CAJ | 학술논문

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 ossiifcation, disharmonious ossiifcation 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.