Schoenecker P L, Cohn A K, Sedgwick W G, Manske P R, Salafsky I, Millar E A
J Bone Joint Surg Am. 1984 Mar;66(3):421-7.
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.
我们研究了21例血小板减少伴桡骨缺如综合征患者的病例。患有该综合征的患者通常双侧膝关节存在关节内发育不良,导致膝内翻以及屈曲和扭转畸形,这些畸形在出生后第一年或第二年就会显现出来。通常在生长过程中畸形会进展,因此常常需要使用支具或进行手术治疗,或两者兼用。尽管进行了矫正截骨术,但内翻成角和胫骨内旋仍有自然复发的倾向,这可能是由于涉及股骨和胫骨髁的关节内畸形所致。在骨骼成熟后,已观察到畸形的进展最小。