Herniation of the brain after conservative treatment of a large congenital skull defect in an infant with Adams-Oliver syndrome

Abstract

We report the case of a newborn baby with a large congenital skull defect owing to Adams-Oliver syndrome. An initial attempt at conservative treatment failed and led to disruption of the leptomeningeal membrane and prolapse of the brain. After local debridement, delayed and stepwise surgical closure was complicated by cerebrospinal fluid leakage, which required the insertion of a cerebrospinal fluid shunt. This case report highlights the potential serious risks of nonsurgical management of largely extended congenital skull defects.