Challenges in the management of cutis congenita aplasia in rural low resource settings: A case report from Kabale regional referral hospital in South-Western Uganda

Abstract

Cutis congenital aplasia (CCA) is a rare congenital abnormality that presents with a localized or widespread absence of skin at birth. It is estimated to occur in approximately 0.5 to 2.8/10,000 newborns, with a male: female ratio of 1: 7.  The most commonly affected part of the body is the scalp at the vertex. Widespread lesions affecting multiple parts of the body have been encountered.  Diagnosis of CCA is mainly by clinical examination at birth. Variable presentations at birth include skin erosion, ulceration, membrane-covered defect, or scar, indicating intrauterine healing. Management of CCA depends on the size of the lesion and the presence of other abnormalities. Lesions< 4 cm heal with proper wound care. Larger lesions greater than 4cm require early surgical intervention which improves healing and reduces the risk of complications