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18. MartinRF, Eldrup-JorgensenJ, Clark DE. Blunttraumato the carotidarteries, J VascSurg 1988; 8: 501-507. 19. YamauraI, SukenagaA, MatsumotoTet al. Traumatic occlusionof the extracranialinternalcarotidartery.Reportof two cases.NeurolMad Chit (Tokyo) 1989; 29: 1144-1147. 20. DavisJW, HilbrookT1, HoytDB et al. Bluntcarotid arterydissection: Incidence.Associatedinjuries,screeningand treatment.J Trauma 1990; 30: 1514-1517. 21. StringerWL, KellyD1. Traumaticdissectionof the extracranialinternalcarotid artery. Neurosurgery1980; 6: 123-130. 22. FitherSE. Carotidthrombosisdue to intra-oraltrauma. New Eng J Mad 1966; 274: 764-767. 23. HigginsGL Ill, MeredithJT. Internalcarotidarterythrmnbosisfollowing penetratingtraumaof the softpalate: an injuryof youth. J FaroPract 1991; 32: 319-322. 24. MorrowsKS, ClevengerFW. Oropharyngealimpalementon a wroughtiron fence. SouthMad J 1993; 86: 1306-1309. 25. VenezisP, ClaydonSM, ChapmanRC et al. Internalcarotidarterythrorabosis followingmanualstrangulation.Mad Sci Law 1993; 33:69-71. 26. WelhingRE, Saul TG, Tew JM et al. Managementof blunt injury to the internalcarotidartery. J Trauma 1987; 27: 1221-1226. 27. SanzoneAG, TorresH, DoundoulakisS. Blunttraumato the carotidarteries. Am J EmergMad 1995; 13: 327-330. 28. OkuderaH, Hara H, KuroyanagiTet al. CT findingsof acute deathfollowing severeheadinjury.Jpn J AcuteMad 1986; 10: 863-869. (Jpn) 29. HongoK, KobayashiS, OkuderaHet al. Noninvasivecerebraloptical spectroscopy:depthresolvedmeasurementof cerebralhaemodynamicsusing indocyaninegreen.NeurolRes 1995; 17: 89-93. 30. KastenbergWL. Reviewof indmal arterial injuries.Surgeryversus conservativemanagement,Ann Surg 1990; 56: 504-506. 31. MajeskyMW, SchwartzSM, ClowesAW.HeparinregulatessmoothmuscleS phase entryin the injuredrat carotid artery.CircRes 1987; 61: 296-300.

M o y a m o y a disease a s s o c i a t e d with basilar tip aneurysm Abesh Kumar Bhattacharjee MBBS, Norihiko Tamaki MD PHD, Hiroaki Minami MD, Kazumasa Ehara MD DMSC Department of Neurosurgery, Kobe University, School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan

Summary Direct surgical intervention to treat ruptured basilar tip aneurysms in patients with moyamoya disease has rarely been attempted, and patients who have undergone such treatment have not fully recovered. We review six cases of surgically treated ruptured basilar tip aneurysm associated with moyamoya disease, including our own case to illustrate aspects of surgical intervention and the difficulties encountered. Patients who underwent surgery after 4 weeks of the onset of symptoms showed impressive results. Of the patients who underwent surgery in the acute stage, two died, including our patient, and one showed excellent recovery. It is emphasized that to achieve satisfactory surgical outcome, the following factors should be considered: (i) delayed operation is preferable, with extracranial-intracranial bypass in selected patients; (ii) careful preservation of moyamoyas and transdural collaterals is mandatory; (iii) intraoperative rupture of the aneurysm should be avoided; and (iv) using a neureanaesthetic technique of induced hypothermia and hypertension may be preferable. Joumalof ClinicalNeuroacience(1999)6(3),268-271 © 1999HarcourtBrace& Co. Ltd

Keywerds: moyamoya disease, basilar tip aneurysm, rupture, surgical treatment.

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Received 20 February 1998 Accepted 17 March 1998 Correspondence to: N. Tamaki, Tel.: +81 78 382 5966 Fax: +81 78 382 5979

INTRODUCTION Moyamoya disease is a rare, occlusive cerebrovascular disease of undetermined aetiology that occurs more frequently among the Japanese and Chinese. 1-3 After the disease was first described by Takeuchi and Shimizu in 1957, 4 more than 1000 cases were reported mainly from Japan 5 and elsewhere.2 3 The association between moyamoya disease and intracranial aneurysms is well documented,5-a but direct surgical intervention to treat ruptured cerebral aneurysms in patients with moyamoya disease has rarely been attempted. 9 In addition, many patients who have undergone such treatment have not fully recovered? Surgical intervention through the conventional approaches seems to be difficult due to the extensive network of collaterals and basal moyamoyas. Here we present a case of ruptured basilar tip aneurysm in moyamoya disease and discuss aspects of surgical intervention in the context of selected reported cases.

CASE REPORT A 51-year-old woman was admitted to a local hospital in November 1993 due to the sudden onset of severe headache followed by vomiting. On admission, she was alert and well oriented (Hunt and Hess grade II). A computed tomography (CT) scan showed subarachnoid haemorrhage (SH) (Fisher grade lI), mainly in the bilateral Sylvian fissures and basal cisterns (Fig. 1A). Subsequent angiography revealed a large saccular aneurysm originating at the basilar bifurcation and pointing to the left (Fig. 2A). Bilateral internal carotid artery occlusion and the basal network of moyamoyas were evident (Figs 2 B and C). Bilateral ophthalmic and posterior communicating arteries along with collaterals became prominent following the diversion of blood flow to the brain parenchyma. After transferring to our service, the patient gradually became somnolent (Hunt and Hess grade III), and she underwent surgery l0 days after the onset of symptoms. The right fronto-orbitozygematic (FEZ) approach was chosen. The dura had rich anastomosis with brain parenchyma, therefore, the dural incision was made carefully to minimize damage. The base of the Sylvian fissure and carotid-optic cistern were opened. The right carotid artery, A1 segment of the right anterior cerebral artery, and M1 segment of the right middle cerebral artery were short, atrophic, whitish and collapsed, but the posterior communicating artery and its perforators were well developed. The arachnoid membrane and Liliequist's membrane were dissected out and the aneurysm was seen between the right internal carotid artery and the optic nerve. The aneurysm neck was clipped with a straight 15 m m Sugita clip and, unfortunately, at that moment the aneurysm ruptured. There was profuse bleeding, and systolic blood pressure decreased to 80 m m Hg. A temporary clip was applied to the basilar artery and, after the bleeding came under control, the neck of the aneurysm was clipped with 12 mm and 15 m m (booster) Sugita clips. Periosteum and fibrin glue were placed over the base of the aneurysm. The patient remained comatose (Glasgow Coma Scale 6) postoperatively. Postoperative angiography showed that the aneurysmal neck was clipped, and the posterior cerebral and superior cerebellar arteries were preserved. On the following day, a CT scan showed a large, diffuse, low-density area in the right cerebral hemisphere (Fig. 1B), and flat electroencephalography was recorded. Brain death was declared 11 days after the operation.

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Moyamoya and basilar aneurysm 269

Fig. 1. (A) CT scan demonstrating subarachnoid haemorrhage. (B) The postoperative CT scan showing a diffuse, low density area in the right cerebral hemisphere.

DISCUSSION A s s o c i a t i o n of a n e u r y s m with m o y a m o y a d i s e a s e Moyamoya disease is characterized by a progressive, bilateral narrowing of the internal carotid artery bifurcation, followed by the development of parenchymal collateral vessels known as moyamoya vessels. It has also been found that the frequency of the basal cerebral moyamoyas and transdural collateral vessels increase with the severity of the internal carotid artery (ICA) bifurcation steno-occlusive lesion? After a case of moyamoya disease associated with intracranial aneurysm was first reported by Pool et al. in 1967, m many cases of such lesions, including the present case, have been reported. Before 1978, not a single basilar artery aneurysm was found because vertebral angiography was not routinely performed. 3 In 1985 Waga and Tochio 7 compiled 76 cases of aneurysms associated with moyamoya diseases, of which 30 (39%) were peripheral aneurysms, and 16 (21%) were vertebrobasilar aneurysms. A large autopsy series showed that only two of 206 aneurysms were located at the basilar bifurcation, j~ The aneurysm in moyamoya disease has characteristic features and sites, and the pattern of occurrence and pathogenesis a r e gradually being clarified. ~2,13 Aneurysms are classified into two varieties according to location. One variety includes aneurysms developing on the moyamoya vessels or on the peripheral arteries contributing to the moyamoya vessels. These aneurysms are thought to be associated with intracerebral or intraventricular haemorrhageJ ,~ The other variety includes aneurysms originating from the circle of Willis, and occur with particularly high incidence on the basilar artery. 9,15

B

C Fig. 2. (A) Left lateral vertebral angiography demonstrating a large aneurysm originating at the bifurcation of the basilar artery. (B) Right lateral common carotid angiography showing obstruction at the carotid siphon and collateral blood flow via prominent ophthalmic artery and superficial temporal arteries are evident. The basal network of moyamoyasare visible. (C) The left internal carotid artery demonstrating occlusion just after giving off the posterior communicating artery (arrow); the rich basal network of moyamoyas are significant. The main trunks of anterior cerebral artery and middle cerebral artery are not visible. The posterior communicating artery is dilated due to diversionof blood flow to the posterior circulation. Collateral blood flow via the prominent ophthalmic artery is evident.

M e c h a n i s m of d e v e l o p m e n t of vertebrobasilar aneurysm Rosen et al. 16 and Adams et al. 17 suggested that vascular disease that produces the moyamoya phenomenon compromises the anterior circulation and thus alters the circulatory dynamics of the vertebrobasilar system. These circulatory changes induce the development of an aneurysm at the basilar artery bifurcation. This haemodynamic strain on the posterior circulation might also play an important role in the causation of subarachnoid haemorrhage from these aneurysms. 5 A number of peripheral aneurysms in moyamoya vessels may disappear, either spontaneously 5,18 or after cerebral revascularization procedures. 7'19 The spontaneous disappearance of a true saccular aneurysm in the circle of Willis is extremely rare? It is highly possible for an aneurysm of the circle of Willis to grow and rupture without spontaneous resolution. Therefore, a direct © 1999 Harcourt Brace & Co. Ltd

surgical approach is thought to be necessary in treating patients with associated subarachnoid haemorrhage. 2~,22

Surgical t r e a t m e n t of basilar tip a n e u r y s m a s s o c i a t e d moyamoya disease Including our case, direct surgical approaches have been reported in ruptured basilar tip aneurysm associated with moyamoya diseases in only six patients (Table 1). The patient ages ranged from 34-54 years with a mean age of 43.5 years (standard deviation _+ 8.2), which is slightly higher than that of typical adult patients with moyamoya disease. Of the six patients, three had multiple aneurysms and five had bilateral carotid occlusions. Three patients who underwent surgery 4 weeks after the onset of Journal of Clinical Neuroscience (1999) 6(3)

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Table 1 Surgical cases of ruptured basilar top aneurysm associated with moyamoya disease Case No.

Authors and year of publication

Age and sex

Carotid occlusion

Preoperative grading of SAH

Surgery after onset

Location of aneurysm

Procedure

Approach

Complications

1

Kodama et al. 1978

42/M

Bilateral

HH grade II

10 weeks

Occulomotor palsy

Adams et al. 1979

45/F

Bilateral

SAH grading

4 weeks

Subtemporal

None

3 4 5

Hanakita et al. 1988 Muizelaar 1988 Kodama et al. 1994

54/F 34/M 35/M

Unilateral Bilateral Bilateral

HH grade V HH grade II HH grade II

9 days On that day 4 weeks

Pterional Pterional Subtemporal

6

Bhattacharjee et al.

51/F

Bilateral

HH grade II

10 days

Both ligation and clipping Packing Clipping Clipping Clipping Clipping Clipping Clipping

Subtemporal

2

BA-SCA BA top BA top PCA BA top BA top BA top BA-SCA BA top

Died None Tr. Occulomotor palsy Died

Fez

BA - Basilar artery; SCA - Superior cerebellar artery; HH - Hunt and Hess; Fez - Fronto-orbito-zygomatic; Tr -transient; SAH - Subarachnoid haemorrhage.

symptoms had good results. On the contrary, the patients, including our case, who underwent surgery in the acute stage (within 2 weeks) showed bad outcome, except the case reported by Muizelaar 2 who underwent surgery within hours from the onset of subarachnoid haemorrhage. Although the number of patients is small, it is clear that the result of surgical intervention in the acute stage is disappointing. Nagai et al.22 have suggested that delayed operation is preferable because operative stress in the acute stage may increase morbidity. Adams et al) 7 have recommended extracranial-intracranial anastomosis prior to operative therapy of the aneurysm. CS Ogilvy et alY in 1996 reported their experiences with 132 consecutive aneurysmal clippings using i.v. mannitol administration and induced systemic hypertension and mild hypothermia prior to and during planned temporary vessel occlusion to avoid stroke during aneurysm surgery. Batjer and Samson 2¢ noted a three-fold increase in the risk of clinical stroke in patients in whom rupture had occurred, Induced hypertension during placement of the temporary clip is an attempt to maximize collateral flow and oxygen delivery to cerebral tissue, z3 Mild hypothermia (31-34°C) is thought to reduce the metabolic demands of cerebral tissue, thereby avoiding ischaemia when the energy demand of this tissue outstrips the supply of oxygen and glucose.23 The use of mannitol alone or in combination with other agents has been suggested to be useful in temporary vascular occlusion because it enhances cerebral blood flow by reducing blood viscosityY

Alternative treatment

Interventional neuroradiological procedures have recently produced encouraging short-term results in treating some aneurysmal lesions. Recent advances in neuroangiography have led to the capability of approaching these aneurysms from a different direction, the intravascular space.26 Embolization may be a better option for the basilar tip aneurysm with moyamoya disease in selected cases.

CONCLUSION

Careful preservation of the transdural anastomosis and basal moyamoya vessels should be maintained during surgery. Intraoperative haemorrhage during aneurysm surgery is considered to be highly associated with increased morbidity and mortality. 24 Surgical outcome is related to intraoperative rupture of aneurysm during surgery, since rupture results in excessive hypotension, and temporary clipping compromises cerebral blood flow. Intraoperative rupture could be prevented by delayed surgery and by using a neuroanaesthetic technique of induced hypertension and hypothermia and mannitol administration because

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patients with moyamoya disease are particularly susceptible to profound cerebral ischaemia resulting in extensive infarction. F e z craniotomy provides wide exposure of the operative field and facilitates removal of the blood clot following subarachnoid haemorrhage. In conclusion, it is emphasized that to obtain a good surgical outcome in patients with this difficult disease, the following factors should be considered, based on the previous reports and our experience: (i) delayed operation is preferable with or without the creation of an extracranial-intracranial bypass; (ii) careful preservation of moyamoyas and transdural collaterals during operation is mandatory; (iii) intraoperative rupture of the aneurysm should be avoided; and (iv) using the anaesthesia technique of induced hypothermia and hypertension may be preferable.

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