Fatal superior sagittal sinus hemorrhage as a complication of aplasia cutis congenita: a case report and literature review

Abstract

Aplasia cutis congenita (ACC) is a rare condition that occurs in around 0.01% of births. Characterized by a localized absence of skin, it affects the midline of the scalp in over 80% of cases. We describe the case of an infant born via vaginal spontaneous delivery with ACC affecting the scalp. This extended to the subcutaneous tissue and bone leaving the dura mater exposed. The patient was managed conservatively. At 4 weeks of age, she had a fatal superior sagittal sinus hemorrhage. In a review of the literature, we found ten previous cases of hemorrhage of the superior sagittal sinus complicating an ACC. Including our case, ACC complicated by hemorrhage of the superior sagittal sinus shows a 36% mortality (4/11). An analysis of these cases suggests that this tends to occur between 1 and 3 months of age, though it may occur in younger neonates.