【摘要】 <正>Poland综合征又称胸大肌缺如短指并指综合征,是一种少见的先天性胸壁及上肢畸形。最早于1841年由Poland在尸体解剖中发现^[1],1962年Clarkson遇到同样的病例并将此先天畸形命名为Poland综合征^[2]。有关其临床特点及治疗策略的研究和报道不断增多,文献报道发病率为1:7 000到1:100 000,男女比例为2:1到3:1,右侧发病占60%⁷5%,累及左侧及双侧的患者少见[3-4],目前还未发现有明显的家族遗传性,但偶有家族性发病的病例报道^[5-6]。更多还原

【Abstract】 Poland syndrome(PS),also called pectoralis major muscle defect,brachydactyly and syndactylia syndrome,is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral upper limb and hand abnormalities in different degrees.The clinical manifestations are various,especially the upper limb and hand deformities,with great individual differences.This article reviewed the domestic and foreign literatures in recent years and summarized the various deformities of the upper limb and hand in the PS patients.(1) The PS patients with upper limb deformities may have the following symptoms,including various degrees of short or even defected upper limb,congenital radioulnar synostosis,congenital high scapula,ulnar longitudinal dysplasia,posterior dislocation of the radial head and so on.(2) The PS patients with wrist deformities may have the following symptoms,including one or more pairs of intercarpal fusion,delay of carpal ossification,disharmonious ossification between the carpal and tubular bone,abnormal carpal morphology,absence of carpal bones and so on.(3) The PS patients with hand deformities may have the following symptoms,including volume decrease of the ipsilateral hand such as brachydactyly,ectrodactyly,split hand and acheiria,partially or completely soft tissue syndactyly,dysplasia or absence of the middle phalanx,contracture of the metacarpophalangeal joint and the interphalangeal joint,finger lateral deformity,congenital constriction band deformity and so on.更多还原