Poland综合征手部畸形的临床分型及治疗策略

Clinical classification and treatment strategy of the hand deformity in Poland syndrome

目的总结和探讨Poland综合征手部畸形的临床形态学特征及治疗策略。方法回顾总结2000年2月至2011年8月由我科诊治的Poland综合征患者63例，系统归纳其手部畸形的形态学特点，依据畸形手形态受影响的程度进行分型，分为典型型和其他型两大类，典型型分为Ⅰ、Ⅱ、Ⅲ、Ⅳ、Ⅴ型，其他型分为Ⅰ、Ⅱ、Ⅲ型。并总结不同类型Poland综合征畸形手的生长发育、手指并连、X线片表现、合并畸形及功能损害等特点。根据上述分型，对其中56例进行外科治疗，并对各个类型的畸形手的治疗策略进行总结和归纳。结果术后对所有手术治疗患者进行随访，时间为6～18个月，平均10．5个月。分指成功率达100％，手术后主要残留指蹼粘连和瘢痕挛缩等问题。除并指外的其他畸形，如短指、缺指、肢体短小、乳房发育不良等，目前仍无法有效治疗手段。结论Poland综合征畸形手形态学改变复杂、多样化，本研究对Poland综合征手畸形的分型，基本反映了Poland综合征手部畸形的形态学改变特点，依据此分型对畸形手进行手术治疗，获得一定的临床效果，伴发畸形仍需进一步治疗。

Objective To review and analyze the clinical morphologic characteristics of the hand deformity in Poland syndrome, and establish a new classification system and treatment strategy. Mefllods Sixty-three cases with Poland syndrome treated by the authors between February 2000 and August 2011 were evaluated retrospectively. The clinical morphologic characteristics of the hand deformity were systemically analyzed. Based on the severity degree of morphologic deformity, the Poland hand was classified into typical type and atypical type. Typical type was sub-classified into type Ⅰ, type Ⅱ, type Ⅲ, type IV and type V,and atypical type was sub-classified into type Ⅰ, type Ⅱ and type Ⅲ. The deformed hands in each type were also summarized with respect to growth and development, syndactyly, X-ray features and impairment of functions. 56 cases of them were treated surgically under the guidance of the new classification, and a new strategy for surgical treatment was established. Results A follow-up of 10.5 months on average （range from 6 to 18 months） postoperatively showed that the success rate of interdigital syndactyly release was 100%. The major postoperative problems included scar contracture and web adhesion. Currently there were still no ideal methods for treatment of associated anomalies（such as short fingers, hypoplasia of forearm and upper arm, aplasia of digits, breast hypoplasia）. Conclusion The hand deformities in Poland syndrome are complicated with multiple manifestations. The new classification system covers clinical and morphologie characteristics comprehensively, and offers a basis for options of surgical treatment of the deformed hand. The associated anomalies still need to be treated in the future.