Cerebrofacial venous anomalies, sinus pericranii, ocular abnormalities and developmental delay

Abstract

The clinical implications of venous cerebrovascular maldevelopment remain poorly understood. We report on the association of cerebrofacial venous anomalies (including sinus pericranii), ocular abnormalities and mild developmental delay in two children. In addition, one child had a seizure disorder. Complex cerebrofacial slow-flow vascular anomalies may herald an underlying developmental aberration affecting the cerebrofacial and orbital regions.

Figure 1

A) First Patient. There is a small left orbit and globe with venous expansion of the left forehead. B) Non-contrast enhanced head CT scan reveals a focal calcification in the left basal ganglia (small arrow) and venous malformation of the scalp (large arrow). C) Enhanced Axial T1-weighted MR Image shows smaller left cerebral hemisphere with DVAs in the right caudate nucleus and thalamus (small arrow). The anomalous transcalvarial and epicranial veins are clearly depicted (large arrow). D) Venous phase of cerebral angiography shows global anomalies of the venous drainage of the brain. Note the presence of persistent falcine sinus (long arrow) and fenestrated, underdeveloped superior sagittal sinus with retrograde filling of the sinus pericranii (short arrows).

Figure 2

A) Second Patient. An overgrown left cheek with venous malformation extending into the upper lip and upper eyelid. B) Sonographic image of the left orbit demonstrates linear echogenic septa (arrow) within the posterior aspect of the vitreous space. C) Venous phase of cerebral angiography show transcalvarial communication between the superior sagittal sinus and the epicranial venous malformation.