Sabapathy S Raja, Venkatramani Hari, Mohan Monusha, Zhang Dafang
Department of Plastic, Hand and Reconstructive Microsurgery, Ganga Hospital, Coimbatore, Tamil Nadu, India.
Department of Orthopaedic Surgery, Brigham and Women's Hospital, Boston, Mass.
Plast Reconstr Surg Glob Open. 2020 Feb 28;8(2):e2614. doi: 10.1097/GOX.0000000000002614. eCollection 2020 Feb.
Split-hand/split-foot malformation with long bone deficiency (SHLFD syndrome) is a rare congenital disorder, which may be sporadic or autosomal dominant with incomplete penetrance. When complete tibial aplasia is seen, the mainstay of treatment is amputation and lower limb prosthesis. This rare constellation of congenital differences presents an opportunity for microsurgical free tissue transfer using the principle of "spare parts" to improve the functionality of the hand. We present a rare case of split-hand/split-foot malformation with a monodactylous right hand and complete tibial aplasia, treated with microsurgical free foot-to-hand transfer at the time of lower limb amputation, reconstructing key pinch. At the latest 8 months follow-up, the patient had no pain, active key pinch, and ambulated independently with prostheses. He was able to use his right hand independently for a number of daily activities, such as stacking blocks, drinking from a cup, and playing with toys.
伴有长骨缺损的裂手/裂足畸形(SHLFD综合征)是一种罕见的先天性疾病,可能为散发性或常染色体显性遗传且具有不完全外显率。当出现完全性胫骨缺如时,主要治疗方法是截肢和安装下肢假肢。这种罕见的先天性差异组合为利用“备用部件”原则进行显微外科游离组织移植以改善手部功能提供了机会。我们报告一例罕见的伴有单指右手和完全性胫骨缺如的裂手/裂足畸形病例,在下肢截肢时采用显微外科游离足到手移植进行治疗,重建关键捏握功能。在最近8个月的随访中,患者无疼痛,具有主动关键捏握功能,可独立使用假肢行走。他能够独立使用右手进行一些日常活动,如堆叠积木、用杯子喝水和玩玩具。