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 or mortality. However, in patients with large scalp and skull defects, there are risks of infection and bleeding. Both surgical and conservative treatment 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 scalp flap by means of tissue expansion at the age of 3 years. The original skull defect, 7 x 10 cm in size, decreased to 1.2 x 1.2 cm after a 4.5-year follow-up. No complications, such as haemorrhage or meningitis, were encountered. The final result was satisfactory.