Abstract

Adams Oliver syndrome (AOS) is characterized by a combination of congenital scalp defects (aplasia cutis congenita) and terminal transverse limb defects. Various expressions of AOS have been reported. It can also be associated with extensive lethal anomalies of central nervous, cardiopulmonary, gastrointestinal, and genitourinary systems. Here we report a newborn diagnosed AOS with severe scalp defect, terminal limb defects and cutis marmorata telangiectatica congenita without systemic involvement. During hospitalization menengitis developed as a major complication and treated with appropriate antibiotheraphy. Scalp defect was treated with local wound care. In conclusion it is important to be aware of different presentations of AOS. All newborn infants with aplasia cutis congenita should be evaluated carefully with physical examination and laboratory workup to rule out associated diseases.

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