A case of Adams-Oliver syndrome associated with acrania, microcephaly, hemiplegia, epilepsy, and mental retardation

Adams-Oliver syndrome (AOS) is a rare congenital disorder, characterized by aplasia cutis congenita (ACC) of the scalp and variable degrees of termina l transverse limb defects. In this article, a newborn infant diagnosed as A OS for a large scalp defect, acrania, and finger malformations is presented . The patient was hospitalized and the scalp defect was successfully repair ed with several surgical operations. During the hospitalization septicemia. meningitis, and convulsions developed, but they were successfully treated with appropriate antibiotics, antifungal. and anticonvulsive agents. He was discharged five months after admission to the hospital. Now, he is 3 years old, and has microcephaly, moderate mental retardation, left spastic hemip legia, and epilepsy.