Poland综合征上肢畸形的临床分析

Clinical analysis of upper extremity deformities with Poland's syndrome

目的对116例Poland综合征上肢畸形的表现进行回顾及分析,提出对既往国内Poland综合征手部畸形分型方法的补充。方法通过查体和影像学检查,对手部、臂部的畸形表现进行归纳总结,主要包括并指情况,手部及上肢合并畸形,手部畸形分型。结果本组116例,107例存在并指畸形,2例拇、示指为骨性并指畸形,其余累及指蹼均为软组织并指畸形。典型型,Ⅰ型3例,Ⅱ型35例,Ⅲ型42例,Ⅳ型10例,Ⅴ型1例,Ⅵ型3例;非典型型,Ⅳ型1例,Ⅴ型1例,Ⅵ型1例。结论 Poland综合征上肢畸形尤其是手部表现复杂,改良的手部畸形分型基本反映了畸形的严重程度和手部合并畸形,有助于指导临床治疗。

Objective To review and analyze the hand and arm anomalies in 116 patients with Poland＇s syndrome, and to propose a complement for the domestic classification of the hand anomalies. Methods Based on the physical and imaging examinations, the presentation of upper extremity anomalies including syndactyly and associating deformities of the hand and arm was reviewed and the hand anomalies were classified. Results A total of 107 of 116 cases were involved with syndactyly. There were 2 cases of bony syndactyly with the thumb and index finger involved, and the other cases of soft-tissue syndactyly with the finger web involved. For the typical type, there were 3 cases of type Ⅰ, 35 type Ⅱ, 42 type Ⅲ, 10 type Ⅳ, 1 type Ⅴ and 3 type Ⅵ. And there was only 1 case of atypical type Ⅳ, Ⅴ and Ⅵ respectively. Conclusions The upper extremity deformities with Poland＇s syndrome are complicated especially in the hand. The modified classification of hand anomalies covers the increasing severity of hand anomalies with extended atypical conditions and help guide the clinical treatment.