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

Clinical article

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Object

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.

Methods

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.

Results

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.

Conclusions

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.

Abbreviations used in this paper: AVM = arteriovenous malformation; IIH = idiopathic intracranial hypertension; SS = straight sinus; SSS = superior sagittal sinus.

Article Information

Address correspondence to: Grant Bateman, M.B.B.S., F.R.A.N.Z.C.R., Department of Medical Imaging, John Hunter Hospital, Locked Bag 1, Newcastle Region Mail Center, 2310 Australia. email: grant.bateman@hnehealth.nsw.gov.au.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Case of a 6-month-old infant with hydrocephalus who had a normal arterial inflow of 700 ml/minute. Left: Axial T2-weighted MR image. Right: An MR venogram showing a tapered stenosis of the dominant transverse sinus (arrow, flow gap). A similar flow gap was noted on the other side. The SSS outflow was limited to 210 ml/minute (a 30% return).

  • View in gallery

    A: An MR flow quantification study from both the internal carotid and basilar arteries in an adult man with IIH showing an inflow of 1500 ml/minute. B: A selected slice from a susceptibility-weighted contrast-enhanced perfusion study. Region of Interest 1 shows a mean blood flow of 187 ml/100 g/min in an area consisting mostly of gray matter. Region of Interest 2 shows the right frontal white matter to average 35 ml/100g/min.

  • View in gallery

    Case of a 12-year-old child who had elevated CSF pressure and hydrocephalus with an elevated arterial inflow above 2000 ml/minute. A: Axial T2-weighted MR image. B: An MR venogram showing no evidence of outflow stenosis. Note the well-depicted smaller cortical veins and the deep venous system. There are extensive collateral veins at the skull base. C: Graphic representation of venous outflow. The total venous outflow, measured below the skull base (incorporating the major sinuses and collaterals), was 33 ml/second or just under 2000 ml/minute.

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