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.