

4. Four skin signs can occur:

a. Petechiae: small reddish-brown, noobalancing macules or slightly raised papules most commonly found on the extremities and mucous membranes. Develops in 10-20% of the cases.

b. Splinter Hemorrhages: 1-3mm brown, red, or black linear- discolorations beneath the nail plate. Occurrence is 20%.

c. Osler's Nodes: red or purple painful nodules on pads of fingers and toes .. Present in 10%. d, Janeway's Lesions: nontender, erythematous macules on palms and soles. Occurrence is IS%.

B. Disseminated Gonococcal Infection

1. N. gonorrhea is transmitted by sexual activity. Disseminated disease occurs when gonococci enter the bloodstream usually from an asymptomatic location (urethra, pharynx, rectum).

2. Clinical features:

a. ArthIalgias: usually involving several joints (wrists, hands, knees). Septic arthritis can occur.

b. Tenosynovitis: pain, swelling, and erythema along tendon sheaths. Pain on movements.

c. Skin Lesions: begin as tiny red papules or petechiae and become vesicular and pustular with a gray necrotic center on an erythematous base. Usually located on extremities. Occurrence is 50-70%. Will usually clear in 4 days but new lesions may appear.

3. Treatment: Ceftriaxone

C. Staphylococcal Scalded Skin Syndrome

1. Infection or mucous membrane colonization with toxin producing S. aureus which causes the skin to cleave at the granular cell layer.

2. Fever and irritability precede a generalized macular eruption which progresses to diffuse erythema. Vesicles and bullae then develop followed by skin separation. Skin sloughs leaving a moist, red base that quickly dries.

3 .. Vesicular fluid is usually sterile. Nikolsky's sign, shearing of normal epidennis in ~_nse_t(L__----_ ----------.lateral-pressoreis~.

4. Occurs mostly in children < 5 years old

5. Treatment: Systemic antibiotics

Possible drainage of abscesses Possible intravenous fluids

VI. VIRAL VESICULAR DISEASES

A Herpes Simplex

I. Characterized by small, grouped vesicles on a red base.

2. Lesions are painful, self-limited, and usualIy recurrent Recurrences average 2 a year.

3. Although the lesions can occur anywhere, HSV-l is most often associated with oral-labial lesions and HSV-2 with genital lesions.

4. Fever blisters and cold sores are the most common presentation of reactivated HSV infection.

Lesions occur at the oral mucocutaneous border and outer portion of the lips. The vesicles progress to ulcerated areas that heal without scars.

5. Lesions usually crust within 4-5 days and completely disappear within 10 days. VIral shedding

ends when lesions have crusted. .

. 6; Primary infection results most often from direct contact with another infected person.

7. Factors that initiate reactivation: UV radiation, fever. emotional stress, fatigue. trauma, menstruation, and pregnancy.

8. HSV's contain a linear double stranded DNA molecule.

7

9. Treatment: Acyclovir

Palliative Care: analgesics, antipruritics

B. Varicella (Chickenpox)

1. Highly contagious disease caused by primary infection with varicella-zoster virus, a member of the herpes family.

2. Experienced by most children, usually in the spring.

3. Characterized by 2 or 3 'successive crops of widespread pruritic, small, clear vesicles with pink halos on the scalp, face, mouth, and tnmk.

4. Lesions begin as macules and progress through papules, vesicles, and pustules that crust and heal usually without scarring. Distinctive feature is that in one area of the body all of the stages of a lesion can be seen.

5. Incubation is about 14 days. Constitutional signs (fever, chills, myalgias, and arthralgias) may precede the rash by 1-3 days. Crusting of the lesion usually occurs by day 6 with complete healing in 1-3 weeks.

6. Disease is acquired by direct contact with lesions or inhalation of infected airborne droplets.

7. Treatment: Isolation

Antipruritics and antipyretics (Nonsalicy1ate- aspirin can cause Reye's Syndrome) Topical antibiotic ointment

C. Herpes Zoster (Shingles)

1. Characterized by painful, sma1I, grouped vesicles occurring on an inflamed base and usually localized to 1 or 2 dennatomes without crossing the midline.

2. Vesicles tend to enlarge and become pustular in 3-4 days then erode and ernst in 7-10 days.

Patient is contagious until vesicles form crust Scarring is common.

3. Rash is usually preceded by a prodrome of fever, malaise, headache, and localized pain and parasthesias lasting 1-4 days. Lymphadenopathy is also common.

4. Varicella results from primary infection and herpes-zoster represents reactivation of the latent virus.

5. Treatment: Usually self-limiting and treatment is unnecessmy Acyclovir

Topical Zostrix (Capsaicin) to reduce pain

D. Hand-Foot-And-Mouth Disease

1. A relatively benign, highly contagious, self-limiting disorder usually seen in infants and young ----------------~chWWen.

2. Acquired by direct contact and caused most commonly by coxsackie virus A16 or enterovirus 71.

3. After an incubation of 1-3 days, 2-8mm bright, red macules appear on the tongue, hard palate, and buccal mucosa. These lesions develop into thin-walled gray vesicles surrounded by a red halo. Healing takes 7-10 days.

4. Cutaneous lesions on the sides and dorsmn of the hands, feet, fingers, and toes occur after the oral lesions. Initially, these lesions appear as red papules with small,tbin, gray vesicles appearing in the center. They are usually oval or linear and run parallel to skin lines and usually resolve in 10-

14 days. .

5. Sore throat and mouth, anorexia, malaise, and fever are also common.

6. Treatment: Symptomatic thempy- disease is usually self-limiting.

vn. VIRAL WARTS (VERRUCAE)

Warts are benign growths caused by papillomaviruses of the papovavirus group. They are slowgrowing, DNA-containing viruses that replicate in the nucleus of cells. They can occur on all areas of the body and are very common on the foot.: Warts are housed in the epidermis. Upon debridement, pinpoint bleeding occurs because the vessels in the dermal papillae are cut Remember, the dermal papillae have finger-like projections that interlock with the epidermis.

8

A Verruca Vulgmis

I. Most common form of wart.

2. Seen on fingers. hands, and knees most commonly in children.

3. Finn nodule exhibiting vegetations with thrombosed capillary loops. These are the warts blood supply and are pathognomonic for warts (looks like black dots on the skin).

B. Vemrca Plantaris .

I. Trauma at pressure sites on plantar surface of the foot allows penetration by wart virus.

2. Hyperkeratotic plaque with thrombosed capillaries form (may not be noticeable until

debridement). .

3. Tender on lateral compression.

C. Mosaic Warts

1. Very large, diffuse group of warts with ill-defined boundaries. May look similar to a callous.

2. No pinpoint bleeding upon debridement because the dermal papillae are not pushed superficially.

Lesion has a more horizontal than vertical arrangement

D. Histology of Warts

I. Large vacuolated "swiss cheese effect" of warty plug.

2. Pyknotic nuclei: small, shrunken cells.

3. Basophilic inclusion bodies surrounded by a peri-nuclear halo.

E. Treatment of Warts

I. Acid Therapy

a. Best conservative approach.

b. 80% Monochloracetic acid (Monocete) is best for sharply circumscribed warts.

c. 60% Salicylic acid can also be used. alone or in combination with the monochloracetic acid

d, For the mosaic warts, it is better to use the monochloracetic acid because it doesn't spread out upon weigbtbearing and destroy surrounding good skin, like the salicylic acid paste does.

e. Treatment takes about 6 weeks, has a success rate of 75-80%, and is often painful

2. Surgical Excision: must be down to the level of the superficial fascia.

3. Cryotherapy (Freezing): very painful and not very effective.

4. Laser Surgery

F. Molluscum Contagiosum

~------i--AnAniepiderma1. DNA poXVIrUS whiCh resIaes in the keratinocyte. The virus multiplies and forms globules of viral proteins (molluscum or Henderson-Patterson bodies).

2. Historically, the disease was recognized as a highly contagious childhood condition More recently, it has become a sexually transmitted disease.

3. Incubation period averages 4-8 weeks.

4. Individual warts occur as asymptomatic smooth-surfaced, :flesh-colored, herni-spherical papules several millimeters in diameter. In 250/0, these lesions are surrounded by a flat, erythematous halo. This central umbilication ispatbognomonic.

5. In children, face, extremities, and sometimes trunk are involved. Lesions spontaneously involute over several months. Total course is about 2 years.

6. In adults, inner thighs, pubis, and genetalia are usually involved. Resolution is quicker than in children

vrn. DERMATOPHYTES AND SUPERFICIAL FUNGI

Dermatophytesare capable of colonizing or infecting keratinized tissue such as hair, nails, and stratum corneum. Infections are caused by members of the genera Microsporum, Trichophyton, and Epiderm.ophytes. A KOH preparation can be performed in order to identify the species by culture.

A Tinea Pedis (Athlete's Foot)

I. T. mentagrophytes, T. rubrum, andE. floccosum are the main causative agents.

9

2. Itching and foul odor are the most common symptoms.

3. Four classifications:

a. Chronic Interdigital: characterized by maceration and dermatitis in the toe webs. Skin is white with a foul odor.

b. Chronic Papulosquamous Hyperkeratotic: characterized by fine, dry, white scales that may be patchy or cover the foot in a moccasin-like distribution. Usually occurs bilaterally and is associated with T. rubrum

c. Vesicular or Subacute: characterized by tense vesicles and bullae containing a serous fluid Usually associated with T. mentagrophytes and is the form most responsible for an id reaction on other parts of the body.

d, Acute Ulcerative: a rapid spread of an eczematoid vesiculopustular process. Secondary bacterial infection ean occur and the vesicular fluid turns purulent Can involve large areas of the foot andean appear with cel1ulitis, lymphangitis, and lymphadenitis.

4. Treatment: .Appropriate hygiene- avoid going barefoot, wash and dry feet daily.

Topical imidazole agents (usually for 2-4 weeks) -Clotrimazole (Lotrimin)

-Miconazole (Mieatin)

-Econazole (Spectazole)

-Ketoconazole (Nizoral)

-Sulconazole (Exelderm)

-Oxiconazole (Oxistat)

B. Tinea Capitis

1. Scalp ringworm caused by T. tonsurans.

2. Non-inflammatory type is characterized by multiple scaly lesions and areas ofbroken hair.

C. Tinea BaIbae

1. Commonly referred to as barber's itch and is most often seen in farm workers in contact with animals.

2. The superficial form appears with scaling, erythema, and broken hair associated with T. rubrum

and T. violaceum. .

3. In deeper forms, perifollicular pustules. crusting, and exudates are seen associated with T. verrucosmn and T. mentagrophytes.

D. Tinea Faciale

1. Presents as erythematous, scaling, pruritic lesions on the face and neck.

2. Causative organisms are T. rubrum, T. mentagrophytes, and M canis.

E. Tinea Corporis

1. Characterized by 1 or more circular, sharply circumscribed, slightly erythematous, dry, scaly patches with progressive central clearing on the trunk and limbs.

2. T. rubrum, and T. mentagrophytes are most commonly involved

3. Majocchi's granuloma consists ofperifollicu1ar and granuIomatous, slightly elevated nodules occurring on the lower leg.

F. Tinea Cruris

1. Commonly known as "jock itch".

2. Appears as well demarcated, scaling, circinate lesions with erythematous raised borders involving the groin and intertriginous areas.

3. T. rubrum, T. mentagrophytes, and E. f1occosum are most common G. Tinea Versicolor

1. Characterized by finely scaling, guttate or nummular patches occurring on the upper trunk and extending onto the neck and arms.

2. Caused by dimorphic yeast, Pityrosporum orbiculate.

H. Tinea Manum

1. Clinical presentation ranges from mild, asymptomatic scaling to discrete vesiculopustular plaques and patches .

2. Involvement of one hand and both feet is common and unexplainable.

3. T. rubrum is most common.

I. Tinea Nigra

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1. A superficial infection of the stratum corneum caused by Exophiala wernecki Usually found in . tropical and subtropical areas.

2. An asymptomatic, DOJJSC3ling, dade brown to black lesion on the palms, soles, and fingers.

IX SUBCUTANEOUS AND DEEP FUNGAL INFECTIONS A Mycetoma CMaduIa Foot>

1. An infection caused by a wide variety of agents (bacteria and fungi found in soil and plants) resulting in an indolent condition consisting of a triad of tumefaction, draining sinuses, and grains (microcolonies of organisms).

2. Infection begins as an initial implantation that forms a locally invasive indolent tumor-like mass.

The mass slowly enlarges and sinus ttacts form, and edema and induration lead to scarring into a wooden firmness in parts of the tissue. New nodules form and more sinus tracts develop and destruction of the tissue and bone occur.

3. Treatment: I&D of abscess and surgical debridement

Antimicrobial therapy depending on the causative organism

X PAPULOSQUAMOUS DISEASES A Psoriasis

1 ~ A chronic, inflammatory rash with increased and rapid epidermal proliferation resulting in accumulation of stratum corneum.

2. Classic lesion is well-circumscribed , erythematous plaque with a dry, silveIy scale appearing on extensor surfaces of the limbs. Lesions are bilateral and symmetrical in distribution.

3. Lesion exlubits the Koebner phenomenon which means replication of skin in an area of pressure

due to scratching.

4. Removal of the scales results in pinpoint bleeding known as the Auspitz' sign.

5. Nails are involved in 50010 of the cases and present with pitted depressions and onycholysis.

6. Different types include: plaque, guttate, erythrodermie; pustular, and artbritic.

7. Histopathology: Epidermis- hyperkeratosis and acanthosis with elongated rete ridges. Also see Munroe's

abscesses (a collection of neutrophils in the stratum corneum).

M~~~:~~:=·s~-~ca~pn=·=my~~pro~lifc=era~b=·o~n_lfl=·=th~pen~·~va=scul~=ar~mfl~amnmti~==~·o~n.~ _

--------------~~_rea1ment. Lobli

Tars

Anthralin (hydrocarbon ointment) Topical corticosteroids

Phototherapy- Ultraviolet B (UVB) radiation Oral psoralen plus ultraviolet A (pUV A)

Psoralen is a drug derived from plants which when activated by long wave UV light is phototoxic and melanogenic. The UV light causes binding of the psoralen to DNA thus inhibiting DNA production; therefore, inhibiting cellular production.

Systemic therapy- methotrexate, retinoids, cyclosporin, hydroxyurea, vitamin D3 B. Reiter's

1. A seronegative asymmetric arthropathy. Syndrome consists of conjunctivitis, urethritis, and arthritis.

2. Disease has a strong male preponderance (9: 1) and most patients have an HLA-B27 haplotype.

3. Classic lesion is termed keratoderma blenorrhagicum. Vel)' similar to pustular psoriasis on the paJmer and plantar skin but lesions of Reiter's have a thick layer of keratin overlying the pustules.

4. Penis may also be involved with moist, red plaques.

5. Treatment: NSAID's Immunosuppressive agents

11

C. Lichen Planus

1. An inflammatory dermatitis of unknown etiology that is primarily papular in origin.

2. Violaceous, polygonal, flat-topped, pruritic papules displaying a Iace-like pattern of white lines on their smfaces (Wickham's striae).

3. Lesions appear on flexor smfaces of the wrists and forearms, lumbar area. penis, ankles, anterior aspects of the legs. and dorsum of the hands. Lesions display the Koebner phenomenon.

4. Mucous membranes are affected in over 50%.

5. Nails are involved in about 10%. Most common changes are thinning and 'longitudinal ridging of the nail plate and onycholysis.

6. Variants include: annular, linear, hypertrophic, atrophic, bullous. ulcerative or erosive, lupus erythematous overlap, pempbigoides, and planopilaris or follicular.

7. Histopathology: Epidermis- byperlceratosis with degeneration of the basal cell layer. Damaged cells release melanin. Acanthosis resulting in a "sawtooth" appearance. Clusters of eosinophilic bodies result from kcratinocyte degeneration and are seen along the epidermal-dermal junction.

Dennis- band-like lymphocytic infiltrate in upper dermis.

8. Treatment: Oral. intIalesionaI. and topical corticosteroids Antihistamines

Phototherapy

Systemic retinoid therapy Dapsone

D. Pitvriasis Rosea

1. Acute, self-limiting. inflammatory disease of unknown but suspected viral etiology affecting children and young adults with the highest incidence in the winter.

2. Lesions are found to oval, erythematous papules and plaques with an inner collarette of scale.

The center usually shows some clearing.

3. First cutaneous manifestation is a "herald patch" usually on the trunk that begins as a small erythematous papule and rnpidly a round to oval patch 2-10 em in diameter. Lesions are distributed along lines of cleavage on the trunk and proximal extremities to produce a "Christmas tree" pattern.

4. Treatment: Topical corticosteroids Ultraviolet ligbt

E. Pitvriasis Rubra Pilaris

1. Characterized by hyperkeratotic follicular papules, salmon colored scaly plaques, and palmer-

plantar keratoderma.

2. Striking feature is "island sparing" ... normal appearing skin within sheets of erythema.

3. Lesions usually appear first on the upper half of the body, often in the scalp, and spread caudally.

4. Plantar lesions can extend up the sides of the feet to produce a sharply demarcated "keratodermic sandal". Fissuring is common.

5. Treatment: Topical steroids, emollients, and keratolytics Systemic retinoids

Methotrexate

Oral Vitamin A

XI. ECZEMATOUS DISEASES

Eczema is a Greek word meaning "boiling over". We use it as a general term. The hallmark of all of the eczemas is the presence of itching. All conditions can appear in the acute, subacute, or chronic phases. Treatment of eczema of unknown etiology is usually topical corticosteroids.

A Atopic Dermatitis ,

1. A genetic disorder sometimes associated with asthma, allergic rhinitis, or hay fever.

2. Frequently immunoglobulin E is increased in the serum.

3. Skin disease usually starts in infancy and many children experience resolution after 2-6 years.

12

4. 3 phases are: infantile, childhood, and adolescent or adult

5. Sites commonly affected are: face, scalp, diaper areas and buttocks, hands, antecubital and popliteal fossae.

6. Aggravating factors: extreme temperature changes, sweating, bacteria, soaps, and detergents.

B. Contact Dermatitis

1. Allergic Contact Dermatitis

a. A cell-mediated, type IV, delayed immunologic reaction.

b. 2 phases: First is sensitization in which the patient becomes allergic to the chemical. The second occurs with continued or repeat exposure to the allergen.

c. Eruptions usually appear as itchy feet, generally of a chronic nature secondary to shoegear breaking dowti and putting the feet into contact with various components of the shoes. Dorsum of the foot is the most common area.

d, 2 common allergens in shoes are mercaptobenzothiazole and tettamethylthimam. which are used in rubber adhesives. Phenolic resins, leather tanning agents, and fonnaldehyde have also been identified as allergens.

e. A diagnosis can be confirmed with a patch test by cutting out a small sample of the shoe and placing it on the p1tients back. A shoe contact dermatitis kit is also available.

2. Initant Contact Dermatitis

a Represents 800/0 of contact dermatoses and results from non-immunologic physical or chemical damage to the skin.

b. Skin reactions may be acute, as with exposure to acids, or chronic, due to repeated damage from a weaker agent

C. Seborrheic Dermatitis

1. Usually occurs at 20-40 years of age and persists for life. .

2. Occurs in hairy areas and clinically consists of mild erythema covered. with a greasy scale.

3. Most common forms:

a Scalp seborrhea can mimic psoriasis but.is less scaly, more diffuse, and more easily managed

b. Facial seborrhea appears with a mild erythema and greasy scale in the paranasal area.

c. Truncal seborrhea occurs as small circinate or petaloid patches in the central chest.

d Flexural seborrhea occurs in the axillae, groin, and in:framammary areas and is similar to the scalp lesions.

4. Patients can have I or several forms simultaneously.

5. Etiology is unknown but evidence favors Pityrosporum ovale.

-------t6r.. -----'1T-reatment:-K-eratolytic shalDpoos- Zinc pynthione (Sebulon, Danex, Head and Shoulders) Selenium sulfide suspension (Selsun Blue)

Salcylic acid and sulfur (Sebulex)

Topical steroids

Tar preparations Antifungals (Ketaconazole)

D. Stasis Dermatitis

I. Characterized by erythema, dependent edema, and hemosiderin pigmentation of the distal lower legs, particularly medially.

2. Most commonly occurs as a consequence of phlebitis or varicose veins, which lead to venous insufficiency, venous hypertension, and eventually stasis dermatitis.

3. Stasis ulcers are a complication of uncontrolled disease.

4. Treatment: Control the venous insufficiency- support stockings, elevation, diuretics

E. Dyshydrosis (pompholyx)

1. A non-inflammatory, idiopathic, recurrent vesicular eruption on the palms and soles.

13

2. Characterized by symmetrical, tense, deep-seated, pruritic, bmning vesicles, 1-5 mm in diameter, on the sides of the digits and the volar aspects of the palms and soles. Vesicles may coalesce to form bullae or evolve into sterile pustules that dry and shrink or rupture.

3. The cause is unknown but the patient is usually a type A personality; thus a psychogenic basis is a possibility. Disorder is exacerbated with stress.

4. Treatment: Usually responds very poorly to all therapy other than systemic steroids Topical corticosteroids and antihistamines

Cool water soaks

XII. CUTANEOUS DRUG REACTIONS A Erythema Multiforme

I. Syndrome characterized clinically by acute, self-limited, but often recurrent episodes and by symmetrically distributed round, fixed skin lesions.

2. Primary lesion is a round, erythematous papule. Over hOlD'S to days the JDPUles evolve in several different patterns.

3. Lesions may enlarge and coalesce to produce, small plaques or may evolve with concentric zones to reveal target lesions. Outer zone of target lesion is erythematous and central portion is white, yellow, or gray.

4. In 250/0, the lesions are limited to the oral cavity.

5. Treatment: Topical corticosteroids Antihistamines

B. Erythema Multiforme Major <Stevens-Johnson Syndrome)

I. Usually follows a 1-14 day prodrome of fever, malaise, cough, sore throat, arthralgia, and myalgia

2. Inflammatory bullous lesions suddenly appear on the mucous membranes which are chaIacteristically covered by hemonbagic crusts.

3. Bilateral pwulent conjunctivitis appears. Nares, pbaIynx, larynx, and lower respiratory tract can

be involved

4. Skin involvement varies from wticarial to target orballous lesions.

5. Complications include: blindness, renal failure, pneumonia, scarring of the skin, and nail loss.

6. Treatment: Large doses of systemic steroids Mortality of patients not treated is 5-15%

C. Erythema Nodosum

I. Most often presents with the sudden onset of ill-defined, tender, erythematous nodules or plaques distributed symmetrically over the anterior lower legs.

2. Initially lesions are red, slightly elevated, and 1-5 em in diameter with diffuse borders. Nodules evolve through color changes of a bruise from red to blue to yellow with a green tint,

3. Nodules may last between 1-2 weeks and usually resolve without scarring. Entire episode subsides over a 3-6 week period

4. Fever, malaise, and arthralgias may precede lesions.

5. Females are affected more than males (9:1).

6. Etiologic factors include: infectious agents, sarcoidosis, inflammatory bowel disease, and drugs such as sulfonamides and oral contraceptives.

7. Treatment: NSAID's .

Oral potassium iodide Steroids are not that effective

XIII. GRANULOMATOUS DISEASES A Necrobiosis Lipoidica Diabeticorum

I. Characterized by several, sharply but irregularly demarcated patches, usually on the shins.

14

2. Indurated patches appear yellowish in center and violaceous at the periphery.

3. About 75% are females and about 2/3 have diabetes mellitus.

4. Histopathology: Degeneration or necrobiosis of collagen with polymorphic cellular infiltrates composed

of lymphoid cells, fibroblasts, and histiocytes. 5. Treatment: Topical corticosteroids

Intralesional steroid injection

B. Granuloma Amm1are

1. A benign, usually self-limiting disorder of unknown etiology.

2. Occurs predominantly in children and young adults. Affects women more commonly than men.

3. 4 clinical types: localized, generalized, perforating, and subcutaneous (deep).

4. Localized is the most common and presents with a limited number of asymptomatic, fleshcolored to red-pmple, 1-5 mm dermal papules, often arranged in an arciform or annular pattern.

5. Lesions found most commonly on the hands and feet Involvement of the arms, legs, trunk. and . face may also occur.

6. Histopathology: Focal degeneration of collagen with an inflammatory infiltrate and fibrosis within the

dermis.

7. Treatment: Spontaneous resolution occurs in 50% within 2 years.

Lovibond's angle> 180 degrees..}

G. Spoon Nails (Koilonychia): faulty iron metabolism, familial, inflammatory skin diseases, idiopathic.

H. Onychogryphosis: hypertrophy and curvature- trauma or circulatory disorder.

I. Anonychia: Stevens-Johnson syndrome, epidermolysis bullosa, absence of nails.

J. Beau's Lines: transverse furrows- systemic illness, trauma

K. Onychoschizia: splitting of distal nail plate into layers, dehydration of nail plate.

L. Half and Half Nails: proximal white, distal red- renal disease.

M. Muehrcke's Lines: narrow, white, transverse bands occurring in pairs- hypoalbuminemia.

N. Mee's Lines: white transverse lines, single or multiple- arsenic poisoning. o. Onychorrhexis: brightness with breakage of nail.

P. Terry's Syndrome: distal 1-2 mm normal pink color, proximal end has white appearance- cirrhosis.

Q. Racquet Nails: inherited disorder.

R Median Nail Dystrophy: inverted fir tree- trauma.

s. Leukonychia: white discoloration of nail plate, bed, or matrix. T. Yellow Nail Syndrome: pulmonary disease and lymphedema.

15

DIABETES MELLITUS

Pathogenesis

Group of disorders characterized by glucose intolerance. Insulin produced by beta cells in the pancreas decreases blood glucose by inhibiting glycogen breakdown and facilitates entry of glucose into the tissues. When tissues fail to use glucose, hyperglycemia results. Diabetes affects 2-5% of the population in the U.S.

Types

a. Type I (IDDM) Insulin dependent diabetes was formerly called juvenile-onset diabetes but now is referred to as type 1 because it is not restricted to the juvenile age group. It is characterized by abrupt onset, polyuria, polydipsia, polyphagia, and often rapid weight loss.

b. Type II (NIDDM) Non-insulin dependent diabetes was referred to as adult onset Symptoms are often less pronounced than type I. Patients with NIDDM present with thirst, pruritus and fatigue. Obesity is present in 60-90% of these patients.

c. Secondary

1. Pancreatic disease: hemochromatosis, pancreatic deficiency, paticreatectomy

2. Hormonal: Cushing's syndrome, acromegaly, pheochromocytoma

3. Drug-induced: thiazides, dimetics, steroids, phenytoin

4. Genetic syndromes: lipodystrophy, myotonic dystrophy, ataxia, telangiectasia

d Impaired glucose tolerance (lG1) , also known as chemical, latent, borderline.or su1x:linical e. Gestational: glucose intolerance with onset during pregnancy

Distinguishing Features of DM

Features IDDM NIDDM

age onset <30 >30

prevalence .2-.3% 2-4%

Ketosis common rare

weight normal obese (80%)

complication frequent :frequent

genetics:HLA yes no

___________ nmn~mK~~n~s--------------~40~~-5~OO~~~----------------------~Conco~~------------ 100%

islet cell antIbody insulin secretion hyperinsulinism insulin treatment

required

insulin resistance defect

yes

severe deficiency

no moderate to

always

usually not

occasional:poor control excess antibody

usual: receptor

Diagnosis

a. Classic symptoms: polydipsia, polyphagia, polyuria

b. Hypergylcemia: fasting plasma glucose level greater than 140mWdl on more than one occasion

c. Oral glucose tolerance test: 75g glucose dose dissolved in 300m1 water after overnight fast; plasma glucose above 200mgldl at both 2 hrs and at least one other time between zero and 2 hrs.

d Hemoglobin Alc: (gylcosylated hemoglobin) Concentration in normal individuals 3-6%; patients with DM have two-to threefold elevation. Is a rough reflection of the mean level of circulating glucose for the previous 2 to 3 months (life of RBC-120 days).

16

Treatment

a. Diet: is the cornerstone of treatment Objectives include providing nutrition with a balance of protein, fat and caIbohydrates and to normalize weight

b. Oral Hypogylcemics: Sulfonylureas are recommended for patients with symptomatic NIDDM who cannot be controlled by diet alone and in whom an addition of insulin is impractical or unacceptable. (For a detailed discussion see pharmacology section.)

c. Insulin : Used primarily for the type I (IDDM) diabetic who is hypoinsulinemic and prone to ketosis. Also can be provided for type 11 diabetic who is not compliant with diet(see pharmacology section for details)

Complications

A Acute:

I.Ketoacidosis: precipitating factors- infection, omission of insulin, new onset diabetes

a cbarac. by pH <7.2 ,hyperglycemia, hyperventilation, inc. anion gap. hyponatremia, hyperkalemia, inc. BUN/Cr ketones in blood and mine

b. absolute insulin deficiency leads to inc. lypolysis. inc. fatty acids in the blood and ketosis

c. Ox. :polyuria. polydypsia, nausea. vomiting, aIxl pain, dehydration, altered mental status, acetone halitosis

d, Tx:

-fluids to restore intravascular volume with monitoring of cardiac and renal function

-bicarbonate ifDKA associated with shock/coma, arterial pH of <7.1, severe hyperkalemia

-K replacement (monitor ECG to ensure no hyperkalemia)

-insulin, monitor electrolytes

-phosphate (insulin inc. cellular uptake of phosphate and dec. plasma levels)

2.Hyperosmolar Nonketotic Hyperglycemic Coma

a glucose>600mgldl without ketosis-see in type 11 more common

b. Signs: lethargy ,confusion, seizures, coma, dehydration, hypertension, polyuria, polydypsia

c. Tx: fluid replacement, insulin, electrolytes(esp. K+ and phosphate) 3.Infection: at increased risk due to triopathy

A) neuropathy, B) :vascular insufficiency,and C) immunopathy

a. immune status decreased due to granulocyte depletion and defective phagocyte ingestion

b. nephropathy can compromise antibiosis .

c. Infections can include epidermophytosis, ascending UTI, pyelonephritis,septicemia and gas infections

d, (see section on diabetic foot infections for details) 4.Hypoglycemia-plasma glucose <5Omgldl

----------~ dUe to I)IDDM -lIDb3lance btw.insulin and glucose,2)reactive-insulin secretion vs, absorption of food 3)insuIin overproduction-pancreatic tumor or alcohol ingestion

b. Dx.: Whipple's trlad-l)riskofhypoglycemia,2)BS 40mgldl orless3)immediate recurrence following admin. of glucose

c. Tx.: related to cause(ie insulinoma-surgery) acljustment of dosage and patient education d, somogyi phenomenon: hypoglycemia results in rebound hyperglycemia-may be due to excess secretion of counterregulatory hormones(glucagon and epinephrine) .

B. Chronic:

l.)\the~lerosis

a. Possibly due to oxidation ofLDL

b. macroangiopathy- plaque occluding larger vessels(MI. IITN, CVA)

c. microangioapathy-increase basement membrane thickening(neuropatby, nephropathy, retinopathy)

d. bigh incidence of lower extremity disease-ant tib. Post. Db and peronael arteries usually affected(see PVD section)

2.Retinopathy

a. increased capillary permeability

b. microaneurysms

c. hemorrhages

d. retinal detachment

17

3.Nephropatby a.Diffuse:

1. widening of glomerular basement membrane

2. mesangial cell thickening

b.Nodular:

I. accumulation of PAS-positive material at the glomerular tufts(IGmmelsteil- Wilson lesion)

2. hylanization of afferent glomerular arterioles

c.Tx:

1. strict control of blood sugar

2. control HTN

3. decrease protein in diet

4.Neuropathy

Anatomic Classification

structure disorder

ggns/symptoms

nerve root

pain,dennatome sensory loss

xadiculopathy

mixed nerve vascular

pain, weakness. sen. loss. reflex change

mononeuropathy

nerve terminal

sen Ioss(stocking-glove) absent reflex mild weakness,

polyneuropathy

nerve terminal(muscle) anterior thigh pain with weakness

amyotrophy

etiology

probably vascular

probably

metabolic

unknown

sympathetic ganglion postural hypotension, impotence,gastrophy, anhydrosis,arhtropathy

autonomic neuropathy

unknown

a. Pathogenic mechanisms

I. sorbitol pathway-schwann cell hyperosmolality due to sorbitol excess,swelling, and

destruction

2. occlusion ofvasa nervorum

3. decreased nerve myoinositol

4. decreased nerve conduction

5. altered myelin synthesis and deficient repair

6. motor and sensory neuropathy

7. autonomic disorders

8. neurotrophic factors:nerve growth factors Treatment:

I. antidepressants-amitriptyline IO-25mg qd, nortriptyline 300mg tid

2. anticonvulsant-carbamazapine

3. topica1-capsaicin

4. analgesics/sedatives

2.

18

Diabetic Charcot Foot:

a. pathophysiology:

1 neuropathy: loss of pain perception, proprioception and sympathetic activity 2.trauma: increased weight bearing promotes joint degeneration and subluxation

b. stages:

I.development: destructive phase, joint laxity, subluxation,osteochondral fragmentation 2.colescence: absorption of debris and fusion of larger fragments to adjacent bone 3.reconstruction: revascularization and remodeling of bone and fragments

c. clinical features:

vascular neuropathic

skeletal

cutaneous

bounding pulse erythema swelling warmth

absent/diminished

pain proprioception vibration

deep tendon reflexes

rocker bottom deformity medial tarsal subluxation digital subluxation crepitus

hypermobility

ulcer hyperkeratosis infection hyperhydrosis

d treatment:

1. castinglimmobilization

2. accommodative foot gear

3. reconstructive surgery

Diabetic Foot meers:

1. abnormal pressure distribution secondary to neuropathy -accentuated by faulty biomechanicslbone distortion

-poorly fitted shoes

2. secondary to cuts or punctme wounds from foreign bodies

3. high infection rate often with multiple organisms

4. prevention is cornerstone oftx. -adequate shoe gear

-proper hygiene

-control of blood sugar

-frequent inspection

Diabetes and Surgery:

1. good metabolic balance prior to surgery -normal vital signs/electrolytes

-blood glucose btw. 100 and 200 mg/dl

-renal and hepatic status

-preopEKG

2. preferable to admit patient to hospital the day before surgery to adjust insulin dose

3. glucose measurements several hrs before and after surgery

Management during Minor Procedures

I. those patients undergoing local anesthesia

2. will be given oral nutrition immediately postop.

3. no change in diet, oral hypogycemic or insulin regimen

4. monitor glucose 1 hour before and 1 hour after surgery

Management during Major Procedures

1.Preop. eva!

-type I vs type IT

-type of therapy (diet, oral, insulin)

19

-diabetic control

-evaluate for any systemic complications

-admit to hospital a day prior to surgery or 2-3 days if poor control

-preop labs (ECG, UA, Chemistry, CBC, glycolsylated hemoglobin)

-schedule early in day-better equilibrium btw insulin dose and caloric intake

-if diet controlled and stable, fasting blood glucose on the morning of surgery

-if oral hypogycemics, disoontinue themoming before the day of surgery

-if on insulin and controlled «25Omgldl), give ~ the usual dose on morning of

surgery

2. Intraoperative and Postoperative Management a. Important considerations

-blood glucose should be between l00-200mgldl

-frequent intraop glucose testing to avoid iatrogenic hypoglycemia

h. Methods of management

preop start N with lOOOml 5% dextrose (D5W) with 4OmEq'L ofKCL run at lOOmllhr (need to dec. rate in pts. with CHF or renal failure)

administer ~ of usual morning insulin,gIucose test immediately after surgery

continue N fluids at 80m1Jbr, monitor blood every 4 to 6 hr with appropriate

regular insulin coverage

-or-

low dose continuous regular insulin

N solution consist of 100 U of regular insulin added to 500 ml of .5% normal saline solution

Glucose level:

following smgery, hydration status and glucose most important info. diet controlled, glucose <300mgldl can manage on diet alone 12 to 24 hrs after surgery

oral hypogycemics, if glucose under control, can start oral agent the day following surgery

taking insulin, requires frequent (every 4 hrs for the first 12 postop hrs)

glucose

testing- insulin given only if patients glucose values> 300mgldl -only regular

insulin is to be used at iniervals of 4 to 6 hrs.

Pbarmacology:

Insulin: a source- beef, pork, human

admin. - is a protein structure usually given subcutaneously adverse rx. - mainly sx of hypoglycemia

-tachycardia

-confusion

-vertigo

-diaphoresis

b. preparations: 1. Rapid acting

-aystalline zinc or regular-can give subq or N

-semilente-only given subq

2.Intennediate

-isophane or NPH(neutra1 protamine hagedoIJi)-only subq

20

3. Prolonged

Protamine zinc-max effect in 24hr

ultralente-given with semilente to form lente an intermediate acting form

4. Humalog 70/30

Oral Hypoglycemics: used in patients with NIDDM mechanism: -stimulate insulin release from beta cells -reduce serum glucagon levels

-increase binding of insulin to target tissues

adverse rx.: -GI disturbances -hypoglycemia

-pruritus

-nausea

-anemia

drug interactions: 1. Displace from plasma proteins -clofibrate

-phenylbutazone

-salicylates

-sulfonamides

2. reduce hepatic metabolism -dicumarol

-chloramphenicol

-monoamine oxidase inhibitors

-phenylbutazone

3. decrease urinary excretion -allopurinol

-probenecid

-salicylates

-sulfonamides

Biguanide: (metformin) ------------_:_-'c-t<las~Sifl~cail'fi;r;on:~· anHritih}'j5ergylcenuc NOT hypoglycemic

mechanism: -decrease intestinal glucose absorption -increased peripheral glucose uptake

-incr, Insulin-mediated glucose uptake

-decreased hepatic glucose production

advantage: -gylcemic control without weight gain (which occurs with

insulin and hypogylcemic agents) -reduces total serum cholesterol

-increases HDL

-decreases trigylcerides

adverse IX: most serious is lactic acidosis

risk factors (contraindications) -renal insufficiency

-hepatic disease

-severe cardiovascular disease

-severe pulmonary disease

temporary discontinue;

-N contrast medium

-infection

-surgery, trauma

-acute MI, angina

21

-stroke

-dehydration

-severe GI illness

Drug rx: oral sulfonylureaslinsulin alcohol-mer lactic acidosis cimetidine-incr metformin levels

nifedipine .

cationic drugs (ranitidine,triarnterine, trimethoprim) nephrotoxic drugs

aggressive use of diuretics

Dose: sOOmg tablets BID Rezulin (Troglitazone)

- Troglitazone is a thiazolidinedione antidiabetic agent that lowers blood glucose

by improving target cell response to insulin .

• Troglitazone decreases hepatic glucose output and increases insulin-dependent glucose disposal in skeletal muscle. Its mechanism of action is thought to involve binding to nuclear receptors Peroxisome Proliferator Activated Receptor (pP AR) that regulate the transcription of a number of insulin responsive genes critical for the control of glucose and lipid metabolism.

-Rezulin is indicated to improve glycemic control in patients with type 2 diabetes mellitus as an adjunct to diet and exercise in combination with (not substituted for):

• A sulfonylurea drug for patients who are not adequately controlled with a sulfonylurea alone or,

• A sulfonylurea drug together with metformin for patients who are not adequately controlled with the combination of a sulfonylurea and metformin or,

• Insulin in patients who are not adequately controlled with insulin alone. -Contraindieations

-Severe idiosyncratic bepatocellular injury bas been reported

during marketed use , Rezulin therapy should not be initiated if the patient exlnbits clinical evidence of active liver disease or increased serum traDsamjnase levels (ALT> 1.5 times the upper limit of normal)

-Thus, dose acljustment in patients with renal dysfunction is not necessary ____________ -=-Reznljn is not.indicated as jnitial therapy~abeitue:;:>s----_-_ -Rezu1in is available in 200, 300 and 400 mg tablets as follows

-Rezulin should be taken with a meal.

Combination Therapy

• Sulfonylureas

• Metformin

• Insulin

22

Infection Work-Up

Definition: Invasion of the body by harmful organisms (pathogens) such as bacteria, fungi, and viruses with an initiation of an inflammatory response by the host.

Infectious Dose: Minimal nmnber ofviral particles necessary to establish a disease state: 1 X 106. This allows for differentiation between colonization and infection.

Key: Diagnose an infectious process quickly and accurately. Diagnosis is made by clinical exam, Nor by ancillary tests. Make diagnosis then implement a proactive treatment plan.

Goals: To establish a treatment plan, work to answer 2 questions: . A Is hospitalization required?

Outpatient versus in-patient therapy. Oral antibiotics versus IV antibiotics

B. Is surgical intervention warranted? .

Bedside debridement versus OR I&D

I. HistOry (Subjective):

A Chief Complaint

B. History of Present Dlness

C. NLDOCAT

1.' Any previous ulceration or infection?

2. Any recent antibiotic usage?

3. Trauma?

4. Last meal?

5. Check shoegear for foreign body or signs of irritation.

6. Constitutional signs & symptoms: fever (highest temp & when?), chills, night sweats, rigors (violent shaking), nausea, vomiting, loss of appetite, diarrhea, weight loss, general

malaise/fatigue, mjalgias. .

History of .... (begin to think: empirically):

Post-op Staph. aureus

IV line sepsis Staph. epidermidis

Implant Staph. epidennidis

________ --=-=-===-=--S--'crat___:_:_:_.:_ch-in-'gL_---.:__-----"T..,.in,."ea~inf,.,.-,-. with~· ",-' """seco_ndary_bacteria) inf = gtam_------negative

Puncture Wound

Cellulitis = Staph & Strep Osteomyelitis =

Pseudomonas

Diabetic Infection, '

IV Drug Abuse Water Related

volnificans,

Dog/Cat Bites Human Bites

Polymicrobial

MRSA, MRSE, Pseudomonas

Pseudomonas, Aeromonas hydrophilia,. Vibrio

Mycobacterium marinum

Pasteurella .multicida

Eikenella corrodens

Fever

Body temperature is regulated by the hypothalamic thermoregulatory center in response to various stimuli. The stimuli affecting the hypothalamus are endogenous pyrogens secreted by leukocytes and Kupfer cells. Other stimuli are bacterial endotoxin&, phagocytosis, and certain immune reactions. The endogenous pyrogen then increases the set point and temperature is raised. Two signs of increased set point are chills and shivering.

Gram negative bacteria tend to cause more of an intense fever. Gram positive bacteria tend to evoke a profound inflammatory response, which releases endogenous pyrogens. It is believed that a certain threshold of endogenous pyrogens is necessary for fever to occur. It is for this reason we may not see fevers with early localized infections.

23

General Guide to Post-Op Temperature Elevations

107 Anesthetic a
106
105 Blood Transfusion Reactions .'
104 Closed Abscesses
103 AteJectasislPneumonitislDrug ReactionsILiver Disease
102 Wound Infection
101 Draining Abscess
100 Benign Post-on FeverlPost-anesthesia Overshoot
99
98 Usual Range of Normal
97
96 Post-op Hypothermia II. Past Medical ffistory:

A Diabetes: What type and for how long? Last BG? Classic triad (neuropathy, nephropathy, retinopathy).

B. Cardiovascular: CABG (how many bypasses and when?), IITN, Murmurs, MVP, MI (last one), phlebitis.

C. LungILiverlRenaI disease.

ill. Medications: Note dosages and frequencies. H on antibiotics: how long and why? IV. Allergies: Penicillin, Local anesthetics, Iodine/Shellfish. Note type of reaction.

V. Past Surgical ruston: list all surgeries, dates, & physicians andlor hospital. Get medical records on recent lower extremity surgeries.

VI. Social Histon:

A Cigarettes, Alcohol, IV Drugs (how long & how much?).

B. Occupation: Is patient on feet at work.

C. HIV risk factors.

VII. Family mstOry: ------------~~==Are~~pmm~~~~~~?J[n~~was~~of~-------------------------------------

B. Person to contact in case of emergency.

Vill. Review of Systems:

A HEENT: headaches, glaucoma, retinopathy, eye or ear infections, sinusitis, sore throat, stiff neck

B. CVS: IITN, MI, CHF, MVP, mmmur, angina, dyspnea, phlebitis, claudication

C. Resp: COPD, asthma, bronchitis. recent cough or cold, m, pneumonia

D. GI: ulcers, GERD's, diarrhea, constipation (last bowel movement), hepatitis

K GU: urgency, frequency, nocturia, hematuria, retention, incontinence, UTI, renal stones, SID's

F. MS: Muscle or joint pain, arthritis (type & how long?). Is patient ambulatory? Any assistive

devices? .

G. Neuro: CV NI1A, seizures, vertigo, syncope, paralysis, NM disorder, peripheral neuropathy

IX. Pbysical Examination (Objective):

A General appearance of patient:

Does patient look sick? Are they diaphoretic, flushed, disheveled. poor attitude?

B. Vital Signs: Preliminary way to detennine the amount of stress the infection has placed upon patient.

1. Temperature: Note Tmax in past 24 hours & Tpres

2. Blood Pressure: May decrease during infection.

3. Pulse: May increase during infection.

4. Respiratory Rate: may increase during infection.

5. Finger Stick Blood Glucose in Diabetics: Usually elevated during infection.

24

Septic Patient: Elevated temperature, bypotensive, tacbycardic:, tacbypnic.

C. Bacteremia:

1. Definition: simple presence of bacteria in the blood stream that have clinical manifestations.

2. Significance: method by which infections can be spread to distant foci.

D. Septicemia:

1. Definition: clinical state in which in addition to bacteremia there are fever, chills, & other clinical symptoms.

2. Significance: represents failure of the body to localize infection.

E. Don't limit exam to the lower extremity:

1. Look for other sources of infection:

a. Eyes, ears. nose. throat

b. Respiratory Tract

c. SBE

d Phlebitis. IV line sepsis

e. Gastrointestinal Tract

f. Genitourinary Tract

g. Skin: decubs or ulcerations on other parts of body.

X Lower Extremity Examination:

A Vascolar Status:

1. Pulses:

a If non-palpable then check with Doppler. If non-audible or weak with Doppler, get noninvasive

arterial studies (PVR's).

b. ABI < 0.45 is inadequate for healing in diabetics. .At least 30mmHg needed to heal digital wounds.

c. May need arteriogram. and vascular surgery consult for possible bypass. d Question for ischemic rest painlre1ief of pain with dependency of limb

2. Capillaxy Refill: Also check color and temperature of digits.

B. Neurological Status:

1. Check all sensory, motor. & reflex functions.

2. Determine level & duration of neuropathy if present

a Use Semmes-Weinstein monofilaments. Comes in 3 sizes. Ifpatient cannot feel the 5.07 ------------. -size;-protective"1breslR>d is lost.

C. Orthopedic Status:

1. Note all honey prominences.

2. Note foot type and deformities.

3. Previous amputations?

4. Pain with range of motion of affected joint versus tenderness upon palpation.

D. Dermatologic Status:

1. Assess for the 5 CARDINAL SIGNS OF INFLAMMATION:

a Dolor - Pain This is the most indicative sign. Even neuropathic patients will have pain.

b. Rubor - Redness

c. Calor - Heat

d, Tumor - Swelling .

e. Functio Lasea - Loss of Function

At the site of inflammation, blood vessels dilate and there is an increase in blood flow to the area causing RUBOR & CALOR Then there is extravasation of phagocytes and fluid into the perivascular space resulting in TUMOR This edema causes stretching of the cutaneous nerve fibers and the patient experiences DOLOR which causes FUNCTIO LASEA.

Always note Intensity of tbe signs of inOammation & compare to tbe contralateral limb.

25

2. Wound Evaluation:

a Location - digits. interspaces. boney prominences, plantar space.

b. Size - always measure after debridement

c. Base - granular, fibrotic, fatty, necrotic, macerated.

d Depth - Does wound probe to bone or track up tendon sheaths or fascial planes? Is bone or tendon exposed?

Wagner Oassification of Diabetic, Neuropathic, & Vascnlar Foot Lesions o - No open lesions, may have keratosis.

I - Full thickness ulceration that does not go beyond skin.

2 - Beyond full thickness of skin Tendon or joint capsule may be exposed. 3 - Ulceration open to bone. Osteomyelitis is present.

4 - Wet or dry gangrene with or without cellulitis localized to the forefoot.

5 - Gangrene to extensive portion of foot or whole foot. Foot salvage is not possible,

e. Drainage - serous, serosanguinous, hemorrhagic, liquefactive, purulence.

f. Odor - foul odor indicative of anaerobes, fruity odor indicative of pseudomonas.

g. Margins of Wound - viable, nonviable. necrotic. undermined, irregular border, punched-out, keratotic, hyperpigmented, indurated, macerated.

h. Surrounding Tissue

1 .. Erythema versus Cellulitis (outline borders with marker to monitor regression or

progression).

Erythema - abnormal redness of the skin due to capillary congestion such as inflammation CeUulitis - an acute spreading infection of the skin and connective tissue. More wide-spread than erythema and boundaries are not clearly demarcated. (Most common organisms

associated with cellulitis are Group A Strep & Staph aureus.) .

2. Edema - as edema decreases, you will see an increase in skin lines.

3. Lymphadenopathy - check for lwnps in groin & behind knee.

4. Lymphangiitis - "blood poisoning". Red streaks up legs along lympbatic cbannels. Infection is drained from the body via lymphatics. Lymph nodes can become swollen during this process due to excess bacteria & increase in pressure from the edema. If lymph nodes become overwhelmed, lymph drainage can be blocked and red streaks can occur (#1 org. is grp. A Strep)

__________ -=Bacte= ria can also seed the blood via lymphatics and cause a bacteremia which can lead to a septicemia.

XI. Medical Imaging:

A Conventional Radiographs:

1. Always order even with soft tissue infections. Need baseline radiographs.

2. Examine for:

a. Soft tissue swelling.

b. Gas in soft tissues. (May also be able to feel crepitation in skin clinically.)

c. Foreign Body. d Trauma

e. Osteomyelitis:

• Periosteal reaction

• Lytic changes with sclerotic border.

• Cortical breaking.

• Osseous destruction

• Sequestrum, involucrum, cloaca, Brodie's abscess (chronic changes). 3. Lag time can be 10-14 days.

26

The following medical imaging modalities should only be used as an atljunct to therapy. They should only be used if the results would alter a treatment pJan or benefit the patient, They should never be relied upon to make a diagnosis. Diagnosis is made by CLINICAL EXAM. Please see the section on Radiology of Infection for complete discussion of each.

B. Nuclear Scinitigraphy (See radiology section).

C. cr Scan. (See radiology section).

D. MRI. (See radiology section).

XII. Laboratory Evaluation:

FIRST LINE TESTS

SECOND LINE reSTS

TESTS·FOR SURGERY

1. CBC with di:ff

2. Chemistry Panel

a. BUN

b. Creatinine

c. Glucose First line tests should be performed upon initial presentation of patient to aid in diagnosis & establish appropriate treatment protocol. Second line tests can be performed after initial presentation. They may help monitor patients progress or help rule-out other sources of infection. Tests for surgery should be performed initially only if patient needs to go to the OR immediately. (Do not orner if they are not medically indicated)

Please see section on "Pre & Postop Management Hospital Charting'" for normal lab values.

A Complete Blood Count (CBC):

1. Hemoglobin (Hgb).

2. Hematocrit (Hct).

3. Platelets (PIts).

4. White Blood Cells (WBC): Usually > 10,000 for infection (Leukocytosis). a. Differential:

1. ESR/CRP

2. Blood Cultures

3. Urinalysis

4. Urine Culture

1. Pf,PIT

2. LFf's, TYr's

3. CXR. 4.EKG

Granulocytes (Contain granules in cytosol)

N~: .

Polymorphonuclear (PMN) cells Band cells

Basophils Eosinophils

Agranulocytes Monocytes Lymphocytes

-----------..~LI'MN'S: Mature neutrophil. Initial killer cell, actively phagocytic. Contains lysozymes & lactoferrin HaIr-life is 6 hours but functions last for 1-2 days. Maturation time is 14 days.

• Band cells: Immature neutrophil. Nucleus is elongated & twisted. It is less deformable & less motile; therefore, less effective in phagocytosis & killing. Increases dtuing acute infection (Left Shift).

• Basophils: Contain heparin. histamine, & other substances to contract smooth muscle &

increase penneability of blood vessels.

• Eosinoehils; Function unknown.

Increased in (NAACP): Neoplasms, Allergens, Addisons, Collagen vascular diseases, Parasites

• Monocvtes: Second line of phagocytic response (replaces neutrophil within 24 hours).

Activated into macrophage at inflammatory site.

A Macrophage is an aggressive phagocytic, bactericidal, & long-lived cell. It processes antigen & delivers them to lymphocytes for specific antibody production.

• Lymphoc;ytes: Possess cellular mediators for immunity.

B cells are produced from bone marrow & produce specific antibody,

T cells are produced from thymus & produce specific sensitized lymphocytes.

27

Laboratory diagnosis of infection:

Leukocytosis fmcrease WBC's) with a shift to tbe left fmcrease band cells).

During acute infection, the body tries to fight the infection by increasing the number of WBC's, specifically neutrophils. Unfortunately, the neutrophils do not have time to mature before they are needed to try to "kill" the infection and band cells are produced.

B. Chemistry Panel: .

I. BUN: Measures hydration state. Less specific for renal function. BUNtCr may be elevated in dehydrated state. Follow levels closely

2. Creatinine: Best hematologic index of renal function. Use to measure creatinine clearance for proper antibiotic dosages.

3. Glucose: Stress of infection may cause an increase & may complicate wound healing.

4. Electrolytes: Na, 1(, Cl, C~.

C. Second Line Tests:

I. Erythrocyte Sedimentation Rate (ESR):

a. Determined by measuring the distance in mm a column of erythrocytes falls in 1 hour.

b. NON-SPECIFIC: inflammation, infection, malignancy, renal disease, connective tissue disease, age.

c. Use as a baseline to monitor effectiveness. Thaw every 5-7 days.

2. C-Reactive Protein: Similar to ESR but more difficult to perform & more expensive.

3. Urinalysis: (+) leukocytes & nitrates (probable UTI). If there are an increase in the squamous epithelial cells, the sample was probably contaminated (need to repeat test). Check urine culture to determine specific organism(s).

4. Blood Cultures:

a. Used to diagnose bacteremia; however, most bacteremias are intermittent & a (+) culture can be difficult to obtain.

b. Take as soon as possible after the onset of a fever (usually 101).

c. Need 2-3 cultures at least 20 minutes apart & from different sites. This increases the chances of catching the organism.

d, Must utilize aspetic technique to avoid skin contaminants.

DECISION MAKING

#1 Does the patient need to be admitted!

• Outpatient versus Inpatient therapy.

• Oral antibiotics versus IV antibiotics,

1. Outpatient Therapy:

a. Localized signs of infection with no systemic manifestations.

b. Benign medical history (patient is not immunocompromised).

c. Can be sent home on OIaI antibiotics.

2. Inpatient Therapy:

a. Extension of infection.

b. Systemic manifestations.

c. Need IV antibiotics.

d Require surgical intervention. e. Immunocompromised host.

• Diabetes

• Peripheral Vascular Disease (vascular surgery consult)

• mv

• Cancer

• Rheumatoid Arthritis

• Steroid Therapy

• Elderly

28

Immunocompromised patients may not be able to elicit an inflammatory response & the infection. may look benign. The typical redness, swelling, & heat may not be present but this does not mean that the infection is localized. Their immune system cannot fight the infection as effectively as a healthy patient; therefore, what may look benign can tum into a rapidly progressing & fatal infection. Caution should be used with these patients and hospitalization even for localized infections should be considered.

#2 Is surgical intervention necessary?

• Bedside debridement versus OR I&D

1. Bedside Debridement: a Localized infection.

b. Neuropathic patients.

c. Better evaluation of the wound

2. Operating Room Incision & Drainage: a Unknown extent of infection.

b. Wound probes to bone or bone is exposed.

c. Wound tracks up fascial planes or tendon sheaths. d Abscess.

e. Gas in soft tissues: Needs immediate I&D.

xm. Treatment:

A Systemic Therapy (Antibiotics):

1. Oral versus N.

2. Choose according to clinical scenario:

a. Diabetes: polymicrobial

b. Cellulitis: Group A Strep, Staph aureus

c. Gas: clostridium

d Foul odor: anaerobes

e. Fruity odor: pseudomonas

f. Interspaces: gram (-)

g. Golden purulence: Staph aureus

3. Start broad & narrow spectrum pending culture results & sensitivities. Good options for

intial therapy are AncefiFlagyl or BactrimJFlagyl or Unasyn___ffi>illyatyJIepeding_ofhosplUi"t3:u.1 _

--------------~nu~cru~.~~~·~=ce~p=ro~:fil~e~)~

4. Obtain cultures before starting antibiotics. May get negative cultures if patient is already on antibiotics,

5. Calculate Creatinine clearance prior to dosing Abx

B. Local Therapy (Wound Care):

I. Incision & Drainage: .

a. Use general anesthesia or spina1 if extent of infection is unknown. Infection may track up leg beyond the boundaries of a local block.

b. Don't use a tourniquet Need to differentiate healthy, bleeding tissue from necrotic tissue. If a tourniquet is used, drop it before final debridement to ensure adequate debridement.

c. Plan all incisions. Final goal is wound closure.

d, Debridement (Remember the 3 D's: debridement, drainage. decompression):

• Do not separate tissue planes. Initial incision should be down to bone.

• Resect all infected, necrotic soft tissue and bone.

• Careful handling of skin edges, especially in patients with PVD.

e. Cultures:

• Soft tissue swabs: gram stain, aerobic, anaerobic, fungal, acid-fast.

• Bone: obtain pieces for culture & biopsy.

• Request sensitivities on all cultures

29

f. Irrigation:

• Need high power for lavage. Pulse lavage apparatus or 60cc syringe with an 18 gauge blunt tip needle. The high power and pressure facilitates debridement.

• Inigant solution is irrelevant, usually use normal saline.

• Adding antibiotics to irrigant solution offers no benefit

g. Wound should be packed open:

• Use plain Nu Gauze packing on smaller wounds & 4X4 gauzes or Kerlix on larger wounds.

• Perform daily dressing changes on wound & monitor for signs of healing.

• When all signs of infection have been irradicated wound can be closed, usually 3-4 days after initial I&D.

2. Daily Wound Care (Bedside):

a. Irrigation,

b. Local debridement.

c. Packing.

d Wet to Dry Dressings:

1. AbsoIbs fluid exudates.

2. Facilitates debridement upon removal.

3. Solutions:

• Saline: isotonic, drying.

• Betadine: drying, antimicrobial, can be caustic to tissues.

• Dilute Betadine: less drying & less caustic to tissues.

• Dakins (hypochlorate, usually %%): antimicrobial, promotes granulation tissue.

• Acetic Acid (usually %%): antimicrobial, good for pseudomonas.

e. Monitor for healthy tissue. Final goal is wound closure.

f. Biologic & Synthetic Membrane Dressing:

1. Not used often, but if desire to use, granulation tissue must be present.

2. Materials:

• Porcine skin graft.

• Amniotic membrane.

• Epiguard: A synthetic membrane with 2 layers. The inner layer is made of

polyurethane. Granulation tissue grows into & attaches to this layer. The outer layer is composed of teflon. This layer is breathable and bacteria impermeable.

g. Elevate limb. This encourages venous & lymphatic drainage to reduce edema & improve local blood flow. Patients with compromised circulation should have legs level in bed, not elevated.

3. Wound Closure:

a. Primary Closure: never use unless all necrotic tissue is excised & no signs of infection exist.

b. Delayed Primary Closure:

• Usually 3-4 days after initial I&D.

• Excise all necrotic, non-viable tissue.

• Obtain bleeding skin edges.

• Irrigation.

• Culture.

• Suture: Avoid closure of deep tissues. Utilize non-reactive materials (Nylon,

Prolene).

c. Secondary Intention: for large wounds that cannot be reapproximated d Skin Grafts (Apligraft}

30

"'Patients with severe PVD require lower extremity bypass surgery or angioplasty prior to closure

Osteomyelitis

1.

Definitions:

A. Hematogenous Osteomyelitis: Usually occurs in children & is primarily a medically treated disease.

When it occurs in adults, it is usually a surgically treated disease. Now increasing incidnce in the elderly >50 yrs

-Primarily long bones LE and the humerus

- Vertebrea in the elderly

-S, Aureus, Strep in neoneates

-GNRs in elderly, immunocompromised patients

-Pseudomonas in IVDAs and long term indwelling urinary catheters

B. Contiguous Osteomvelitis(direct extension): Direct infection of bone from and exogenous source or

spread of infection from a near by infected focus. .

-Prothesis, implants, open fractures primary concerns con

C. Osteomyelitis in a patient with vascular impairment: These patients have difficulty mounting an inflammatory response. They also have poor delivery of antibiotic to the infection site. -vascuIarity needs to be addressed to insure adequet healing

D. Acute Osteomyelitis: Suppurative infection accompanied by edema, vascular congestion, & small vessel thrombosis.

E. Chronic Osteomyelitis: Nidus of infected dead bone or scar tissue, an ischemic soft tissue envelope, & a refractory clinical course.

F. Sequestrum: Piece of necrotic tissue, usually bone, that has become separated from surrounding healthy tissue.

G. Involucrum: The sheath of new bone that forms around a sequestrum.

R Brodie's Abscess: A chronic abscess of bone surrounded by dense fibrous tissue and sclerotic bone.

Lew and Waldvogel, NElM: 336: 1997

II. ClaSsification:

Waldvogel Hematogenous Osteomyelitis Contiguous Focus Osteomyelitis

Osteomyelitis associated with Vascular Disease Chronic Osteomyelitis

Cleroy-Mader

Anatomic Stage

I. Medullary Osteomyelitis 2. Superficial Osteomyelitis 3, Localized Osteomyelitis

__________________________________________________ ~m:~4~.~·~emayclm~-----------Physiologic Stage

A Normal Host

B. Systemically Compromised Host (BS)

Locally Compromised Host (BL)

C. Treatment is worse than the Disease

III. Clinical Evaluation: (please see infection work-up.)

IV. Imaging Modalities:

A. Radiographs:

1. Need 30-50% resorption to detect osseous changes on x-ray. This can take 10-14 days.

2. Always take x-rays for an infection. Can act as a baseline & monitor the regression or progression of the infectious process.

3. Signs (In order by which appears :first):

• Soft tissue swelling.

• Periosteal reaction (thickening or elevation).

• Focal osteopenia.

B. Please refer to Radiology of Infection for bone scans, MRI, & CT.

31

V. Diagnosis:

A. Bone biopsy:

1. Definitive diagnosis.

2. Bone culture & microscopic examination of bone.

3. Utilize imaging modalities to determine best area to take biopsy.

B. Hematogenous Osteomyelitis maybe diagnosed with positive blood cultures witha positive bone scan.

VI. Treatment:

A. Primary Goals:

1. Adequate drainage.

2. Thorough debridement

3. Obliteration of deadspace.

4. Antimicrobial coverage.

B. Primary Surgical:

1. Debridement of all nonviable & marginally viable tissue.

2. Antibiotic Polymet:hylmethacrylate Impregnated Beads (PMMA):

a. Indications for use:

• Deadspaee maintenance followed by bone grafting, bone fusion, or primary closure.

• Debridement & closure.

• Combined debridement & internal fixation procedure.

• Systemic disease which contraindicates adequate systemic levels of organ toxic antibiotics, such as aminoglycosides.

b. Gentamycin is the traditional antibiotic used with PMMA beads because it is not heat labile and the curing process of the PMMA is an exothermic process. Other antibiotics may be used; however, the antibiotic must be added in the dough phase of the PMMA. This . prevents the heat degradation of the antibiotic. Antibiotics that have been used include:

• Gentamycin

• Cepbalosporins- Cefazolin, Moxalactam, Cefotaxime

• Tobramycin

• Vancomycin

• Ticarcillin

c. Procedure:

• Mix & construct the beads on a wire and place in the wound, ------------it--Glose-the-wound-over-the-beads:::-. -------------------------

• Leave the beads in for about 2-4 weeks.

d. Mechanism

• Immediate ans sustained release of antibiotic in the local area

• Release of antibiotic declines exponentially with time

3. Ingress-Egress Systems:

a. Closed suction irrigation. A closed system in which continual flushing of the wound is accomplished through inflow tubing (ingress) & outflow tubing (egress). There are many problems with this system, such as contamination of the outflow tubing (usually pseudomonas).

4. Bypass Surgery: (Consult vascular surgery for options.)

C. Secondary Surgical - Reconstructive:

1. Papineau bone graft: This technique is for filling bone defects. Small cancellous chips are packed into bone deficits when the wound is granular. This graft is given time to take then various closures can be used.

2. Bone Fusion: Used after resection when infections are near articular surfaces.

3. Delayed primary closure.

4. Tissue transfer/Skin grafting.

5. Bead therapy, bone grafting, & closure.

32

vn. Follow-Up:

A. Goal is to prevent recurrence.

B. Antibiotics:

I. Customarily 6-8 weeks following last surgery or since initial clinical resolution of infection when no surgery has been performed.

2. The word "cure" is inappropriate. It is possible for an area to activate for up to 20 years.

3. Follow-up intervals: .

• First 6 months: every month.

• 6 months-I year: every 3 months.

• I year-2 years: every 5 months.

Local Antibiotics/Antibiotic Beads

The implantable admixture of antibiotic to any of several carriers for the purpose of localizing increased antibiotic concentrations at specific target tissue sites.

Selected antibiotic should meet the following criteria:

A H2O soluble

B. nontoxic to tissue

C. bactericidal (preferably)

D. available in powder form

E. heat stable ifused with PMMA

Carriers may be:

A. biological

I. cancellous bone graft

2. demineralized bone matrix

3. Ca ++ hydroxyapatite B. biologically inert

1. polymethylmethaaylate (PMMA)

2. plaster of paris

* commercially prepared antibiotic carriers are available in Europe, and are expected to be available in the United States after further investigation of possible toxicities

----------Mrclmrend comparoollie eluhon of tobiamycm from various carriers, and found:

A cancellous bone graft eluted 70% of its antibiotic within the first 24 hours of implantation. and

had non-measureable levels at 14 days .

B. demineralized bone matrix released 45% of its antibiotic within the first 24 hours of implantation, and had non-measurable levels at 14 days

c. plaster of paris eluted 17"/0 of its antibiotic in the first 24 hours after implantation, with measurable levels of antibiotic up to 21 days post-implantation

D. PMMA eluted 7% of its antibiotic within the first 24 hours of implantation, with trace amounts measurable up to 14 days post-implantation

PMMA, the most commonly used carrier for implantable antibiosis, usually comes in half-packs (20 g) or full-packs (40-60 g); consists of

A monomer (liquid)

B. polymer (powder)

* the monomer and polymer are mixed together (an exothermic reaction) with the antibiotic powder until a doughy consistency is achieved, at which time small beads are fashioned and placed on 26 or 28 gauge wire like pearls on a necklace. The hardened beads are then implanted into a dead space in which high local levels of antibiotic are desired, typically within an area of debrided osteomyelitic bone or the space previously occupied by osteomyelitic bone after resection. (N.B. no one wearing contacts should be in the surgical suite during preparation of the beads, as offensive vapors have been linked to morbidity with those individuals)

33

Suggested Ratio of Antibiotic:PMMA

Antibiotic Amount of Anttbiotic Amount ofPMMA
Cefazolin 4-S g 40-60 g
Cefotaxime 4-Sg 40-60g
Nafcillin 4-Sg 40-60 _g_
Tobramycin 5-10 g 40-60g
Vancomycin 4-Sg 40-60g
Ticarcillin 6-13g 40-60g
Clindamycin 4-Sg 40-60g Diffusion (Elution) of Antibiotic from PMMA depends on:

A. type of cement used (Palacos vs. Simplex: vs. CMW)

B. coefficient of diffusion for each antibiotic

C. concentration of antibiotic within the bead

D. size/surface area/roughness of the bead

E. amount of fluid that moves past the bead

Bead Properties

A. smaller beads = more surface area to total volume, leading to ina-eased elution rates

B. higher concentrations of antibiotic within a bead leads to increased pore size and roughness,

each leading to increased rates of elution .

. C. Ciemy proposed a ratio of 1:5 when adding antibiotic powder to the PMMA powder; e.g. for every 109 of powdered PMMA polymer, add 2 g powdered antibiotic, stating that any higher ratio would prevent proper hardening of the PMMA

D. most studies conducted with 1-2 g antibiotic to 40-60 g PMMA in literature

Complications with Antibiotic-Impregnated PMMA Beads: very few

A if the cement is used for fixation, mechanical strength is compromised with greater than 100/0 antibiotic content (not an issue with beads)

B. care should be used in patients with renal disease, though local antibiotic concentrations 10-50 times greater than the toxic serum levels can be achieved without the serum ever absorbing enough to achieve those toxic levels

C. no allergic reactions have been reported to date in the literature

Disadvantages

A second procedure required for bead removal, typicallyafter 10-14 days

SEPTIC ARTHRITIS

Infectious arthritis stands apart from other rheumatic disorders because cure rather than mere control is possible. Whether or not this goal can be achieved depends upon the type of organism, promptness in initiating . therapy, medical status of the individual, and prior status of the involved joint Almost any pathogen may affect articular cartilage and periarticular tissues. A multitude of bacteria, viruses, mycobacteria, and fungi have been associated with arthritis.

MECHANISMS OF SEPTIC ARTHRITIS

1. DIRECT JOINT INVASION BY THE MICROBE: pathogens may invade the joint directly and produce on-going inflammation and destruction. Examples include direct joint innoculation (puncture WOWld) or hematogenous spread.

2. JOINTS MAY BE AFFECIED INDIRECTLY AS A RESULT OF:

34

A the resulting immune response of the affected host. For example, in Hepatitis-B arthritis circulating immune complexes appear to mediate the polyarthritis that accompanies the prodromal stage of this infection.

B. microbial debris that may persist in the joint and perpetuate an inflammatory response, even after the organism is no longer in the joint This appears true in some cases of gonnococcaI arthritis in v.hich an ongoing synovitis is associated with sterile joint fluid that is non-inflammatory, so called n post-infectious

sterile arthritis. " .

c. unknown mechanisms. This applies to almost all of the reactive arthritides, such as those associated with gastrointestinal infectioos with some of the enteric gram negative organisms (Shigella sp .. Yersinia sp.).

ACUTE BACTERIALARTBRITIS

PREDISPOSING FACfORS AND ASSOCIATED CONDmONS

I. Rhewnatoid arthritis

possible mechanism. for association damaged joint is a good nidus for infection compromised defenses from medications defective phagocytosis (1)

important clinical features usually due to Staph. aureus treatment results often poor

2. Crystal-induced arthritis

possible mechanism. for association

synovial fluid acidosis may promote crystal deposition enzymatic n strip-mining" of cartilage

important clinical featmes

identification of crystals in the joint does not rule out septic arthritis

3. Severe osteoarthritis, Charcot joints, Hemarthroses

possible mechanism for association

• joint d.isorgani7at:ion, chronic synovitis, and bloody effusions all provide nidus for bacteria

important clinical features

• always send bloody joint fluid for culture

4. Chronic systemic disease (SLE, sickle cell anemia, cancer, etc.)

possible mechanism for association

• impaired generalized defenses due to chronic illness, phagocytic deficiencies, and medications

important clinical features

• often due to Staph aureus or gram negative bacilli

• consider Salmonella sp. in patients with sickle cell

35

ACUTE BACTERIAL ARTHRITIS

PREDISPOSING FACfORS AND ASSOCIATED CONDmONS

1. Rheumatoid arthritis

possible mechanism for association damage4joint is a good nidus for infection compromised defenses from medications defective phagocytosis (1)

important clinical features usually due to Staph. aureus treatment results often poor

2. Crystal-indoced arthritis

possible mechanism for association

synovial fluid acidosis may promote crystal deposition enzymatic " strip-mining" of cartilage

important clinical features .

identification of crystals in the joint does not rule out septic arthritis

3. Severe osteoarthritis, Charcot joints, Hemarthroses

possible mechanism for association

• joint disorganization, chronic synovitis, and bloody effusions all piovide nidus for bacteria

important clinical features

• always send bloody joint fluid for cultu:re

4. Chronic systemic disease (SLE, sickle cell anemia, cancer, etc.)

possible mechanism. for association

• impaired generalized defenses due to chronic illness, phagocytic deficiencies,. and medications

important clinical features

• often due to Staph aureus or gram negative bacilli

• consider Sa1monel1a sp, in patients with siclde cell

PATHOGENESIS

Acute bacterial arthritis occurs most often by hematogenous dissemination from a primary source of infection. However, joint sepsis may also result from the extension of an a<ijacent soft tissue infection or aQjoining osteomyelitis.

Three stages of cartilage destruction have been described:

36

1. Initial events include liberation of lysosomal enzymes from PMN's and synoviaiJining cells resulting in the loss of proreoglycan .from cartilage. If the infectious process is resolved early, a restoration of proteoglycan matrix.may be achieved and chondrocyte damage avoided.

2. During the second stage. increased mechanical stress and inadequate nutrition result in chondrocyte damage.

3. In the last stage, enzymes released :from PMN's and synovial lining cells with the altered joint mechanics gradually destroy the collagen network.

CLINICAL FEATURES

Septic arthritis usually affects one joint, but may involve two or more joints. The involved joint is generally red, hot, swollen and acutely painful. There is also extreme pain and limitation with range of motion. The knee followed by the hip and ankle are the most commonly involved joints in non-gonococcal septic arthritis.

Systemic features include fever ( may be low grade ), chills and rigors. A careful physical exam may reveal an infectious focus involving any site, but the usual sources include the skin, nasophaIynx. sinuses, lungs, cervix, and the rectum.

JOINT DISTRIBUTION

JOINT

%OFCASES

ADULTS

CHILDREN

knee 55 40
hip 11 28
ankle 8. 14
shoulder 8 4
wrist 7 3
elbow 6 11
others 5 3
LABORATORY TESTS Peripheral leukocytosis occurs in the majority of patients with acute bacterial arthritis. An elevated ESR is also generally found

~-----Patients suspected of haVlllg septic arthritis ShOUld have blood cuhures and cultures of regions that may be the primary source of infection ( physical examination will help to determine areas that may need to be cultured ). Blood cultlires may be positive in up to 75% of patients.

A diagnosis of septic arthritis is established by gram stain and a positive synovial fluid culture. A typical joint infection demonstrates a purulent effusion with a synovial fluid white count greater than 50,000 celisIcnun and more than 9()01o PMN's. Gram stain and culture are obligatory if septic arthritis is suspected.·

INCIDENCE OF ORGANISMS IN ACUTE BACTERIAL ARTBRITIS IN ADULTS

Gram-positive cocci

45%

Gram-negative cocci ( N. gonorrhoeae )

50010

Gram-negative bacilli

5%

TREATMENT

1. Treatment should begin immediately after the suspicion and/or confirmation of the diagnosis.

37

2. Antibiotics are chosen primarily on the basis of the synovial fluid gram stain and are acljusted based on the culture results.

3. Closed needle aspiration should be performed at I~ once daily to drain the affectedjoint.

4. Open smgical drainage is indicated if there is a lack of response to therapy ( joint :fluid cu1tmes remain persistently positive or patients remain febrile after 34 days of antibiotic therapy ); Some studies espouse innnediate open surgical drainage.

5. The infectedjoint should be kept at rest to prevent mechanical stress; generally usmg a splint will provide

adequate immobilization and reduce pain and trauma. _

6. As joint symptoms resolve, passive range of motion exercises should be started to avoid joint contractures.

7. Weigb1bearing should be deferred until inf1alJ'llMtion has completely resolved.

SYNOVIAL FLUID EXAMINATION (IMPORTANT POINTS)

Cultures are nearly 100010 positive in non-gonococcal bacterial arthritis, but only 25-500/0 positive in gonococcal arthritis.

Gram stain smears are aAxoximatcly 15% positive with gram positive cocci, 50010 with gram negative bacilli. and less than 25% in gonococcal artluitis.

The leukocyte and differential leukocyte counts are generally greater than 50,000 ceDslcmm and greater than 80% PMN's, but these results can also occur with noninfectious inflammatory arthritis.

Synovial fluid glucose is less than 5OO1o:fasting, simultaneous serum glucose but may not be present and may be seen with Rheumatoid arthritis.

RADIOGRAPmc TESTING

Radiographic examination of affected joints may reveal only soft tissue swelling early in the comse of the disease. Juxta-articular osteopenia appears about 7-10 days after the onset ·of joint infection. If tbe joint infection is not eradicated, progressive joint space narrowing may develop. Late l3diographic finding-; include joint subluxation and eventual joint destruction.

Radioisotope scanning may be helpful in localizing sites of infection, however the findings are non-specific and ________ _::radi=·o=nuclide=·=-=-.una.=c.· =~ generally has little role in the initial evaluation o~.e_septi~~-------~

RADIOGRAPHIC-PAmOLOGIC CORRELATION

PAmOLOGIC ABNORMALITY

RADIOGRAPHIC ABNORMALITY

edema and hypertrophy of soft tissue

joint effusion, swelling

synovial membrane with fluid production

hyperemia

osteoporosis

inflammatory pannus with chondral destruction

joint space loss

pannus destruction of bone

fibrous or bony ankylosis C~DHOODSEYnCARTInUTIS

marginal and central erosions bony ankylosis

BACTERIA (# OF CASES) < 2 YRS

2-6YRS

>6YRS

10TAL

38

S_ aureus 49 36 81 166(34%)
R influenza 154 19 1 174(36%)
Streptococci 27 23 24 74(15%)
Gramneg_ 24 9 6 39 (8"10)
bacilli
S. pneumoniae 23 4 3 30(6%)
482 From series by Fink and Nelson. Speiser et al., PeltoIa and Vahvaven, Borella et el., and Samilson et al,

39

INCIDENCE OF ORGANISMS IN CHILDHOOD SEPTIC ARTHRITIS

Gram-positive cocci

70010

Gram-negative cocci

5%

Gram-negative bacilli

<15%

H. infIuenzcl

10010

GONOCOCCALAR~

(DISSEMINATED GONOCOCCAL INFECflON)

Gonococcal arthritis is the most common fonn of acute bacterial arthritis. Joint involvement occurs during dissemination of the gonococcal infection from a primaIy source of infection.

CLINICAL MANIFESTATIONS

The organism irritially infects nmcosal cell smfaces. Typically the primaIy infection involves the reproductive organs, but may involve the phaIynx or rectum.

Disseminated gonococcal infections occur in approximately 1-3% of all primary infections, Dissemination seems to occur more commonly in females, generally around the time of menstruation. *** An important note is that patients with complement deficiencies are at an increased risk of gonococcal bacteremia,

Most patients with disseminated gonococcal. infections are less than 40 years of age and relate a history of promiscuity and unprotected sexual intercourse. Again the primary sites of infection are the urethra in males, cervix in females, anfthe rectum. and pharynx in both sexes.

Patients di.c;seminated disease may have systemic finding:; that include fever, cbills, and malaise. A migratort polyarthritis is the most common initial joint manifestation, and subsequent finding" may localize to one or

~~~ __ ~~~m""o~re"-..Jj,-=oi=n=k:..:Any=[joint may be involved.

Peri-articular pain and swelling are common, and tenosynovitis is found in a significant number of patients. Typical skin lesions are a frequent manifestation, generally beginning as small erythematous macules, which may develop into pustular lesions characterized by a grey, necrotic center with a hemhorragic base.

LABORATORY TESTS

Peripheral leukocyte counts may be normal or modeIateIy elevated. Synovial fluid analysis reveals an inflammatory fluid with a variable nmnber of white cells ranging from a few thousand to several htmdred thousand per cubic millimeter. Joint fluid cultures are positive in less than halfof all patients even under ideal conditions.

DIAGNOSIS

Disseminated infection should be suspected in young, sexually active [patients who develop an acute migratory polyarthritis, tenosynovitis, and/or a characteristic rash, Primary source and joint cultures along with synovial fluid analysis should be performed.

40

TREATMENT

1. Antibiotics: Ceflriaxone ( Rocephin ) IMIIV or Ceftizoxime ( Cefizox ) IV or

Cefotaxime ( Claforan ) IV then, Cefuroxime axetil ( Ceftln ) po or Amoxicillinlclavu1anate ( Augmentin) po or Ciprofloxacin ( Cipro ) po

Parenteral antiobiotics are genem1ly continued for 24-48 hours until symptoms begin to resolve, and then patients are switched to enteral antibiotics to complete a 7-10 day course of therapy.

2. Joint aspiration

3. Joint immobilization

VIRAL ARTHRITIS

Viral arthritis often begins with non-speci:fic symptoms such as malaise and fatigne, headache, neck stiffness, sore throat, and nausea and vomiting. Joint involvement is generally polyarticular, and the arthritis is usually short-lived and rarely results in joint damage. Laboratory tests are non-specific. The following are some of the most connnon causes of viral arthritis :

1. Hepatitis-B - joint manifestations may be seen in 10-30010 ofpatiems. The arthritis is generally self-limiting,

2. Rubella - may occur in natural rubella infection or after innmmization with the live attenuated virus.

3. Parvovirus B 19

4. HIV related arthropathy

FUNGAL ARTHRITIS

A wide variety offungi may cause joint infection, which may arise either from direct extension from a focus of . osteomyelitis or by hematogenous dissemination The arthritis is usually monolpauciarticu1ar. most often in large weightbearing joints. and generally follows a chronic or indolent course. Synovial fluid analysis and culture are variable, often providing little to no diagnostic value. Fungi that may be associated with infectious

arthritis may be grouped as superficial or deep organsims, Although normal hosts may develop fungal arthritis, there is usually some predisposing fuctor that increases suscepb.bility to infection The diagnosis can be made by synovial fluid culture or synovial tissue biopsy.

SUPERFICIAL Sporothrix schenckii Candida albicans Actinomyces israelii " Maduromycoses H

DEEP

Aspergillus fumigatus Histoplasma capsu1atum. Cryptococcus neoformans

Coccidioides immuns, Blastomyces dermatitidis

MYCOBACTERIAL BONE AND JOINT INFECTIONS

ExtrapuImonary tuberculosis occurs in approximately 1-2.6% of patients with tuberculosis.

. It is generally believed that skeletal 1B is caused by dissemination of bacilli by hematogenous spread or lymphatic drainage from another area of TB. TB arthritis most frequently involves weightbearingjoints and is usually monoarticular. A positive tuberculin skin test is obtained in most patients, and in about 50% a previous personal or fiunily history is present.

Synovial pathology reveals a chronic granulomatous reaction with giant, langhan-type cellular infiltration. Acid-fast staining of the synovium or smrounding tissue may reveal the organisms.

41

Radiographically the affected joint may reveal only soft tissue swelling or more advanced changes with destruction of peri-articular bone, narrowing of the space, and osteoporosis. Chest X-ray abnonnalities of past or present 1B may be present in SOOIo of patients.

ARTHROCENTESIS AND SYNOVIAL FLUID ANALYSIS

Synovial fluid is an invaluable source of diagnostic information One of the most impOltaIJt reasons to perfonn synovial fluid analysis is to rule out. bacterial infection in severely inflamed joints. There is no other sufficiently reliable way to differentiate septic art1nitis from acute crystal-induced arthritis. Arthrocentesis may be therapeutic as well as diagnostic. For tense effusions in which the intra-articular pressure is high. removal of the fluid will relieve symptoms and at least theoretically decrease joint damage

TECHNIQUE

I. The joint should be moved through its range of motion to resuspend its contents.

2. Anatomic landmarks defining the joint surface should be identified and may be outlined with a pen or marker.

3. The specific area of the joint to be aspiIated is identified and may be marked with the retIacted portion of a

bill point pen.

4. The skin should be cleansed of obvious dirt with soap and water. S. The skin can be infiltrated with a local anesthetic ..

6. A brief scrub with a betadine solution followed by an alcohol swab should provide adequate protection.

7. Do not touch the skin at the aspiration site after cleansing!

8. It is a mistake to select a small needle with the intention of sparing the patient pain VISCOUS material flows

with great difficulty, if at all, through needles smaller than 20 gauge. .

9. Stretch the skin slightly, penetrate the skin, aspirate gently, and advance the needle slowly until fluid appears in the syringe.

10. After removaloftbe needle, apply gentle compression at the puncture site for several mimrtes Then apply

a band-id or light dressing.

C. COMPLICATIONS OF ARTIlROCENTESIS

1. Iatrogenic infection of a previously sterile joint (it bas been estimated. that infection occurs in less than 1 in

10,OOOinstancesofdiagQostic~.J'.__ _

2. Bleeding at the puncture site and presumably within the joint; in anticoagulated patients who develop acute

arthritis, anticoagulation itself is not a contraindication to arthrocentesis.

3. Possible iJYmy to cartilage by the needle.

4. Occasionally patients may experience a vasovagal ( syncopal ) episode during or after the procedure.

SYNOVIAL FLUID ANALYSIS

I. GROSS EXAMINATION

I. VOLUME: can serve as one measure of the severity of a process, but low volume does not mean the

absence of an important intra-articular process,

2. VISCOSITY : fluid of normal viscosity holds together and stretches approximately one inch. before separating. Low viscosity fluid drops from a syringe like water. VIscosity is generally decreased in inflammatory joint fluids.

42

3. COLOR .AND CLARITY: if newspaper print cannot be read through the fluid, it generally suggests an

inflammatory process. The plastic of some syringes makes fluids appear falsely cloudy, so examine the

fluid in a glass container (ie. test tube ). Normal and noninflammatory joint fluids are a transparent straw or

yellow color depending on the amount of albumin or bilirubin present.

n. MICROSCOPIC EXAMINATION

1. WET MOUNT: Useful for identification of cells, cartilage fra~ lipid droplets, cytoplasmic inclusions, and some crystals. Sickled red ceIls may be seen in effusions of patients with sickle c:en disease.

2. CRYSTAL ANALYSIS: A polarizing light microscope provides the gold standard for crystal identification.

Monosodium urate crystals of gout are needle shaped or long with blunt ends, and disp1ay a strong negative birefringence. CPPD crystals display a weak positive birefringence. The number of crysIals associated with human disease is increasing and includes calcium oxalate associated with renal failare, crystals or protein in dysproteinemic states, cholesterol crystals in chronic inflammatory diseases. and apatites.

3. GRAM'S STAIN : Useful iftbere is any suspicion of joint infection. Based on gram stainfindin~ bacteria can classified into broad groups to allow empirical therapy. The following steps are used in pIeparing a slide:

A flame the smear R add crystal violet

C. Gram's iodine

D. wash with 95% alcohol

E. safianin

4. OTIIER. STAINS:

Prussian blue: stain for iron that may show iron in synovial lining cells in pigmented viIIonodular synovitis or hemochromatosis.

Ziel-Nielson : may be helpful in evaluating possible

tUbercUlOSIS.

Congo red : amyloid deposits show an apple-green birefringence on polarized light examination.

Alizarin red S : a calcium stain used for apatite crystals.

ill. SPECIAL LABORATORY reSTS

1. CULTURES: if joint infection is suspected, the fluid should be sent for culture and sensitivity. The following type of cultures can be sent: aerobic, anaerobic, gonococcal, fungal, and tuberculosis.

2. MUCIN a...ar lEST: several drops of synovial fluid are added to about 20 ml of 5% acetic acid in a small beaker allowing one minute for a clot to form. A " good clot " from normal or osteoarthritis fluid forms a firm mass that does not fragment on shaking. A " poor clot n like that from many inflammatory fluids fragments

easily and forms :flakes, shreds. and cloudiness in the sunounding fluid " Good mucin clot " generally

reflects the normal integrity ofhya1uronate.

43

3. LEUKOCYIE COUNT AND DIFFERENTIAL: will provide information about the level of joint inflammation.

4. GLUCOSE: synovial fluid glucose concentmtion is normally sligbtly less than that of blood glucose. A very

low level of glucose in the synovial fluid suggests joint infection. Occasional effusions in Rheumatoid

arthritis have a synovial fluid level of less than half that ofblood

·RHEUMATIC DISEASES CHARACTERIZED BY SYNOVIAL FLUID ANALYSIS

NONINFLAMMATORY Osteoarthritis

Trauma

Osteochondritis dessicans Neuropathic arthropathy Sickle cell disease ' Osteochondromat Hypertrophic osteoarthroplth Ochronosis

Hemochromatosis Acromegaly

Amyloidosis

INFLAMMAIDRY Rheumatoid disease

Reiter's syndrome

Psoriatic arthritis

Ankylosing spondylitis Ulcerative colitis

Acute aystal synovitis Systemic lupUs erythematosus VIral or fungal infections

SEPTIC

Bacterial infections

HEMORRHAGIC

Trauma with or without fracture Postsurgical

Pigmented villonodular synovitis Synovioma

Neuropathic arthropathy Hemangioma

Coagulation disorders

Hemophilia

von Willebrand's disease Therapeutic anticoagulation

------,Sic1de-eell-4mj· t;a:;c;__---------------------------.---~ Thrombocytopenia

Eblers-Danlos syndrome

Scmvy

CLASSIF1CATION OF SYNOVIAL EFFUSIONS

EXAM NORMAL· NONINFLAMM. INFLAMM. SEPTIC
color straw strawlyellow yellow variable
WBC(mmJ) <200 200-2,000 2,000-75,000 >100,000
PMN(%) <25 <25 >50 >75
culture (-) (-) (-) often(+)
mucin finn finn friable . friable
clot 44

Tuberculosis of BODes and Joints

Amer Thoracic AssocAmerJ Resp and Clin Care Med, 149: 1994

INTRODUCTION

1. The prevalence of tuberculosis has been rising since 1986, with morbidity increasing 14% from 1985 to 1993.

2. Urban areas of developed countries are more likely to encounter patients who have tuberculosis

3. Factors that contribute to increased rate include:

a. suppression of the inunune system

b. development of drug-resistant strains of Mycobacterium

c. aging population

d increased number of health-care workers who are exposed to the disease

4. mv remains the leading known risk for the reactivation of latent tuberculosis infection

5. Spinal tuberculosis has existed for at least 5000 years

6. Pott noted the association between tuberculosis involvement of the thoracic spine and paraplegia

7. The sanitarium setting, in the era before antitubercular drugs, was considered to be successful treatment in that era, still had a 60% mortality rate within six years after discharge from the sanitorium

THREE RELA1ED ORGANISMS

1. Mycobacterium tuberculosis - most common

2. Mycobacterium africanmn - found outside Northwestern Africa

3. Mycobacterium bovis - found in areas not utilizing milk pasteurization

MYCOBACTERIUM TUBERCULOSIS

.....fuin rod, with round ends, nonmotile, without capsule, resists decolorization with strong mineral acids and alcohol; hence, is considered an acid-alcohol-fast or acid-fast bacillus.

-grows only on enriched medium containing egg and potato base or sennn (albumin) base

-visible colonies appear at around 2 to 4 weeks, secondary to oxygen tension. Increased oxygen tension, as

in the lung, allows the organism to grow :freely.

INCIDENCE

-one-third of the global population is infected with Mycobacterium tuberculosis

-M tuberculosis is the cause of 3 million deaths per year

-ten million persons are presently infected. and 90% of new activated cases come from this infected pool of

individuals

-In non-Hispanic white people, the median age at diagnosis is 61 years

-among the American minority, the median age of diagnosis is 39 years

-one-third of patents with tuberculosis who are also infected with mv will have extrapulmonary disease,

with or without a pulmonary component

SITE OF INFECTION

-Tuberculosis has been reported in all bones of the body

-In the U.S., the spine is involved 500/0 of the time

Thoracic spine 500/0

Cervical spine 25% Pott's Disease- TB of the vertebra

Lumbar spine 25%

-Other reported areas are less common,

pelvis 12%

hip & femur 10%

knee & tibia 10%

ri~ 7%

ankle or shoulder 2%

elbow or wrist 2%

multiple sites 3%

-extrapulmonary tuberculosis is more common in children than in adults

-most common extrapulmonary involvement in children is the superficial lymph nodes(scrofula)

45

DIAGNOSIS OF MUSCULOSKELETAL TIJBERCULOSIS CLINICAL

1. Localized pain

2. Associated fever

3. weight loss

4. tnmcal rigidity

5. muscle spasm

6. neurological signs

7. cold abscess (swelling without inflammation) - strongly suggestive of TB osteomyelitis

® Tubercular skeletal lesions demands further evaluation of other organ involvement, i.e. lungs, intestinal tract, and kidneys. One-third of patients with bone or joint 1B have a history of pulmonary involvement

PLAIN RADIOGRAPHS

l. NO SPEClF1C RADIOGRAPIllC FINDINGS 2.~

3. soft-tissue swelling

4. minimal periosteal reaction

5. narrowing of joint space

6. cysts in bone acljacent to joint

7. enlargement of the epiphySis in children

8. subchondral erosions

9. new-bone formation

BONE SCANS - not tembly heJpful SKIN TESTS

-Koch discovered the material tubercnlin that was the antigenic component of Mycobacterium tuberculosis. This was later precipitated out to reveal the purified protein derivative (PPD) -Interpretation of the PPD test depends on exposure to M tuberculosis, immunosuppresion of the host and previous exposure to bacille Calmette-Guerin (bCG).

-at least 200/0 of debilitated or malnomished patients who have extensive disease have a falsenegative skin test.

-HIV infected patients with concomitant TB are prone to be anergic and wUltberefore_proridea-----

-------------n~tiv~~~

OTIIER TESTS

l. ESR - neither specific nor completely reliable

2. ELISA (enzyme-linked immunosotbent assay) - reported sensitivity of 60 - 800/0, but these

tests may be negative for patients who have advanced disease.

3. Chromatography-not widely available

4. Nucleic Acid Probes-not widely available

5. Polymerase Chain Reactions-not widely available

CULTURE

-ultimate diagnosis depends on recognition of Mycobacterium tuberculosis on either histologic study or culture or both.

BIOPSY

-Biopsy and possible operative treatment are reserved for patients who fail to respond to adequate

chemotherapy, who have substantial neurological impairment(as in TB infected vertebrae), or in whom either resistant strains or other disease entities are suspected

46

TREATMENT

1. If patient is suspected of having tuberculosis, that patient must be placed in an isolation room

2. operative intervention should be delayed until the patient is no longer infectious

3. Medical treatment

a. contact Center for Disease Control for most recent guidelines

b. consult an in:fectious-disease speciaJist

4. Antibiosis

a minimum of three drugs to which the organism is susceptible

b. at least one of these drugs must be bactericidal

c. Possible antibiotic selection includes, but is not limited to,

Isoniazid 3-5 mglkglday

Pyridoxine 10 mg QD

Rifampin 10 m~day

Pyrazinamide 20-25 mglkglday

Ethambutol 15-25 mglkglday

Streptomycin15-20 m~day

5. Optimum duration of treatment, 6-9 months for patients with pulmonary involvement alone.

Patents with extrapulmonary tuberculosis require 12-18 months of treatment

MANAGEMENT ofHIV

L Definition: a human retrovirus that infects lymphocytes and other cells bearing the CD4 surface marker. Infection leads to lymphopenia, CD4lymphocyte deficiency and dysfunction, impaired cellmediated immune response, and polyclonal B-cell activation with impaired B-cell response to new antigens.

I. AIDS: characterized by opportunistic infections and unusual malignancies

ll. Standard of Care

1. Document CD4 count every 3-4 months as long as CD4>50. Do not need to follow CD4 count<50.

Viral load testing every 3-4 months.

2. All mv -infected patients should have the following regardless of CD4 count A PPD/anergy panel

1. If PPD is (+) give INH for I year.

2. Anergy panel-Checks the ability to host an immune system response. Want it red and

raised,

B. RPRIFI'A: IfRPR is reactive, get an LP.

C. Pneumococcal vaccine: Get every 3-5 years.

D. Influenza) shot: Get every year. 1bis is controversial.

E. Hepatitis screen and heptavax series if HBV serology is negative.

F. Cervial pap smear for all women eveyr 6-12 months.

G. Toxoplasma titer.

3. CD4 of 500 begin combination antiretroviral therapy using:

A AZf 200mg TID + a second reverse transcriptase inhibitor +1- a protease inhibitor.

1. AZf: can cause megaloblastic anemia, neutropenia, GI effects, anxiety, dark blue nails.

B. Other nucleoside reverse transcriptase inlnbitors include:

1. DDI: can cause pancreatitis, peripheral neuropathy.

2. DOC: can cause mouth ulcers, peripheral neuropathy.

3. D4T

4. 3TC: has the least amount of side effects.

C. Protease inhibitors; (saquinavir, indenovir, nalfinevir, ritonavir, .amprenovir)

1. Decrease viral load markedly and prolong life.

2. Be selective with patient: Have to be compliant with medication. If doses are missed resistance can form

47

D. Nonnucleoside nbonucIease reverse transcriptase inhIbitors:

1. viramine

2. deIavadine

3. efavirenz

4. CD4,200 begin PCP (Pneumocystis carinii pneumonia) prophylaxis:

A PCP prophylaxis should be started regradless of CD4 count if patient has oropharyngeal candidiasis . (thrush), fevers, weight loss/wasting syndrome.

B. PCP prophylaxis drug therapy:

1. Bactrim I DS on Mon., Wed, and Fri. This is the drug of choice .: Can cause rash, fever, neutropenia, GI effects, liver dysfunciton.

2. Dapsone: Second drug of choice. Must check for G6PD-can cause hemolytic anemia.

May also use dapsone plus pyrimethamine.

3. Aerosolized pentamidine: Third drug of choice. Can still get PCP in upper lobes. Can cause cardiomyopathy, damage to retina. Can also give monthly IV pentamidine.

C. To diagnose PCP:

1. LDH wil be increased

2. If gallium scan is (=) possible PCP, if (-) definitely Nor PCP.

3. Get ABG, ifp02<75, start tapering doses of steroids (ie, prednisone 40mg BID x 5 days,

40 mg QD x 5 days, 20 mg QD x 5 days). .

4. Bronchoscopy.

D. PCP tretment

1. Bacttim

2. Pentamidine: Second drug of choice. Must correct for renal insufficiency. Can cause pancreatitis, life-threatening hypoglycemia and hypertension.

5. CD4<75 begin MAC (Mycobacterium aviwn complex) prophylaxis:

A Rifabutin: Drug of choice for prophylaxis. Can cause GI effects and hepatotoxicity .. Second line choices include clarithromycin or azithromycin.

B. Can cause bone marrow suppression and pancytopenia Need to check AFB in blood May also be found in respiratory or GI tract

C. Treatment for MAC is for life. Usually c1arithromycin + one other drug (ethambutol. clofazimine, ciprofloxacin, rifabutin).

6. CMV (cytomegalovirus): ---~A--R.etinits;__Most-common-p1ace.it.presents.-Usua1l}Lpatientis.at.endstage-disease.-TreatforJife

B. May be found in bone marrow, liver, urine, blood.

C. Treatment (There is no prophylaxis available):

I. Gancic1ovir. Can cause bone marrow suppression and neutropenia Oral form can be used for CMV colitis.

2. Foscamet: Assocaited with renal failure and electrolyte abnormalities. May have some anti-retroviral properties.

D. Recurrences occur frequently and require reinstitution of high dose induction therapy.

E. +Note-CMV, MAC, lymphoma all can present with fever, bone marrow suppression, pancytopenia, and an increase in LFf's.

7. Kaposis

A Can be found in skin, mouth, GJ tract, liver (can cause biliary obatruction), lung (these bleed easiIy- prognosis very poor).

B. Treatment is chemotherapy. Intralesion interferon can be injected for skin lesion.

8. TB

A Rise with increase in lllV. With this see an increase in extra-pulmonary TB.

B. If patient has been incarcerated, homeless, or lived in a shelter, should be tested for TB.

C. Treatment for patients with HIV and TB should be for one year.

9. Peripheral neuropathy:

A About 50% of lllV patients will have this. B. Treatment:

I. Elavil: as much as their blood pressure can tolerate.

48

2. Morphine.

3. Mexilitine.

10. Otherpossible infections:

A Toxoplasmic encepahlitis: Prevention-do not eat raw meat, wash all vegetables, stay away from

cats. Prophylaxis is Bactrim or dapsone plus pyrimethamine.

B. Cryptosporidosis: Found in contaminated drinking water. No available prophylaxis.

C. Cryptococcus: Avoid sites contaminated with pigeon droppirigs. Treatment is fluconazole.

D. Coccidiomycosis: Found in soil, farms. Prefened treatment is fluconazole .. Alternate drugs include itraconazole, ketaconazole, amphotericin B.

E. Histoplasmosis: Found in chicken coops, caves, bird roosting sites. Treatment is fluconazole.

F. Candidiasis: Found on mucosal surfaces and skin. Treatment is an om azole (fluconazole).

Can also use topical nystatin or clotrimazole.

Herpes Simplex: Treatment is acyclovir unti.llesions have resolved. Can use IV

foscarnet for strains resistant to acyclovir.

H. Varicella-Zoster: Effectiveness of acyclovir is questionable. 1. Human Papillomavirus: May need biopsy of cervix

1. Bacillaty angiometosis: Looks similar to Kaposis. Treatment is erythromycin. K. Seborrheic Dermatitis.

NEUROLOGY

Neurologic disOIders may lead to various lower extremity manifestations including cavus deformities, equines, pes planus, spasticity, paralysis, gait abnormalities and diminished sensation

I. DIAGNOSTIC WORKUP:

LHistmy of illness

- Should be obtained from all available sources.

- Utilize a problem oriented format ie.NLDOCAT.

a Patient

b. Family/Guardian

if patient is a child, ascertain pregnancy course de1iveIy, APGAR score, developmental milestones.

~~~~~~~~~~~~iliare~~

2.Past medical history

3.Past surgical history

4. Current medications

5.Family history

6.Social history - occupation, EtOH, illicit drugs, SID'sIfllV

n. PHYSICAL EXAM

-a complete neurologic exam should be performed. Neurology consultation is highly recommended.

I. Mental status check - obtained by observation and during history taking.

- Assess general appearance, level of consciousness, orientation, affect, cognition

49

2. Cranial nerves

• o

f)t)

I. OIfactory- sense of smell

Il, Optic- visual acuity and fields aanopsia- loss in 1 eye

b. bi-temporal hemianopsia-loss temporal fields

c.homonymous hemianopsia-Ioss complete field,L or R (see Fig. I) m. Occulomotor »>

N. Trochlear »»» extraocular eye motions VI. Abducens »>

V. Trigeminal- Motor- muscles of mastication

- Sensory- entire face via 3 divisions VIT. Facial- Motor- muscles offacia1 expression

- Sensory- anterior 113 tongue vn. Vestibulocochlear - hearing

IX. Glossopharyngeal - sensmy posterior 113 tongue X Vagus -uvula midline, normal voice, gag response

XI. Spinal accessory - Motor sternocleidomastoid and trapezius XII. Hypoglossal- motor tongue

3. Motor exam - evaluate symmetry, function and muscle tone

a Lower extremity

Tl2-L3 - Iliopsoas

L2-4 - Adductors, Quadriceps IA - Tibialis anterior

L5 - Abductors, EHL, ED!.. EDB

SI - Gluteus maximus, PL, PB, 1P, Gasttosoleus S2-3 - Instrinsics

b. Upper extremity

C5 -Deltoid C~-Biceps

C6 - Wrist extensors

C7 - Triceps, Wrist flexors, Fmger extensors C8 - Finger flexors,

C8- Tl - Hand intrinsics

c. Manual muscle test grading

5 - Normal- Complete ROM against gravity with full resistance 4 - Good - Complete ROM against gravity with some resistance 3 - Fair - Complete ROM against gravity

2 - Poor - Complete ROM with gravity eliminated

1 - Trace - Evidence of slight contIactility. No ROM o - Zero - No evidence of contractility

50

4. Sensation - determine if sensory loss is localized to a single dermatome or stocking-glove distribution

a Posterior columns - VibJ:atory

-2-point discrimination

-Position sense

b. AilteI'ior spinothalamic -Light touch

c. Lateral spinotbalamic

-Pain and temperature

5. Reflexes

a Deep tendon

lA - Patellar tendon Sl - Acbilles tendon C5 - Biceps tendon

C6 - Brachioradialis tendon Cl- Triceps tendon

Grading of01R's +0 -absent

+1 - hypoactive +2 -normal

+3 - hyperactive

+4 - pathologically brisk! clonus

51

b. Pathological reflexes - assesses UMN dysfunction Babinski - not present- plantarflexion oftoes

- Present - dorsiflexion ofhaIlux and fanning oflesser toes.

6. Cerebellar tests

aRhomberg

b. Rebound

c. Rapidly alternating movements d Heel to shin

e. Tandem walking (heel to toe)

7. Orthopedic lower extremity exam

a assess deformities present

- mild, moderate. or severe

- flexible or rigid

- location - rearfoot, midfoot, forefoot or combination of deformities

- compensatory mechanisms present

- evaluate weigbtbearing and non-weigbtbearing

- gait analysis

-sensory ataxic - demyelinating peripheral neuropathies, spinocerebellar degeneration, Vit B12 deficiency. M.S.

-steppage - peripheral or spinal motor nerve lesions, polio. C1\tIT. progressive muscular atrophy

-myopathic -Muscular dystrophy and other myopathies

-cerebeIlar ataxic - cerebelIar lesions

-extrapyra1nidal- Parkinson's

-spastic - CV A. tumor. trauma, corticospinaI lesion, MS .• C.P.

-apractic - Alzheimer's. normaI pressure hYarocepbafuS

-hysterical

8. Labs and Special Studies

may include CPK.. aldolase. EMGlNCV, muscle and nerve biopsies. lumbar puncture. cr

scan, MRI, X-rays and other procedures.

m. Differentiation on Upper Motor Neuron (UMN) and Lower Motor Neuron (LMN) dysfunction.

UMN LMN

Voluntary control Tone

Reflex arc Pathologic reflexes Atrophy

lost spastic present present

little or none

lost flaccid absent

not present significant

52

IV. Neurologic Diseases affecting the lower extremities

1. Cavus Foot - 75% of patients with a pes cavus deformity will have an underlying neurologic problem (Brewerton et al.)

Congenital causes:

CHARCOT-MARIE- TOOTH DISEASE (Peroneal muscular atrophy)

-most common neuromuscular disorder resulting in cavus foot Symmetric hereditary motor and sensory neuropathy that is progressive.

-3 modes of inheritance

1. Autosomal dominant- affects 30/100,000 and is slowly progressive

2. X-linked recessive- affects 3.6/100,000 and is more severe, onset during adolescence

3. Autosomal recessive- affects 1.4/100,000, is :rapidly progressive and severely

disabling

females> males 3-5:1. Seen primarily in Caucasians

defonnities- cavus, cavovarus, equinocavovarus due to overpowering of . muscles. Rigid or Flexible

CHARCOT-MARJE- TOOTH DISEASE (cont) PL> TA produces a pIantar:Oexed medial column 1P> PB produces a rearfoot varus

-Contracture of plantar fascia increases cavus

-FDL > EDL and intrinsics leads to clawtoes

generalized IE atrophy leads to the development of "stork: leg" or "inverted champagne bottle" deformity.

-present with high steppage or marionette gait

-progressive atrophy of distal UE leads to "monkey fist" or "skeleton hand"

deformity.

-Diminished sensation in stocking-glove distribution

-NCV are delayed by approximately 50"10

-Diagnosis based on positive family history and clinical findings, Recently

available is a blood test to diagnose the Type I autosomal dominant form.

DEJERINE-SOTTAS DISEAE (Hypertrophic interstitial polyneuropathy)

-------------------------------~~e

-progressive disease with onset in childhood to early adulthood.

-distal weakness and sensory loss of primarily the lower extremities.

-peripheral nerves are enlarged and frequently palpable due to "onion bulb"

formation of the nerves .

-nerve biopsy reveals concentric proliferation of Schwann cells around an area of demyelination.

FREIDRICH'S ATAXIA -recessively inherited disease

-idiopathic degeneration of the posterior and lateral columns of the spinal cord.

-onset in late childhood and early adolescence, rapidly disabling.

-weakness oflower extremities, anterior and 1ateral compartments. Later upper

extremity

weakness, primarily ulnar nerve distribution. -stocking-glove sensory loss.

-cerebellar involvement with incoordination, nystagmus, dysarthric speech, ataxia

-no lab abnormality is diagnostic however, sensory action potentials are absent or

mark:edly

decreased. NCV remain normal or decrease slightly with disease progression.

53

ROUSSY-LEVY SYNDROME

-autosomal dominant inherited disease

-slowly progressive

-common :findin~ include resting tremor, clumsy gait, atrophy of the distal LE's

and UE's, abnormal equilibrium, and absent DlR's.

REFSUM'S DISEASE (Heredopathia atactia polyneuritiformis) -autosomal recessive, hypertrophic sensory-motor polyneuIUpathy

-metabolic neuropathy caused by a distmbance in lipid metabolism which leads to

an accumulation of phytanic acid -males = females

-periods of acute exacerlJations

-retinitis pigmentosa, ataxia, ichthyosis, deafness, distal extremity weakness with

gait distmbances, diminished DlR's.

-sennn phytanic acid levels are >500/0 above normal.

SPINAL DYSRAPHISM

-fuilure of mesoderm and neuroectoderm to fuse and form the neural tube

-Types:

1. Spina bifida-lack of closure of vertebral arches, primarily lumbar

2. Spina bifida occulta- not accompmiedby protrusion of meningeal or spinal

elements

3. Spina bifida cystica- protrusion of meninges

4. Meningorndiculocele- protrusion of meninges and nerve roots

5. Meningomyelocele- protrusion of meninges and spinal cord.

-leg weakness with feet in equinovarus position

-diminished DTR. sensory loss feet and saddle area

-may be associated with hydrocephalus

-associated with congenital abnormalities and mental retardation

CEREBRAL PALSY

-caused by static brain lesion acquired in utero, during delivery or months afterbirth.

-commonly due to trauma, asphyxia or premature birth

-irreverstDle, non-progressive brain deformity

-classification:

-Spastic (65%)

1. Spastic diplegia- BIL LE spasticity- scissoring gait, variable mental impairment

2. Spastic quadriplegia- severe BIL DE and LE spasticity, severe intellectual impUrment

3. Spastic hemiplegia- unilateral spastic hemiparesis, less severe mental impairment

-Athetoid (20%)

-Ataxic

-Rigid

-Tremor

-Atonic

54

-treatment of CP

-Physical therapy

-Surgical intervention-release contractures

-Muscle relaxants

MUSCULAR DYSTROPHY

-familial disorders that lead to degeneration of nmscle fibers, due to enzymatic defect

-Types:

I. Duchennel Pseudohypertrophic - most common sex-linked inherited myopathy, diagnosed during early childhood due to slow motor development SOO/o show pseudohypertrophy of calf musculatme due to fat deposition which can be seen on biopsy. Girdleweakness leads to the classic

"Gower's sign" utilized when going from the floor to standing. Ankle equinus and equinovarus deformities are common. Progressive and the majority of patients are wheelchair bound by age 20. Elevated CPK levels are noted

2. Becker MD. - similar pattern of muscle weakness as Duchenne however, less aggressive. Patients with Becker MD. often walk to

approximately age 35 and have longer life spans. ..

3. Facioscapulohwneral MD. - mildly progressive, affects facial and shoulder muscles with weakness and atrophy. Lower extremity weakness usually occurs 20-30 }'C3'S later.

4. Myotonic MD. - multisystemic disorder, onset ranges from birth to 50 years of age. Presents with tonic muscle spasms. Often a foot drop develops.

5. Limb girdle MD. - diagnosis of exclusion, has inconsistent features. Initial pesentation of shoulder and pelvic girdle weakness and atrophy.

SYRINGOMYELIA

-progressive disorder, onset usually 2nd-3rd decade

----------------------~~~~l~n~~=oo~------------------------------

anesthesia, paraparesis with neurotrophic changes including neurogenic osteoarthropathy (Charcot), loss ofDTR.

-caused by a tubular dilatation in the spinal cord most commonly in the cervical

-diagnosis easily made withMRI or cr

2. Peripheral nemopathy ( COMMON CAUSES)

-Hereditary

-orr

-Dejerine-Sottas

-Refsum's disease

-Amyloidosis

-Acquired

-Diabetic

-Myeloma

-Guillain-Barre'

-Malignancy

-Uremia

-Cryoglobulinemia

-HIV

-Tangier's disease

-Fabry's disease

-Abeta1ipoprotei

-Freidrich's ataxia

-Hypothyroidism

-Sarcoidosis

-Alcoholic

-Collagen-vascular

-Acromegaly

-VitaminB

55

-Toxic (iedrugs, chemicals)

-chloramphenicol

-isoniazid

-nitrofurantoin

-Dapsone

-cisplatin

-metronidazole

-hydralazine

-phenytoin

-lead

-cyanide

-thallium

-zerit

-Infectious

-leprosy

-diphtheria

-nrv

-En1Iapment

-carpal and tarsal tunnel

-lithium

-pyridoxine

-disulfuram

-ethionamide

-vinaistine

-gold

-amiodarone

-colchicine

-arsenic

-mercury

-trichloroethylene

~

-heIpes zoster

-Lyme disease

3. Various neurologic disease ..

1. PARKINSON'S DISEASE

-progressive disorder, characterized by cytoplasmic eosinophilic inclusions in the neurons of the substantia nigra.

-characterized by rigidity, resting tremor, bradykinesia, loss of postural reflexes, festinating gait Dementia occurs in SOO/o of patients.

-males=femaIes, onset ~ years of age

-multiple drugs utilized in treatment including levodopa, caIbidopa, amantadine,

bromocriptine, pergalide. Physical therapy helpful to improve function.

2. MULTIPLE SCLEROSIS

:::cJiiODiC eNS cnsease OfUDkriOwn ongm

-characterized by inflammatory demyelinating plaques in white matter of CNS. Infiltration oflymphocytes, plasma cells and macrophages.

-onset at around 30, 2: 1 female:male .

-course is relapsing and remitting

-optic neuritis is most common presenting symptom

-LE affected> UE often with spasticity and fatigue

-cerebellar involvement with ataxia, scanning dysarthria and intention tremor

-autonomic symptoms- incontinence, impotence and orthostatic hypotension

-often see personality changes, memory loss, dementia and emotional lability.

-MRI diagnostic in >90% of cases

-mean survival approx. 25yrs and is improving

-avoid physical and emotional stress, infections and prolonged exposure to

extremes of'temperature,

3. CEREBRAL VASCULAR ACCIDENT

-rapid onset of a neurologic deficit involving a vascular territory of the brain and lasting for greater than 24 hours, < 24 hours considered a transient ischemic attack: (TIA).

-most often due to thrombosis or embolic event as well as hemorrhage from aneurysm or A V malformation.

56

-presentation varies with the vessel involved and portion of the brain infarcted.

-er scan diagnostically helpful

-GenernIly, early flaccid pualysisfollowed by increasing spasticity and

development of synergistic panems,

-Physical therapy and orthotic fabrication can improve functional ability

4. EPILEPSY

-characterized by abnormal, recurrent, excessive discharges from neurons.

-SOO/o have no underlying neurologic disorder

-other etiologies include EtOH withdrawal, head trauma, hypoglycemia, meningitis,

encephalitis

. -most seizures last 30-90 seconds -Classification

l.Focal (Partiallocalr originate from a localized portion of the brain -Simple focal

-motor- focal motor with march Jacksonian

-sensory- somatosensory, visual,auditory, gustatory

-autonomic- epigastric sensation, pallor, sweating, flushing,

piloerection, pupillary dilatation

-psychic- dysphasia, affective, cognitive. illusions.

hallucinations.

-Complex focal

-simple focal followed by impairment of consciousness

-impaired consciousness at onset sometimes with automations.

-Any focal seizure may evolve to a secondary generalized seizure.

2.Generalized - no focal component,therefore no prodrome, aura, or focal motor or sensory symptoms.

-Absence (Petit Mal)

-impairment of consciousness only, may show mild

clonic,atonic,tonic component. -Myoclonic seizures

-Tonic

-Tonic-Clonic (Grand mal)

----~---------------------------~-4M~u~q~~-------------------------------------

3. Unclassified

-Status epilepticus

-condition in which patient has a series seizures without total recovery-of

consciousness. Most common cause in compliance . with seizure medications.

-Anticonvu1sants (ie. phenytoin, cmbamazepine) are most commonly utilized. Need to

obtain therapeutic blood levels.

5. MYASTHENIA GRAVIS

-Acetylcholine (Ach) receptor antibodies destroy the receptor sites on the }X>Stsynaptic membrane. Results in miniature end plate potentials that are reduced in size and number.

-Due to an autoimmunopathy

-HaIlmaI'k is exercise induced fatigue that is resolved with rest

-Often get double vision and ptosis, difficulty with chewing. Only involves skeletal

muscle.

-Ach receptor anbbody found in the serum of>9()01o

57

-Tensilon (edrophonimn) is helpful diagnostically.

-EMG shows classic changes of decrement

-5% of patients have an associated thymoma which is most easily diagnosed with

CTscan.

-Treatments include long term anticholinesterase meds or disease modifying drugs such as Immman. Also p1asmaphoresis has been found to be effective.

MG(cont)

- *** Eaton-Lambert syndrome is similar to myasthenia gravis however, it involves a defect in neuromuscular transmission due to a decreased number of Ach packets released at the presynaptic terminal

- use certain meds such as aminoglycosides and tetracycline with caution

6. Gun..LAIN-BABRE' SYNDROME (Acute idiopdhic polyneuritis) -innnunologically mediated demyelinating polyneuropathy

-affects all ages and sexes

-bimodal peaks of occurrence-15-35 yrs and 50-75yrs

-usually preceded by a viral or mycoplasma infection, surgery, immunization or

in patients with systemic lupus erythematosis (SLE) and lymphomas. ~ by rapidly progressing symmetrical motor weakness in a distal to proximal fushion. Can lead to severe respiratory paralysis and occasionally cranial nerve involvement

-causes stocking-glove paresthesias

-decreased or absent DIR's

-may cause autonomic dysfunction including-bradycardialtachycardia,

hypotensionlhypertension.

-diagnosis-lumlm puncture reveals elevated CSF protein and the presence of a few mononuclear leukocytes.

-EMG reveals decreased NCV and prolonged latencies

-functional recovery occurs usually 2-4 weeks

BONE TIlMORS

c:cmrlaoLF1aanms D~SIA

~~~~ -- __ a

i ~~am~~~

. .. 2:

i

;iMlfau. __ atllD:~ -:::..J.jCl--_~AIlUlJ'I "VIt f$ 8C1t€"

i

"15

1 . iV'rSftASIA !PIJ'HYSG&LIS

---- H9tUMB..IQ)----

58

Classification: Histologically based on the cell or tissue type from which the lesion originates. Osseous, Cartilaginous and Fibrous are the origin of most primaIy benign and malignant neoplasms in bone.

Diagnosis:

Subjective S}'lDIloms(NLDOCAT) Past medical histmy

Clinical evaluation of objective signs

Radiographic evaluation:

Helps to determine the rate and aggressiveness of the bone tumor

1. Pattern ofbone destruction:

a) Geographic -least aggressive, well defined

b) Moth eaten -more aggressive, less defined

c) Permeative - highly aggressive

2. Scleroticborder:

-slowly growing lesions are associated with reactive sclerosis of the surrounding bone

-sclerosis can be of ,7ulable thickness and partially or completely surround the lesion

3. Cortical involvement:

-bone cortex serves as a barrier to lateral expansion of the tumor

-nonaggressive medullary lesions may cause little change in the endosteal surface,

however, some benign lesions

such as aneurysmal bone cysts can expand rapidly

-aggressive lesions cause endosteal erosion and can penetrate the cortex in more than one place

4. Periosteal reaction: 1. Contimlous

a COrtex destroyed

smooth shell - due to expansile pressure benign lesions (giant cell, enchondroma, fibrous dysplasia)

lobulated shell - due to variation of growth rate

ridged shell - AKA "soap bubble" due to slowing rate of proliferation (non-ossifying fibroma, enchondroma)

b. Cortex present

solid - implies slow growth rate, can be seen in osteoid osteoma, enchondroma and eosinophilic granuloma

undulating - variant of solid, seen with periostitis, hypertrophic osteoarthropathy

lamellar - may be single or onion skin in appearance parallel or spiculated "hair on end" -often associated with malignancy (Ewing's sarcoma)

2. Interrupted

a Buttress

solid appearing wedge of bone, forms at margins of slowly enlarging lesion(chondromyxoid

fibroma, malignant change of long-standing benign lesion)

b. Codman Triangle .

suggests aggressive malignant lesion seen in

59

osteosarcoma and chondrosarcoma c. Larnella1ed Reaction

onion skin appearance can be seen in malignant (osteosarcoma) and benign lesions (eosinophilic granuloma)

3. Complex

Sunburst pattern, highly suggestive of osteosarcoma

5. Size and Shape: .

In general, malignant tumors are larger than benign tumors, often >6Cm Elongated lesions in which the greatest lesional diameter is 1.5 times the least diameter may be indicative of Ewing's sarcoma, histiocytic lymphoma, chondrosarcoma and angiosarcoma

6. Soft tissue involvement:

Iflesion leads to cortical breakthrough than it is aggressive

7. Skeletal location oflesion:

Tumors may predominate in areas of red, or hematopoietic,

marrow. These include metastatic disease, plasma cell myeloma, Ewing's sarcoma and histiocytic lymphoma.

The tendency for these tumors to involve appendicular and axial skeleton in the young and only the axial skeleton in aged is related to the changing distribution of red marrow.

Some tumors are more prevalent in areas of rapid bone growth such as distal femur and proximal tibia

11 bia: increased incidence of adamantinoma, nonossifying fibroma and chondromyxoid fibroma, osteosarcoma, osteoid osteoma, Giant cell tumor

Fibula: non-ossifying fibroma, aneurysmal bone cyst, Ewing's sarcoma, lipoma Foot chondromyxoid fibroma, aneurysmal bone cyst.

chondroblastoma, osteoid osteoma, osteob1astoma, enchondroma, lipoma

8. Location oflesion in bone: (Fig.I)

Transverse plane: .

Central Solitary bone cyst, enchondroma

Eccentric .Anemysmal bone cyst, giant cell tumor,

osteosarcoma. chondrosarcoma, fibrosarcoma, chondromyxoid fibroma

____________________ ~CO~m=·cd== .. ~ ... ~ .. =.N=o=n~o~~fibro~~~~o~st~eo~i~d~o~steo~ma~ _

JuxtacorticallParosteal. ... Osteochondroma, juxtacortical . chondromas, parosteal osteosarcoma

Longitudinal plane:

Epiphysis. Chondroblastoma

Metaphysis. Osteosarcoma

Diaphysis .Ewing's sarcoma. retinaculum cell sarcoma, multiple myeloma and

metastases

Physeal Giant cell tumor

MetalDiaphyseal. Unicameral bone cyst. enchondroma

9. Trabeculation:

-may reflect residual trabecu1ation displaced by tumor or new bone formation due to the tumor

Pattern

delicate, thin

coarse, thick lobulated

delicate, horizontally oriented

striated, radiating

Tumor

Giant Cell tumor Chondromyxoid fibroma

non ossifying fibroma aneurysmal bone cyst

hemangioma

60

10. Matrix Pattern:

-certain tumors produce matrix that calcifies or ossifies Cartilage tumors:

-calcific circles (rings) -due to endochondral bone formation

-flocculent

-fleek-Iike (stippled)

11. Patient age: (Fig.2)

~ Lesion

I .. 1 year Metastatic nemoblastoma

2. 1 year to IOyrs. 3. 10 years to 20 yrs.

Ewing's sarcoma

AnemysmaI bone cyst

4. 10 years to 30 yrs.

5. Skeletally immature ~

6. Skeletally mature to 5Oyrs.

Chondrob13stoma Lesion

. Giant cell tumor

7. 30 years to 60 yrs.

Chondrosarcoma PrimaIy lymphoma

Malignant fibrous histiocytoma Fibrosarcoma

8. 50 years to 80 yrs.

Multiple myeloma Metastasis

Size

Soft tissue mass Periosteal reaction

------Margms

Zone of transition Trabeculation Cortical destruction Overall appearance

Benign versus Malignant Bone Lesions Benign Small

Malignant Large

None Large

Rare Common

Sclerotic None

Narrow Wide

Yes

Rare Geographic

No Usual

Permeative or Moth-eaten

Other diagnostic tests include:

-Bone scans

provides insight on multiple sites of involvement Increased uptake of the nucleotide in bone reflects the hypermetabolic osteoblastic activity

-Computerized tomography

helps to determine more precisely the location and extent of involvement of the tumor

-Magnetic resonance imaging

also help; to determine the location and extent of involvement of the tumor, however, better in assessing

the soft tissues and tmnor characteristics.

-Laboratory studies

A1ka1ine phosphatase(blood and wine) Complete blood count

Chest xray

61

age and gender specific studies may include serum protein electrophoresis(SPEP) urine protein electrophoresis(UPEP) prostate specific antigen(PSA) thyroid:function tests(TFf's)

liver function test (LFl) caIcinoembIyonic antigen(CEA)

-Biopsy

Incisional: cutting into the lesion to remove a sample for pathology evaluation -required for malignant bone lesions

-carries risk of spreading lesion by lymphatic or

blood seeding

-<XDDpIications are minimized with careful incision planning, strict hemostasis, minimal dissection.

avoidance of neurovascular structures and tight wound closure Excisional: removal of entire lesion (done for benign tumors)

Common Tumors of the Distal Leg and Foot:

BENIGN TUMORS: 1. Osteoid osteoma

-benign osteoblastic lesion characterized by a well demarcated core(nidus)of usually less

than 1 em. and a distinctive surrounding zone of reactive bone formation. -age: 10-25 years (rare>30 years) males>females

-location: femur 32% tibia 24% foot 11%

-presentauon: point tenderness over the lesion. nocturnal pain. local soft tissue swelling in

the foot. the lesion usually appears at the junction of the anterior mid-third

of the talus or os calcis, salicylates may completely relieve symptoms by prostaglandin

inlubition

-radiographic features: lesion is located most commonly in the cortex. The lesion bas a radiolucent nidus with a striking

thickening of 3(ljacent cortical bone and periosteal new bone formation.

-pathologic features: lesion is characterized by varying degrees of osteoid and highly

~~g~~~~~~~·~e~. __

-treatment. excision of the nidus and surrounding bone

2. Osteoblastoma

-benign, rapidly growing tumor that is histologically similar

to osteoid osteoma. Characterized by the absence of any reactive perifocal bone formation. -age: 20-30 years of age (9()01o are <30 years of age)2: I males:females

-locaiion: diaphyseal and metaphyseal in the foot the tumor

occurs most commonly in the dorsal aspect of the anterior portion of the talus -presentation: dull. aching, localized pain. Not noctmnal

pain. Pain is not relieved by salicy1ates. Localized swelling

-radiographic features: lesions are well circumscribed, radio-

lucent and usually expansive with occasionally a thin shelf of reactive bone. -pathologic features: tumor is hemorrhagic, gritty and friable. Large lesions may show central softening and cystic degeneration

-treatment: curettage and packing of the defect

3. Enchondroma

-benign hyaline cartilage growth that develops within the medullary cavity of a single bone

-age: usually manifest during 3rd or 4th decade; equal distribution between the sexes

-location: metaphyseal to diaphyseal - most occur in the small

62

tubular bones in the foot, seen in metatarsals and phalanges

-presentation: lesions develops slowly and gradually and is well established before clinical symptom. Often painless swelling is the first manifestation Painful lesions without tramna should arouse suspicion that a malignant transformation is occmring. -radiographic features: lesions are well circumscribed with

distinct ares of rarefaction-the lesion may show speckled calcification -pathologic features: hyaline cartilage with scattered calcification

-treatment: curettage .

** The syndrome of multiple enchondromas is known as OIlier's

disease. If also associated with soft tissue hemangiomas, the disorder is known as Maffucci's syndrome.

4. Osteochondroma

-benign, slow growing mushroom shaped hyaline cartilage capped lesions. Classically arise from a growth plate in long bones, growth ceases with skeletal maturity

-age: 2nd decade, 2: 1 male:female

-location: metaphyseal to diaphyseal- exostosis points away from adjacent joint -commonly seen in

the distal femur and proximal tibia

-presentation: often an incidental finding on x ray most commonly a painless. slow growing

lesion - pain may becaused by impingement of surrounding anatomic structures. .

-radiographic features: sharply pedunculated or sessile tumor

at the level of the metaphysis that points away from the neatby joint.

-pathologic features: cortex and periosteum of the tumor are continuous with underlying bone. Hyaline cartilage is usually 2-3 mm. thick and this diffentiates an osteo-chondroma from a subungual exostosis which has a fibrocartilage cap.

-treatment excision

**less than 1% give rise to chondrosarcoma

5. Non-ossi:fving fibroma

age: 1st and 2nd decade

location: cortically based metaphyseal lytic lesion. eccentrically located-most common in distal femur and proximal tibia

presentation: usually asymptomatic unless associated with a pathologic fiacture with accompanying pUn, swelling and disability.

---------~-rOliiugraphlc JeatUres: LeSion IS an active and proliferating form of :fibrous cortical defect seen in infants and chi1drenLucent, eccentric lesions that may be loculated, with a sclerotic rim

pathologic features: dense collagen. giant cells and lipid filled histiocytes treatment. curettage

6. Chondromyxoid :fibroma

rare, benign tumor composed of chondroid, :fibrous and myxoid tissues age: 2nd to 3rd decades, male>females

location: occur in the metaphyses oflong bones offoot - 16%

presentation: usually complain of localized, dull, achy pain possibly swelling and tenderness

radiographic features: lucent tumor, usually >5cm. with well-defined sclerotic borders pathologic features: nodules of poorly formed hyaline cartilage and rnyxoid tissue delineated by fibrous septae

treatment. curettage

7. Aneurysmal bone cyst

primary tumor like lesion which initates an A V :fistula and thereby creates, via hemodynamic forces, a secondary reactive bone lesion

age: most occur in patients under 30 years of age, equal sex distribution

63

location: may affect any bone - foot-8%

presentation: mild to moderate pain as the lesion develops, patient may limp radiographic features: small lytic lesion seen early

later, areas resembling Codman's triangle may develop, at the blowout stage the bone may appear expanded with no shell around the lesion Late lesions as a result of fibrous septae

and bony spicules appear loculated.

**angiogrnphy may help to define the vascular malformation treatment: curettage and packing with bone chips

8. Simple bone CYSt (Unicameral bone cyst) -fluid containing solitary unilocular cyst

-age: 8O-9()01o <20 years old, 2: 1 males:females

-location: metaphyseal region of long tubular bones in juxtaposition to the epiphyseal plate

-presentation: indistinct discomfort makes the cysts presence known. In 50%, pathologic

fractme is the cause of diagnosis

-radiographic features: from its principle metaphyseal location, the cyst extends into the diaphysis. Characteristica, they occupy a central location, its length is

greater than its width. The overlying cortex is eggshell thin brt always intact.

***fallen fragment sign-radiologic finding describing a free fiagment of cortical bone :fullen by gravity in the fluidcontaining cyst.

-pathologic features: serous usually bloody tinged fluid is expelled upon opening. Cavity is unicystic with a whitish. thin glistening lining.

-treatment: curettage and packing with bone chips

9. Giant cell tumor

-uncommon, locally aggressive tumor so named because it contains multinucleated, 0steoclast type giant cells.

-age: arise during 3rd to 5th decades

-location: adults: involve both epiphysis and metaphysis

(adolescents: confined proximally by the growth plate and are limited to the metaphysis-most arise about the knee)

-presentation: location of the tumor near ends ofbones causes

arthritis type symptoms-occasionally pathologic fracture is presenting concern. Swelling, intermittent pain and limited range of motion also can be seen.

----------------~adiographie~atures:~~~ytic~~esimm~---------------which are eccentric may have a thin, perilesional sclerotic shell

-pathologic features: large and red-brown and frequently

undergo cystic degeneration Composed ofuniform oval mono-

nuclear cells which are the proliferating component of the

tumor. Scattered within this background are giant cells having 100 or more nuclei. -treatment: curettage, recurrence rate is 40-60%

10. Fibrous dysplasia

-Iocalized, progressive replacement of bone by a fibrous proliferation intermixed with poorly formed, haphazardly arranged trabeculae of woven bone.

-age: 2nd to 3rd decade most common

-Iocation:monostotic-involvement of single bone

polyostotic-involves more than one bone, can be seen with various endo-crinopathies -presentation: symptoms depend on bone(s)involved and degree

of involvement-pain and swelling may accompany pathologic :fracture.

-radiographic features: "ground glass" appearance with a well-defined sclerotic rim - central meta-diaphyseal in tubular bones

-pathologic features: lesional tissue composed of curvilinear

64

trabeculae of woven bone surrounded by a moderately cellular fibroblastic proliferation. -treatment: excision or curettage in monostotic fOnDS.

11. Chondroblastoma

age: 2nd decade, 2:1 male:female

location: epiphyseal, eccentrically located - foot 100/0

presentation: painful and due to location often lead to art1nitis type symptoms with joint effusions and Jimited range of motion.

radiographic features: well-defined geographic lucency that commonly has spotty calcifications

pathologic features: composed of sheets of compact polyhedral chondroblasts with well defined cytoplasmic borders

treatment: curettage

MAliGNANT TUMORS

1. Osteosarcoma

-ggressive mesenchymal tmnor in which neopJastic cells produce bone matrix age: most common in persons <20 years old, 2: 1 male:female

location: 80-90010 arise in the medullary cavity of the metaphyseal ends oflong bones but any bone can be involved.

presentation: pain, local tenderness and swelling - sudden fracture is sometimes the presenting syndrome - at time of diagnosis 200/0 of patients have demonstrable lung metastases

radiographic features: large destructive,mixed lytic and blastic mass with penneative margins. Periosteal reaction = Codman's triangle

treatment: chemotherapy and inadiation to debulk lesion

followed by resection/amputation.

2. Chondrosarcoma

second most common matrix-producing tumor of bone behind osteosarconia common feature is the production of neoplastic cartilage- often asymptomatic age: most commonly seen in ~ year age group, 1.5:1 male:female

__________ ___::olocati==·o=n,_,_: =co=rnm= only arise in the~portiQDS..ofJhe-skeletOD,...pehds..and.shoulUlder;a-----------girdle, proximal femur.

presentation: painful, progressively enlarging masses

radiographic features: localized area of bone destruction punctuated by mottled densities from calcification or ossification.

pathologic features: conventional chondrosarcomas are composed of malignant hyaline cartilage. Myxoid variants are viscous and gelatinous-spotty calcifications are typically present

treatment amputation

3. Fibrosarcoma

rare collagen-producing metastatic neoplasm

age: can occur at any age, however, most common in 2nd 4th decade, equal sex distribution

location: arise in metaphysis oflong bones and pelvic flat bones - most common in femur presentation: enlarging painful masses which may be palpable and tender often present with pathologic fracture

radiographic features: permeative and lytic lesion that often extends into the soft tissueslight sclerosis at margins of tumor

pathologic features: composed of malignant fibroblasts arranged in herringbone pattern treatment amputation

65

4. Ewing's Sarcoma

uncommon primary small round cell tumor that exhibits a primitive neural phenotype

age: 10-15 years old, male>female, blacks rarely affiicted

location: arises in the diaphysis of long tubular bones especially the femur

presentation: painful enlarging mass. Affected site is frequently tender, warm and swollen. Pathologic fractures may occur

radiographic features: affected bones may demonstrate non-specific "onion skinning" and lytic lesions

pathologic features: composed of sheets of uniform. small. round cells that are slightly larger than lymphocytes whichappear clear due to increased glycogen content.

treatment chemotherapy and surgical resection with or without radiation

5. Multiple Myeloma

most common malignant bone tumor age: 40-70 years old, males>females

location: multiple sites, metaphysis oflong bones, spine, skull, and pelvis

presentation: fever, malaise, weight loss, fatigue, anemia,1hrombocytopenia and renal failure, Pain in one or more bones that increases with weightbearing- monoclonal gammopathy on SPEP. Proteinuria due to renal damage and characteristic Bence-Jones Protein.

radiographic features: vague radiolucent, oval lesions

METASTATIC TUMORS

-pathways of spread include:

I. direct extension 2.lympbatic and

3. intraspinal. seeding

carcinomas of the breast, prostate, lung, kidney, and thyroid are most common sources skeletal metastases are typically multifocal, however, carcinomas of the kidney and thyroid may produce solitaIy lesions.

most metastases induce a mixed lytic and blastic reaction

in lytic lesions, the metastatic cells secrete substances such as prostaglandins. interleukins,

and parnthyroid hormone re1ated. ~ that stimu1ates...o.Steoclastic..hone..resol.L}rpbAl·LUODlI...---------Carcinomas of the kidney, lung, GI and melanoma produce this type of bone destruction.

metastases that produce a sclerotic response stimulate osteoblastic bone formation

Prostatic adenocarcinomas typically produce this type of response.

lIJ lD

. n~C""~I*~Q'buuaN&~ 9.,li ... 1'_ ", ..

AB ~ ................ "-ir,_ .......... II-.n.. __ ~~ID ...... a__..w..""m.

0._ -

.. - t:hlaoid_~a......-_ioIljb_~_

I I

F

.liD-

J ~

tiI ... ~ Il0l1 ..........

66

Soft-Tissue Masses

Benign verses Malignant

Benign

- Resemble derivative tissue

- Automous growth rate

- Local invasion Tare

- Low recurrence rate

- Small

- Cystic/fluid-filled

- Movable

Malignant - Sarcoma'

- Aggressive

- Rapid destructive growth

- Recurrence/metastasis

- Large

- Solid

- Fixed

Evaluation

- Visually inspect the lesion for edema, color, overlying skin, location and transillumination.

- Palpate for size, consistency, mobility and tenderness/pain.

MRI - Benign verses Malignant

Benign

- Well-defined margins

- Homogeneous signal intensity

- No N- V or bone involved

Malignant

- Poorly defmed margins

- Heterogeneous signal

- N- V or bone involvement

Biopsy

Definition: Removal and examination of tissue. cells and fluid from the living body.

Types of Biopsy:

_______ -----'l~._C~los_e_d - obtain tissue_percutaneously-with-a-needle----------------- fine needle - done with 25-gauge needle to aspirate mass

- core needle - done with cannulated needle with inner trocar

* Closed biopsies are easy to perform and indicated for local recurrence or infection. However, they oftentimes result in insufficient tissue retrieval and diagnostic inaccuracy.

2. Open - tissue is obtained through a surgical incision - excisional - removal of entire mass

- done for small subcuataneous, obviously benign lesions

- incisional - removal of a portion of mass leaving main mass in situ

- performed for suspected malignant masses

Incisional Placement:

longitudinal on extremities length short as possible over midportion of mass

67

Common Benign Tumors

Ganglion Cyst

well circumscribed, soft, cystic mass usually in dorsal foot or ankle

close relationto tnndon sheath or joint

aspirate for definitive diagnosis - straw colored fluid treatment - marginal local excision

Plantar Fibroma

subcutaneous thickening of plantar fascia usually affects medial and central bands bilateral 10-15%

treatment - wide local excision recurrence common

Lipoma

most common benign tumor subcutaneous, soft, movable mass 2 types:

- superficiaJ - well-circumscribed, static

- deep - no margin

treatment - marginal excision

Neurofibroma

tumor of spindle cell origin fusiform expansion of nerve solitary or multiple cutaneoud or deep

multiple associated with von Recklinghausens disease -----------,=---treatment=-excise--cutaneous;-very-difficult-to--excise-deep-without-nerve-------destruction

Neurolemoma

tumor of peripheral nerve sheath discrete, tender nodule

usually affects major nerve treatment - blunt marginal excision

68

DEEP VENOUS THROMBOSIS AND PULMONARY EMBOLISM

1. Definitions

A DVT - blood clots which usually form in the deep venous system of the lower extremity .

B. PE - a detached thrombus, usually from the deep veins in the leg (95%), which travel to and lodges in the arteries of the lung

Il, Promoting Factors - Vrrchow's Triad

1. stasis

2. abnormalities of the vessel waIl or damage to the waIl

3. "bypercoagulability"

ill. Pathogenesis / Pathophysiology Promoting Factors (Vrrchow's Triad)

White Thrombus - nidus of platelets

Red Thrombus -->Fibrinolysis -->Resolution

(fibrin thrombus) or

Organization Pulmonary Embolus

-red thrombus - develops rapidly (minutes) and is very unstable at:first (It takes 7-10 days to stabilize.)

-fibrinolysis - the process by which fibrin is degraded

-organization - a 7-10 day process during which the thrombus stabilizes resulting in vessel narrowing

IV. Conditions associated with risk of~sis

1. post partum 5. lower extremity tramnalfracture

2. ventricular failure 6. chronic deep venous insufficiency

3. prolonged bedrest 7. using estrogen/oral contraceptive

4. carcinoma 8. recent pelvic or lower extremity

surgery

V--DiagnoSis

ADVT

1. Classical - pain, heat, swelling in the affected limb

2. PE - presents as the :first sign in 7()O/o of patients with DVT

3. Homan's Test - dorsiflexion of the foot elicits caIfpain

4. Pratt's Sign - compression of calf causes pain

B. PE

1. sudden onset

2. "classic" triad (only experienced in 14% of patients) a dyspnea

b. chest pain

c. hemoptysis (tachycardia is actuaIIy more common)

3. 85% of patients with fatal PE have some warning sign such as prior embolic event or symptomatic venous disease

VI. Diagnostic Tests for DVT

A Non-invasive Tests

1. Impedance Plethysmography - a rapid test to evaluate for DVT proximal to the thigh

2. Doppler Technique - an operator dependent technique which demonstrates DVT by lack of venous compression

69

3. Radiofibrinogen - a very sensitive test for the diagnosis of thrombosis in the calves

B. Invasive Tests

I. Contrast venography

VII. Diagnostic Tests for PE

A Blood Gas - Pa02 < SOOIo rom Hg; PaC02 < or = to normal

B. Chest X-ray - SOO/o are normal (may see a hemidiaphragm. atelectasis, density, Pulmonary wedge)

C. EKG - Tachycardia .

D. Ventilation-Perfusion Scan (V/Q Scan) - a mismatch demonstrating an area of ventilation but no perfusion suggests PE

I. Perfusion Scan - Inject technetium 99 labeled hwnan serum aIbwnin 2. Ventilation Scan - Inhalation ofxenon l33

E. Pulmonary Angiography

I. Definitive diagnosis thus indicated when VlQ scan is inconclusive

2. Diagnostic signs are: intraImninal filling defect, an abrupt vessel cutoff: or loss of side branches

F. Catheterization of the right side of the heart and measure the pulmonary artery and right ventricular diastolic presswe (increased pulmonary resistance)

VIII. Prophylaxis

A Subcutaneous Heparin

L Pre-op - 5,000 u SQ 2 hours before surgery

2. Maintenance - 5,000 u SQ q 8-12 hours

3. Conqnession stockings

4. Intermittent pneumatic compression devices

5. Early ambulation or range of motion B. Aspirin

L 325mg po qd (baby ASA 81 mg po qd)

IX Treatment

A Anticoagulation

L Intravenous Heparin

a Loading dose - 10,000 - 15,000 u

b. Maintenance via N drip. start with 1,000 u Ihr --------~(nux-~25~,0001l m 250CC ofD5 1/2 NSS)

c. Monitor PIT daily - titrate heparin to maintain 1.5 - 2 times patient control

d Protamine sulfate reverses heparin (Ratio: 1 mg protamine sulfate per 100 u heparin)

• caution should be used there are many possible side affects of protamine and due to haparin's short ha1flife (1/2 -2 hours)

Note: Weight Based Pneumogram of Heparin Dosage (variations exist)

Bolus 80 u/kg Heparin N with a maintenance rate of 18 u/kflbr, check PIT in 6 hours, then if

PIT <35

and 36-45 46-70 71-90 >90

BOLUS CHANGE RAm

80 uIkg (lean weight) increase 4 uIkgIhr

Need to beme check stools prior to

40 uIkg increase 2 uIkgIhr

--take no action --decrease 2 u/kglhr

hold heparin 1 hour and decrease 3 uIkgIhr

during initiation of anticoagulation

70

2. Coumadin (warfarin)

a. Onset of action is delayed (peak effect 3-5 days)

b. Started after heparin is therapeutic

c. Dosage 2.5-15 mglday

d Titrate dosage to 1.5-2.0 times control e. VItamin K reverses Comnadin

3. Lovenox

A Venous interruption operations - intracaval umbrella, balloon or wire filter placed in the inferior vena cava below the renal veins

B. Thrombolytic therapy - Urokinase or streptokinase to dissolve clot (must be initiated within 24-48 hours)

C. Pulmonary Eri:J.bolectomy

X Differential Diagnosis for DVT A Cellulits

B. Compartmetn syndrome

C. Venous stasis

XI. Miscellaneous

A Fat Embolism

1. Usually occurs with large:fractures of the lower extremity (from bone marrow fat)

2. Three organ systems with symptoms: Pulmonary (hypoxia), CerebIal (confusion), Cutaneous (petechiae)

3. Treatment -largely supportive

- IV fluids, respiratory support, fracture care, steroids, and hypertonic glucose and insulin

B. Superficial Thrombophlebitis

1. Presents as a palpable cord along the distribution of a vein with erythema, edema, and pain locally.

2. Treatment - supportive consisting oflocal heat, elevation and rest Antiinflammatory agents may be given.

D. Emboli secondary to endocarditis

1. Most common cause of endocarditis is Strep. VIridans -----------? ~h: Aureus

Peripheral Vascular Disease

I. Arterial Disease:

Definitions:

Arteriosclerosis: generic term for a group of vascular diseases which cause thickening and inelasticity of arteries, decreasing the blood supply to the tissues.

Atherosclerosis: diminished size of vessel lumen due to the formation of fibrofatty plaques along the intimal surface of the artery.

Acute arterial occlusion: arterial embolic event usua1Iy derived from the heart (atrial fibrillation, l\.1I, va1vuIar disease, PfCA, CABG) which cause occlusion of small end arteries with ischemia to corresponding tissues.

MODckeberg's medial calcific sclerosis: calcification of the media of the artery. Does not decrease the vessel lumen but does falsely elevate ABI's.

71

Major Risk Factors: -Diabetes Mellitus

-Hypen:bolesterolemia

-Hypertension

-Smoking

Clinical Presentation: -Acute arterial occlusion

Pain

Pallor Pulselessoess Paresthesias Paralysis

-Chronic arterial occlusive disease

• Intermittant claudication _

location related to level of obstruction, calf most common complain of cramping brought on by a certain amount of exercise symptoms are reproducible and relieved by rest

• RestPain

noctumal and diffuse in foot while lying with legs elevated legs placed in dependency for relief

• Ischemic ulceration I gangrene most advanced complication

genemlly occur in distal portion of the foot

ulcers look necrotic and do not bleed with debridement

Minor Risk Factors: -Obesity

-Stress

-Age

-Genetic predisposition

-Deereased exercise

Clinical Signs ofPVD:

-Color: pallor. cyanosis. rubor on dependency

-Temperature: decreased

-Quality and Quantity of skin, nails and hair:

skin: shiny, scaly, atrophic nails: thickened, dystrophic

hair: diminished .

-Edema: may be increased due to continuous dependency

Vascular Examination -Pulses

palpate - femoral, popliteal, dorsalis pedis and posterior tibial arteries doppler

-Elevational pallor

-elevate legs above heart for at least 30 seconds

-evaluate color and grade 0 to 4 with +4 indicating marked pallor

- Venous filling time

-after elevational pallor test have patient sit up quickly

-measure time it takes to fill dorsal venous arch

-Normal 10-15 seconds, Significant disease 30-60 seconds

-dependent rubor may follow 2-3 minutes later

Non-invasive vascular testing -Ankle ann index

-ankle systolic pressure I brachial systolic pressure

-quick and reliable test, offers little information about anatomic level of occlusion

-Segmental limb pressures

-systolic pressures measured with cuffs at groin, above knee, below knee. ankle and

translnetatarsallevels

-should note a gradual decrease of approx. 20mmHg down the ipselaterallimb

72

-both limbs should be within 20mmHg at similar levels

-medial calcinosis results in falsely elevated pressures resulting in unreliable information

Indices 1.00- 0.9 0.89 - 0.7 0.69-0.5 0.49 - 0.3 0.29- 0.0

normal

mild

moderate impending gangrene distal neCrosis

-Segmental plethysmography

-air cuff technique (Raines) utilizes same cuffs as segmental pressure measurements

-inflate cuffbelow DBP - allows small changes in extremity volume to be translated as a

pressure change which is plotted versus time (PVR- pulse volume recording) crest- rapid vessel expansion in systole

normally <35%

dicrotic notch- due to elasic recoil

amplitude- normally 8 -12 mm, decreased in arteriosclerois

-other plethysmography techniques .

impedence: measures volume changes by assessing differences in resistance between 2 electrodes

mercury strain gauge: elastic tube filled with mercury is wrapped around extremity, as tube elongates with systolic expansion resistance changes. Photoplethysmography: sensor probe is placed on digit which contains infrared light and a sensor. The light reflected and absorbed by tissues is related to capillary bed volume.

Transcutaneous oxygen measurements

assesses tissue metabolism as a function of perfusion

< 20-30mmHg high correlation with dec. abilityfmability to heal extremity dependency increases TcP02 values

----------------~·tres~ct~esponse:

ambulate on a treadmill at 100% gradient at 2MPH for 5 minutes

at completion, perform AB! and repeat every 2 minutes until pre-exam level obtained, normal patients have no appreciable drop.

Magnetic Resonance Angiography:

avoids use of ionizing radiation and need for contast media as in arteriography allows multiple imaging planes

consider in pts. with renal failure

Spin Echo (SE) -blood appears dark ( low signal intensity) Gradient Echo (GRE) - blood appears bright ( high signal intensity)

Arteriography:

involves the injection of a organic iodine contrast medium in the arterial system which is then visualized with radiographs

usually reserved for patients that may be candidates for bypass surgery contraindicated in patients with a iodine allergy

need to stabilize Cr to <2.0

pts need to be Npo

anticoagulation given after procedure (plavix)

73

Treatment: (Conservative) Exercise

No tobacco Vasodilators Trental

Treatment: (Surgical) .

Balloon angioplasty - balloon catheter is fed into a localized area of stenosis and inflated to dilate the artery.

Bypass surgery - utilizes either autogenous vein graft or synthetic arterial prosthesis (gortex) to circumvent areas of occlusion and improve distal circulation

Laser angioplasty - laser probe is passed. percutaneously and then activated at the site of occlusion / stenosis to recanalize the vessel.

Atherectomy - utilizes a high speed rotational burr to ablate stenosis and improve flow.

II. VENOUS DISEASE:

Superficial Thrombophlebitis:

-palpable cord along distribution of superficial vein with local erythema, pain and edema

-treatment consists of-local warm compresses, elevation, rest and anti-inflammatory medications

-discontinue heplock if on the involved extremity

Deep Vein Thrombosis

Usee section on DVT**

MAGENTIC RESONANCE IMAGING GENERAL CONCEPTS AND PATHOLOGY

I. MRI Pbysics

A Based on the hydrogen atom.

1. Hydrogen atom is mack up of a single proton in its nucleus.

2. Hydrogen is abundant throughout om body.

3. Hydrogen has the highest gyromagnetic ratio known

a. Precession is when a proton spins around its axis when placed in a magnetic field (Exampleatop)

b. The fIeQUency of this precession is determmedbj1 the Larmor Equation:

(i) ='YXj3

ro = Precessional (Larmor) Frequency

"( Gyromagnetic Ratio

j3 = Strength of External Magnet

c. Since hydrogen has the largest gyromagnetic ratio, it precesses at a higher frequency.

B. Atoms with an odd number of protons and neutrons in their nucleus are small, rotating magnets that align themselves in the direction of the magnetic field

1. Nuclei in a sample tissue are randomly oriented until a magnetic field is applied.

2. BulkMagni{ication Vector (M) is the sum of the magnetic moments of all of the hydrogen nuclei

in a sample tissue. .

C. A radiofrequency is applied and protons are stimulated.

1. Resonance is a process where nuclei make transitions between low energy states (parallel to the magnetic field) and high energy states (anti-parallel).

2. A radiottequeney (RF) pulse induces transitions between energy states and tends to decrease the number of nuclei pointing to the main magnetic field

3. Before a 90° RF is induced:

a. Protons are in a longi tudmal, relaxed state parallel to the z axis.

74

b. Protons precess out of'phase with one another.

z

_H ...... t_X.V

I

e.

4. After a 90° RF is induced:

a. Protons are excited and flip into the transverse xy plane.

b. Protons precess in phase with one another.

M

--------~ .. ~~X-y

D. After the termination of the RF, the protons relax, radiowaves are emitted and an image is reconstructed onto the computer. The signal produced depends on the nwnber of nuclei present and the time it takes for the nuclei to relax.

1. Tl Relaxation (Spin-Lattice Relaxation):

a. Time it takes for nuclei to realign parallel (assume a low energy state) to the magnetic field M must return to the z axis.

b. Also called longitudinal relaxation.

c. Reflects the chemical environment of the proton. Strength in which the nuclei are bound to the chemical backbone (i,e, water, fat).

2. T2 Relaxation (Spin-Spin Relaxation):

a. An exponential decay of the xy component ofM back to O. ---------~b~gofthenuclei.

c. Also called transverse relaxation.

d, Reflects the relationship of the proton to the surrounding nuclei.

II. Pulse Sequences

A Spin Echo: Utilizes a 90° excitation pulse followed by a 1800 refocusing pulse. This istbe most common pulse

sequence used.

SpinEci10

9C]' 180-

~laJl nlo...----_

~--------~----------

1. TE (Time to Echo): Time between a 900 excitation pulse and the production of a spin echo.

2. TR (Tune to Recovery): Time between 90° excitation pulses.

75

B. TI-Weighted Pulse Sequence:

1. Short lE (l5-3Omsec).

2. Short TR (200-600msec) .:

3. Main focus is on TR As TR increases, the difference in signal intensity (SI) decreases.

1. Tl is called the FAT image. Fat displays a high

S1 lONGI"TUOOW.. RELAXATION (Ttl

2. Used for normalAM4TOMY.

lima (m)

1"41ot. _"'~~_irl_~

c. T2-Weighted Pulse Sequence: t21-

1. Long lE (60-8Omsec).

2. Long TR (2000msec).

3. Main focus is on lE. As lE increases, the difference in SI increases.

4. T2 is called the WA TER image. The following display a high SI:

a. Fluid, edema., hematoma.

b. Infection, inflammation.

c. Tumor,

5. Used for PATHOLOGY.

.E'W"U"h.I.L..11o.M- I'~"""""" ... ~1

. .

D. Proton Density Qntennediate-Weighted Pulse Sequence):

1. Short lE (20-4Omsec).

2. Long TR (1500-2000msec).

3. Has a high signal to noise ratio (SIN). .

a. SIN is the ratio between the strength of the signal coming back from the nuclei and the

. ~m=· t_~.tt of the noise from the patient and theB,m~~~c;h!!!j~n~e.~ _

4. Good for delineating basic anatomy.

T .... ("Iii)

It&. '~7. 1'IU4 __ .~ __ """'_

...... ~_"'rr_

E. Fast Spin Echo:

I. Quicker than normal spin echo 1'2 sequence.

a. For each spin echo pulse sequence, multiple samples are used.

b. Instead of one data line per phase pulse, there are several sample echoes in the train of each TR sequence.

2. Sacrifice slight resolution for speed.

a. An increase in speed results in a decrease in phase direction resolution 3. Slight shift in contrast clue to longer TR.

a. Typically use longer TR (4000-5000), but multi-echo train still makes it faster than normal T2.

F. STIR (Short Tl Inversion Recovery):

1. A 1800 excitation pulse followed by a standard spin echo.

2. IfTI is very short, the 900 pulse of the spin echo will occur at a time when theM of fat is near O. a. Any tissue with a short TI, such as fat, will be suppressed. Not specific for fat

3. Tissues with longer Tl values will be non-zero.

a Any tissue with a long TI, such as water, will produce a high SI.

4. Useful for evaluating edema in high lipid regions, such as bone marrow.

a. Also useful in evaluating articular cartilage because the joint fluid is bright and the cartilage is darker.

76

5. When contrast agents that shorten TI are used, such as gadolinium. the desired contrast enhancement may be suppressed.

..". ....

..... '!'

:

-

u.

G. Fat Saturation:

I. Utilizes RF pulses prior to the spin echo sequence.

2. RF pulse centered at the lipid resonant frequency will saturate lipid protons and rotate them into the ."':y plane.

8. Subsequent pulses rotate the lipid protons out of the xy plane and their resonance is inhibited

3. Water protons are unaffected because there are differences in the resonant frequencies between the lipid and water nuclei.

4. This technique is specific for fat, whereas, the STIR. technique is not.

5. Excellent technique to use with gadolinium to suppress high SI of fat.

H. Gradient Echo:

L Starts with 8 RF pulse < 90°.

a. Protons are not completely rotated into the xy plane.

b. Sufficient magnetization remains along the z axis which allows for short 1R times. Shorter TR times means shorter imaging times.

2. A short TR time does not allow the nuclei to completely dephase before the next RF is induced.

a Common in tissues with long T2 relaxation.

3. Magnetization is "recycled".

4. Also called steady state magnetization.

5. Good for joint imaging.

6. Bone marrow has a low SI.

I. Gadolinium:

1. Paramagnetic metal ion.

a. Chelated with DTP A to avoid side effects.

b. Shortens Tl.

2. Good to use with fat saturation.

3. Two methods of use:

a. Intravenous:

I. Quickly distributed into- intracellular fluid

2. Areas with increased vascularity, such as neoplasms and inflammation, enhance rapidly and retain contrast longer. **Cellulitis and walls of abscesses will enhance but pus will not.

b. Intraarticular:

1. Assess cartilage integrity, such as talar dome lesions.

III. Pathology

A Bone Contusions (Bruises):

1. Trabecular microfractuIe with edema and hemorrhage.

2. Poorly defined, inhomogenous, low SI on T1.

3. High SI on T2 because of acute hemorrhage.

B. Stress Fractures:

1. Gradual and progressive resorption of lamellar bone and replacement of dense osteonal bone.

2. Characterized by local hyperemia, edema, and osteoclastic activity.

3. Tl- linear zone of decreased SI surrounded by less defined area of lower SI.

77

4. TI-linear zone continues to have decreased SI. but surrounding edema has a higher SI.

5. STIR- increase in SI because fatty bone marrow is suppressed.

6. Callous formation has an intermediate SI.

c. Neuropathic Osteoarthropathy:

1. Difficult to differentiate from osteomyelitis.

a Marrow signal changes without involvement of the periosteum/cortex is Dot suggestive of

osteomyelitis.

2. Tl- marked decrease in SI within medullary and cancellous bone.

3. T2- mild increase in SI (less than with osteomyelitis).

4. STIR- marked increase in S1

D. Acute Osteomyelitis:

1. T'l- involvement of cortex/medullary bone shows slight areas of increased SI against background of normally low SI cortex.

2. TI and STIR- increased SI in the cortexlmedullary portion of involved bone. a Reflects a decrease in the normal fat content and localized pusledema.

E. Chronic Osteomyelitis:

1. Sinus tracts- linear areas of increased SI on TI.

2. Foci may demonstrate the "rim sign".

a Low SI area smrounded by an area of local, active disease.

F. Soft Tissue Infections:

1. TI and STIR sequences are preferred for detection of cellulitis and abscesses.

a. Increased SI causing distortion of superficial soft tissues is seen in cellulitis.

b. Well-marginated, homogenous, high SI collections are seen with abscesses.

c. Gas contained within abscesses display low SI on all sequences.

G. Osteonecrosis (AVN):

1. Hallmark is a reactive interface.

a A distinct layer of inflammatory fibromesencbymal tissue between viable and infarcted bone. 2. Tl- well defined line of decreased SI.

a. Represents granulation replacement offat.

3. T2- decreased SI.

4. STIR and long TI- "double rim sign" (Mitchell & Kressel).

a. Inner margin- increase in SI. Represents granulation tissue.

______ -----"'b"---. _Qut~unal:gin~-dec-r-easejn-S-LRepLeSents-min-eralizatiQn.~----------------- 5. Staging System:

a. Class A- characteristics similar to fat

1. T 1- increase SI.

2. T2- intermediate SI.

b. Class B- reflects subacute hemorrhage with chaIacteristics of blood. 1. Tl and T2- increase SI.

c. With increasing fluid development in necrotic tissue; SIon Tl decreases and SIon T2 increases.

d, During the late stages when fibrosis and sclerosis take place, there is a decrease in SI on Tl andT2.

R Osteochondral Lesions:

1. Tl- decreased SI within subchondral bone.

2. TI- SI may be increased

3. STIR- marked increase in SI.

4. Gadolinium may be helpful. Will have a high SI on Tl.

I. Tendons:

1. Low SI on all sequences due to lower water content

2. T2 and STIR- optimizes the contrast between the dark tendon and the abnormal increase in water content of the tendon.

3. Classification ofPf Tendon Tears:

a. Type J- incomplete, hypertrophy 4-5 times normal.

78

b. lJ!J!g_jl- partial tears, longitudinal splitting. Tendon becomes attenuated and atrophic and assumes a long, ovoid configuration.

c. Type IlI- complete tear, retraction of ends with obvious gap. Ends may hypertrophy. (Need to distinguish from Type I.)

J. Soft TISSUe Masses:

I. Most demonstrate low to intermediate SI on TI and high SI on TI.

2. Certain benign lesions, such as lipoma, hemangioma, and ganglions reveal. characteristic morphology.

K. Bone Tumors:

1. Most reveal low to intermediate SI on Tl and high SI on T2.

2. Lesions with high SI on Tl contain either fat or blood..

3. Very low SI seen on T2 images in cellular, densely fibrous lesions, and in lesions containing bone.

L. Septic Arthritis:

1. T2 and STIR- increased SI within surrounding soft tissues as well as joint fluid is suggestive.

2. Need clinical correlation. .

M. Metal6c Artifact:

1. Two mechanisms:

a. Ferromagnetic objects have their own magnetic field They cannot be utilized in the MRI machine.

b. Most orthopedic implants are non-ferromagnetic.

1. Distorts RF uniformity.

2. Causes low SI adjacent to implant but no more than 1 em away.

N. Magnetic Resonance Angiography:

1. Most of the experience is with extra-cranial carotid and intra-cranial circulation.

2. Non-invasive technique.

3. Lower extremity indications:

a. PVD.

b. DVT.

c. Neoplasms.

d. Anatomic study preoperatively.

Radiology of Infection

I. Nuclear Medicine Studies:

A Technetium - 99m:

1. Most commonly administered in the form ~c methylene diphosphonate (fc-MOP) & hydroxymethylene diphosphonate (Tc-HMDP).

2. Binds directly to calcium hydroxyapatite to form soluble salts. Referred to as a "bone seeking" agent.

3. Isotope concentrates in active new bone formation due to osteoblastic activity related to bone injury.

4. Most widely used nuclear medicine study.

5. NON-SPECIFIC - Positive Scans For:

Osteomyelitis Recent Sur~ry
Neuropathic Osteoarthropathy Arthritis
Bone TumoIS RSD
Fracture Ischemic Necrosis of Bone 79

6. Half life = 6 hours.

7. Remains positive for extended periods in cases of osteomyelitis & post-osseous surgery because of continued remodeling and osteogenesis.

8. Increased levels of isotope in the following sites in nonnaI bone:

• Cancellous bone> cortical bone

• Metaphyseal bone

• Periarticular regions, especially sacroiliac joints

• Epiphyseal plates

• Tips of the scapula

• Petrous portion of the skull

• Costochondral margin of the ribs

• Sternum

9. Extra-skeletal sites where the isotope occurs;

• Kidneys

• Bladder

• Nasopharynx

• Orophmynx

• Glandular tissue of the breasts

• Lacrimal appuatus

10. Phases of Bone Scanning:

a. Angiogram (Immediate. Early, 1st phase):

• After N injection of the isotope, several images are taken 1-3 seconds apart as the isotope travels to the extremity.

• Displays arterial blood flow to the extremity.

b. Blood Pool (2"" phase):

• Images are taken 2-5 minutes post N injection.

• Quantitatively describes "blood pool" occurring in the capillaIy beds & veins.

c. Delayed (3rd phase):

• Isotope seeks bone & demonstrates the amount of osteoblastic activity present

• Images are taken 2-4 hours post N injection.

• Represents the bone uptake for repair or maintenance. d, Fourth Phase:

• Images taken 5-24 hours post IV injection.

~ • __ The.delay-in_the.timepost_injection-demonstrates-more-bone-activity-and-Iess-soft-tissue

activity.

• There is a decrease in the sensitivity because the half-life is 6 hours.

11. Interpretation;

a. Evaluate all phases simultaneously.

b. The angi~ & blood pool phases will be "hot" in both soft tissue and bone infections.

c. Use the 3 or 4th phase bone scan to differentiate soft tissue infections from bone infections.

Blood Pool

3rdPbase

4th Phase

Osteomyelitis

focal uptake

hot

hot

Cellulitis

diffuse uptake

neg

neg

Septic Arthritis

diffuse uptake

diffuse uptake

diffuse uptake

B. Gallium -67 Citrate:

1. Localization in areas of acute inflammation & infection by binding mechanisms;

a. Direct bacterial uptake by bacterial siderophore complex.

b. Lactoferrin, a plasma protein released by leukocytes.

c. Direct leukocyte labeling (only 6%).

2. Scan is taken 48-72 hours after injection of the isotope.

80

3. Like technetium, it is non-specific. It is not indicated for detection of osteomyelitis but sequential technetium - gallium scans can be used.

4. Returns to normal after infection and inflammation have resolved.

5. Positive scan may represent

• Infection

• Fractures
• Inflammation
• Epiphyseal plates
• Surgical trauma
• Neoplasms
• Gout C. SeQUential Technetium -Gallium Scan:

1. 4 hour technetium bone scan followed by a 48-72 hour gallium scan.

2. Increases the specificity for infection.

3. Positive sequential Tc - Ga scan if the gallium uptake is greater than the technetium. uptake.

4. Normal scan if the gallium uptake is spatially congruent but is less than the technetium scan

Technetium

GaUhim

Acute Osteomvelitis

+

+

Septic Arthritis

+

+

Chronic Osteomyelitis

+

Cellulitis

+

D. Indium -111 Oxime:

1. WBC's are isolated from blood taken from the patient & labeled with indium -111. The tagged cells are then reinjected back into the patient The scan is performed 24 hours later.

2. Highly sensitive & specific for acute soft tissue & osseous infections.

3. May not be effective in diagnosing chronic osteomyelitis because it is predominantly a lymphocytic response & will not cause localization of indium labeled neutrophils.

a Acute & subacute osteomyelitis = neutrophil response = + scan.

______ ---'b. C.hrQnic_osteom}'elitis = predomjnan~c = neg~l~-------~-------

4. Can be helpful in differentiating osteomyelitis versus osteoarthropathy.

5. Half life = 67 hours. -

6. Interpretation;

a (+) Scan - Presence of indium labeled WBC's - Higher uptake than surrounding bone

- Acute osteomyelitis

b. (-) Scan - No localization of indium labeled WBC's - Chronic osteomyelitis, osteoarthropathy

7. False positives may occur with:

a. Aseptic soft tissue and bone inflammation such as periostitis secondary to Charcot changes.

b. Hyperemia & hypervascularity which mayor may not be associated with inflammation such as fractures, arthritis, inflammatory bone disease.

E. Technetium - 99m - HMPAO Leukocytic Scintigraphy:

1. Developed in order to have a tagged WBC study which is technically easier & with less radiation than indium. This allows for more radioactive material to be used & increased anatomical contrast.

2. Tagging molecule = Hexylroetbyl propylene amine oxime.

3. Performed by obtaining 50ml of whole blood and spinning offWBC's then tagging/labeling them. 2ml of the preparation is then reinjected. The scan is performed 3 hours later.

81

4. May show advantages over other nuclear medicine studies in diagnosis of early osteomyelitic changes; however, it is non-specific and any area of inflammation will be hot,

5. A negative scan can rule out a septic process with high precision (94%).

6. A positive scan may not discern septic from aseptic inflammation with sufficient accuracy.

Positive predictive value = 62%. Note - a subtraction technique is available and may yield higher results.

Il. Magnetic Resonance Imaging:

Please refer to the MRI General Concepts & Pathology Section for basic principles. A Cellulitis:

I. Increase signal intensity (S1) on T2-weighted & STIR images.

2. Diffuse, infiltrative pattern throughout subcutaneous tissues.

3. On TI-weighted images, a decrease in SI occurs due to the inflammato:ry process infiltrating & replacing normal high SI for subcutaneous fat

B. Abscesses:

1. Increase in SI on T2 & STIR images.

2. Localized, well-demarcated, relatively homogeneous signal.

3. Can add contrast agent, such as gadolinium, to improve contrast between normal tissue & pathological lesions.

4. Note- If abscess is filled with necrotic tissue or purulence, instead of inflammatory fluid, the SI decreases.

C. Osteomyelitis:

I. T2 preferred over STIR image for detection of osteomyelitis.

2. Increase SI on T2 & decrease SI on Tl.

3. Detects changes in the medullary canal, cortex, and periosteum.

• Must have changes in both the medullary canal & cortex to be indicative of osteomyelitis.

D. Chronic Osteomyelitis:

I. Opposed to acute osteomyelitis, where the cortex & medullary cana1 are destroyed, they are being remodeled in the chronic state.

2. Cortical changes are more extensive than the marrow & are low SI on TI.

________ ~3~_Areas_of_acthrejnfection_CJlD stiU be seen as increase_sLon-12-contrasted-agaj.nst-Iower-SLon----thickened surrounding bone. This is known as the "rim sign".

• The rim of low SI reflects fibrous tissue surrounding focal, active disease.

4. "Healed" osteomyelitis displays an increase in SI on Tl. This represents the infiltration offat back into the marrow after resolution of the infection.

E. Septic Arthritis:

I. Joint fluid is best detected with T2 & STIR images.

2. To differentiate septic arthritis from non-infectious effusions, an increase of SI of surrounding soft tissue must also be seen.

F. Advantages ofMRI:

1. More sensitive and specific for infection than other imaging modalities.

2. Assistance in pre-operative planning;

a Helps localize infection & determine the proximal &distal boundaries.

• Particularly helpful with long bones & the calcaneus.

• Helps determine necessary level of amputation or debridement; 3. Evaluate for other possible sources of infection:

• Example: Patient with cellulitis not responding to current treatment protocol. Need to rule out abscess or osteomyelitis.

4. Monitor infectious process:

82

ill. Computed Tomograpby (CU:

A Basic Principles:

1. Coronal and transverse plane images only. Sagittal plane images available via reconstruction

on computer.

2. High-energy ionizing radiation.

3. Metallic implants will result in artifacts & distortion of images.

4. Windows vary density

Highest Density Lowest Density

Cortical Bone

Calcification

Cancellous Bone

Nerve

Tendon

Ligament

Cartilage

Fat Air

B. Clinical application of CT to evaluate infection:

1. Use of Soft tissue window can demonstrate abnormal soft tissue density which may represent

suppuration, reactive granulation tissue, edema,. or fibrosis. .

2. Can be used to evaluate plantar compartments of the foot to diagnose plantar space infections.

3. Use of the bone window can demonstrate the osseous and articular alterations.

a. Early stages of osteomyelitis is noted as an increase in the density of the bone marrow. b, Can be used to establish the extent of the infection & help determine the approximate level of amputation or debridement

Rheumatic Diseases

GOUT

Gout is a metabolic disorder in which tissue deposition of monosodium urate crystals occurs from supersaturated extracellular fluids and results in one or more clinical manifestations which include gouty arthritis, tophi, gouty nephropathy, and uric acid calculi in the kidneys. *** Asymptomatic hyperuricemia in the absence of gout is not a disease state.

Gout is predominately a disease of adult men with a peak incidence in the fifth decade. It is the most common cause of inflammatory arthritis in men over age 30. Gout rarely occurs in men before adolescence or women before menopause. Serum urate concentIations rise from normal childhood mean values of 3.5-4.0 mgfdl to adult levels during peberty in young men. In contrast urate levels remain rather constant in women until menopause. The discrepancy in serum urate levels between the sexes during ttre reproductive years appears to stem from the action of estrogens which promote renal excretion of uric acid Serum urate levels in women rise after cessation of the menses. The disease is widely distributed amongst many races .

.. PURINE BIOSYNTHESIS (KEY STEPS ) 1. RAW LIMITING S1EPS

Mg2+

nbose-5-phosphate+ AlP ---------.> PRPP

PRPP synthetase

PRPP + glutamine r===================;" 5 phosphonbosyl-I-amine I amidophosphoribosyJ transferase I

83

2. SALVAGEPATIIWAYS

Hypoxanthine + PRPP -----------> Inosinic Acid

HGPRT

Gmmme+PRPP----------------~GmnrylicAcid

HGPRT

3. URIC ACID FORMATION

Hypoxanthine Xanthine Uric Acid

Xanthine Oxidase Xanthine Oxidase

KEY

Enzymes listed in bold print

PRPP = phosphoribosyl pyrophosphate

HGPRT = HypoxanthiJle..Gaun Phosphonbosyltnmsferase

PAlHOGENESIS

1. Gout in humans arises from the species-wide lack of the enzyme uricase which oxidizes uric acid to allantoin.

2. In humans uric acid is derived both from the ingestion offoods containing purines and endogenous synthesis of purine nucleotides.

3. Under steady state conditions renal excretion is the major route of uric acid disposal The kidney accounts for approximately two-thirds of uric acid excretion. Bacterial oxidation of urate excreted into the gut is the major mechanism of extra-renal disposal

4. Urinary uric acid excretion is the major a<ljustable mechanism for maintaining urate hemostasis. The capacity of extra-renal uric acid disposal is greatly limited in comparison to the kidney.

5. Increased uric acid production or diminished uric acid excretion by the kidney operating alone or in combination have been demonstrated to eontn"bute substantially to the hyperuricemia of gout

PAmOLOGY

L Urate crystallizes as a monosodium salt in over-saturatedjoint tissue.

2. Decreased solubility of sodium urate at lower temperatmes of peripheral structures such as toes and ears may explain why urate crystals deposit in these areas.

3. Histopathology of tophi reveals a chronic foreign body granuloma surrounding a core of monosodium urate.

4. The inflammatory reaction around the crystals consists mostly of mononuclear cells and giant cells.

5. Urate crystals appear to be directly able to initiate and sustain intense attacks of acute inflamniation because of their capacity to stimulate the release of several inflammatory

mediators.

STAGES OF GOUT

I. Asymptomatic Hyperuricemia

2. Acute Gouty Arthritis

3. Intercritical Gout

4. Chronic Tophaceous Gout

~~CATIONSOFGOUT

I. PRIMARY : refers to those circumstances in which elevated serum urate levels or urate deposition appear to be consequences of inherent disorders of uric acid metabolism not associated with another acquired disorder and in which gout is a prominent feature of the clinical picture.

84

2. SECONDARY: refers to those circumstances in which gout is a minor clinical feature secondary to any mnnber of genetic or acquired processes.

3. URIC ACID OVERPRODUCTION: approximately 100/0 of petients with hyperuricemia or gout excrete excessive quantities of uric acid into the urine. Overproduction of uric acid occurs with some frequency in a variety of acquired and genetic disorders characterized by excessive rates of cell and nucleic acid turnover such as myeloprolifeIative and lymphoproJiferaitve disorders, hemolytic anemias, and psoriasis ( i.e. secondary gout ).

Uric acid overproduction can also occur secondary to inherited derangements in mechanisms of endogenous purine biosynthesis. In both partial deficiency of HGPRT and PRPP synthetase overactivity early onset gout and renal urate calculi constitute the usual clinical manifestations. Severe HGPRT deficiency is associated with spasticity, choreoathetosis, mental retardation, and compulsive self-mutilation (Lesch-Nyham Syndrome ) ( i.e, primary gout ).

4. URIC ACID UNDERSECRETION : the majority of'padents with hyperuricemia or gout (up to 90 %) show a relative deficit in the renal excretion ofmic acid Vutually all plasma urate is filtered by the glomerulus, with greater than 95% of the filtered load undergoing proximal tubular resorption. Subsequent proximal tubular secretion contributes the major share of excreted uric acid A diminished tubular secretory rate may contribute to hypermicemia, as well as increased tubular reabsorption or dinrinjsherl uric acid filtration.

CLINICAL FEATURES

1. Acute gouty arthritis is the most common early clinical manifestation.

2. The 1st MIP joint is involved most often and is affected at some point in 75% of patients. In approximately 100/0 of patients there is never a recurrence, but up to 6()O/o of patients experience a second attack in less than a year.

3. The ankle, tarsal area, and knee are also commonly affected.

4~ The first episode of acute gouty arthritis frequently begins abruptly in a single joint, often dming the night, so that the patient awakens with dramatic, unexplainedjoint pain and swelling.

5. Affected joints are usually red, hot, swollen, and extremely tender. Diffuse erythema may be present which can be confused with cellulitis or thrombophlebitis.

6. Acute attacks of gouty arthritis may be associated with high grade fever and systemic symptoms which may be falsely interpreted as infection.

'7~The--most~common--sites-toi--wplll are tDe1iiSe of the great toe, AChilles tendon, olecranon bursae, knees, wrists, and hands.

8. Approximately 10-200/0 of patients with primary hypermicemia develop uric acid kidney stones.

9. Renal disease is the most common complication of gout except for the arthritis.

RADIOGRAPIDC FINDINGS

1. The earliest changes in gout are soft tissue swelling andjoint effusions.

2. A lace pattern of erosion may be seen as an early change with a fine striated pattern of periosteal reaction along the cortex acljacent to a tophus.

3. Bony erosions tend to be round or oval with a sclerotic margin and have been described as " rat-bite erosions ", cyst-like, or

" punched-out " erosions.

4. A feature distinguishing gout from other arthritides is the presence of destructive lesions in bone that are remote from the articular surface.

5. Many of the lesions are expansile with overhanging margins (Martel's sign) that are displaced away from the axis of the bone.

6. Generally joint spaces are preserved until late in the course of the disease.

7. Ankylosis andjoint subluxation may occur in very advanced cases.

8. Tophi may be visualized within the soft tissues.

85

LABORATORY FINDINGS

1. *** Demonstration of synovial fluid monosodium urate crystals is now generally considered mandatory for establishing the diagnosis of gout as an explanation of acute arthritis. Monosodium urate crystals are needleshaped and display a negative birefringence (bright yellow) when viewed under a polarizing light microscope parallel to the axis of slow vibration marked on the polarizing microscope.

2. Synovial fluid leukocytes are elevated ( 20,000-100,000 cells per cubic millimeter) with a predomination of neutrophils.

3. The value of serum uric acid levels in the diagnosis of acute gout is limited; levels can be normal at the time of an acute gouty attack

4. The ESR ( erythrocyte sedimentation rate ) is generally elevated at the time of an acute gouty attack.

5. An elevated serum WBC count may also be seen with an acute attack

TREATMENT

ACUTE GOUT

L COLClllCINE: a potent inhIbitor of inflammation, bot also a very toxic drug. The mechanism of action appears to be interference with several steps of the inflammatory response in which neutrophils play a central role. Intracellular interference with the organization of labile fibrillar microtubular systems concerned with cell structme and movement may lead to micotubular disaggregation and to decreased neutrophil motility, chemotaxis, release of chemotactic factors, and lysosomal degranulation

Dose: 1-2 mg IV over 2-5 minutes

0.5 mg po taken hourly until pain reduction. GI toxicity, or max. dose of 8 mg

It has been suggested that the prompt response of arthritis to colchicine is diagnostic of gout, bot it should be noted that other arthropathies have been shown to respond to colchicine, and the response to colchicine may be variable if it is started 24 hours after an acute gouty attack

ARTHROPATHIES WHICH MAY RESPOND TO COLCHICINE ADMINISTRATION

1. Gout 7. Sweet's Syndrome

2. Pseudogout 8. Serum Sickness

3.Fmnilim~M~~~·---·~F-ev-er------------~9~.~HY~dro~~~pm~·re~-----------------------------

4. Sarcoidosis 10. EIythema Nodosum

5. Behcei's Syndrome II. Rheumatoid Arthritis

6. Amyloidosis

The oral administration of colchicine may cause gastrointestinal toxicity in up to SOOIo of patients.

Nausea, vomiting, diarrhea, and cramping abdominal pain may be severe. The gradual use of smaIl repeated doses is meant to minimize GI toxicity. The maximum dose should not exceed 8 milligrams over a 24 hour period

Avoidance of GI toxicity is one of the advantages of intravenous colchicine. WIth proper use the only anticipated side effects are thrombophlebitis if not properly diluted and skin sloughing if extravasated Excessive intravenous dosages may produce bone marrow suppression, renal failure, myopathy, disseminated intravascular coagulation, severe hypocalcemia, cardiopulmonary arrest, seizures, and death. The maximum daily dose should not exceed greater than 4 milligrams over a 24 hour period After intmvenous therapy no more colchicine should be given for at least 7-10 days.

2. NSAIDS: are also potent inhibitors of inflammation The mechanism of action appears to be inhibition of the enzyme cyclooxygenase, In general NSAIDS are started at the maximum daily dosages at the :first sign of an acute gouty attack and gi"adually tapered and continued until the arthritis has resolved Although less toxic than colchicine, NSAIDS may cause significant side effects involving the GI tract, kidneys, and hematologic

86

systems. Among NSAIDS most physicians prefer indomethacin for acute gouty arthritis. Other NSAIDS with proven efficacy include sulindac, naproxen, piroxicam, ketoprofen, tolmetin sodium, and meclofenamate sodium.

3. Corticosteroids : generally not recommended for parenteral use in acute gouty arthritis because the effects are inconsistent and rebound attacks are :frequent upon discontinuation. Intra-articular steroid injections are of benefit in conjunction with colchicine or NSAIDS, or in patients who are unable to take colchicine or NSAIDS due to various medical conditions.

4.· ACIH : is produced by the pituitaIy gland and causes release of glucocorticoids :from a normal functioning adrenal gland. Exogenous forms can be administered with effects similar to corticosteroids.

CHRONIC GOUT

1. COLCJnCINE : generally given as 0.5-0.6 mg by mouth two to three times daily as a prophylactic measure.

2. ALLOPURINOL : potent inhibitor of uric acid formation by blocking the action of the enzyme xanthine oxidase. Dosed as 300 mg po qdlbid

3. URICOSURICS ( PROBENICID, SULFINPYRAZONE) : both increase uric acid excretion by the kidneys and effectively lower.serum uric acid levels.

*** Allopurinol, probenicid, and sulfinpyrazone should not be started during an acute gouty attack. Rapid mobilization of uric acid and sudden changes in plasma urate levels may predispose an individual to an acute gouty attack.

4. CHANGE IN DIET

SURGICAL PROPHYLAXIS

To prevent periopeiative acute gouty attacks colchicine 0.5-0.6 mg orally t.i.d 3 days before and 3 days after surgery is recommended

DIFFERENTIAL DIAGNOSIS OF PODAGRA

I. GOut

2. CPPD Disease

3. Rheumatoid Arthritis

4. Sarcoidosis

5. Sesamoiditis

6. Infection

7. Seroneg. Spondyloarthropathies

8. Osteoarthritis

9. Type n Upoproteinemia

10. Calcium Hydroxyapatite

11. Calcium Oxalate

12. Paget's Disease

13. Calcific Tendonitis

14. Trauma

PATIENTS AT RISK AND PRECIPITATING FACTORS FOR GOUT

- male patient with 1st M1P joint arthritis and hyperuricemia

- patients with a history of uric acid renal calculi

- alcoholics .

- chronic renal insu::fficiency

- post N contrast dye

- post treatment for hematologic malignancy

- stress, trauma, or surgery

87

CONDmONS ASSOCIATED WITH GOUT AND HYPERURICEMIA

- medications

low dose salicylates diuretics

TB medications warfarin nicotinic acid

- hemopoietic and neoplastic disease

- hypertension, ischemic heart disease. and hyperlipidemia

- acute myocardial infarction

- diabetes mellitus

- chronic lead poisoning ( satmnine gout )

- glycogen storage disease

- sarcoidosis

-psoriasis

- chronic renal insufficiency

- sickle cell anemia and other hemoglobinopathies

- pernicious anemia and polycythemia vera

- hyperparathyroidism

-obesity

- regular alcohol consumption

-myxedema

- toxemia ofpregnancy

- chronic berrylinm disease

- Down's syndrome

CALCIUM PYROPHOSPHATE DIHYDRATE CRYSTAL DEPOSITION DISEASE

CPPD crystal disease, also called pseudogout., is an inflarrimatmy process wherein crystals are deposited in tendons, ligaments, articular capsules, synovium, and cartilage. The incidence of clinically

-symptomatic-disease-is-about-one-half-that-Qf-elassie-geut.-'fhe-male-to-female-ratio-is--about-H·~-!-;-Radiologic studies show a steady increasing prevalence with age so that nearly 5()O/o of individuals have evidence of chondrocalcinosis ( radiographic appearence of calcified cartilage ) by the ninth decade of life.

CLASSIF1CATION OF CPPD CRYSTAL DEPOSmON DISEASE

1. HEREDITARY

2. SPORADIC ( IDIOPATIllC )

3. ASSOCIATED WITH METABOLIC DISEASE Hyperparathyroidism

Hypothyroidism

Hemochromatosis

Hemosiderosis

Hypomagnesemia

Gout

Amyloidosis

4. ASSOCIATED WITH JOINT TRAUMA OR SURGERY

CLINICAL FEATURES

1. Acute pseudogout is marlred. by inflammation in one or more joints lasting for several days or more.

2. These self-limited attacks can be as abrupt in onset and as severe as true acute gout, although the average attack is less painful.

88

3. The knee is the site of almost one-half of all attacks, although involvement of nearly all joints, including the 1st MIP joint, has been noted

4. Provocation by surgical stress, acute medical illnesses, and trauma can cause acute attacks of arthritis.

5. Due to both the inflammatory and degenerative features of CPPD crystal disease, it may simulate diseases such as rheumatoid arthritis, ankylosing spondylitis, and neuropathic osteoarthropathy.

RADIOGRAPHIC FEATURES

1. The typical appearence of punctate and linear densities in articular hyaline or fibrocartilaginous tissues is diagnostically helpful.

2. Calcific deposits may also occur in articular capsule, ligaments, and tendons.

3. Subchondral bone cysts and hook-like osteophytes may be seen.

LABORATORY FINDINGS

1. *** The precise diagnosis of pseudogout is made by identification of CPPD crystals in joint fluid The crystals generally have a needle-shaped or rhomboid appearence and display positive birefringence when viewed under a polarizing light microscope.

2. Synovial fluid leukocytes may be elevated ( 1,000-50,000 celIs per cubic millimeter) with a predomination of neutrophils.

3. A systemic leukocytosis and elevated ESR may be present

TREATMENT

Unlike gout, there is no way to effectively remove CPPD crystals from the joint Acute attacks in large joints can be treated by thorough aspiration alone or combined with injection. of microcrystalIine corticosteroids. NSAIDS are often very useful. Colchicine given intravenously is effective, whereas the effectiveness of oral colchicine is less predictable. But both the number and duration of acute attacks can be reduced. by colchicine. It is important to note that treatment of associated diseases does not result in resorption of intIa-articualar CPPD crystal deposits.

NEUROPATHIC OSTEOARTHROPATHY

WIthout any appreciable external cause we may see, between one day and the next, the development of a general and often enormous tumefaction of a limb, most commonly without any pain whatever, or any febrile reaction. At the end of a few days the general tumefaction disappears, but a more or less considerable ~~~~~-swelling-ofthejoint--remains.-owing101he-t'ormatiollo--rahy&art1lus; arid sometimes to the accumulation of a liquid in the periarticular serous bursae also. On puncture being made, a transparent lemon-colored liquid has been frequently drawn from the joint

One or two weeks after the invasion, sometimes much sooner, the existence of more or less marked cracking sounds may be noted, betraying the alteration or-the articu1ar surfaces which, at this period. is already profound. The hydrarthus becomes quickly resolved, leaving after it an extreme mobility in thejoint, Hence consecutive luxations are frequently found, their production being largely aided by the wearing away of the heads of the bones which has taken place. I have several times observed a rapid wasting of the muscular masses of the limbs affected by the articular disorder ... Besides the wearing down of the articu1ar surfaces ... you may notice the presence of foreign bodies. of all the customary accompaniments of arthritis deformans. .. I am led to believe that .. they are all produced in an accidental manner, and to all appearances chiefly by the more or less energetic movements to which the patient sometimes continues to subject the affected limbs. ..

JM Charcot, 1868

I. DEFINTI10N

neuropathic osteoarthropathy implies disease of nerves that leads to underlying bone and joint abnormalities first described by J.M Charcot in 1868

n. PATHOGENESIS

Exact cause is still unknown, but both neurovascular and neurotIaumatic theories exist and may both play a crucial role in the development of this disorder

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Neurovascular Theory ( French Theory ) : damage to. trophic nerve centers with an alteration in the sympathetic control of blood flow to. bones and joints leads to. persistent hyperemia and active bone resorption

Neurotrawnatic Theory ( German Theory ) : An extreme progression of degenerative joint disease following loss of proprioception and protective pain sensation

III CAUSES

Diabetes Mellitus Syringomyelia

Tabes Dorsalis ( Lues) Excess ethyl alcohol Myelomeningocele Spinal cord injmy Poliomyelitis CMTDisease Riley-Day Syndrome Spina Bifida Congenital Insensitivity Acrodystrophic neuropathy

Amyloidosis

Leprosy

Tuberculo.sis

Uremia

Pernicious anemia peripheral nerve injwy Multiple Sclerosis Dejerine-Sottas Disease Tumor invading nerve Brain iniury Intra-articular corticoPhenylbutazone and indomethacin

*** Diabetes mellitus, Syringomyelia, and Tabes Dorsalis are the three most common causes of Cbarcot Joints.

Specific diseases with a predilection for the lower extremity :

Diabetes Mellitus - tarsal, tarsometatarsal. and MIP joints Amylo.id neuropathy - ankle, tarsal joints Myelo.meningocele - ankle, tarsal joints

Acrodystrophic nemopathy - ankle, MfP joints

Congenital insensitivtity to. pain - ankle, tarsal joints

IV. INCIDENCE OF CHARCOT JOINTS

** The most common came of Cbarcot Joints is Diabetes Mellitus **

5-10010 o.f patients with Diabetes Mellitus 10% of patients with Tabes Dorsalis 25% of patients with syringo.myelia

V. CLINICAL PRESENTATION

>red, hot, and swollenjoint(s) >defo.rmed, UDSIableJhypermobile joint(s)

>PAIN may or may not be a feature owing to the degree, progression, and duratio.n of the underlying illness, and when present is often mild considering the amount of joint distension and destruction that may be evident

>variable neurologic exam with decreased sensation, atrophy o.fintrinsic muscles, and diminished patellar! Achilles reflexes

>variable vascular exam. but generally pulses are easily palpable >spontaneous :fractureIdislocatio.ns

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>deformity possible in all three cardinal body planes, but especially common in the sagital plane ( rocker- bottom foot)

>possible ulcerations over displaced/dislocated bony prominences

VI. RADIOGRAPIllC FINDINGS

The two classic radiologic forms are atrophic and hypertrophic arthropathy. Atrophic arthropathy is encountered more commonly in the 1JA'ef extremity, whereas hypertrophic arthropathy is encountered more commonly in the lower extremity. The underlying disease does not determine which form predominates.

A1ROPIDC NEUROPATIllC OSJEOARTIfROPATIIY

>extensive resorption of bone ends

>osteoporosis is present, and destruction ofbone may lead to :fracture&Idislocations >no osteophytes, sclerosis, fragmentation, or soft tissue debris is preseo1

>tapering of the distal aspect of the bones may be present, with a " mortar and pestle "or "pencil-incup" deformity

***a potential diagnostic problem which could be mistaken for cellulitis/osteomyelitis or an aggressive bone tumor

HYPER1ROPIllC NEUROPATIllC OS1EOARTIIROPATIN

>joint space narrowing with marked bony sclerosis >osteoporosis does not occur with this form >fractures and fragmentation of the articular smfaces

>a large amount of bony soft tissue debris forms and later fuses into a large, dense, well-organized bony mass

with an integral cortex

>periosteal new bone formation may occur

>subluxation and dislocation proceed to destruction, maJaIignment of articular smfaces, and:finally to total >disorganization of the joint, which appears as ifit were pounded by a sledge-hammer

VII. STAGES OF DEVELOPMENT

StageI:ACUTEorDEVELOPMENTAL

joint laxity, subluxation, osteochondral fragmentation, and debris formation,

Stage IT : COALESCENCE .

absoIption of debris and fusion ofbony fragments Stage ill: RECONSTRUCTION

remodelling and revascularization ofbony fragments

*Stages are based on pathological findings. **Disease course is variable and progression may be very rapid

VII. DIFFERENTIAL DIAGNOSIS

Acute septic arthritis Pigmented Villonodular Synovitis
Osteomyelitis Synovial Chondromatosis and other tumors
Apdite Crvstal_De1'Qsition Disease Gout
CPPD Crystal I ~on Disease Psoriatic Arthritis
Osteonecrosis ( A vascular Necrosis ) Rheumatoid Arthritis 91

IX. PATIIOLOGY

>multiple shards ( fragments) of bone and cartilage embedded in the deep layer of synovium are characteristic

findings in acutely resorbedjoints, bone is replaced by highly vascular:retinaculum >bone is actively resorbed by osteoclasts

>in acute stages synovium is edematous, congested, and infiltrated with PMN's >noninfJammatory and serosanginous or hemonbagic e:ffusioris are frequently described >bone and cartilage ftagments as well as CPPD crystals can be identified in synovial fluid

X TREATMENT

Conservative

>strict cessation of weightbearing

>compression cast ( Jone's-type cast ) to control edema >cast immobilization for fractures ( generally 8-12 weeks ) >gradual increase to :full weightbearing based on healing

>long term aocomadative foot wear (ie. orthopedic shoes, custom molded shoes, AFO, Pm, CROW)

Surgical

>should not be done during the acute phase

>ulcer excision ( primary closure if possible; may require skin grafting ) >exostectomy

>saucerization

>digital stabilization

>metataIsal head rescetion

>Lisfranc arthrodesis

> Triple and panta1ar arthrodeses >amputation

LYME DISEASE

Lyme disease is a complex multisystem il1ness caused by the tick-borne spirochete Borrellia burgdorferi. The spirochete is transmitted primarily by certain ixodid ticks ( Ixodes dammini is the principal vector in the Northeastern and Midwestern U.S.; Ixodes pacificas is the vector in the West). The illness which closely mimics other rheumatic diseases usually occurs in stages with remissions, exacerbations, and different clinical manifestations at each stage. It was :first recognized in 1975 in Lyme, Connecticut.

CLINICAL FEATURES·

EARLY DISEASE: After an iricubation 00-32 days, erythema cbronicum migrans OCCUIS at the site of tick bite in about 80% of patients. Although the lesion can occur anywhere, the thigh, groin, and axilla are particulary common sites. The lesion is warm to the touch and is often painless. WIthin several days after the onset of erythema chronicum migrans, 50010 of patients develop multiple secondaIy lesions which may persist from 1 day to 14 monthes. These lesions are similiar in cq:.pearanc:e to erythema chronicum migrans, but are usually smaller, migrate less, and lack indurated centers. Skin involvement is commonly accompanied by low grade fever and lymphadenopathy.

IN1ERMEDIAlE DISEASE : Generally occurs approximately 1-6 monthes after the initial exposure. Neurologic abnormalities include meningitis, cranial neuritis, motor and sensory radiculopathy, mononemitis multiplex, alone or in various combinations. The most common cardiac abnormality is fluctuating degrees of atrioventricular block. Less commonly peri- and myo- carditis may occur. The typical pattern of arthritis is one of migratory pain in joints, tendons, bmsae, muscles, and bone.

LAlE DISEASE : Occurs monthes to years after initial exposure. The most common form of chronic CNS involvement is a subtle encephalopathy affecting memory, mood, or sleep. Most of these patients also have an axonal polyneuropathy manifested by either distal parasthesias or spinalIradicular pain, About 60% ofuntreated patients in the U.S. develop frank artlnitis. The typical pattern of involvement is brief: intermittent attacks of monoarticular or oligoarticular arthritis in a few large joints, especially the knee.

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LABORATORY FEATURES

Serologic testing is currently the only practical laboratory aid in diagnosis. IgM antibodies peak at approximately 3-6 weeks; IgG antibodies peak monthes to years after exposure. Direct blood, CSF, synovial fluid and skin lesion cultures are rarely positive.

lREATMENT

EARLY ADULT

Doxycycline 100 mg po bid Tetracycline 250 mg po qid Amoxicillin 500 mg po qid CHllDREN

Amoxicillin 50 m~day in divided doses Erythromycin 30 mglkglday in divided doses

NEUROLOGICABNO~S

Ceftriaxone 1 gr/day IV x 30 days

Penicillin G 20 million unitslday in divided doses

ATRIOVENTRICULAR BLOCK Intravenous anbbiotics Corticosteroids Cardiac monitoring

ARTHRITIS

Oral antIbiotics Intraarticu1ar corticosteroids NSAIDS

? synovectomy ifsymptoms persist > 6-12 monthes

*** During the initial 24 hours after antIbiotic therapy is started, 10-15% of Jntients experience a JariscbHeribeimer

reaction characterized by high fever and worsening of symptoms.

LYME DISEASE: TIMETABLE OF A TRIPHASIC ILLNESS

STAGE

SIGNS AND SYMPTOMS

STAGE 1 : initial weeks of illness

constitutional symptoms erythema cbronicum migrans

STAGE 2 : weeks to monthes after stage 1

recurrent skin lesions neurologic manifestations cardiac abnormalities

STAGE 3 : monthes to years after stage 1

arthritis

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RHEUMATOID ARTHRITIS

Rheumatoid arthritis is a systemic autoimnnme disorder of unknown etiology. 'The major distinctive feature is chronic, symmetric, and erosive synovitis of peripheral joints. The disease is known to affect multiple organ systems in acktition to the joints. The average age of onset for adults is 40 years of age, but the disease may strike for the first time at any age. The disease tends to affect women slightly more commonly than men.

PAmOLOGY

Rheumatoid arthritis is a synovial disease with secondary pathologic changes taking place in the synovial fluid, cartilage. periarticular structures, and tendons. Although data are limited, the initial pathologic event appears to be activation and/or injuIy of the synovial microvascular endothelial cells which leads to edema in synovial tissue and effusions within the joint cavity. Synovitis is the initial lesion. As the disease progresses to chronic stages the synoviwn becomes massively hypertrophic and edematous. The fonnation of proliferative and invasive granulation tissue defined as pannus leads to periarticular bone and cartilage erosions and destruction. 'There is no known etiology.

CLINICAL FEATURES SYMPTOMS

- The mode of presentation can be quite variable ( indolent, episodic, or progressive polyarthralgias ).

- Monoarticular, oligoarticular, or polyarticular joint involvement may occur.

- Constitutional symptoms such as malaise, fatigue, low-grade fever, anorexia, and weight loss may

occur.

- Stiffness, particularly after prolonged rest, is usually severe and lasts nearly 60 minutes or more.

SIGNS

ARTICULAR MANIFESTATIONS

- joint swelling and periarticular tenderness

-Iocal wannth over affected joints

- limitation of motion of affected joints

- symmetry of joint involvement

- frequently affected joints include : MCPIMfP's, PIP's, wrists, knees, shoulders, hips, elbows, ankles,

------·-----------mi~~~·-------------------------------------------------

- tenosynovitis

- deformities ( swan neck, boutonniere, hallux valgus, hammertoes, ankle valgus, fibuIarIulnar

deviation of digits )

- calcaneal erosions, plantar fasciitis, retrocalcaneal bursitis

EXTRA-ARTICULAR MANIFESTATIONS

1. Rheumatoid nodules : tend to develop over pressure points. Rheumatoid nodules have characteristic histopathologic findings when. biopsy is perfonned. A central zone of necrosis is surrounded by palisading histiocytes, fibroblasts, and monocytes. The outer layer of inflammatory granulation tissue is composed of lymphocytes, histiocytes, and plasma cells

2. Pulmonary : the lungs are commonly affected by Rheumatoid arthritis. Commmon complications include:

- pleurisy with or without effusions

- diffuse interstitial lung disease

- pulmonary rheumatoid nodules

- Caplan's syndrome ( nodules with pnemnoconiosis )

- pulmonary vasculitis and hypertension

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