Aplasia cutis congenita: clinical management of a rare congenital anomaly.

BACKGROUND

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by absence of skin and adjacent tissue that usually affects the scalp, but any part of the body may be affected. Although ACC is more often superficial and small, it can be large and involve the underlying structures such as skull and dura, thus increasing the risk of hemorrhage, infection, and mortality. Controversy exists regarding nonsurgical versus surgical intervention for this condition. This study reviews indications and modalities for treatment of this rare congenital anomaly.

RESULTS

Management of this anomaly depends on size, location, and structures at risk. Small lesions with intact underlying structures and lesions affecting extremities are treated in a conservative fashion with dressings and ointments followed by delayed scar excision. Aplasia cutis congenita scar excision often requires complex tissue rearrangement, tissue expansion, or skin grafting. Larger ACC lesions or lesions with exposure of vital structures require early surgical intervention. Initially, exposed vital structures and bony ridges can be protected using conservative measures. Delayed definitive repair can then be performed using scalp flaps, split- and full-thickness skin grafts, cultured epithelial autografts, delayed split rib cranioplasty, tissue expansion, and composite cranioplasty.

CONCLUSIONS

Aplasia cutis congenita should be individually evaluated based on size, depth, location, and tissues involved. Using conservative and surgical modalities, one can achieve complete closure of the defect, thus avoiding risks of infection, hemorrhage, and further trauma.