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8 Cards in this Set
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8 mo girl comes clinic for a second opinion congenital deformity Fig A & B. surgeon #1 recommended an open tenotomy of one slip (FDB) tendon & reconstruction of the plantar skin defect. What is your recommendation Tx |
Observation; Curly toe is congenital def-flexion & varus defrmty->MC aff IPJ 3rd & 4th toes & most pts don't have Sx. Tx was unncssary. Surg if > 3 yo sx + (pain, diffty w/shoe wear, nail prob)
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An enzymatic mutation leading to abn carbonic anhydrase function in osteoclasts would lead to a condition best illustrated by what on Xray?
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classic "erlenmeyer flask" femurs & "rugger jersey spine" lateral radiograph seen in osteopetrosis. abnormal carbonic anhydrase function within the osteoclast.
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22yo male sustained a dis radius fx 2^ fall down the stairs. ER Doc noticed abn bone on the x-ray & referred skeletal survey & eval. Pelvis & spine xrays FigA & B. What etiology bony dz?
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Defective osteoclast function; dense bone, loss of trabeculations, and narrow femoral canals. spine xray demonstrates the classic "rugger jersey" spine with very dense vertebral bodies.
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5yo boy has sustained multiple fx since birth. A pelvis xray taken 4 yrs ago is Fig A. Current spine xray is Fig B. Which of the following describes the mode of inheritance of this disease?
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AR-infantile form, aka"malignant," is AR (maps to chrom 11q13).
AD-adult form, "benign," is AD. |
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AR are_____ & AD are _______?
Mnemonic for autosomal dominant disorders is : |
AR are enzyme deficiencies
AD are structural prob. "Very Powerful DOMINANT Humans 2" V- Von willibrands disease/ Von hippel lindau P – Pseudo hyPO-PARA-THYroidism D – Dystrophia myotonica O – Osteogenesis imperfecta/Osler-weber-rendu M – Marfans syndrome I – Intermittent porphyria N – Neurofibramatosis A – Achondroplasia, N – Noonans syndrome T- Tuberous sclerosis H – Huntington’s disease H – Hypertrophic obstructive cardiomyopathy |
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What is the cellular mechanism responsible for osteopetrosis (Albers-Schönberg Disease)?
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Inactive osteoclast carbonic anhydrase; Osteopetrosis, or marble bone disease, a defect in the osteoclast, normally responsible for bone resorption and remodeling within Howship's lacunae
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10-mo child fell off of the couch and has L elbow pain & swelling. x-ray Fig A. All are char injury pattern EXCEPT: 1High risk ulnar n palsy; 2 PM displt; 3 High assoc. w/child abuse 4. High risk cubitus varus defrm 5. High risk AVN medial condyle
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1 ulnar nerve palsy ; PM displacement of the radial & ulnar shafts relative distal humerus; assoc w/ child abuse, may->cubitus varus def; AVN medial condyle. Tardy ulnar nerve palsy is lat.l condyle fx nonunions & cubitus valgus.
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7-mo girl cries when the mother touches her swollen elbow. Xray Fig A. What is the most appropriate Tx? 1 ORIF 2. CRPP 3. CR & casting 4. Functional bracing 5.CR & hinged ex-fix
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2 CRPP; distal humeral physeal separation and is most appropriately treated with CRPP
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