Management of Aplasia cutis congenita of the scalp

Abstract

BACKGROUND: Aplasia cutis congenita (ACC) is a rare condition commonly affecting the scalp in which there is a focal deficiency of cutaneous tissues of varying severity ranging from an absence of skin through to full thickness defects involving deeper elements such as bone and dura. Lesions of the scalp can be associated with complications including infection, hemorrhage, thrombosis, and seizures. Opinions in the current literature regarding management of this condition are varied with both conservative and surgical management advocated. Conservative treatment consists of regular wound dressings and systemic antibiotics, while surgical management commonly involves skin grafting and local flaps. METHODS: A retrospective case review was performed to audit the outcomes of patients with ACC of the scalp managed at the Women's and Children's Hospital (WCH) in Adelaide, Australia from 2002 to 2012. Cases were identified from admission coding diagnoses and data was retrieved from patient case notes. RESULTS: Seventeen cases of ACC were identified. The most common location involved was the scalp vertex. Thirteen patients were managed conservatively and 4 had primary surgical intervention. Of the cases that were managed with primary surgery, 2 had complications. None of the conservatively managed patients had complications in the acute setting. CONCLUSIONS: At the WCH, we advocate adopting a conservative approach to management of ACC of the scalp. Defects can be successfully managed with a combination of regular dressings and systemic antibiotics. Regular wound monitoring is essential to detect any complications early to instigate appropriate treatment and determine the need for emergency surgical management.