Facial nevi associated with anomalous venous return and hydrocephalus

The authors describe two children with anomalous intracranial venous return associated with bilateral facial nevi, macrocrania, and cephalic venous hypertension. Both children had functional absence of the jugular bulbs, forcing the intracranial venous effluent to exit through persistent emissary pathways. Both children had sustained intracranial hypertension, with one child developing symptomatic communicating hydrocephalus that responded satisfactorily to shunting. The relationship between these patients and those with Sturge-Weber syndrome is discussed. The embryologic abnormality producing the anomalous venous return is characterized. The link between venous hypertension and the development of hydrocephalus is discussed. The increased cranial compliance seen in this age group may predispose certain pediatric patients to develop hydrocephalus when stressed by venous hypertension.