Extensive congenital cutis aplasia of the scalp treated with conservative management: a case report

Abstract

Introduction: Congenital cutis aplasia (CCA) is a rare disorder characterized by the localized absence of skin at birth, primarily affecting the scalp, with lesions ranging from superficial defects to extensive lesions associated with osseous or systemic involvement. Methodology: We present the case of a full-term newborn (FTN) born to a mother with gestational diabetes (GD) and delivered by emergency cesarean section, who presented with an extensive CCA lesion of the scalp. Clinical evaluation considered the extent, location, and depth of the lesion, as well as the presence of neurological, osseous, vascular, or systemic involvement. To guide therapeutic decision-making, a narrative review of the available literature on CCA was conducted, focusing on evaluation criteria, factors associated with complications, and conservative and surgical management options. Results: A multidisciplinary evaluation was performed, with no evidence of associated systemic involvement. The patient showed a favorable clinical course with conservative management. Discussion: The extent of the lesion and its proximity to critical vascular structures represented a significant clinical risk; however, the absence of radiographically identifiable osseous involvement, together with clinical stability and the absence of hemorrhagic or infectious complications, allowed conservative management to be maintained under close monitoring. Conclusion: This case highlights the importance of a comprehensive approach to the evaluation of extensive CCA, as well as the role of conservative management in selected patients without major complications.