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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

References

REFERENCES:

1. Takagi, T, Ushimaru, N, Hirata, M. [A case of “moyamoya” disease found out after cesarean section.] Sanka To Fujinka 1977; 44: 949954.Google Scholar
2. Fujita, K, Yamasaki, S, Tamaki, N, et al. [Cerebro-vascular accident during pregnancy.] No Shinkei Geka 1978; 6: 989995.Google ScholarPubMed
3. Karasawa, J, Kikuchi, H, Furuse, S. Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 1978; 49: 679688.CrossRefGoogle ScholarPubMed
4. Karasawa, J, Kikuchi, H, Furuse, S. Subependymal hematoma in “moyamoya” disease. Surg Neurol 1980; 13: 118120.Google ScholarPubMed
5. Enomoto, H, Goto, H. Moyamoya disease presenting as intracerebral hemorrhage during pregnancy: case report and review of the literature. Neurosurgery 1986; 20: 3335.CrossRefGoogle Scholar
6. Hashimoto, K, Fujii, K, Nishimura, K, et al. Occlusive cerebrovascular disease with moyamoya vessels and intracranial hemorrhage during pregnancy. Neurol Med Chir 1988; 28: 588593.CrossRefGoogle ScholarPubMed
7. Amin-Hanjani, S, Kuhn, M, Sloane, N, et al. Moyamoya disease in pregnancy: a case report. Am J Obstet Gynecol 1993; 169: 395396.CrossRefGoogle ScholarPubMed
8. Komiyama, M, Yasui, T, Kitano, S, et al. Moyamoya disease and pregnancy: case report and review of the literature. Neurosurgery 1998; 43: 360369.CrossRefGoogle ScholarPubMed
9. Dias, MS, Sekhar, LN. Intracranial hemorrhage from aneurysms and arteriovenous malformations during pregnancy and the puerperium. Neurosurgery 1990; 27: 855866.CrossRefGoogle ScholarPubMed
10. Hendricks, CH. The hemodynamics of a uterine contraction. Am J Obstet Gynecol 1958; 76: 969982.CrossRefGoogle ScholarPubMed
11. Kerr, MG. Cardiovascular dynamics in pregnancy and labour. Br Med Bull 1968; 24: 1924.CrossRefGoogle Scholar
12. Ueland, K, Hansen, J. Maternal cardiovascular dynamics: II. Posture and uterine contractions. Am J Obstet Gynecol 1969; 103: 118.CrossRefGoogle ScholarPubMed
13. Sharshar, T, Lamy, C, Mas, JL. Incidence and causes of strokes associated with pregnancy and puerperium. Stroke 1995; 26: 930936.CrossRefGoogle ScholarPubMed
14. Amias, AG. Cerebral vascular disease in pregnancy: I. Hemorrhage. J Obstet Gynecol Br Commonw 1970; 77: 100120.CrossRefGoogle Scholar
15. Robinson, JL, Hall, CJ, Sedzimir, CB. Subarachnoid hemorrhage in pregnancy. J Neurosurg 1972; 36:2733.CrossRefGoogle ScholarPubMed
16. Robinson, JL, Hall, CJ, Sedzimir, CB. Arteriovenous malformations, aneurysms, and pregnancy. J Neurosurg 1974; 41: 6370.CrossRefGoogle ScholarPubMed
17. Matsuoka, M, Kamata, T, Okuba, H. [Pregnancy and delivery of the patient with moyamoya disease.] Sanfujinka Chiryo 1976; 33: 572575.Google Scholar
18. Bingham, WF, Beguin, EA, Ramirez-Lessepas, M. Moyamoya disease in pregnancy. Wis Med J 1980; 79: 2125.Google ScholarPubMed
19. Sasaki, J, Mezaki, N. [Considerations concerning the management of pregnancy and delivery in “moyamoya” disease.] Sanka To Fujinka 1984; 51: 109116.Google Scholar
20. Miyakawa, I, Lee, HC, Haruyama, Y, et al. Occlusive disease of the internal carotid arteries with vascular collaterals (moyamoya disease) in pregnancy. Arch Gynecol 1986; 237: 175180.CrossRefGoogle ScholarPubMed
21. Ogawa, A, Yoshimoto, T, Suzuki, J, et al. Cerebral blood flow in moyamoya disease. Part 1: correlation with age and regional distribution. Acta Neurochir 1990; 105: 3034.CrossRefGoogle ScholarPubMed
22. Kawaguchi, S, Sakaki, T, Kakizaki, T, et al. Clinical features of the haemorrhage type moyamoya disease based on 31 cases. Acta Neurochir 1996; 138: 12001210.CrossRefGoogle ScholarPubMed
23. Viscomi, CM, Wilson, J, Bernstein, I. Anesthetic management of a parturient with an incompletely resected cerebral arteriovenous malformation. Reg Anesth 1997; 22: 192197.Google ScholarPubMed
24. Colosimo, C Jr, Fileni, A, Guerrini, P. CT findings in eclampsia. Neuroradiology 1985; 27: 313317.CrossRefGoogle ScholarPubMed
25. Lazebnik, N, Pazmino, R, Dierker, LR, et al. Maternal intracranial hemorrhage complicating severe superimposed pre-eclampsia. J Reprod Med 1989; 34: 857860.Google Scholar
26. Vandenplas, O, Dive, A, Dooms, G, et al. Magnetic resonance evaluation of severe neurologic disorders in eclampsia. Neuroradiology 1990; 32: 4749.CrossRefGoogle ScholarPubMed
27. Digre, KB, Varner, MW, Osborn, AG, et al. Cranial magnetic resonance imaging in severe pre-eclampsia vs eclampsia. Arch Neurol 1993; 50: 399406.CrossRefGoogle ScholarPubMed
28. Drislane, FW, Wang, A. Multifocal cerebral hemorrhage in eclampsia and severe pre-eclampsia. J Neurol 1997; 244: 194198.CrossRefGoogle ScholarPubMed
29. Giannina, G, Smith, D, Belfort, MA, et al. Atraumatic subdural hematoma associated with pre-eclampsia. J Matern Fetal Med 1997; 6: 9395.Google ScholarPubMed
30. Maymon, R, Fejgin, M. Intracranial hemorrhage during pregnancy and puerperium. Obstet Gynecol Surv 1990; 45:157159.CrossRefGoogle ScholarPubMed
31. Hunt, HB, Schifrin, BS, Suzuki, K. Ruptured berry aneurysms and pregnancy. Obstet Gynecol 1974; 43: 827837.Google ScholarPubMed
32. DeGrood, RM, Beemer, WH, Fenner, DE, et al. A large meningioma presenting as a neurologic emergency in late pregnancy. Obstet Gynecol 1987; 69: 439440.Google ScholarPubMed
33. D’Haese, J, Christiaens, F, D’Haens, J, et al. Combined cesarean section and clipping of a ruptured cerebral aneurysm: a case report. J Neurosurg Anesthesiol 1997; 9: 341345.CrossRefGoogle ScholarPubMed
34. Korula, G, Farling, P. Anesthetic management for a combined cesarean section and posterior fossa craniectomy. J Neurosurg Anesthesiol 1998; 10: 3033.CrossRefGoogle ScholarPubMed
35. Ogawa, A, Nakamura, N, Yoshimoto, T, et al. Cerebral blood flow in moyamoya disease. Part 2: autoregulation and CO2 response. Acta Neurochir 1990; 105: 107111.CrossRefGoogle ScholarPubMed
36. Kuwabara, Y, Ichiya, Y, Sasaki, M, et al. Response to hypercapnia in moyamoya disease. Cerebrovascular response to hypercapnia in pediatric and adult patients with moyamoya disease. Stroke 1997; 28: 701707.CrossRefGoogle ScholarPubMed