Rossillon D, Rombouts J J, Verellen-Dumoulin C, Vanwijck R, Vincent A, de Coninck A
Department of Plastic Surgery, St Luc Hospital, Catholic University of Louvain, Brussels, Belgium.
Br J Plast Surg. 1988 May;41(3):270-7. doi: 10.1016/0007-1226(88)90111-7.
The authors report 19 cases of congenital ring-constriction syndrome of the limbs. Complications arose in eight of the pregnancies. The limb anomalies were always congenital ring-constrictions together with distal amputation. Moreover, 13 patients also had syndactyly and 15 associated anomalies were registered in 9 patients--8 of the limbs, 3 craniofacial and 4 visceral. One patient had a family history of hypospadias and cardiac malformation. Treatment usually required several operations, staged Z-plasty being the procedure of choice in surgical release of constriction bands. The acrosyndactylies were released early in life. More extensive fusions required division and skin grafting even if the webs did exist. The aetiology of the condition is still under discussion. The number of cases in the literature that cannot be explained by the exogenous theory of the congenital ring-constriction syndrome is increasing. Among them are four cases in our series.
作者报告了19例先天性肢体环状缩窄综合征病例。8例妊娠出现了并发症。肢体畸形总是先天性环状缩窄合并远端截肢。此外，13例患者还患有并指畸形，9例患者共记录到15处相关畸形——8处肢体畸形、3处颅面畸形和4处内脏畸形。1例患者有尿道下裂和心脏畸形的家族史。治疗通常需要多次手术，分期Z成形术是手术松解缩窄带的首选方法。并指畸形在患者幼年时进行松解。即使存在指蹼，更广泛的融合也需要进行分离和植皮。该病的病因仍在讨论中。文献中无法用先天性环状缩窄综合征的外源性理论解释的病例数量正在增加。我们的系列中有4例属于此类。
