Hyperemic hydrocephalus: a new form of childhood hydrocephalus analogous to hyperemic intracranial hypertension in adults

Abstract

In the majority of adults with idiopathic intracranial hypertension (IIH), there is an elevation in venous pressure associated with a venous outflow stenosis. In about 15% of IIH patients the elevated venous pressure is associated with an elevation in blood flow but little or no evidence of a stenosis. Venostenotic IIH and idiopathic hydrocephalus in children with a normal blood inflow have been shown to be equivalent. The aim of this study was to test whether children with hydrocephalus and an elevated arterial inflow have a vascular pathophysiology that is analogous to the hyperemic form of IIH in adults. Nine children with idiopathic hydrocephalus underwent MR imaging with flow quantification and were found to have arterial inflows 2 SDs above the mean for normal controls. Measurements of the head circumference, ventricular enlargement, total blood inflow, superior sagittal sinus (SSS)/straight sinus (SS) outflow, and the degree of collateral venous flow were performed. The results were compared with findings in 14 age-matched controls. In hyperemic hydrocephalus the cerebral blood inflow was elevated but the SSS and SS outflows were in the normal range. The sinus outflow as a percentage of the inflow was reduced by 8 percentage points in the SSS territory and 5 percentage points in the SS territory compared with findings in the controls (p = 0.04, p = 0.003, respectively), suggesting blood was returning via collateral channels. Similar to patients with hyperemic IIH, children with hyperemic hydrocephalus show a significant elevation in collateral venous flow, indicating that the same venous pathophysiology may be operating in both conditions.