Hyperemic hydrocephalus: a new form of childhood hydrocephalus analogous to hyperemic intracranial hypertension in adults.
[hydrocephalus with stenosis of the aqueduct of sylvius]
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.
