Management of Aplasia Cutis Congenita: An 11-Year Experience

Aplasia cutis congenita (ACC) is a rare congenital condition characterized by focal absence of skin and soft tissue, most commonly on the scalp. Although many lesions are superficial, involving the epidermis and dermis, deeper structures such as muscle, bone, or dura may be affected. Small lesions often heal with minimal intervention, but severe cases can lead to complications such as cerebrospinal fluid leaks, hemorrhage, or infection, necessitating surgical intervention. This study reviews the management of ACC at a large academic pediatric hospital.

A retrospective chart review and analysis of patients diagnosed with ACC who were treated from January 2012 to October 2023 was conducted.

Of 162 identified patients, complete records were available for 144. Fifty-five percent (79/144) were female, and 47% (67/144) had comorbidities, including 12% (17/144) with internal organ abnormalities. The average lesion size was 7.05 cm² (range: 0.12-96 cm²). Patients with lesions averaging 1.87 cm² were managed with education and follow-up alone (42%), those with 7.14 cm² lesions received local wound care (21%), and those with 16.19 cm² lesions (range: 0.70-96 cm²) underwent surgical intervention (26%). Twelve percent of patients were lost to follow-up.

ACC primarily affects the scalp's superficial tissues and is often associated with limb anomalies or comorbidities such as cardiac or neurological defects. Conservative management is suitable for most lesions, but surgical intervention may be required to prevent life-threatening complications. Further research is needed to establish indications for surgery and optimal wound care strategies for rapid closure.