Surgical Management of Cutis Aplasia with High-Flow Sinus pericranii

Abstract

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.

Sinus pericranii: diagnosis and management in 21 pediatric patients

“…Finally, SP has been reported in association with several malformations and syndromes, as shown in Table 3. 5,6,9,10,16,17,24,26,27,31,32,47,50 The natural history of SP is not clearly understood. Although SP is generally considered benign, the outcome may not be predicted easily.…”

Two different management modalities in a two sibling case report of Adams Oliver syndrome

“…In the long-term, reconstitution of the bony calvaria and scalp with protection of the underlying brain, and appropriate cosmesis and hair growth is desirable. Various papers have been published related to ACC, yet there is no consensus on the therapeutic approach 11. The management decision hinges upon balancing the risks of spontaneous haemorrhage from the risk of surgical intervention in different sizes of lesions and varying degrees of depth of defect.…”

Aplasia cutis congenita of the scalp

“…Certain instances of ACC are probably related to an underlying in- Computerized tomography scan showing the skull defect close to the midline (arrows). The brain was normal voluted leptomeningeal angioma or even to sinus pericranii [16,21].…”

“…Differential diagnosis is established against atretic cephalocele, sinus pericranii, and heterotopic scalp tissues [16,18]. Prenatal diagnosis with ultrasound seems to be unreliable.…”

“…In our patient, we assessed his general condition by clinical examination, and that of the brain by performing an urgent CT scan [14,16]. After careful evaluation of these clinical data, we decided to undertake early surgical treatment of the lesion in order to avoid infection or hemorrhage.…”