Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage

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Object. In this study the authors investigated whether patterns of intracranial venous drainage in children with complex craniosynostosis associated with raised intracranial pressure (ICP) were abnormal and, thus, could support the theory that venous hypertension is a major contributor to raised ICP that can lead to impaired visual function or even blindness in these patients.

Methods. The authors analyzed the anatomy of intracranial venous drainage as demonstrated in the results of 24 angiography studies obtained in 23 patients, all of whom had either a craniosynostosis-related syndrome (18 patients) or a nonsyndromic multisutural synostosis (five patients). Twenty-one patients had experienced raised ICP (in 19 patients diagnosis was based on invasive ICP monitoring and in two patients on clinical grounds alone) 1 to 6 weeks before undergoing angiography. Of the two remaining patients (both with Apert syndrome) whose ICP monitoring was normal immediately before angiography, each had undergone two previous cranial vault expansion procedures.

On results of 18 angiography studies a 51 to 99% stenosis or no flow at all could be observed in the sigmoid—jugular sinus complex either bilaterally (11 patients) or unilaterally (seven patients). In 11 of these patients a florid collateral circulation through the stylomastoid emissary venous plexus was also seen. Two angiography studies were performed in one patient with Crouzon syndrome. A comparison of the two studies demonstrated a progression of the abnormal venous anatomy in that case. The authors found no obvious correlation between each patient's baseline ICP and the degree of abnormality of their venous anatomy, as judged on the basis of a venous-phase angiography severity score.

Conclusions. Based on their findings, the authors assert that in children with complex forms of craniosynostosis in whom other factors, such as hydrocephalus, are absent, abnormalities of venous drainage that particularly affect the sigmoid—jugular sinus complex produce a state of venous hypertension that, in turn, is responsible for the majority of cases of raised ICP. The incidence of these changes is unknown, but an analysis of the ages of the children in this study indicated that the period of particular vulnerability to the effects of venous hypertension lasts until the affected child is approximately 6 years old. After that age the collateral venous drainage through the stylomastoid plexus will likely become sufficient to allow ICP to normalize.

Article Information

Address reprint requests to: Richard Hayward, F.R.C.S., Department of Neuroradiology and Craniofacial Surgery, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, United Kingdom. email: richard.hayward@gosh-tr.nthames.nhs.uk.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 3. Angiogram demonstrating normal left transverse and sigmoid sinuses and a patent jugular bulb. On the right side there is a nonopacified transverse sinus and a nonopacified sigmoid sinus.

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    Case 2. Angiogram revealing severe stenosis (S++) of the sigmoid sinus on the right side with prominent stylomastoid collateral veins (+). Nonopacification of the sigmoid sinus can be observed on the left side and there is nonvisualization of the jugular bulbs bilaterally.

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    Case 7. Angiogram demonstrating nonopacification of the right transverse sinus, sigmoid sinus, and jugular bulb.

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    Left: Case 1. Angiogram demonstrating stylomastoid emissary veins having prominent collateral veins. Right: Case 4. Angiogram demonstrating stylomastoid emissary veins having a florid collateral vein circulation.

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    Case 5. Left: Initial angiogram revealing nonopacification of the left sigmoid sinus and severe stenosis (S++) of the left jugular bulb and segment. Right: Angiogram obtained during the second angiography session demonstrating progression on the right side to mild narrowing (S+) of the jugular bulb and a florid stylomastoid venous plexus, and progression on the left side to nonopacification of the jugular segment.

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    Graph showing the lack of any relationship between intracranial pressure and severity score.

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