Surgical Management of Sinus Pericranii With Crouzon Syndrome.

Abstract

Sinus pericranii (SP) is a rare vascular malformation which connects the intracranial dural sinuses to the extracranial venous drainage system. Although the majority of SP cases are caused by trauma, some of them are congenital. Furthermore, a few SP cases have been reported in association with craniosynostosis. The authors' objective is to discuss the surgical management of SP with Crouzon's syndrome in children. Three-Dimensional reconstruction with enhanced CT scan was used for evaluate the condition of SP with Crouzon's syndrome in all 4 cases. Two cases with small single-hole defect on skull were only treated by cranioplasty with distraction osteogenesis. In the management of the other 2 SP patients with large skull defect, titanium mesh was used for compression of dilated venous sinus to inhabit filling and promote shrinking. Four cases of SP with Crouzon's syndrome were treated in the authors' department. With cranioplasty with distraction osteogenesis only, 2 patients with single-hole skull defect experienced SP spontaneous involution at 4 to 6 months postoperatively. With titanium mesh compression, SP with large skull defect shrank significantly without clinical recurrence, but long-term follow-up was still in need. For small single-hole skull defect in SP patient with Crouzon's syndrome, the only application of cranioplasty for decreasing intracranial pressure can promote SP spontaneous involution. 3D printed titanium mesh compression renders a safe and efficient approach for SP with large skull defect. Long-time follow up and further accumulation of cases may establish the efficacy of the authors' management.