A Marfan syndrome child with contractural arachnodactyly deformity of both hands was admitted to our unit in June 2015. The left and right middle fingers and ring fingers of the child received the continuous " Z" skin flap and " V-Y" plasty to reduce the palmar tension on the skin and release the contracture. Due to the improper therapeutic strategy and the underestimation of effect of underlying lesions of Marfan syndrome on the therapeutic strategy, the right middle finger of child was with ischemic necrosis and amputated, and the finger tip was accompanied with distension and paroxysmal neuropathic pain at a specified future date. The skin flaps of the other three surgical fingers were with local necrosis and scar healing. The child was complicated by distal interphalangeal joints stiffness and some serious complications post-discharge. Combined with this case, the similar disorders are not recommended to carry out one-stage operation.
2015年6月，笔者单位收治1例马方综合征双手蜘蛛样指伴畸形挛缩患儿。对该患儿的左、右手中指和环指均采用连续"Z"字瓣＋"V－Y"成形术来减轻手指掌侧皮肤牵张，松解挛缩。由于治疗方案选择失当，以及轻视了马方综合征的基础病变对该手术治疗的影响，术后患儿右手中指缺血坏死并截指，远期并发指端膨隆和阵发性神经源性疼痛；其余手术3指皮瓣局部坏死，瘢痕愈合。出院后，患儿继发远侧指间关节僵硬等严重并发症。结合本病例，类似疾病不推荐行Ⅰ期手术治疗。.
Keywords: Arachnodactyly; Contracture; Hand deformities, congenital; Marfan syndrome; Surgical procedures, operative.