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Hemorrhagic Moyamoya Disease during Pregnancy

Published online by Cambridge University Press:  04 August 2016

John C.L. Sun
Affiliation:
Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
Margaret Yakimov
Affiliation:
Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver, BC, Canada
Ismail Al-Badawi
Affiliation:
Department of Obstetrics and Gynaecology, University of British Columbia, Vancouver, BC, Canada
Christopher R. Honey*
Affiliation:
Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
*
Division of Neurosurgery, University of British Columbia, 3rd Floor, 910 West 10th Avenue, Vancouver, B.C. Canada V5Z 4E3.
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Abstract:

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Background:

Intracranial hemorrhage in pregnant patients with Moyamoya disease is rare. We review the case of one such patient who presented with pre-eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties.

Methods:

A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a progressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubated, ventilated and given intravenous mannitol and magnesium sulfate. She underwent a simultaneous craniotomy and Cesarean section. Post-operatively the patient's ICP and jugular venous saturation were monitored in the intensive care unit.

Results:

The patient delivered a 1185g infant who did well. The patient's ICP was well controlled until the tenth post-operative day when she developed malignant brain edema and died.

Conclusion:

This case highlights three important points. First, simultaneous craniotomy and Cesarean section can be performed. Second, intraoperative control of bleeding Moyamoya vessels is described. Third, the difficult post-operative management of these cases is highlighted. The literature regarding Moyamoya disease and pregnancy is reviewed and some recommendations for the management of this rare but potentially deadly condition are presented.

Résumé:

RÉSUMÉ:Introduction:

L'hémorragie intracrânienne chez les patientes enceintes présentant la maladie de Moyamoya est rare. Nous revoyons le cas d'une patiente qui s'est présentée avec de la prééclampsie et une hémorragie intracérébrale catastrophique afin de souligner les difficultés rencontrées dans la prise en charge de telles patientes.

Méthodes:

Il s'agit d'une femme enceinte de 31 semaines qui a été amenée à l'urgence parce qu'elle présentait de l'hypertension et une détérioration progressive de la conscience. Elle a développé rapidement une mydriase à droite et une posture en extension à gauche. Un scan cérébral a montré une hémorragie intracérébrale importante dans le putamen. Elle a été intubée, ventilée et on lui a administré du mannitol intraveineux ainsi que du sulfate de magnésium. Elle a subi simultanément une crâniotomie et une césarienne. Dans les suites postopératoires, on a monitoré sa pression intracrânienne et son niveau de saturation au niveau de la jugulaire aux soins intensifs.

Résultats:

L'enfant pesait 1185 g et son évolution a été favorable. La pression intracrânienne de la patiente a été bien contrôlée jusqu'au dixième jour postopératoire alors qu'elle a développé un tdème malin du cerveau et elle est décédée.

Conclusion:

Ce cas illustre trois points importants. Premièrement, on peut faire simultanément une crâniotomie et une césarienne. Deuxièmement, nous décrivons le contrôle du saignement pendant la chirurgie, au niveau des vaisseaux atteints de la maladie de Moyamoya. Troisièmement, nous soulignons les difficultés du traitement postopératoire de ces cas. Nous revoyons la littérature concernant la maladie de Moyamoya et la grossesse ainsi que certaines recommandations pour la prise en charge de cette maladie rare mais potentiellement fatale.

Type
Case Report
Copyright
Copyright © The Canadian Journal of Neurological 2000

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