Sinus Pericranii Complicated by Hydrocephalus: Case Report and Literature Review.

Background: Sinus pericranii (SP) is a rare vascular condition that results when abnormal communication between the intracranial and extracranial venous systems is present. Here we report a rare case of hydrocephalus revealed through a SP scalp mass with a review of literature.

Case Description: A 13-year-old girl presented with a history of bulging left eye, bilateral gradual deterioration of vision, nausea, and progressive headaches. The family reported the sudden appearance of a scalp mass prior to these symptoms. Brain imaging showed supra- and infratentorial communicating hydrocephalus and subcutaneous vascular collaterals from the midportion of the superior sagittal sinus. After ventriculoperitoneal shunt insertion, interval reduction of the ventricular size and disappearance of extracranial veins without associated intra- or extracranial vascular anomalies was observed.

Conclusions: SP is divided into two main types: (1) Spontaneous type, in which lesions are formed due to acquired causes without a syndromic association or other vascular anomalies or stenosis. The most commonly reported cause is trauma in which SP may develop at the trauma site rather than the midline. (2) Congenital type, in which case the most commonly reported association is craniosynostosis. It can also be seen with vein of Galen hypoplasia, vein of Galen malformations, dural sinus malformations, solitary developmental venous anomalies, and intraosseous arteriovenous malformations. We are reporting the first case of idiopathic SP with hydrocephalus without a congenital association or acquired cause, including trauma. The sudden change in SP size can indicate a change in intracranial pressure and the development of hydrocephalus.