Congenital ring-constriction syndrome of the limbs; a report of 19 cases.

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.