罕见膝反屈型先天性多发性关节挛缩症的手术治疗

目的：探讨罕见膝反屈型先天性多发性关节挛缩症(Arthrogryposis面上multiplex congenita，AMC)的手术治疗方法及疗效。方法：3例膝反屈型AMC中，2例接受了手术治疗，每例均分两期施行16个手术。第1期下肢手术包括腱切断、腱延长、关节凹侧关节囊切开和切除术，股骨下端旋转性截骨及畸形足的三关节融合术；第2期上肢手术为肱骨下端切骨术及手部肌力平衡术。结果：2例均获十分满意的结果，无畸形复发。结论：选择恰当的术式治疗膝反屈型AMC是很有效的。

Operative Treatment of a Rare Knee Hyperextension type of arthrogryposis multiplex congenita

Objective:To discuss the operative treatment for a rare hyperextension of knee deformity of AMC.Methods:Three patients with knee hyperextension type of AMC were reviewed.Two patients under went operative treatment and each received 16 operative procedures by two stages.The first stage was aimed at the lower exteremity,including tenotomies,tendon lengthening,capsulotomy and capsulectomy on the concave side of the joint,distal femoral rotational osteotomy,and triple arthrodesis for clubfoot.The second stage was aimed at the upper extremity,including distal humeral osteotomy and muscle balance procedures of the hand.Results:Both cases obtained quite satisfactory results.No recurrence of the deformity was observed.Conclusion:Adequate operation is effective for the treatment of severe knee hyperextension type of AMC.