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135 Cards in this Set
- Front
- Back
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Achondroplasia
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FGFR3. AD. Spinal stenosis. Lumbar kyphosis, wide pelvis
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Pseudoachondroplasia
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COMP. AD. Cervical instability. Normal facies. Precocious OA.
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SED
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Type II collagen. Congenita- AD/ Tarda- X-linked. Epiphyseal fragmentation w/ spine involvement. Early OA. Dislocated hips. Retinal detachment (congenita)
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Kniest's syndrome
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Type II collagen. AD. Retinal detachment. Dumbbell-shaped bone.
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Jansen's metaphyseal chondroplasia
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PTHrP. AD. Hypercalcemia w/ metaphyseal expansion. Most severe form.
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Schmid's metaphyseal chondroplasia
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Type X collagen. AD. Coxa vara, genu valgum. Looks like rickets
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McKusik's metaphyseal chondroplasia
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COMP. AR. Cartilage-hair dysplasia. Odontoid hypoplasia. Immunocompromised. Intestinal malabsorption.
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MED
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Type II collagen. AD. Bilateral hip involvement- looks like symmetric bilateral Perthes. Double layer patella.
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Diastrophic dysplasia
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sulfate transport protein. AR. Cauliflower ears, kyphoscoliosis- twisted dwarf. Cervical kyphosis- risk quadriplegia.
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Cleidocranial dysplasia
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CBFA1 (transc factor for osteocalcin). AD. Aplasia of clavicles, coxa vara
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Hurler's syndrome
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AR. MR. Cloudy cornea. Dermatan/heparan sulfate in urine. Worst form.
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Hunter's syndrome
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XR. MR. Clear cornea. Dermatan/heparan sulfate in urine.
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Sanfilippo's syndrome
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AR. MR. Clear cornea. Heparan sulfate in urine. Nml kids until 2yo.
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Morquio's syndrome
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AR. Nml intelligence. Karatan sulfate in urine. Most common. Odontoid hypoplasia.
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Beckwith-Wiedemann syndrome
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organomegaly/omphalocele/macroglossia. Hemihypertrophy w/ spastic CP. Risk Wilm's tumor.
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FAS
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hip dislocations, congenital fusions, congenital scoliosis, myelodysplasia
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Gauchers disease
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B-glucocerebrosidase deficiency--> accumulation of cerebroside in reticuloendothelial system. Patchy sclerosis, erlenmyer flask distal femur, hepatosplenomegaly, lipid-laden histiocytes (gaucher's cells)
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Nieman Pick disease
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AR. Accumulation of sphingomyelin in reticuloendothelial system.
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Sickle cell
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ON, septic arthritis, salmonella infections, dactylitis. Preop oxygenation/exchange transfusion. Hydroxyurea-->pain relief during pain crisis.
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Hemophilia A disorder
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Factor VIII
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Hemophilia B disorder
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Factor IX
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Grading hemophilia
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mild- 5-25%/ moderate- 1-5%/ severe <1% factor available
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Hemophilia
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iliacus hematoma-->femoral n paralysis. Cartilage atrophy s/p hemarthroses. Mild-moderate A: tx desmopressin.
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Factor replacement- hemophilia
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Acute hemarthrosis or soft tissue surgery- >50%. Skeletal surgery- 100% preop then >50% x 10 days. IgG inhibitors- relative CI to surgery
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Rickets
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Dec Ca. Si/Sx: physeal cupping/widening; bowing long bones; Looser's lines; ligamentous laxity; enlarged costal cartilages; dorsal kyphosis. Histo: widened osteoid seams/ large trabecula.
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Osteogenesis imperfecta
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Type I collagen defect (COL1A2 gene) --> abnormal cross-linking/ decreased collagen secretion. Fx healing nml. Fx less common after puberty. Tx: brace early/ telescoping IM rods after 2yo/ bisphosphonates dec fx risk. Surgery if scoliosis over 50deg--> large blood loss expected.
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Idiopathic juvenile osteoporosis
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8-14yo. Osteopenia/growth arrest/bone pain. Nml Ca/Phos levels. Resolves 2-4yrs after puberty.
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Osteopetrosis
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Failure of osteoclastic resorption. Defect in thymus. Dense bone/ rugger jersey spine. Loss of medullary canal--> aplastic anemia/loss of optic/oculomotor nerves. Tx: BMT
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Infantile cortical hyperostosis
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Caffey's disease. Soft tissue swelling/ cortical thickening s/p febriel illness. 0-9mo old. Self-limiting.
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Marfan's syndrome
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AD. Fibrillin defect. Pectus deformity. Scoliosis. Acetabular protrusio. Aortic dilation. Superior lens dislocation.
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Ehlers-Danlos syndrome
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AD. Cigarette paper skin. Hypermobile and unstable joints. Soft tissue procedures ineffective.
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Homocysteinuria
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AR. Methionine metabolism error. Osteoporosis. Marfanoid habitus. Inferior lens dislocation. Dx: cyanide-nitroprusside test--> homocysteine in urine. Tx: vitamin B6/ low methionine diet
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Obstetric brachial plexus palsy- prognostic indicators
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Poor prognostic indicator: no antigravity biceps at 6mo, Horner's sign.
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Obstetric brachial plexus palsy- treatment
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Contracture release, lat and teres major tx to shoudler external rotators (L-Episcopo's). Tendon tx for elbow flexion. Proximal humerus rotational osteotomy. Nerve grafting.
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Congenital muscular torticollis
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SCM contracture. 90% resolve w/ stretching. Z-plasty if over 1yo. Associated w/ AA instability
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Congenital pseudarthrosis of clavicle
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R side. ORIF w/ bone grafting for cosmesis or fragment mobility or scapular winging at ages 3-6yo. Union predictable
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Erb's palsy
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C5-C6 palsy. Lose deltoid/cuff/biceps/wrist dorsiflexors--> waiter's tip position. Best prognosis
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Klumpke's palsy
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C8-T1 palsy. Lose wrist flexors/intrinsics. + Horner's syndrome.
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Walking ability hemiplegic CP
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all eventually can walk
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Predicting walking ability CP
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Persistence of 2+ primitive reflexes
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Mechanism of botulinum toxin
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Inhibition of presynaptic release of Ach
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Hip at risk
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Abduction less than 45 degrees w/ partial uncovering of femoral head. Tx: adductor/psoas release- done early.
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Dega acetabuloplasty
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Take 3 triangles of iliac crest and wedge into pelvis superior to acetabulum
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Larsen's syndrome
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multiple joint dislocations- bilateral knee's. Scoliosis/clubfeet/cervical kyphosis
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Red/warm/swollen limb in myelodysplasia
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Fracture until proven otherwise
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Level of myelodysplasia needed for functional ambulation
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Must be distal to L2--> need quads
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Level of myelodysplasia needed for community ambulation
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distal to L4
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Hip dislocation in myelodysplasia
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Most common at L3-L4 level. Tx: containment only if functioning quadriceps.
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Most affected muscles in CMT
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TA and PB
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Myasthenia gravis
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easy fatigability. Competitive inhibition of Ach receptors at motor end plate by antibodies from thymus. Tx: cycloposrin/anti-ach tx/ thymectomy.
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Polio
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viral destruction of anterior horn cells
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Proteus syndrome
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overgrowth of hands/feet w/ bizarre facial disfigurement.
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Hemihypertrophy.
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many causes. Most common- NF. Assoc w/ Wilm's tumor.
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Tx windswept hips
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brace adducted hip. Release abduction contracture of other hip.
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Tx hyperextension knee gait
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rectus femoris lengthening
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Tx stiff-leg gait
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if EMG: continuous then trnasfer rectus to hamstrings
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Genetic defect McCune-Albright
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activating mutation cAMP
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Genetic defect CMT
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PMP22
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Genetic defect Duchenne/Becker's
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Dystrophin
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Genetic defect hypophosphatemic rickets
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PEX
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Most common direction of thoracic scoliosis
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right apex
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Threshold of normal scoliometer reading
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7deg- correlates w/ 20 degree coronal curve
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Indications for MRI scoliosis
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1. left thoracic. 2. apical kyphosis. 3. cavus/cavovarus feet. 4. asymmetric neuro exam. 5. structural abnormalities. 6. onset <11yo. 7. rapid progression. 8. Pain
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Predictor peak growth velocity
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before onset of menarche/ Risser 1
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Age categories of scoliosis
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infantile: <3yo/ juvenile: 4-10yo/ adolescent: >10yo
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Milwaukee brace
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CTLSO- use for apex above T7
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Boston brace
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underarm TLSO- use for apex at or below T8
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Indications for circumferential fusion in idiopathic scoliosis
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1. deformity >75yo. 2. Crankshaft prevention (Risser 0/ age <10yo (F) or <13yo (M)/ before peak growth velocity)
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Stable vertebra in scoliosis
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most proximal vertebra that is most closely bisected by center sacral line
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Lowest instrumented vertebra
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Single thoracic: 1-2 proximal to stable vertebra. Double/Triple major curves: distal end vertebra
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Late rod breakage after PSF for scoliosis
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indicator of pseudarthrosis
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Risk of sublaminar wires for scoliosis correction
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Increased risk of neuro injury
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Determinants of progression risk in IIS
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Curves less than 25deg/ RVAD less than 20deg resolve- need no tx
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Indication for fusion in JIS
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Curve >50deg
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Features of neuromuscular scoliosis
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pelvic obliquity, progression after deformity, bony deformities, cervical involvement, more rapid progression
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Galveston technique of fixation to pelvis
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bend caudal ends of raods from lamina of S1 to pass into PSIS between tables of ilium
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Indication for scoliosis fusion in Duchenne's
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curve >30deg- fuse T2-pelvis. Need preop PFT (need >40% predicted values) and cardiac function eval
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Congenital vertebral anomaly w/ highest liklihood of progression
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Unilateral bar w/ contralateral fully segmented hemivertebra- indication for OR at presentation
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Complication spinal fusion in NF
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Pseudarthrosis more common/ neuro injury
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Treatment diastematomyelia
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if symptomatic or deformity correction planned- needs resection- o/w observation only.
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Treatment Scheuermann's kyphosis
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Milwaukee brace if progressive curve and growth remains. PSF w/ dual rods if >75deg and painful or progressive. ASF if >90deg or very stiff
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Treatment rotatory AA subluxation
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if secondary to retropharyngeal inflammation- traction/bracing early/ fusion late. If traumatic: soft collar if early/ fusion late (>1mo)
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Pseudosubluxation of cervical spine
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up to 40% or 4mm in kids less than 8yo. Will have continued alignment of posterior interspinous distances and spinolaminar line. Pseudosubluxation reduces w/ cervical extension.
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Intervertebral disc calcification
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Pain, decreased ROM, low grade fevers, inc ESR, disc calc w/out erosions. Tx: symptomatic
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Basilar invagination
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weakness, paresthesias, hydrocephalus. Tx: transoral resection/ occipital laminectomy, occipitocervical fusion
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Sprengel's deformity
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Undescended scapula. Associated w/ winging/hypoplasia/omovertebral connections. Tx for decreased abduction- distal advancement of muscles/scapula/ detachment and movement of scapula. Tx best when 3-8yo. Concomitant clavicular osteotomy to avoid brachial plexus palsy.
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Treatment metatarsus adductus
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If passively correctible- stretching. If not correctible- cuneiform osteotomies and medial release after 5yo.
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Tx increased tibial torsion
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Only tx severe cases- supramalleolar osteotomy
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Tx increased femoral anteversion
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if <10 deg external rotation- consider derotational osteotomy (intertrochanteric)- cosmetic only NOT functional
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Potential obstructions to hip reduction in DDH
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iliopsoas tendon, pulvinar, contracted inferomedial capsule, transverse acetabular ligament, inverted labrum
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Hip US- DDH
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Best test for age <4-6mo. NML: alpha angle >60deg/ femoral head bisected by line down ilium
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Normal acetabular index
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25deg
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Complications Pavlik harness
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excessive abduction: osteonecrosis (compression posterosuperior retinacular br medial femoral circumflex). Excessive flexion: femoral nerve palsy. Pavlik disease: erosion of pelvis superior to acetabulum.
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Tx DDH 6-18mo
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closed reduction/ arthrogram/ hip spica
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Tx DDH over 18mo
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open reduction/adductor tenotomy/femoral shortening. - anterior approach if >12mo old
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Medial approach for hip reduction in DDH
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Ok if age <12mo. Less blood loss, directly addresses blocks to reduction, does not allow for capsulorrhaphy, assoc w/ more ON
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Salter osteotomy
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opening wedge osteotomy through ileum. Need concentric reduction/ age >8yo. May lengthen leg 1cm
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Pemberton's osteotomy
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opening wedge osteotomy through acetabular roof to triradiate cartilage. Need concentric reduction/ age >8yo.
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Sutherland's osteotomy
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Salter's plus pubic osteotomy
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Steel's osteotomy
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Salter's plus osteotomy of both rami (triple osteotomy)
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Ganz osteotomy
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Periacetabular osteotomy. Must have closed triradiates
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Chiari osteotomy
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through ilium above acetabulum- makes new roof. Salvage procedure. Depends on fibrocartilagenous metaplasia.. Shortens affected leg.
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Shelf osteotomy
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lateral acetabular augmentation. Salvage in patients >8yo
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Benefits Ganz PAO
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3D correction. Allows immediate weight-bearing (post column intact). Spares abductors. Single incision. Can access and visualize joint
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Congenital coxa vara
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defect in ossification of femoral neck. Si/Sx: waddling gait (bilat) painless limp (unilat). XR: triangular ossification defect inferomedial femoral neck. Tx: Hilgenreiner's angle (hilgenreiners angle to femoral physis) <45- obs and will correct. 45-60deg- obs and may not correct. >60deg- valgus osteotomy +/- greater troch transfer to restore mechanics
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Lateral pillar classification
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A: little involvement- good B:>50% maintained- good if <6yo o/w bad. C: <50% maintained- bad. B/C border group: <50% hieght but very narrow/little ossification/depressions
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Catteralls' head at risk signs
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1. lateral calcifications. 2. V-shaped defect at lateral physis. 3. lateral subluxation. 4. metaphyseal cyst. 5. horizontal growth plate
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Site of defect in SCFE
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weak perichondrial ring/ slip through hypertrophic zone. Slip metaphyseal in renal osteodystrophy
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Loder's classification SCFE
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stable- able to bear weight (0% ON). Unstable- unable to bear weight (47% ON)
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Radiographic grading SCFE
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grade I: 0-33%; II: 34-50%; III: >50%. Look for lateral overhang in relation to Klein's line
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Proximal femoral focal deficiency
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developmental defect. Short bulky thigh- flexed/abducted/ER. Relative shortening remains constant. Associated w/ coxa vara, fibular hemimelia, knee ligament deficiency/contracture. Tx: if femoral length <50%- prosthetics/ >50%- lengthening +/- contralateral epiphysiodesis. If absent femoral head- amputation/femoral-pelvic fusion/Van Ness/ lengthening.
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Normal knee alignment kids
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varus when <2yo--> valgus at 2.5yrs--> physiologic valgus by 4yo
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Infantile Blount's disease
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Bilateral. Drennans' angle over 16deg (bet metaphyseal beaks). Tx: bracing early/ osteotomy if >4yo or failed bracing/ epiphyseolysis if bony bridge
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Adolescent Blount's
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>10yo. Usually milder/ unilateral. Tx: tib-fib epiphysiolysis. Osteotomy if physes closed or large deformity.
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Indications for surgery for genu valgum
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kids >10yo of >10cm between medial malleoli or >15deg valgus. Tx: hemiepiphysiodesis
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Posteromedial tibial bowing
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physiologic. Associated w/ calcaneovalgus feet/ LLD. Spontaneously corrects.
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Anteromedial tibial bowing
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Associated w/ fibular hemimeila/ankle instability/equinovarus foot/tarsal coaligtion. Significant LLD results. Tx: reconstruction w/ resection fibular anlage or amputation if severe shrotening/stiff nonfunctional foot at 10mo old.
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Anterolateral tibial bowing
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Associated w/ congenital pseudarthrosis of tibia. 50% assoc w/ NF. Tx: total contact brace/ IM fixation w/ hamartomatous tissue excision and bone grafting for nonhealing fx/ vascularized fibular graft or Ilizarov/ Syme's amp indicated after 2-3 failed surgeries. Osteotomies contraindicated.
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Worst prognosis conservative tx OCD
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synovial fluid behind lesion. Also poor if lateral femoral condyle or patella.
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Tx LLD
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2-5cm: epiphysiodesis/ >5cm: lengthening (obtain 1mm per day)
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Skeletal growth by location
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distal femur: 9mm/ 6mm proximal tibia/ 3mm proximal femur. Growth stops at age 14 (F)/ 16 (M)
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Joints with intra-articular metaphyses
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hip/elbow/shoulder/ankle
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Transient synovitis vs septic hip criteria
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1. WBC >12,000. 2. ESR >40. 3. Inability to bear weight. 4. fever >101.5. if 3/4 than >90% risk of septic arthritis
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Gonococcal arthritis
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Preceding migratory polyarthralgia, small papules. Multiple joint involvement. WBC usually <50,000. Does not require drainage. Tx; High dose PCN.
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Subsequent surgery in TEV
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TAL in 90%/ ant tib tx in 15-20%
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Resistant insensate clubfeet >8yo
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Talectomy. Triple arthrodesis contraindcated.
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Resistant sensate clubfeet >8yo
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Triple arthrodesis
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Resistent clubfeet 6-9mo old.
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soft tissue release w/ tendon lengthening. DP artery insufficient. Add osteotomies in kids >3yo
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Skewfoot
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Metatarus adductus + valgus hindfoot
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Congenital vertical talus
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Plantarflexion lateral XR- Meary's angle >20deg (if corrects then congenital oblique talus). Tx: 12wks casting. Lengthen extensors/peroneals/Achilles w/ recon spring lig and talonavicular reduction @6-12mo
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Tarsal coalitions
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Sx: spastic peroneal flatfoot. Calcaneonavicular most common in 10-12yo/ subtalar more common 12-14yo. Multiple in 20%. XR: anteater sign on lat/irregular middle facet on Harris view. Tx: CN coalition: resection. Subtalar: <50%- resection/ >50%- subtalar arthrodesis
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Calcaneovalgus foot
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Intrauterine positioning/ myelomeningocele @L5 level. Tx: stretching/ obs
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Kohler's disease
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ON of tarsal navicular. Tx: activity modification. +/- immobilization
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Atavistic great toe
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congenital hallux varus: deformity us at MTPJ- short/thick 1st MT w/ firm band of abductor hallucis longus. Tx: release
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Overlapping toe
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Tx: stretching/ buddy taping. OR: tenotomy/dorsal capsulotomy/ syndactylization.
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