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Surg Neurol 1992;37:46-50

Atypical Moyamoya Disease Associated with Brain Tumor Noritaka Aihara, M.D., Hajime Nagai, M.D., Mitsuhito Mase, M.D., Hideki Kanai, M.D., Shigeo Wakabayashi, M.D., and Hideo Mabe, M.D. Department of Neurosurgery, Nagoya City University Medical School, Nagoya, Japan

Aihara N, Nagai H, Mase M, Kanai H, Wakabayashi S, Mabe H. Atypical Moyamoya disease associated with brain tumor. Surg Neurol 1992;37:46-50.

A 4-year-old boy with right retinal hemorrhage, mental retardation, and multiple minor anomalies was referred to our hospital. Computed tomography scanning revealed a cystic brain tumor at the vermis. Angiography showed stenosis of both internal carotid arteries at the supraclinoid portion and the Moyamoya vessels. The right ophthalmic artery was dilated as wide as the internal carotid artery. Stenosis of the basilar artery was also observed. Collateral circulation via the posterior inferior cerebellar artery and Moyamoya vessels in the area of the posterior cerebral artery was observed. KEY WORDS: Moyamoya disease; Brain tumor; Retinal hemorrhage; Ophthalmic artery; Basilar artery

Moyamoya disease is a kind of occlusive arteriopathy. Whether the pathogenesis is congenital or acquired is unknown. Suzuki and Kodama [13] suggested in their reviews that Moyamoya is an acquired disease in which both an immunological vascular reaction and subsequent inflammation play an important role. On the other hand, a connection between congenital diseases and familial cases has been reported [2,4,7,8,17]. These findings suggest that Moyamoya disease may be congenital. These congenital diseases are known to be sometimes associated with brain tumor and/or anomaly. Angiographic findings of Moyamoya disease are stenosis of the internal carotid arteries (ICAs) at the supraclinoid portion and Moyamoya vessels in the region of the basal ganglia. Dilatation of the ophthalmic artery and stenosis of the basilar artery are not common. The clinical symptoms of Moyamoya disease in childhood usually arise from brain ischemia. Intracranial Address reprint requests to: Noritaka Aihara, Department of Neurosurgery, Nagoya City University Medical School, 1 Kawasumi, Mizuho-Cho, Mizuho-Ku, Nagoya 467, Japan. Received November 26, 1990; accepted May 24, 1991.

(c~ 1992 by Elsevier Science Publishing Co., Inc.

hemorrhage occurs in adults. Retinal hemorrhage has not been reported in any case of Moyamoya disease. We report a case of Moyamoya disease with associated brain tumor, retinal hemorrhage, and multiple anomalies. Case Report A 4-year-old boy complaining of ataxic gait was admitted to our department in July 1989. H e was born after a normal pregnancy and delivery on January 7, 1984. His Apgar score was completed at 5 minutes. However, he had respiratory distress syndrome 6 hours after birth and he was cared for in the neonatal intensive care unit. His first retinal hemorrhage occurred on the right side when he was 2 months old, with repeated retinal hemorrhage occurring. In addition, he had multiple minor anomalies: bilateral syndactyly of his toes, high arched palate, and bilateral simian crease. Neurological examination on admission revealed truncal ataxia, mental retardation, and right visual loss caused by retinal hemorrhage. H e had no clinical symptoms of increased intracranial pressure (ICP), such as headache or vomiting. Computed tomography scanning showed posthemorrhagic changes in the right orbit and a cystic lesion in the cerebellar vermis (Figure 1). N o hydrocephalus was seen. Truncal ataxia was attributed to this cystic lesion. Magnetic resonance imaging showed a mass lesion in the cerebellar vermis, bilateral dilated ophthalmic arteries in the orbits, and multiple, small, low-intensity areas in the basal ganglia bilaterally (Figure 2). Stenosis of both ICAs at the supraclinoid portion, dilatation of both ophthalmic arteries, especially on the right side, and dilated ethmoidal arteries and Moyamoya vessels were seen in the carotid angiogram (Figures 3 and 4). The vertebral angiogram showed severe stenosis of the basilar artery before the ramification, and collateral circulation via the posterior inferior cerebellar artery (PICA) and Moyamoya vessels in the area of the posterior cerebral artery (PCA) (Figure 5). An operation was performed on the mass lesion in the vermis. Pathological diagnosis was pilocytic astrocytoma (Figure 6). 0090 a,019/92/S ~,.50

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Surg Neurol 1992;37:46-50

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A

Figure 1. C T scan shows posthemorrhagic change at the right orbit (A) and a cystic mass lesion enhanced with contrast medium in the cerebellar vermis (B).

Discussion Several cases of associated occlusive arteriopathy and brain tumor have been reported [1,10,12]. Mori et al [10] have speculated on possible causes: compression of the carotid bifurcation by a slowly growing basal tumor; secondary arterial changes caused by radiation therapy for a basal tumor; associated symptoms of neurocutaneous syndrome; coincidence. The tumor location in the present case exerted no influence on the anterior circulation, and this patient had not received any radiation therapy. However, he had minor anomalies: bilateral syndactyly of toes, high arched palate, and bilateral simian crease. The finding of two or more minor anomalies usually reveals the presence of a major anomaly [9]. In patients with Down's syndrome and Wilms' tumor, specific congenital anomalies can be seen more often than in the general population. Cases of association between these congenital diseases and Moyamoya disease have been reported [5,16]. Many other congenital diseases,

such as von Recklinghausen's disease, tuberous sclerosis, Fanconi's anemia, and sickle cell anemia, are sometimes linked with a kind of occlusive arteriopathy termed Moyamoya syndrome [2,4,7,8,17]. The angiographic and pathological findings of these diseases are similar to that of Moyamoya disease [6,7]. Intrafamilial occurrence of Moyamoya disease has been reported [11]. These findings and associations suggest a congenital pathogenesis of Moyamoya disease. Although the present case had a normal karyotype by G banding in peripheral blood leukocytes, more detailed investigations may elucidate chromosomal abnormality. The angiographic findings of Moyamoya disease are as follows: stenosis or occlusion beginning at the termination of the intracranial ICA, and also at the origin of the anterior cerebral artery and the middle cerebral artery (MCA); abnormal vascular network (Moyamoya vessels) in the region of the basal ganglia. Many cases of abnormal vascular networks have been seen in the area of the PCA by vertebral angiogram [15]. However, changes before the ramification of the basilar artery are not usually seen. The symptoms of Moyamoya disease in childhood are caused by brain ischemia whereas intracranial hem-

orrhage is seen in adulthood. The reason for this difference is unknown. Retinal hemorrhage has not been reported as a clinical symptom o f Moyamoya disease. In the present case dilatation of ophthalmic arteries bilaterally and abnormal netlike (Moyamoya) vessels were observed by angiogram. These abnormal netlike vessels have been called ethmoidal Moyamoya [14]. Blood flow was supplied via these abnormal netlike vessels to the ischemic brain. The ophthalmic artery was dilated as if to increase the blood flow. Therefore, retinal hemorrhage may have contributed to the overloading o f retinal vessels. The possibility that the retinal hemorrhage was caused by an increased ICP state must be ruled out because the patient had no hydrocephalus or clinical symptoms of ICP elevation. The findings of fundus oculi did not change after removal of the vermian tumor. Surgical treatment for Moyamoya disease, that is, EDAS or S T A - M C A bypass, may prevent recurrent retinal hemorrhage because it may decrease the overloading of retinal vessels. However, the efficacy of these surgical treatments has not been firmly established

Figure 2. MRI shows multiple, small, low-intensity areas that represent Moyamoya vessels in the basal ganglia bilaterally.

Figure 3. Right carotid angiogram: (Left) anteroposteriorview; (Right) lateral view. Stenosis of the right ICA and Moyamoya vesselsare seen. Right ophthalmic artery is extremely dilated.

i

Figure 4. Lej? ~arotid a~lgloqram: (Top) ~znteropo*terior rieuv (Bottom) Lttera/ rleu'. 5"teptod.~ lJ/ the /~[? ICA. di/ata/ioJi ~/ the I~/? ophtha[mic artery,, am] l~|o~'amo]w t'e.*.*e]~are *eel/.

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Figure 5. LeJt t'ertebral angiogram: (Top) anteroposterior t'ieuv (Bottom) ]atera/ rieu'. Collateral circulation t'ia the P I C A and Moyamoya ressels in the area o/the PCA a n d .,teno.ws ~f the basi/ar artery before the ral?llfi¢~lll'O?l,

glee .~e¢'?l.

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Surg N e u r o l t992;37:46-50

Aihara et al

Figure 6. Photomicrograph of the surgical specimen. The pathological diagnosis was pilocytic astrocytoma (hematoxylin and eosin stain × 400).

for hemorrhagic attack. Surgical treatment, such as S T A - M C A bypass, results in improvements principally of symptoms related to the territory of the MCA, including motor weakness, sensory disturbances, and aphasia [3]. The present patient did not have any obvious symptom related to the territory of the MCA. In addition, he lost right visual acuity. Therefore, the effect of an increase in blood supply to the territory of the MCA cannot be evaluated. This patient did not receive surgical treatment for Moyamoya disease; he is being followed as an outpatient. References 1. Bayer RA, Paden P, Sobel DF, Flynn FG. Moyamoya pattern of vascular occlusion after radiotherapy for glioma of the optic chiasma. Neurology 1986;36:1173-78. 2. Brook BS, Gammal TE, Adams RJ, Harlage PL, Smith WB. MR imaging of moyamoya disease in neurofibromatosis. AJNR 1987;8:178-9. 3. Endo M, Kawano N, Miyasaka Y, Yoda K. Cranial burr hole for revasculization in moyamoya disease. J Neurosurg 1989;71: 180-5. 4. Horae AM, Keogh AJ. Cerebrovascular moyamoya disease. Br Med J 1974;1:430-2. 5. Kitamura K, Kurokawa T. Annual report of the research committee on spontaneous occlusion of the circle of Willis (Moyamoya disease). Japan Ministry of Health and Welfare, 1982:73-9. 6. Lamas ED, Lobato R, Cabello A, Abaci JM. Multiple intracranial arterial occlusions (moyamoya disease) in patients with neurofi-

bromatosis: one case report with autopsy. Acta Neurochir (Wien) 1978;45:133-45. 7. Leone RG, Schatzki SC, Wolopow ER. Neurofibromatosis with extensive intracranial arterial occlusive disease. AJNR 1982;3:572-6. 8. Makoyo PZ, Rapoport AM, Fleming RJ. Moya-moya disease in black adults. Arch Neurol 1977;34:130. 9. Marden PM, Smith DW, McDonald MJ. Congenital anomalies in the newborn infant, including minor variations. J Pediatr 1964;64:357-71. 10. Mori K, Takeuchi J, Ishikawa M, Handa H, Toyama M, Yamaki T. Occlusive arteriopathy and brain tumor. J Neurosurg 1978;49:22-35. 11. Narumi S, Nishimura K, Fuchizawa K, Hidaka T. Three cases of moyamoya disease found in an inbred family. Brain Nerve (Tokyo) 1976;28:1201-5. 12. Numaguchi Y, MarcJA, Balsys R. Unusual angiographic manifestation of craniopharyngioma: a case report. Neuroradiology 1976;11:215-8. 13. Suzuki J, Kodama N. Moyamoya disease--a review. Stroke 1983;14:104-9. 14. Suzuki J, Kodama N, Takaku A. Cerebrovascular "Moyamoya" disease. Brain Nerve (Tokyo) 1970;22:417-24. 15. Suzuki J, Takaku A. Cerebrovascular"Moyamoya" disease. Arch Neurol 1969;20:288-99. 16. Watanabe Y, Todani T, Fujii T, Toki A, Umemura S, Koike Y. Wilms' tumor associated with moyamoya disease: a case report. Z Kinderchir 1985;40:114-16. 17. Yonekawa Y, Handa H, Okuno T. Moyamoya disease: diagnosis, treatment, and recent achievement. In: Barnett HJM, Stein BM, Mohr JP, Yatsu FM, eds. Stroke. New York: Churchill-Livingstone, 1986:805-29.

Atypical Moyamoya disease associated with brain tumor.

A 4-year-old boy with right retinal hemorrhage, mental retardation, and multiple minor anomalies was referred to our hospital. Computed tomography sca...
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