Surgical management of aplasia cutis congenita of the scalp and skull defect in a resource-limited setting: A case report.

Background: Aplasia cutis congenita (ACC) is a rare congenital condition marked by the absence of skin layers and sometimes underlying structures. Its etiology is unclear, with up to 70% of cases involving the scalp. We report the first document case of ACC in Ghana. Case Description: A 1-day-old female, born through spontaneous vaginal delivery after an uneventful pregnancy, was referred for management of a scalp defect noted at birth. Examination revealed an 8 × 5.5 cm central scalp defect with absent cranial vault, partial fronto-parietal bone loss, dural defect, exposed arachnoid membranes, and visible superior sagittal sinus. Other physical findings were normal. Brain magnetic resonance imaging (MRI), whole-body MRI, and echocardiography were unremarkable. A brain computed tomography confirmed a skull defect. The patient underwent a duraplasty using bovine pericardium and received serial wound dressings with epithelial growth factors. The defect reduced to 4.5 × 3.6 cm post-surgery and continues to improve pending potential cranioplasty at 2 years. ACC is primarily diagnosed clinically, and this patient was diagnosed with type 1 ACC. Management depends on subtype, location, defect size, and infection risk. In this case, surgery was employed due to the size of defect and the risk it posed. Prognosis is generally favorable, but limited resources may delay and increase complications in low-income settings. Conclusion: ACC with skull and dural involvement poses serious risks to infant survival in resource-limited settings, where systemic challenges are pervasive. This case highlights the importance of care that is locally adapted, affordable, and delivered through strong multidisciplinary collaboration.