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The Journal of Bone and Joint Surgery, Vol 66, Issue 3 421-427, Copyright © 1984 by Journal of Bone and Joint Surgery, Inc

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Dysplasia of the knee associated with the syndrome of thrombocytopenia and absent radius

PL Schoenecker, AK Cohn, WG Sedgwick, PR Manske, I Salafsky and EA Millar

We studied the cases of twenty-one patients with the syndrome of thrombocytopenia and absent radius. Patients with this syndrome usually have associated intra-articular dysplasia of the knee joint bilaterally, causing genu varum and a flexion and torsional deformity that become manifest in the first or second year of life. Usually there is progression of the deformities during growth and therefore treatment with braces or operations, or both, will frequently be needed. Despite correctional osteotomy, the varus angulation and internal tibial rotation have a natural tendency to recur, possibly because of the intra-articular deformity that involves the femoral and tibial condyles. After skeletal maturity has been reached, minimum progression of the deformity has been observed.
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