, Volume 29, Issue 4, pp 535-541
Date: 29 Dec 2012

Dilemmas and challenges in the management of a neonate with Adams–Oliver syndrome with infected giant aplasia cutis lesion and exsanguination: a case-based update

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Abstract

Background

Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with a variable extent of defective formation of the scalp. Adams–Oliver syndrome is a condition mainly characterized by the congenital absence of skin, known as “aplasia cutis” which is usually limited to the vertex scalp and transverse limb defects.

Case report

A 17-day-old term female neonate was referred to us with an infected scalp lesion of the vertex. The lesion which is about 10 × 9 cm had signs of infection with necrotic eschar. We started the neonate on systemic parenteral antibiotics with local dressings. On day 3 of conservative management, the neonate had exsanguination due to bleeding from the midline with severe hemodynamic compromise requiring cardiopulmonary resuscitation. After controlling the bleeding with local tamponade and resuscitating the child, she was taken for early surgery. Debridement and bipedicled rotation flap of the scalp to cover the raw area was performed. On day 18, the flap started showing signs of necrosis. The neonate was taken up for debridement, and subsequently, maternal allograft of split-thickness skin was placed as a temporary wound cover. Meanwhile, the wound showed progressive epithelialization. At 1 year, the patient continued to have a non-healing area, which was later successfully covered with a split-thickness skin graft. We plan to revaluate the need for cranioplasty at around 3–4 years of age.

Discussion

We discuss the dilemmas and challenges involved in the successful management of a neonate with Adams–Oliver syndrome with infected aplasia cutis and an episode of life-threatening exsanguination.

Conclusion

Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with defective formation of the scalp to a varying extent and severity, requiring various timely strategies.