Treatment of a Large Skull Defect and Brain Herniation in a Newborn With Adams-Oliver Syndrome

Adams-Oliver syndrome (AOS) is a rare congenital disorder characterised by a wide variety of clinical expression ranging from the occurrence of aplasia cutis congenita (ACC), transverse limb defects, and cutis marmorata telangiectica to extensive lethal anomalies. In this article, we present the conservative and surgical management of a male newborn infant diagnosed with AOS. Surgical treatment included wound management, the removal of protruding brain, and treatment of cerebrospinal fluid (CSF) leakage. After spontaneous reepithelization of the wounds, conservative treatment was chosen instead of reconstruction with an occipital flap; this was continued until the total healing of the dermal defect after eight months, during which the patient was continuously treated with antibiotics. At 17 months, the child was in good physical condition with a three-month development delay in comparison with infants of his age and no evidence of neurological deficit.