Hydrocephalus in an achondroplastic child treated by venous decompression at the jugular foramen

Case report

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✓ A 10-month-old child with achondroplasia with progressive head enlargement, ventriculomegaly, and wide subarachnoid spaces over the hemispheres was referred for evaluation. A steady-state lumbar infusion test revealed increased cerebrospinal fluid (CSF) outflow resistance (14 mm Hg/ml/min), and intra-arterial digital subtraction angiography (DSA) demonstrated bilateral venous outflow obstruction due to stenosis of the jugular foramen. Surgical decompression by opening the right jugular foramen relieved the clinical signs of intracranial hypertension. During the following year, the patient's head enlargement was moderate with relative normalization of size. Repeat DSA demonstrated improved venous runoff on the right side, and a steady-state lumbar infusion test demonstrated reduced CSF outflow resistance (10 mm Hg/ml/min). Venous decompression is causal therapy and may prove to be preferable to shunting in children with hydrocephalus and bilateral stenosis of the jugular foramen.

Article Information

Address reprint requests to: Tryggve Lundar, M.D., Department of Neurosurgery, Rikshospitalet, N-0027, Oslo 1, Norway.

© AANS, except where prohibited by US copyright law.

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Figures

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    Computerized tomography scans demonstrating ventriculomegaly and enlarged subarachnoid spaces at 6 months of age (left), preoperatively at 9 months (center), and at 14 months (4 months postoperatively) with slightly rerinred ventricular size (right).

  • View in gallery

    Left: Intra-arterial digital subtraction angiography (DSA) demonstrating impaired venous runoff due to bilateral high-grade venous stenosis (75% to 90% luminal obstruction) at the level of the jugular foramen. Right: Postoperative DSA showing improved venous runoff on the decompressed right side.

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    Plot of the head circumference. The arrow indicates performance of the operation at the age of 10 months and is followed by reduced circumference (48.8 to 47.7 cm).

References

1.

Kobayashi AHashi K: Secondary spinal canal stenosis associated with long-term ventriculoperitoneal shunt. J Neurosurg 59:8548601983Kobayashi A Hashi K: Secondary spinal canal stenosis associated with long-term ventriculoperitoneal shunt. J Neurosurg 59:854–860 1983

2.

Marmarou AShulman KRosende RM: A nonlinear analysis of the cerebrospinal fluid system and intracranial pressure dynamics. J Neurosurg 48:3323441978Marmarou A Shulman K Rosende RM: A nonlinear analysis of the cerebrospinal fluid system and intracranial pressure dynamics. J Neurosurg 48:332–344 1978

3.

Rosman NPShands KN: Hydrocephalus caused by increased intracranial venous pressure: a clinicopathological study. Ann Neurol 3:4454501978Rosman NP Shands KN: Hydrocephalus caused by increased intracranial venous pressure: a clinicopathological study. Ann Neurol 3:445–450 1978

4.

Sainte-Rose CLaCombe JPierre-Kahn Aet al: Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:7277361984Sainte-Rose C LaCombe J Pierre-Kahn A et al: Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:727–736 1984

5.

Steinbok PHall JFlodmark O: Hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension. J Neurosurg 71:42481989Steinbok P Hall J Flodmark O: Hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension. J Neurosurg 71:42–48 1989

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