Aplasia Cutis Congenita Treatment & Management

Updated: May 12, 2017
  • Author: Joy Wan, MD; Chief Editor: William D James, MD  more...
  • Print
Treatment

Medical Care

The decision to use medical, surgical, or both forms of therapy in aplasia cutis congenita (ACC) depends primarily on the size, depth, and location of the cutaneous defect and any therapy indicated for associated defects. [61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71]

Local therapy includes gentle cleansing and application of a bland ointment or topical antibiotic ointment to prevent desiccation of the defect.

Antibiotics are only indicated if overt signs of infection are noted.

Other treatment is rarely necessary because the erosions and ulcerations almost always heal spontaneously.

A variety of specialized dressing materials have been used. [72, 73]

Next:

Surgical Care

Surgical repair is not usually indicated in aplasia cutis congenita (ACC) 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 may require excision with primary closure, if feasible, or the use of tissue expanders and rotation of a flap to fill the defect. Occasionally, skin or bone grafting may be required.

Patients with large, full-thickness scalp defects may face a treatment dilemma: conservative versus surgical therapy of the defect. Complete osseous regeneration of a large skull defect associated with aplasia cutis congenita has been obtained with a conservative approach of skin care and topical antibiotics. No surgical treatment of bone or soft tissue reconstruction was necessary. On the other hand, aplasia cutis congenita of the scalp may be complicated by sagittal sinus hemorrhage or thrombosis, and primary closure with scalp flaps may prevent a potentially fatal outcome.

Truncal and limb defects, despite their large size, usually epithelialize and form atrophic scars, which can later be revised if necessary.

Patients should be evaluated for evidence of epidermolysis bullosa before a surgical endeavor is undertaken.

Previous
Next:

Consultations

Genetic counseling should be considered if associated anomalies are noted. Consultations based on other findings are appropriate to evaluate these conditions.

Previous