Management of large scalp and skull defects in a severe case of Adams-Oliver syndrome

Abstract

Adams-Oliver syndrome is a rare congenital disorder that includes congenital scalp and skull defects, variable degrees of terminal transverse limb anomalies, and cardiac malformations. Cutis aplasia occurring in 75% of patients is a potentially life-threatening condition. Large skin defects that cannot be closed primarily present a management dilemma, and may require skin grafting or flaps, or a combination of both operative and conservative modalities. The authors' experience in management of huge scalp and bone defects with the Integra Dermal Regeneration Template and regular dressing changes showed good scalp repair and no serious complications attributed to this approach.