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Moyamoya disease associated with intracranial aneurysms
20
Surg Neurol 1983;20:20-4
Moyamoya Disease Associated with Intracranial Aneurysms Michio Yabumoto, M.D., Kazuyoshi Funahashi, M.D., Toru Fujii, M.D., Seiji Hayashi, M.D., and Norihiko Komai, M.D. Department of Neurological Surgery, Wakayama Medical College, Wakayama, Japan
Yabumoto M, Funahashi K, Fujii T, Hayashi S, Komai N. Moyamoya disease associated with intracranial aneurysms. Surg Neurol 1983;20:20-4.
A case of moyamoya disease associated with multiple intracranial aneurysms is reported. The aneurysms were located at the bifurcation of the basilar artery and at the precommunicating segment of the right posterior-cerebral artery. They were found to be unruptured and clipped successfully through a right frontotemporal craniotomy. Outcome of the operation was satisfactory. We propose the operative indication for these cases on the basis of operative and radiological findings, and also discuss characteristic aspects of this disease. KEY WORDS: Moyamoya disease; Intracranial aneurysm; Operative indication
Since a case of m o y a m o y a disease associated with intracranial aneurysm was first reported by Pool et al in 1967 [20], 56 cases of such lesions, including the present case, have been reported [3,5-7,11,13-15,18,19,22,24,25]. The association of aneurysm in moyamoya disease has characteristic features described later. Although direct operation for these aneurysms was performed successfully in seven cases, the operative indication and problems, especially the possibility of damaging the moyamoya vessels, were not fully discussed. We report a case of m o y a m o y a disease associated with intracranial aneurysms and propose the operative indication for these cases.
Case Report A 42-year-old woman experienced sudden onset of headache and vomiting on N o v e m b e r 5, 1980, followed by nuchal rigidity, left hemiparesis, left hemihypesthesia, left h o m o n y m o u s hemianopsia, and a stuporous state. A lumbar puncture revealed bloody cerebrospinal Address reprint requeJts to." Michio Yabumoto, M.D, Department of Neurological Surgery, Wakayama Medical College, 1-7 Bancho, Wakayama City, Wakayama, Japan.
© 1983 by Elsevier Science PublishingCo., Inc.
fluid (CSF). H e r neurological condition improved gradually at bedrest. The patient was admitted to us on January 8, 1981 to receive further neurological examination. On admission, she was alert and had same neurological findings at the onset. Laboratory and other physical findings were normal. C o m p u t e d tomography (CT) demonstrated two abnormal low-density areas in the right frontal and occipital lobes, the latter of which showed a ringlike, high-density area by contrast enhancement (Figure 1 ). These findings suggested an old intracerebral hemorrhage from abnormal vascular origin, such as arteriovenous malformation, aneurysm, and moyamoya disease. Right carotid angiography on January 22, 1981 revealed obstruction of the C-1 segment of the right internal carotid artery and the basal moyamoya (Figure 2). The loop-shaped, right posterior cerebral artery was supplied through the enlarged right posterior communicating artery. T w o aneurysmlike shadows were seen at the bifurcation of the basilar artery and at the precommunicating segment of the posterior cerebral artery. Left carotid angiography revealed obstruction of the A-1 segment of the anterior cerebral artery and the basal m o y a m o y a (Figure 3). Vertebral angiography on January 26, 1981 very clearly revealed two aneurysms (Figure 4). Since scant transdural cerebral anastomoses and m o y a m o y a vessels in the right subtemporal region and around the aneurysms were recognized angiographically, we performed a direct operation.
Operation We performed a direct operation on February 6, 1981 through a right frontotemporal craniotomy. A small amount of transdural cerebral anastomoses were noticed. Under the operating microscope, a few abnormal vascular networks, consisting of small arteries and veins, were recognized on the medial surface of the temporal lobe. The distal portion of the internal carotid artery was narrow, yellow-white, and terminated into the occluded A-1 and M-1 segments. The posterior communicating artery was very enlarged and elongated, and had two elongated perforating arteries supplying the basal 0900~30 l 9/83/S~.00
Moyamoya Disease and Aneurysms
Surg Neurol 1983;20:20-4
21
Figure 1. CT showing two abnormal low-density areas in the right frontal (A) and occipital lobe with ringlike enhancement (B)
moyamoya. The basilar top aneurysm was located behind the enlarged posterior communicating artery, so it had to be clipped with very careful and intermittent retraction of the artery. The aneurysm at the precommunicating segment of the right posterior cerebral artery was also clipped with minimal retraction of the temporal lobe because there were many abnormal vascular networks arising from the crural segment of the posterior cerebral artery. An especially careful maneuver on the vessels in this disease is essential in order Figure 2. The lateral view of the right carotid angiography showing obstruction of the C-1 segment of the internal carotid artery and the basal moyamoya. Two aneurysmlike shadows were also visible (arrow).
to lessen the damage of the fragile moyamoya vessels (Figure 5). Postoperative Course Postoperatively, she was somnolent with a right oculomotor palsy, left-medial, longitudinal fasciculus syndrome, and left hemiparesis. Three days after the opFigure 3. The lateral view of the left carotid angiography showing obstruction of the A-I segment of the anterior cerebral artery and the basal moyamoya. ........
22
Surg Neurol 1983;20:20-4
Figure 4. The anteroposterior view of the vertebral angiography showing two aneurysms located at the bifurcation of the basilar artery and at the precommunicating segment of the right posterior cerebral artery.
Yabumoto et al
eration, a convulsion occurred beginning at the angle of the left lip and extending to the left upper extremity including the left side of the face. On the seventh day after the operation, status epilepticus developed followed by stupor and increased left hemiparesis. Electroencephalogram demonstrated the epileptic focus in the right frontopolar lead. Administration of diazepam and phenytoin decreased the convulsion, and improved the hemiparesis and level of consciousness. Postoperative right carotid and vertebral angiography, performed on March 31, revealed occlusion of the right posterior communicating artery and disappearance of the basal moyamoya. Two aneurysms were successfully clipped (Figure 6). Left carotid angiography on April 7 demonstrated that middle meningeal artery developed and supplied the right frontopolar area. The patient improved gradually and was discharged on May 1 with minimal diplopia. She was last seen and examined on November 13, 1982. She was asymptomatic and working as a housewife. The postoperative symptoms and signs may depend on the obstruction of the posterior communicating artery or direct damage of the perforating arteries into the midbrain in spite of a very careful procedure during the operation. The postoperative obstruction of the right Figure 6. Vertebral angiography showing successful clipping of the aneurysm after surgery.
Figure 5. Schematic drawing of the surgical view: Narrowing of C-I segment of the internal carotid artery (ICC) and enlargement of posterior communicating artery (Pcom) are noted. Aneurysm (AN) at the bifurcation of the basilar artery (B__AA)is seen only by retracting the enlarged posterior communicating artery. Aneurysm at the precommunicating segment and many vascular networks arising from the posterior cerebral artery can be seen.
Surg Neurol 1983;20:20-4
Moyamoya Disease and Aneurysms
posterior communicating artery may indicate that vessels in moyamoya disease are susceptible to compression and other operative procedures. Discussion Over 1000 cases of moyamoya disease had been reported up to 1979 [ 17]. In those cases, intracranial aneurysms were found in 56 cases. The incidence of concomitant aneurysm is approximately 5 or 6% in all moyamoya disease, which is more frequent than that of aneurysm in the general population (0.5%-1.0%) reported by McCormick in 1976 [12]. The location of the aneurysms is shown in Table 1. Compared with the report on the Cooperative Study by Locksley [10], it is evident that aneurysms in the vertebrobasilar system have a much larger incidence in moyamoya disease. The unusual number of aneurysms arising from the intercavernous portion of the internal carotid artery [2], and the low incidence of aneurysm on the middle cerebral artery in addition to the high incidence in the vertebrobasilar system, may support the following speculation of aneurysm formation. The hemodynamics in the cerebral circulation depends on the posterior circulation due to the obstruction of the internal carotid artery. Therefore, aneurysms in moyamoya disease may be formed more easily in unusual locations. It is also suggested that hemodynamic stress can play an important role in the formation of aneurysm as exhibited in the cases of association of arteriovenous malformation and intracranial aneurysm [4]. Two types of aneurysms are recognized in moyamoya disease in angiographic follow-up. One is saccular aneurysms in the circle of Willis, which sometimes increased in size and never disappeared. Another is "pseudoaneurysm" (one of which is confirmed histologically [27]) in the basal moyamoya or peripheral vessels, some of which disappeared in follow-up angiography. The ratio of these two types ofaneurysm is approximately even in 61 aneurysms (30:31). Direct surgery was performed in eight cases, during which 12 aneurysms were found [1,8,16,23,26]. The results of the operation were all good, and there were no deaths after surgery. Clipping was performed in six
23
cases (seven aneurysms), and wrapping in four cases (5 aneurysms). In one case, it was impossible to perform direct surgery because of moyamoya vessels around the aneurysms [8]. Aneurysms in the present case have a high probability of rupture, because they were saccular and more than 1 cm in diameter [28]. Furthermore, they might receive much hemodynamic stress resulting from the moyamoya disease. Rich collaterals around the aneurysms will disturb clipping of the aneurysms, and rich transdural anastomoses in the frontotemporal region will decrease the blood supply to the cerebrum after craniotomy in this region. We predicted that there would be no special problems in the operation because of scant collaterals around the aneurysms and little transdural cerebral supply in the right frontotemporal regions angiographically. Since careful estimation of the blood supply must be performed on the preoperative angiography, magnification angiography should be utilized [21]. Even if estimation of the vessels before surgery and careful surgical procedures are carried out, there still exists a difficulty in clipping, and transient neurological deficits may appear after surgery. These deficits would mainly be due to alteration of the cerebral circulation caused by damage of moyamoya vessels or the posterior communicating artery. Judging from the present case and our review of the literature, we propose the surgical indications for aneurysms associated with moyamoya disease as follows: (a) saccular aneurysm; (b) scant transdural cerebral anastomoses; and (c) scant collaterals around the aneurysm. If the second condition is not satisfied, reconstruction of cerebral circulation such as superficial temporal art e r y - m i d d l e cerebral artery anastomosis should be performed on the aneurysms before the radical operation. If the third condition is not satisfied, radical operation should not be performed.
References 1. Adams H P Jr, Kassell NF, Wisoff HS, Drake CG. Intracranial saccular aneurysm and moyamoya disease. Stroke 1979;10:174-
9. 2. DebrunG, LacourP. A new caseofmoyamoyadiseaseassociated
Table 1. Distribution of the Aneurysms Associated with Moyamoya Disease Location
ACA
ICA
MCA
VB
BG
Unknown
Total
Case no.
5 (7.6) (33.5Y
13 (19.7) (41.2y
4 (6.1) (19.8y
28 (42.4) (5.3Y
11 (16.7)
5 (7.6)
66
Source: aLocksley, 1966.
ACA: anterior cerebral artery; ICA: internal carotid artery; MCA: middle cerebral artery; VB: vertebrobasilar system; BG: basal ganglia region.
24
Surg N e u r o l 1983;20:20-4
with several intracavernous aneurysm. Neuroradiology 1974;7:27782. 3, Galligioni F, Andrioli GC, Marin G, Briani S, lraci G. Hypoplasia of the internal carotid artery associated with cerebral pseudoangiomatosis. Report of 4 cases. AJR 1971;112:251-62. 4. Hayashi S, Arimoto T, ltakura T, Fujii T, Nishiguchi T, Komai N. The association of intracranial aneurysm and arteriovenous malformation of the brain. Report. J Neurosurg 1981 ;55:971-5. 5. Izawa M, Sento S. A case of cerebrovascular moyamoya disease associated with basilar artery aneurysm. No Shinkei Geka 1981;9:365-70. 6. Kamisasa A, Hiratsuka H, Inaba Y. Aneurysm arising from the abnormal vascular network at the base of the brain. Case report and etiological study. No To Shinkei 1972;24:463-8. 7. Kodama N, Mineura K, Suzuki J, Kitaoka T, Kurashima Y, Takahashi S. Cerebrovascular moyamoya disease associated with aneurysm at the peripheral portion of the posterior choroidal artery. No Shinkei Geka 1976;4:985-91. 8. Kodama N, Suzuki J. Moyamoya disease associated with aneurysm. J Neurosurg 1978;48:565-9. 9. Lee MLK, Cheyng EMT. Moyamoya disease as a cause of subarachnoid hemorrhage in Chinese. Brain 1973;96:623-8. 10. Locksley HB. Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Part I, Section 5: Natural history of subarachnoid hemorrhage, intracranial aneurysms, and arteriovenous malformations based on 6368 cases in the cooperative study. J Neurosurg 1966;25:219-39. 11. Mauro AJ,Johnson ES, Chikos PM, Alvord EC,Jr. Lipohyalinosis and miliary microaneurysms causing cerebral hemorrhage in a patient with moyamoya. A clinicopathological study. Stroke 1980;11:405-12. 12. McCormick WF, Nofzinger JD. Saccular intrecranial aneurysms. An autopsy study. J Neurosurg 1965;25:155-9. 13. Momma F, Kowada M, Kikuchi K, Kugo U. Cerebrovascular moyamoya associated with basilar artery aneurysm. Report of a case. Rinsho Hoshasen 1979;24:285-8. 14. Muraki M, Kaneko M, Yamamoto T, Iwamoto K, Uemura K, Nakajima S. A clinical study of 9 cases of adult type of moyamoya disease associated with intracerebral hematoma. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. GummaITokyo:Neuron-Sha, 1981;64-70. 15. Nakao S, Fukumitsu T, Ogata M, Nishimura H. Intracranial hemorrhage associated with moyamoya disease--intracerebral hematoma and intraventricular hemorrhage. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. Gumma/Tokyo:Neuron-Sha, 1981 ;64-70. 16. Nagamine Y, Sonobe M, Takahashi S. Multiple aneurysms as-
Y a b u m o t o et al
sociated with moyamoya disease. No Shinkei Geka 1980;8:27582. 17. Nishimoto A. Moyamoya disease. Neurol Med Chir (Tokyo) 1979;19:221-8. 18. Ohkuma A, Ohshita H, Funakoshi T. A case of aneurysm in the cerebral-moyamoya vessel--Aneurysmal rupture during cerebral angiography and spontaneous regression of the aneurysm. No Shinkei Geka 1980;8:181-5. 19. Ohno K, Fujimoto T, Komatsu K, Hiratsuka H, Inaba U, Kamisasa H, Shibata T. Abnormal intracranial vascular network containing an aneurysm--especially its morphological findings by operation microscope. No To Shinkei 1976;28:353-64. 20. PoolJL, Wood EH, Maki Y. On the cases with abnormal vascular networks in the cerebral basal region in the United States. In Kudo T, ed. A disease with abnormal intracranial vascular networks--Spontaneous occlusion of the circle of Willis. Tokyo:Igaku-Shoin, 1967;63-8. 21. Takahashi M. Magnification angiography of cerebral aneurysms associated with moyamoya disease. AJN 1980;1:547-50. 22. Takeyama E, Matsumori K, Sugimori T, Kagawa M, Fukuyama S. A case of the anterior choroidal artery aneurysm combined with the abnormal intracranial vascular network. No Shinkei Geka 1976;4:1075-80. 23. Tanaka Y, Takeuchi K. Ogashiwa M, Akai K, Omata K. Moyamoya phenomenon combined with cerebral aneurysm. No To Shinkei 1978;30:687-95. 24. Tani S, Kawamura Y, Yamanouchi Y, Kurimoto T, Matsumura H. Computed tomographic follow-up study of a posterior choroidal artery aneurysm associated with moyamoya disease. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. Gumma/Tokyo:Neuron-Sha, 1981 ;99104. 25. Yamada F, Fukuda S, Kawamoto S, Kudo T. Spontaneous occlusion of the circle of Willis--Magnification angiography study of miliary aneurysms, the cause of hemorrhage and its incidence in juvenile and adult groups. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. Gumma/ Tokyo:Neuron-Sha, 1981 ;94-8. 26. Yasargil MG, Smith RD. Association of middle cerebral artery anomalies with saccular aneurysms and moyamoya disease. Surg Neurol 1976;6:39-43. 27. Yuasa H, Tokito S, Izumi K, Kirabayashi K. Cerebrovascular moyamoya disease associated with an intracranial pseudoaneurysm. J Neurosurg 56:131-4. 28. Wiebers DO, Whisnant JP, O'Fallon WM. The natural history of unruptured intracranial aneurysms. N Engl J Med 1981 ;304:6968.
Surg Neurol 1983;20:20-4
Moyamoya Disease Associated with Intracranial Aneurysms Michio Yabumoto, M.D., Kazuyoshi Funahashi, M.D., Toru Fujii, M.D., Seiji Hayashi, M.D., and Norihiko Komai, M.D. Department of Neurological Surgery, Wakayama Medical College, Wakayama, Japan
Yabumoto M, Funahashi K, Fujii T, Hayashi S, Komai N. Moyamoya disease associated with intracranial aneurysms. Surg Neurol 1983;20:20-4.
A case of moyamoya disease associated with multiple intracranial aneurysms is reported. The aneurysms were located at the bifurcation of the basilar artery and at the precommunicating segment of the right posterior-cerebral artery. They were found to be unruptured and clipped successfully through a right frontotemporal craniotomy. Outcome of the operation was satisfactory. We propose the operative indication for these cases on the basis of operative and radiological findings, and also discuss characteristic aspects of this disease. KEY WORDS: Moyamoya disease; Intracranial aneurysm; Operative indication
Since a case of m o y a m o y a disease associated with intracranial aneurysm was first reported by Pool et al in 1967 [20], 56 cases of such lesions, including the present case, have been reported [3,5-7,11,13-15,18,19,22,24,25]. The association of aneurysm in moyamoya disease has characteristic features described later. Although direct operation for these aneurysms was performed successfully in seven cases, the operative indication and problems, especially the possibility of damaging the moyamoya vessels, were not fully discussed. We report a case of m o y a m o y a disease associated with intracranial aneurysms and propose the operative indication for these cases.
Case Report A 42-year-old woman experienced sudden onset of headache and vomiting on N o v e m b e r 5, 1980, followed by nuchal rigidity, left hemiparesis, left hemihypesthesia, left h o m o n y m o u s hemianopsia, and a stuporous state. A lumbar puncture revealed bloody cerebrospinal Address reprint requeJts to." Michio Yabumoto, M.D, Department of Neurological Surgery, Wakayama Medical College, 1-7 Bancho, Wakayama City, Wakayama, Japan.
© 1983 by Elsevier Science PublishingCo., Inc.
fluid (CSF). H e r neurological condition improved gradually at bedrest. The patient was admitted to us on January 8, 1981 to receive further neurological examination. On admission, she was alert and had same neurological findings at the onset. Laboratory and other physical findings were normal. C o m p u t e d tomography (CT) demonstrated two abnormal low-density areas in the right frontal and occipital lobes, the latter of which showed a ringlike, high-density area by contrast enhancement (Figure 1 ). These findings suggested an old intracerebral hemorrhage from abnormal vascular origin, such as arteriovenous malformation, aneurysm, and moyamoya disease. Right carotid angiography on January 22, 1981 revealed obstruction of the C-1 segment of the right internal carotid artery and the basal moyamoya (Figure 2). The loop-shaped, right posterior cerebral artery was supplied through the enlarged right posterior communicating artery. T w o aneurysmlike shadows were seen at the bifurcation of the basilar artery and at the precommunicating segment of the posterior cerebral artery. Left carotid angiography revealed obstruction of the A-1 segment of the anterior cerebral artery and the basal m o y a m o y a (Figure 3). Vertebral angiography on January 26, 1981 very clearly revealed two aneurysms (Figure 4). Since scant transdural cerebral anastomoses and m o y a m o y a vessels in the right subtemporal region and around the aneurysms were recognized angiographically, we performed a direct operation.
Operation We performed a direct operation on February 6, 1981 through a right frontotemporal craniotomy. A small amount of transdural cerebral anastomoses were noticed. Under the operating microscope, a few abnormal vascular networks, consisting of small arteries and veins, were recognized on the medial surface of the temporal lobe. The distal portion of the internal carotid artery was narrow, yellow-white, and terminated into the occluded A-1 and M-1 segments. The posterior communicating artery was very enlarged and elongated, and had two elongated perforating arteries supplying the basal 0900~30 l 9/83/S~.00
Moyamoya Disease and Aneurysms
Surg Neurol 1983;20:20-4
21
Figure 1. CT showing two abnormal low-density areas in the right frontal (A) and occipital lobe with ringlike enhancement (B)
moyamoya. The basilar top aneurysm was located behind the enlarged posterior communicating artery, so it had to be clipped with very careful and intermittent retraction of the artery. The aneurysm at the precommunicating segment of the right posterior cerebral artery was also clipped with minimal retraction of the temporal lobe because there were many abnormal vascular networks arising from the crural segment of the posterior cerebral artery. An especially careful maneuver on the vessels in this disease is essential in order Figure 2. The lateral view of the right carotid angiography showing obstruction of the C-1 segment of the internal carotid artery and the basal moyamoya. Two aneurysmlike shadows were also visible (arrow).
to lessen the damage of the fragile moyamoya vessels (Figure 5). Postoperative Course Postoperatively, she was somnolent with a right oculomotor palsy, left-medial, longitudinal fasciculus syndrome, and left hemiparesis. Three days after the opFigure 3. The lateral view of the left carotid angiography showing obstruction of the A-I segment of the anterior cerebral artery and the basal moyamoya. ........
22
Surg Neurol 1983;20:20-4
Figure 4. The anteroposterior view of the vertebral angiography showing two aneurysms located at the bifurcation of the basilar artery and at the precommunicating segment of the right posterior cerebral artery.
Yabumoto et al
eration, a convulsion occurred beginning at the angle of the left lip and extending to the left upper extremity including the left side of the face. On the seventh day after the operation, status epilepticus developed followed by stupor and increased left hemiparesis. Electroencephalogram demonstrated the epileptic focus in the right frontopolar lead. Administration of diazepam and phenytoin decreased the convulsion, and improved the hemiparesis and level of consciousness. Postoperative right carotid and vertebral angiography, performed on March 31, revealed occlusion of the right posterior communicating artery and disappearance of the basal moyamoya. Two aneurysms were successfully clipped (Figure 6). Left carotid angiography on April 7 demonstrated that middle meningeal artery developed and supplied the right frontopolar area. The patient improved gradually and was discharged on May 1 with minimal diplopia. She was last seen and examined on November 13, 1982. She was asymptomatic and working as a housewife. The postoperative symptoms and signs may depend on the obstruction of the posterior communicating artery or direct damage of the perforating arteries into the midbrain in spite of a very careful procedure during the operation. The postoperative obstruction of the right Figure 6. Vertebral angiography showing successful clipping of the aneurysm after surgery.
Figure 5. Schematic drawing of the surgical view: Narrowing of C-I segment of the internal carotid artery (ICC) and enlargement of posterior communicating artery (Pcom) are noted. Aneurysm (AN) at the bifurcation of the basilar artery (B__AA)is seen only by retracting the enlarged posterior communicating artery. Aneurysm at the precommunicating segment and many vascular networks arising from the posterior cerebral artery can be seen.
Surg Neurol 1983;20:20-4
Moyamoya Disease and Aneurysms
posterior communicating artery may indicate that vessels in moyamoya disease are susceptible to compression and other operative procedures. Discussion Over 1000 cases of moyamoya disease had been reported up to 1979 [ 17]. In those cases, intracranial aneurysms were found in 56 cases. The incidence of concomitant aneurysm is approximately 5 or 6% in all moyamoya disease, which is more frequent than that of aneurysm in the general population (0.5%-1.0%) reported by McCormick in 1976 [12]. The location of the aneurysms is shown in Table 1. Compared with the report on the Cooperative Study by Locksley [10], it is evident that aneurysms in the vertebrobasilar system have a much larger incidence in moyamoya disease. The unusual number of aneurysms arising from the intercavernous portion of the internal carotid artery [2], and the low incidence of aneurysm on the middle cerebral artery in addition to the high incidence in the vertebrobasilar system, may support the following speculation of aneurysm formation. The hemodynamics in the cerebral circulation depends on the posterior circulation due to the obstruction of the internal carotid artery. Therefore, aneurysms in moyamoya disease may be formed more easily in unusual locations. It is also suggested that hemodynamic stress can play an important role in the formation of aneurysm as exhibited in the cases of association of arteriovenous malformation and intracranial aneurysm [4]. Two types of aneurysms are recognized in moyamoya disease in angiographic follow-up. One is saccular aneurysms in the circle of Willis, which sometimes increased in size and never disappeared. Another is "pseudoaneurysm" (one of which is confirmed histologically [27]) in the basal moyamoya or peripheral vessels, some of which disappeared in follow-up angiography. The ratio of these two types ofaneurysm is approximately even in 61 aneurysms (30:31). Direct surgery was performed in eight cases, during which 12 aneurysms were found [1,8,16,23,26]. The results of the operation were all good, and there were no deaths after surgery. Clipping was performed in six
23
cases (seven aneurysms), and wrapping in four cases (5 aneurysms). In one case, it was impossible to perform direct surgery because of moyamoya vessels around the aneurysms [8]. Aneurysms in the present case have a high probability of rupture, because they were saccular and more than 1 cm in diameter [28]. Furthermore, they might receive much hemodynamic stress resulting from the moyamoya disease. Rich collaterals around the aneurysms will disturb clipping of the aneurysms, and rich transdural anastomoses in the frontotemporal region will decrease the blood supply to the cerebrum after craniotomy in this region. We predicted that there would be no special problems in the operation because of scant collaterals around the aneurysms and little transdural cerebral supply in the right frontotemporal regions angiographically. Since careful estimation of the blood supply must be performed on the preoperative angiography, magnification angiography should be utilized [21]. Even if estimation of the vessels before surgery and careful surgical procedures are carried out, there still exists a difficulty in clipping, and transient neurological deficits may appear after surgery. These deficits would mainly be due to alteration of the cerebral circulation caused by damage of moyamoya vessels or the posterior communicating artery. Judging from the present case and our review of the literature, we propose the surgical indications for aneurysms associated with moyamoya disease as follows: (a) saccular aneurysm; (b) scant transdural cerebral anastomoses; and (c) scant collaterals around the aneurysm. If the second condition is not satisfied, reconstruction of cerebral circulation such as superficial temporal art e r y - m i d d l e cerebral artery anastomosis should be performed on the aneurysms before the radical operation. If the third condition is not satisfied, radical operation should not be performed.
References 1. Adams H P Jr, Kassell NF, Wisoff HS, Drake CG. Intracranial saccular aneurysm and moyamoya disease. Stroke 1979;10:174-
9. 2. DebrunG, LacourP. A new caseofmoyamoyadiseaseassociated
Table 1. Distribution of the Aneurysms Associated with Moyamoya Disease Location
ACA
ICA
MCA
VB
BG
Unknown
Total
Case no.
5 (7.6) (33.5Y
13 (19.7) (41.2y
4 (6.1) (19.8y
28 (42.4) (5.3Y
11 (16.7)
5 (7.6)
66
Source: aLocksley, 1966.
ACA: anterior cerebral artery; ICA: internal carotid artery; MCA: middle cerebral artery; VB: vertebrobasilar system; BG: basal ganglia region.
24
Surg N e u r o l 1983;20:20-4
with several intracavernous aneurysm. Neuroradiology 1974;7:27782. 3, Galligioni F, Andrioli GC, Marin G, Briani S, lraci G. Hypoplasia of the internal carotid artery associated with cerebral pseudoangiomatosis. Report of 4 cases. AJR 1971;112:251-62. 4. Hayashi S, Arimoto T, ltakura T, Fujii T, Nishiguchi T, Komai N. The association of intracranial aneurysm and arteriovenous malformation of the brain. Report. J Neurosurg 1981 ;55:971-5. 5. Izawa M, Sento S. A case of cerebrovascular moyamoya disease associated with basilar artery aneurysm. No Shinkei Geka 1981;9:365-70. 6. Kamisasa A, Hiratsuka H, Inaba Y. Aneurysm arising from the abnormal vascular network at the base of the brain. Case report and etiological study. No To Shinkei 1972;24:463-8. 7. Kodama N, Mineura K, Suzuki J, Kitaoka T, Kurashima Y, Takahashi S. Cerebrovascular moyamoya disease associated with aneurysm at the peripheral portion of the posterior choroidal artery. No Shinkei Geka 1976;4:985-91. 8. Kodama N, Suzuki J. Moyamoya disease associated with aneurysm. J Neurosurg 1978;48:565-9. 9. Lee MLK, Cheyng EMT. Moyamoya disease as a cause of subarachnoid hemorrhage in Chinese. Brain 1973;96:623-8. 10. Locksley HB. Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Part I, Section 5: Natural history of subarachnoid hemorrhage, intracranial aneurysms, and arteriovenous malformations based on 6368 cases in the cooperative study. J Neurosurg 1966;25:219-39. 11. Mauro AJ,Johnson ES, Chikos PM, Alvord EC,Jr. Lipohyalinosis and miliary microaneurysms causing cerebral hemorrhage in a patient with moyamoya. A clinicopathological study. Stroke 1980;11:405-12. 12. McCormick WF, Nofzinger JD. Saccular intrecranial aneurysms. An autopsy study. J Neurosurg 1965;25:155-9. 13. Momma F, Kowada M, Kikuchi K, Kugo U. Cerebrovascular moyamoya associated with basilar artery aneurysm. Report of a case. Rinsho Hoshasen 1979;24:285-8. 14. Muraki M, Kaneko M, Yamamoto T, Iwamoto K, Uemura K, Nakajima S. A clinical study of 9 cases of adult type of moyamoya disease associated with intracerebral hematoma. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. GummaITokyo:Neuron-Sha, 1981;64-70. 15. Nakao S, Fukumitsu T, Ogata M, Nishimura H. Intracranial hemorrhage associated with moyamoya disease--intracerebral hematoma and intraventricular hemorrhage. In Kawafuchi J, ed. Proceedings of the 10th Japanese conference on surgery of cerebral stroke. Gumma/Tokyo:Neuron-Sha, 1981 ;64-70. 16. Nagamine Y, Sonobe M, Takahashi S. Multiple aneurysms as-
Y a b u m o t o et al
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