J Cerebrovasc Endovasc Neurosurg.  2013 Sep;15(3):191-199. 10.7461/jcen.2013.15.3.191.

Thirteen-year Experience of 44 Patients with Adult Hemorrhagic Moyamoya Disease from a Single Institution: Clinical Analysis by Management Modality

• Choi WS
• Lee SB
• Kim DS
• Huh PW
• Yoo DS
• Lee TG
• Cho KS

• KMID: 1491438
• DOI: http://doi.org/10.7461/jcen.2013.15.3.191

Abstract

Our objective was a retrospective assessment of the management modalities that provided the most beneficial treatment in hemorrhagic moyamoya disease during the last 13 years at our institution. The clinical results of 44 patients with hemorrhagic moyamoya disease were investigated, comparing revascularization surgery (direct, indirect, and combined bypass) or conservative treatment. Angiographic features, rebleeding, and clinical outcome were investigated. Six of the 35 patients (17.1%) with revascularization surgery experienced rebleeding, as did 4 of 9 patients (44.4%) with conservative treatment. However, patients who underwent bypass surgery had a lower chance of rebleeding. No significant difference in chance of rebleeding was observed between bypass surgery and non surgery groups (p > 0.05). Cerebral angiography performed after bypass surgery showed that for achieving good postoperative revascularization, direct and combined bypass methods were much more effective (p < 0.05). While the risk of rebleeding in the revascularization group was generally lower than in the conservative treatment group, there was no statistically significant difference between treatment modalities and conservative treatment. Although statistical significance was not attained, direct and combined bypass may reduce the risk of hemorrhage more effectively than indirect bypass.

Keyword

Hemorrhagic moyamoya disease; Revascularization surgery; Intracranial hemorrhage; Surgical results

MeSH Terms

Adult
Cerebral Angiography
Hemorrhage
Humans
Intracranial Hemorrhages
Moyamoya Disease
Retrospective Studies

Figure

• 

  Fig. 1 Kaplan-Meier plots of stroke-free time demonstrates that patients with revascularization surgery had longer stroke-free times than those with conservative treatment, but there was no statistical difference (p = 0.09).

Reference

1. Asfora WT, West M, McClarty B. Angiography of encephalomyosynangiosis and superficial temporal artery to middle cerebral artery anastomosis in moyamoya disease. AJNR Am J Neuroradiol. 1993; Jan-Feb. 14(1):29–30. PMID: 8427106.

2. Fukui M. Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis ('Moyamoya' disease). Clin Neurol Neurosurg. 1997; 10. 99(Suppl 2):S238–S240. PMID: 9409446.

3. Golby AJ, Marks MP, Thompson RC, Steinberg GK. Direct and combined revascularization in pediatric moyamoya disease. Neurosurgery. 1999; 7. 45(1):50–58. discussion 58-60. PMID: 10414566.

4. Han DH, Nam DH, Oh CW. Moyamoya disease in adults: Characteristics of clinical presentation and outcome after encephalo-duro-arterio-synangiosis. Clin Neurol Neurosurg. 1997; 10. 99(Suppl 2):S151–S155. PMID: 9409427.

5. Horowitz M, Yonas H, Albright AL. Evaluation of cerebral blood flow and hemodynamic reserve in symptomatic moyamoya disease using stable xenon-CT blood flow. Surg Neurol. 1995; 9. 44(3):251–261. discussion 262. PMID: 8545777.

6. Houkin K, Kamiyama H, Abe H, Takahashi A, Kuroda S. Surgical therapy for adult moyamoya disease. Can surgical revascularization prevent the recurrence of intracranial hemorrhage? Stroke. 1996; 8. 27(8):1342–1346. PMID: 8711799.

7. Ikezaki K, Fukui M, Inamura T, Kinukawa N, Wakai K, Ono Y. The current status of the treatment for hemorrhagic type moyamoya disease based on a 1995 nationwide survey in Japan. Clin Neurol Neurosurg. 1997; 10. 99(Suppl 2):S183–S186. PMID: 9409433.

8. Kim H, Kim YW, Joo WI, Park HK, Joo JK, Lee KJ, et al. [Effect of direct bypass on the prevention of hemorrhage in patients with hemorrhagic type of moyamoya disease]. Korean J Cerebrovasc Surg. 2007; 3. 9(1):14–19. Korean.

9. Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H. Cerebral revascularization using omental transplantation for childhood moyamoya disease. J Neurosurg. 1993; 8. 79(2):192–196. PMID: 8331399.

10. Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H. Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg. 1992; 7. 77(1):84–89. PMID: 1607976.

11. Kashiwagi S, Yamashita T, Katoh S, Kitahara T, Nakashima K, Yasuhara S, et al. Regression of moyamoya vessels and hemodynamic changes after successful revascularization in childhood moyamoya disease. Acta Neurol Scand Suppl. 1996; 166:85–88. PMID: 8686450.

12. Kim DS, Yoo DS, Huh PW, Kim JK, Cho KS, Kang JK. Recent surgical treatment of moyamoya disease. J Korean Neurosurg Soc. 2001; 6. 30:800–804.

13. Kawaguchi T, Fujita S, Hosoda K, Shose Y, Hamano S, Iwakura M, et al. Multiple burr-hole operation for adult moyamoya disease. J Neurosurg. 1996; 3. 84(3):468–476. PMID: 8609560.

14. Kobayashi E, Saeki N, Oishi H, Hirai S, Yamaura A. Long-term natural history of hemorrhagic moyamoya disease in 42 patients. J Neurosurg. 2000; 12. 93(6):976–980. PMID: 11117870.

15. Matsushima Y, Fukai N, Tanaka K, Tsuruoka S, Inaba Y, Aoyagi M, et al. A new surgical treatment of moyamoya disease in children: A preliminary report. Surg Neurol. 1981; 4. 15(4):313–320. PMID: 7245020.

16. Matsushima Y, Inaba Y. Moyamoya disease in children and its surgical treatment. Introduction of a new surgical procedure and its follow-up angiograms. Childs Brain. 1984; 11(3):155–170. PMID: 6723433.

17. Morimoto M, Iwama T, Hashimoto N, Kojima A, Hayashida K. Efficacy of direct revascularization in adult moyamoya disease: Haemodynamic evaluation by positron emission tomography. Acta Neurochir (Wien). 1999; 141(4):377–384. PMID: 10352747.

18. Nakagawa Y, Abe H, Sawamura Y, Kamiyama H, Gotoh S, Kashiwaba T. Revascularization surgery for moyamoya disease. Neurol Res. 1988; 3. 10(1):32–39. PMID: 2899853.

19. Okada Y, Shima T, Nishida M, Yamane K, Yamada T, Yamanaka C. Effectiveness of superficial temporal artery-middle cerebral artery anastomosis in adult moyamoya disease: Cerebral hemodynamics and clinical course in ischemic and hemorrhagic varieties. Stroke. 1998; 3. 29(3):625–630. PMID: 9506603.

20. Rankin J. Cerebral vascular accidents in patients over the age of 60. II. Prognosis. Scott Med J. 1957; 5. 2(5):200–215. PMID: 13432835.

21. Saeki N, Nakazaki S, Kubota M, Yamaura A, Hoshi S, Sunada S, et al. Hemorrhagic type moyamoya disease. Clin Neurol Neurosurg. 1997; 10. 99(Suppl 2):S196–S201. PMID: 9409437.

22. Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 2004; 2. 100(2 Suppl Pediatrics):142–149. PMID: 14758941.

23. Suzuki J, Kodama N. Moyamoya disease- A review. Stroke. 1983; Jan-Feb. 14(1):104–109. PMID: 6823678.

24. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969; 3. 20(3):288–299. PMID: 5775283.

25. Ueki K, Meyer FB, Mellinger JF. Moyamoya disease: The disorder and surgical treatment. Mayo Clin Proc. 1994; 8. 69(8):749–757. PMID: 8035631.

26. Wanifuchi H, Takeshita M, Izawa M, Aoki N, Kagawa M. Management of adult moyamoya disease. Neurol Med Chir (Tokyo). 1993; 5. 33(5):300–305. PMID: 7687036.

27. Yoshida Y, Yoshimoto T, Shirane R, Sakurai Y. Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: An extensive follow-up study. Stroke. 1999; 11. 30(11):2272–2276. PMID: 10548656.