Constriction Band Syndrome.

Abstract

Constriction band syndrome is a rare congenital deformity that occurs in 0.5 to 1.1 per 10,000 births and presents as a range of malformations from constriction bands to autoamputation. The etiology of constriction band syndrome is incompletely understood, but the leading theory suggests that partial rupture of the inner amnion prior to fusion with the outer chorion creates fibrous chorionic bands that constrict fetal parts. Given the heterogeneity of malformations, a comprehensive physical examination is essential for accurate diagnosis. Depending on the severity of the constriction band, surgical intervention can range from simple excision of constriction bands to on-top-plasties in cases of digit deficiencies. Importantly, each patient should receive an individualized approach based on their anatomy, which considers the urgency of constriction band release, preservation of the neurovascular structures, viability of the distal body part, and maximization of functionality. With these goals in mind, surgical management of constriction band syndrome regardless of presentation can greatly improve function and cosmesis in affected children.