Amniotic band syndrome.

Abstract

Amniotic band syndrome is an uncommon, congenital fetal abnormality with multiple disfiguring and disabling manifestations. A wide spectrum of clinical deformities are encountered and range from simple ring constrictions to major craniofacial and visceral defects. Lower extremity limb malformations are extremely common and consist of asymmetric digital ring constrictions, distal atrophy, congenital intrauterine amputations, acrosyndactyly, lymphedema and clubfoot. Although debated, early amnion rupture with subsequent entanglement of fetal parts (mostly limbs and appendages) by amniotic strands is the primary theory of pathogenesis. The sporadic nature of this congenital anomaly is discussed, as well as a case study involving the surgical correction of an associated rigid clubfoot deformity.