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Results in defect in
endochondral bone formation
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PATHOPHYSIOLOGY
• Failure of enchondral ossification
• Intramembranous and periosteal ossification
is undisturbed
• Fibrous tissue present in the zone of
provisional calcification
• Bones remain short
• Intramembranous ossification is normal
(diameter of bones are normal)
Tachdjian, Padiatrics Orthopaedic, 4th ed. 2008 p. 1683-1684
HISTOLOGIC
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• Pelvis: Constriction at triradiate cartilage with a flat acetabular roof and relative flaring
of the ilia, producing champagne pelvis; and coxa vara
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DIFFERENTIAL DIAGNOSIS
• Hypochondroplasia
• Thanatophoric dysplasia
• Pseudoachondroplasia
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TREATMENT
•Lumbar hyperlordosis
nonoperative: observation
•treatment typically not required
•Foramen magnum stenosis
operative
•surgical decompression of foramen
magnum (Sleep apnea or cord compression)
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TREATMENT
• Genu varum
– operative
• tibial +/- femur osteotomies (based on CORA)
– indications
» pain or fibular thrust
» progressive deformity
• Short stature
– operative
• lower limb lengthening
– indications (very controversial)
» feasible due to soft tissue redundancy
• upper extremity lengthening
– indications
» upper extremity lengthening required to maintain ADL's
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