Large scalp and skull defect in aplasia cutis congenita

Aplasia cutis congenita (ACC) is a rare disease, which may involve any site on the body but usually the vertex with scalp and even skull involvement. Superficial lesions may heal spontaneously and seldom result in morbidity o r mortality. However, in patients with large scalp and skull defects, there are risks of infection and bleeding. Both surgical and conservative treatm ent have been proposed. We report a case of successful treatment of ACC by early debridement and wound closure with split-thickness skin graft (STSG) in the neonatal stage followed by replacement of the STSG with a hairy scal p flap by means of tissue expansion at the age of 3 years. The original sku ll defect, 7 x 10 cm in size, decreased to 1.2 x 1.2 cm after a 4.5-year fo llow-up. No complications, such as haemorrhage or meningitis, were encounte red. The final result was satisfactory. (C) 2000 The British Association of Plastic Surgeons.