Ricci Joseph A, Desai Naman S
Division of Plastic Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
World J Plast Surg. 2019 Jan;8(1):97-105. doi: 10.29252/wjps.8.1.97.
Thumb hypoplasia, a congenital deficiency of bony and soft tissue of thumb, is often associated with systemic syndromes like Holt-Oram syndrome, and is the second most common thumb anomaly after duplicated thumb. It has traditionally been classified into six categories, which help guide treatment including reconstruction versus pollicization (transfer of the index finger to thumb). Amputation of the thumb and pollicization is the traditional treatment for a IIIB hypoplastic thumb. A five-year-old girl presented with a classic type IIIB hypoplastic thumb in which she has absent motor function, aplasia of the metacarpal, shortened first web space, and an unstable but present carpometacarpal (CMC) joint. Instead of amputation, the thumb was reconstructed by capsulodesis to stabilize the CMC joint, Huber Transfer Opponensplasty and abductor pollicis longus transfer to restore motor function, W-plasty to deepen the first web space, and distraction to lengthen the metacarpal. The patient tolerated the multi-stage reconstruction and bony distraction well. She was followed for one year postoperatively and has demonstrated a functional thumb. This case questions the surgical algorithm for hypoplastic thumbs and suggests a revised classification system for hypoplastic thumbs which would further divide class III based on not only the stability of the CMC joint but the presence or absence of the joint as well. We propose that amputation be reserved for type III hypoplastic thumbs in which the CMC joint is absent, (revised class IIIC) and reconstruction be attempted when the joint is present irrespective of stability (revised classes IIIA and IIIB).
拇指发育不全是拇指骨骼和软组织的先天性缺陷，常与诸如霍尔特-奥拉姆综合征等全身性综合征相关，是继多指畸形后第二常见的拇指异常。传统上它被分为六类，这有助于指导治疗，包括重建与拇指化（将食指转移至拇指）。拇指截肢和拇指化是IIIB型发育不全拇指的传统治疗方法。一名五岁女孩患有典型的IIIB型发育不全拇指，其运动功能缺失、掌骨发育不全、第一蹼间隙缩短，且腕掌关节（CMC）不稳定但存在。该拇指未进行截肢，而是通过关节囊固定术稳定CMC关节、进行休伯转移对掌成形术和拇长展肌转移以恢复运动功能、采用W形皮瓣手术加深第一蹼间隙，并进行牵张以延长掌骨。患者对多阶段重建和骨牵张耐受良好。术后对她进行了一年的随访，结果显示拇指功能良好。该病例对发育不全拇指的手术方案提出了质疑，并建议对发育不全拇指采用修订的分类系统，该系统将不仅根据CMC关节的稳定性，还根据关节的有无对III类进一步细分。我们建议，对于不存在CMC关节的III型发育不全拇指（修订后的IIIC类）应保留截肢治疗，而对于存在该关节的情况，无论其稳定性如何，均尝试进行重建（修订后的IIIA类和IIIB类）。
