Adams-Oliver Syndrome

Abstract

A term infant born in a resource-poor setting (Mozambique) presented with extensive disepithelization of the cranial vertex with absent underlying bony plate, disepithelization of the posterior median line, and bilateral amputation of the fifth toe. These iconic clinical features are indicative of extensive Aplasia Cutis with associated transverse limb defects, a condition known as Adams-Oliver Syndrome. Management is typically conservative; increased need for fluids, risk of electrolyte derangements and infections are possibly associated issues in the first days of life, especially in case of large lesions, and have to be carefully monitored and adequately addressed.