Bouvet J P, Maroteaux P, Briard-Guillemot M L
Nouv Presse Med. 1976 Jan 24;5(4):185-90.
Study of malformations of the hand makes possible the definition of three forms of progressive severity in the Poland syndrome, in which there is unilateral symbrachydactylie associated with homolateral aplasia of the pectoralis major. The three forms share the predominance of involvement of the 2nd, 3rd and 4th fingers. The existence of alterations in palmar creases and dermatoglyphia of the involved side alone confirms the absence of any lesion, even infra-clinical, of the other hand. Genetic study favours a pluri-factorial inheritance. Topographical data and the comparative study of vascularisation suggest the possibility of hypoplasia of the proximal portion of the sub-clavian at the origin of this malformative association.
手部畸形的研究使得确定波兰综合征三种逐渐加重的形式成为可能,在该综合征中,单侧短指畸形与同侧胸大肌发育不全相关。这三种形式的共同特点是第2、3和4指受累为主。仅受累侧手掌褶皱和皮纹的改变就证实了对侧手部不存在任何病变,即使是亚临床病变。遗传学研究支持多因素遗传。地形学数据和血管化的比较研究表明,这种畸形关联起源于锁骨下近端发育不全的可能性。