What is the prognosis of aplasia cutis congenita (ACC)?

Updated: Jul 31, 2018
  • Author: Joy Wan, MD, MSCE; Chief Editor: William D James, MD  more...
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Answer

The prognosis for aplasia cutis congenita (ACC) is usually excellent. If the defect is small, recovery is uneventful, with gradual epithelialization and formation of a hairless, atrophic scar over several weeks. Small underlying bony defects usually close spontaneously during the first year of life. Surgical repair of large or multiple scalp defects with excision and primary closure, if feasible, or with the use of tissue expanders and rotation of a flap, may be considered. Truncal and limb defects, despite their large size, usually epithelialize and form atrophic scars, which can later be revised if necessary.

If aplasia cutis congenita is associated with other anomalies, the prognosis is dependent on the severity of the associated abnormalities. Underlying or associated defects may significantly affect mortality and morbidity. Full-thickness defects of the scalp, skull, and dura are associated with a mortality rate of greater than 50%. Even large defects on areas other than the scalp usually heal well with conservative skin care using topical antibiotic ointment. The rare larger scalp defects are prone to complications of hemorrhage and infection, placing patients at risk for death. Extensive aplasia cutis congenita of the scalp may be associated with an increased risk of sagittal sinus thrombosis. For these reasons, surgical intervention may be required for large, full-thickness scalp defects.


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