Surgical management of cutis aplasia with high-flow sinus pericranii.

Cutis aplasia (cutis aplasia congenita cystica) is a rare congenital disorder with failure of completion of the extracerebral coverings, involving a defect in the scalp and often a cranial defect. A case of cutis aplasia is reported with an associated high-flow venous fistula or sinus pericranii, resulting in altered venous drainage of sagittal sinus blood through dilated scalp veins. Radiological workup to ensure patency of the superior sagittal sinus was critical to the surgical management in this case.