Aplasia Cutis Congenita. Case report and literature review

Abstract

Aplasia Cutis Congenita (ACC) is a rare condition characterized by congenital absence of epidermis, dermis and, in some cases, like this one, subcutaneous tissue and bone. It usually involves the scalp vertex. The estimated incidence is 3 in 10,000 births resulting in a total number of 500 reported cases till nowadays. The lesion can occur on everybody surface, but scalp is the most affected region (70% of the cases). In about 20% of cases there is bone lesion. ACC can occur as an isolated defect or can be associated with a number of other congenitally anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised. Case presentation: We describe a child with ACC involving almost all skull, where it could be seen the brain only with arachnoid membrane. At presentation there was no Cerebral Spinal Fluid (CSF) leakage. And it was managed surgically. The child was operated on one time. It was covered with non-absorbable matrix and the skin was approached. Conclusion: There are multiple treatments for this condition. But there is no consensus on treatment strategies. Conservative treatment can be tried, especially when there is no bone lesion. The treatment must be individualized for each case.