Ireland D C, Takayama N, Flatt A E
J Bone Joint Surg Am. 1976 Jan;58(1):52-8.
Forty-three consecutive cases of Poland's syndrome were analyzed and the relevant literature was reviewed. The syndrome is not hereditary and is of unknown origin. It affects males more frequently than females. The clinical features are variable but always include congenital aplasia and syndactyly. The middle phalanges are hypoplastic or absent so that effectively there is only one interphalangeal joint. The syndactyly is usually incomplete and simple. It may involve all fingers and frequently includes the thumb, which then lies in the same plane as the fingers. Poland's syndrome may also include hypoplasia of the nipple and breast, hypoplasia of the upper ribs, herniation of the lung, contracture of the anterior axillary web, and elevated scapula. The arm and more frequently the forearm are hypoplastic. The right side is more often affected than the left. Surgical treatment by separating the syndactyly is recommended. In some cases a digit is removed to produce a three-fingered hand. Surgery is initiated by the age of one year and is completed by the time the child enters school, although periodic revisions may be necessary. Although the hand remains hypoplastic and functional capacity is limited by the inherent skeletal anomalies, surgical treatment improves functional capacity and cosmetic appearance in the majority of patients.
对连续43例波兰综合征患者进行了分析，并对相关文献进行了综述。该综合征非遗传性，病因不明。男性比女性更易受累。临床特征多样，但总是包括先天性发育不全和并指。中指骨发育不全或缺失，因此实际上只有一个指间关节。并指通常不完全且为单纯性。可累及所有手指，常包括拇指，此时拇指与其他手指处于同一平面。波兰综合征还可能包括乳头和乳房发育不全、上肋骨发育不全、肺疝、腋窝前皱襞挛缩以及肩胛骨抬高。手臂，更常见的是前臂发育不全。右侧比左侧更常受累。建议通过分离并指进行手术治疗。在某些情况下，切除一个手指以形成三指手。手术在1岁时开始，在孩子入学时完成，不过可能需要定期复查。尽管手部仍发育不全，功能能力因先天性骨骼异常而受限，但手术治疗可改善大多数患者的功能能力和外观。
