(Stroke. 1999;30:2272-2276.)
© 1999 American Heart Association, Inc.
Original Contributions |
From the Department of Neurosurgery, Tohoku University School of Medicine (Y.Y., T.Y., R.S.), and the Neurosurgical Department, Stroke Center, Sendai National Hospital (Y.S.), Sendai, Japan.
| Abstract |
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MethodsThis study included 28 moyamoya patients with episodes of intracranial hemorrhage between 1976 and 1988. The mean follow-up period was 14.2 years. There were 4 males and 24 females, aged 7 to 69 years (mean 39.2 years). Cerebral angiography and CT scans were performed for all patients. Surgical treatment was performed in 19 patients (67.9%), and 10 patients (35.7%) underwent revascularization surgery. We observed the clinical course of all 28 patients. We also studied the relationship between the efficacy of surgical treatment and long-term outcome.
ResultsFive of the 28 patients (17.9%) died of the initial intracranial hemorrhage, and 2 patients died of other causes. Rebleeding occurred in 6 of the remaining 21 patients (28.6%). The interval to rebleeding ranged from 2 to 20 years (mean 7.3 years). Of these 6 patients, 4 died of rebleeding. Rebleeding was observed in 1 of 8 patients who underwent bypass surgery and in 5 of 13 patients who did not, which suggested that rebleeding was less likely to occur in patients who had undergone bypass surgery. However, there was no significant difference in rebleeding ratio or mortality between patients with and those without revascularization surgery (P>0.05).
ConclusionsIn this study, we compiled the results of meticulous follow-up conducted over the past 10 years for patients with hemorrhagic moyamoya disease. Because hemorrhagic moyamoya disease is known for its high rate of mortality at the time of rebleeding and often causes rebleeding long after the initial episode (as much as 20 years later), implementation of long-term preventive measures for rebleeding is necessary. This suggests that a long-term prospective study of a large number of patients with hemorrhagic moyamoya disease is required to determine whether bypass surgery prevents rebleeding of hemorrhagic moyamoya disease.
Key Words: cerebral revascularization intracerebral hemorrhage moyamoya disease
| Introduction |
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| Subjects and Methods |
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Types of Hemorrhagic Episodes
Types of hemorrhage in the above 28 patients included 7
intracerebral hemorrhages, 10
intraventricular hemorrhages, 6
intracerebral hemorrhages with
intraventricular hemorrhage, and 5
subarachnoid hemorrhages (Table 2
). Five of the 28 patients (17.9%) died
of hemorrhage at first admission (Table 3
).
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Surgical Treatment
Eighteen of the 28 hemorrhagic moyamoya patients were
transferred to 4 major facilities with neurosurgical departments, where
they underwent surgical treatment, including ventricular
drainage in 6, hematoma evacuation in 1, aneurysmal clipping in
3, and ventriculo-peritoneal shunting in 2 patients. Of these patients,
10 (35.7%) underwent revascularization surgery for
16 hemispheres: 7 patients with encephalo-duro-arterio-synangiosis
(EDAS), 1 hemisphere in 1 patient with encephalo-myo-synangiosis (EMS),
1 hemisphere in 1 patient with STA-MCA anastomosis, and 2 hemispheres
in 1 patient with STA-MCA anastomosis combined with EDAS (Table 2
). All the patients were followed up from 1976 to 1998, over a
period ranging from 10 to 22 years (mean 14.2 years). The 7 patients
who died at first admission were excluded.
| Results |
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Effect of Revascularization Surgery
Rebleeding occurred in 5 of 13 patients who underwent no
revascularization surgery (38.5%). Four of the
five died of rebleeding. Rebleeding occurred in 1 of 8 patients who
underwent revascularization surgery (12.5%); this
patient also died of rebleeding. The mortality rate attributed to
rebleeding did not differ between patients with and those without
bypass surgery. The rebleeding ratio was 12.5% for patients with
bypass surgery and lower than the 38.5% for those without bypass
surgery. However, there was no significant difference in rebleeding
ratio between patients with and without
revascularization surgery (P>0.05,
2 test) (Table 3
).
Long-Term Outcome
Of the 21 patients, 6 (28.6%) died of rebleeding and 2 died of
other causes. Fourteen patients (66.7%) attained long-term survival,
including 6 patients with revascularization surgery
and 8 without revascularization surgery (Figure 4
). The survival rates were 75.0%
and 61.5% for patients with and without
revascularization surgery, respectively. There was
no significant difference (P>0.05,
2 test) in survival rates between the 2
groups. The mortality rates were 25.0% and 38.5% for patients with
and without revascularization surgery,
respectively. In this case as well there was no significant difference
between the 2 groups (P>0.05,
2
test) (Table 3
).
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| Discussion |
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The incidence of moyamoya disease is generally <1 in 100 000 individuals, but in Japan it is reported to be much higher. However, even with the study results available in Japan, it is difficult to prove that bypass surgery has a preventive effect on rebleeding. Prospective studies of large numbers of patients may be necessary to achieve further advances in the field of moyamoya disease. Because therapeutic methods vary depending on medical institutions, the overall characteristics of moyamoya disease and effective therapeutic strategy are difficult to assess, particularly for hemorrhagic moyamoya patients. Because patients have undergone revascularization surgery with different methods, results of the assessment of the incidence of rebleeding may be unclear. Appropriate comparisons between groups of large numbers of patients with the same therapeutic method are thus important. Although the clinical course, surgical management, and long-term outcome of moyamoya disease are still unclear, despite its being considered a single entity for more than 30 years, we can summarize the present findings for our patients with hemorrhagic moyamoya disease as follows. (1) Peak age of first bleeding is during the third and fourth decades of life. (2) Rebleeding tends to be common within 7.3 years after the first bleeding, although some cases of rebleeding occur after a long period. In some cases, rebleeding occurred after 20 years. (3) There is still no clear evidence that revascularization surgery significantly prevents rebleeding in patients with hemorrhagic moyamoya disease, even in our study, with observation performed over a mean of 14.2 years. (4) A preventive effect of bypass surgery on rebleeding is expected. However, a long-term prospective study that targets a large number of patients is necessary, with evaluation of cerebral circulatory metabolism.
| Acknowledgments |
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| Footnotes |
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Received July 1, 1999; revision received August 23, 1999; accepted August 23, 1999.
| References |
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2. Karasawa J, Kikuchi H, Furuse S, Kawamura J, Sasaki T. Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg. 1978;49:679688.[Medline] [Order article via Infotrieve]
3. Karasawa J, Kikuchi H, Furuse S, Sasaki T, Yoshida Y, Ohnishi H, Taki W. A surgical treatment of moyamoya disease encephalo-myo-synangiosis. Neurol Med Chir (Tokyo). 1977;17(part I):2937.
4. Yoshida YK, Shirane R, Yoishimoto T. Non-anastomotic bypass surgery for childhood moyamoya disease using dural pedicle insertion over the brain surface combined with EGMS. Surg Neurol. 1999;51:404411.[Medline] [Order article via Infotrieve]
5. Mizoi K, Kayama T, Yoshimoto T, Nagamine Y. Indirect revascularization for moyamoya disease: is there a beneficial effect for adult patients? Surg Neurol. 1996;45:541549.[Medline] [Order article via Infotrieve]
6. 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;99:183186.
7. Aoki N. Cerebrovascular bypass surgery for the treatment of Moyamoya disease: unsatisfactory outcome in the patients presenting with intracranial hemorrhage. Surg Neurol. 1993;40:372377.[Medline] [Order article via Infotrieve]
8. Fujii K, Ikezaki K, Irikura K, Miyasaka Y, Fukui M. The efficacy of bypass surgery for the patients with hemorrhagic moyamoya disease. Clin Neurol Neurosurg. 1997;99:194195.
9.
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;27:13421346.
10. Saeki N, Nalazaki S, Kubota M, Yamaura A, Hoshi S, Sunada S, Sunami K. Hemorrhagic type moyamoya disease. Clin Neurol Neurosurg. 1997;99:196201.[Medline] [Order article via Infotrieve]
11.
Okada Y, Shima T, Nishida M, Yamane K, Yamada T,
Yamanaka C. Effectiveness of superficial temporal arterymiddle
cerebral artery anastomosis in adult moyamoya disease: cerebral
hemodynamics and clinical course in ischemic
and hemorrhagic varieties. Stroke. 1998;29:625630.
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