J Neurosurg 48:1026-1029, 1978

M oyamoya syndrome as a complication of radiation therapy Case report

ANITI SERVO, M . D . , AND MATTI PURANEN, M . D .

Roentgen Division, Department of Neurosurgery, Helsinki University Central Hospital, Helsinki, Finland ~" A case is reported with occlusion and stenosis of the internal carotid arteries in association with basal telangiectasia. Fifteen years after postoperative irradiation for an optic glioma, radiological signs typical of the moyamoya syndrome were observed. Radiation therapy is discussed as the cause of the vascular damage in this case. 9 moyamoya syndrome 9 occlusive vascular disease 9 radiation therapy 9 carotid artery occlusion 9 optic glioma

KEY WORDS

T

HE etiology of the disease of multiple progressive intracranial arterial occlusion, also known as the moyamoya syndrome, is unclear. A congenital etiology has been suggested) ,6,9,18 but acquired forms have also been shown to exist. 1'1~176 In the neurocutaneous syndromes of children, radiographic features similar to those in m o y a m o y a are found. 1' Intracranial arterial occlusive disease in combination with neurofibromatosis has previously been reported in 14 cases. 2~ Nine of these had the m o y a m o y a appearance, and three of the nine patients had received previous radiation therapy for optic gliomas. Intracranial occlusive disease secondary to radiation has been reported. 7,~6 We report an additional case of neurofibromatosis with cerebral arterial occlusive disease after radiation therapy. 1026

Case Report

This 17-year-old boy presented for neurological evaluation. At the age of 11/2years, he had received a course of irradiation, with a total of 4200 rads, postoperatively for an optic glioma. The irradiation was given over a period of 4 weeks through two opposing temporal ports and one anterior port. During the operation before irradiation, the anterior cerebral arteries were identified on both sides and seen to be normal. The glioma compressed the optic chiasm and extended below the arteries. After irradiation the patient recovered well, except that the left eye was blind and vision in the right eye was impaired. Ten years later, at the age of 12 years, the patient ceased to grow. Panhypopituitarism was diagnosed and substitution therapy was started. J. Neurosurg. / Volume 48 / June, 1978

Radiation therapy and moyamoya syndrome

FIG. 1. Left common carotid angiogram, lateral view. Left: Early arterial phase showing transdural meningocortical anastomoses from the middle meningealand superficial temporal arteries to the parietal branches of the anterior cerebral artery (arrows). Right: Mid-arterial phase. The internal carotid artery (large arrow) is occluded at the origin of the ophthalmic artery, which is thick and tortuous and displaced inferiorly (small arrows).

For the past 4 years before his present admission, the patient had had periodic attacks of headache, nausea, and vomiting. He also had numerous caf6-au-lait spots. He had never suffered hemiparesis. On admission, 15 years after radiation therapy, left common carotid angiography showed that the internal carotid artery was occluded at the origin of the ophthalmic artery (Fig. 1 right). A dense network of small ethmoidal arteries arose from the ophthalmic artery, which was thick, tortuous, and displaced inferiorly. There was early opacification of transdural meningocortical anastomoses from the middle meningeal and superficial temporal arteries (Fig. 1 left). These anastomoses and the ethmoidal network together supplied the parietal branches of the anterior cerebral artery. Since the middle cerebral artery was not visualized, the artery obviously originated from the basilar artery by collaterals. Right common carotid angiography showed that the internal carotid artery had a stenosis at the origin of the ophthalmic artery. An ethmoidal arterial network was seen here, too, but there were no transdural meningocortical anastomoses. Above the stenotic carotid siphon originated an intricate and dense vascular network, which involved the whole region of the basal nuclei (Fig. 2). The posterior cerebral artery was thick and

J. Neurosurg. / Volume 48 / June, 1978

the posterior choroidal artery participated in this basal arterial network. A rapid collateral circulation to both pericallosal arteries emerged from this network (Fig. 3 left), and there was collateral circulation to the

FXG. 2. Right common carotid angiogram, anteroposterior view. Basal telangiectasia (arrows) above the stenotic carotid siphon. 1027

A. Servo and M. Puranen

FIG. 3. Right common carotid angiogram, lateral view. Mid-arterial (left) and late arterial (right) phase. Basal telangiectasia (arrowheads) above the stenotic carotid siphon. Collateral circulation to the pericallosal arteries (arrows) and to the branches of the middle cerebral artery (double arrows) from the posterior cerebral artery.

branches o f the middle cerebral artery from the posterior cerebral artery (Fig. 3 right). These findings are t y p i c a l o f the moyamoya syndrome.

a r t e r y m a y also be o c c l u d e d ? 1 Table 1 s u m marizes five previously reported cases 7,1'-1e with t y p i c a l r a d i o g r a p h i c pictures of m o y a m o y a s y n d r o m e . All five patients had received r a d i a t i o n therapy several years before the radiological e x a m i n a t i o n was Discussion made. N o a n g i o g r a m s were m a d e on these Vascular lesions o f the cerebral arteries in patients before irradiation; the diagnosis o f n e u r o c u t a n e o u s s y n d r o m e s are well known. ~4 optic or h y p o t h a l a m i c gliomas was based on In neurofibromatosis, occlusive lesions are e c h o e n c e p h a l o g r a p h y . In t h r e e o f t h e f o u n d m o s t l y in t h e p e r i p h e r a l lepto- patients ~4'~5 the occlusions m a y have resulted meningeal arteries, TM but the internal carotid f r o m n e u r o f i b r o m a t o s i s as well as irradiation.

TABLE 1 Clinical summary of five cases with moyamoya syndrome after irradiation Authors & Year Debrun, eta/., 1975 Hilal, et al., 1971

Diagnosis

Neurofibromatosis

Irradiation Dosage (rads)

Time Interval*

hypothalamic ghoma optic glioma

--

5500

15 mos

+

3000

10 yrs

optic glioma

+

+

7 yrs

Klatte, optic glioma et al., 1976 Lee& Hodes, 1967 optic glioma Servo & optic glioma Puranen, 1978

+

2400

-H-

5500 4200

Angiogram Occlusion Basal TelSide angiectasia fight left right left ? ?

? 3 yrs 15 yrs

-ff + -+

-+ + -+

--

--

right left

+ +

+ q-

? right left

+ -+

qq--

*Time elapsed between irradiation and radiological examination. ]028

J. Neurosurg. / Volume 48 / June, 1978

Radiation therapy and moyamoya syndrome In the two patients without neurofibromatosis, T M the carotid occlusion was probably secondary to radiation therapy. There have been reports of arterial stenosis following irradiation in infancy and carotid thrombosis after therapeutic radiation in adults) ,~,17 Postirradiation myelomalacia has also been reported, where a selective spinal cord arteriography was made before and after radiotherapy, and spinal cord arterial occlusion was seen in the p o s t i r r a d i a t i o n arteriogram, s In young patients, the basal telangiectasia has been considered to be a nonspecific response to a slow occlusive process in the circle of Willis. 13 Although in our case no angiogram was made before radiation therapy, at the operation of the glioma the anterior cerebral arteries on both sides had been identified and regarded as normal. The occlusive process may therefore have been caused by irradiation. Opinions differ on the advisability of irradiation of optic gliomas in children. 2 In two recent reports radiation therapy was considered contraindicated in anterior chiasmal gliomas.t2, 22

References 1. Calliauw L: Moyamoya. Neurochirurgie 18:383-390, 1972 2. Chutorian AM, Schwartz JF, Evans RA, et al: Optic gliomas in children. Neurology 14: 83-95, 1964 3. Colquhoun J: Hypoplasia of the abdominal aorta following therapeutic irradiation in infancy. Radiology 86:454-456, 1966 4. Conomy JP, Kellermeyer RW: Delayed cerebrovascular consequences of therapeutic radiation: a clinicopathologic study of a stroke associated with radiation-related carotid arteriopathy. Cancer 36:1702-1708, 1975 5. van Damme W, Beeckman P, Verbruggen R: Moyamoya syndrome probably associated with hydrocephalus. Neuroradiology 9:39-42, 1975 6. Debrun G, Lacour P: A new case of moyamoya disease associated with several intracavernous aneurysms. Neuroradiology 7:277-282, 1974 7. Debrun G, Sauvegrain J, Aicardi J, et al: Moyamoya, a n6nspecific radiological syndrome. Neuroradiology 8:241-244, 1975 8. Di Chiro G, Herdt JR: Angiographic demonstration of spinal cord arterial occlusion in postradiation myelomalacia. Radiology 106:317-319, 1973 9. Galligioni F, Andrioli GC, Marin G, et al: Hypoplasia of the internal carotid artery J. Neurosurg. / Volume 48 / June, 1978

10. 11.

12.

13.

14.

15.

16. 17. 18. 19.

20.

21.

22. 23.

associated with cerebral pseudoangiomatosis. Report of 4 cases. Am J Roentgenol Radium Ther Nucl Med 112:251-262, 1971 Handa J, Handa H: Progressive cerebral arterial occlusive disease: analysis of 27 cases. Neuroradiolugy 3:119-133, 1972 Harwood-Nash DC, McDonald P, Argent W: Cerebral arterial disease in children. An angiographic study of 40 cases. Am J Roentgenol Radium Ther Nucl Med 111: 672-686, 1971 Heiskanen O, Raitta C, Torsti R: Management and prognosis of gliomas of the optic pathways in children. Mud Probl Paediatr 18:216-219, 1977 Hilal SK: Arterial occlusive disease, in Newton TH, Putts DG (eds): Radiology of the Skull and Brain, Volume 2. Angiography. St. Louis: CV Mosby, 1974, Book 4, pp 2286-2309 Hilal SK, Solomon GE, Gold AP, et al: Primary cerebral arterial occlusive disease in children. Part II: Neurocutaneous syndromes. Radiology 99:87-93, 1971 Klatte EC, Franken EA, Smith JA: The radiographic spectrum in neurofibromatosis. Semin Roentgenol 11:17-33, 1976 (Cited in Reference 21) Lee KF, Hodes PJ: Intracranial ischemic lesions. Radiol Clin North Am 5:363-393, 1967 Painter M J, Chutorian AM, Hilal SK: Cerebrovasculopathy following irradiation in childhood. Neurology 25:189-194, 1975 Pecker J, Simon J, Guy G, et al: Nishimoto's disease: significance of its angiographic appearances. Neuroradioiogy 5:223-230, 1973 Suzuki J, Takaku A: Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288-299, 1969 Taveras JM: Multiple progressive intracranial arterial occlusions: a syndrome of children and young adults. Am J Roentgenol Radium Ther Nucl Med 106:235-267, 1969 Tomsick TA, Lukin RR, Chambers AA, et al: Neurofibromatosis and intracranial arterial occlusive disease. Neuroradiology 11:229-234, 1976 Wong IG, Lubow M: Management of optic glioma of childhood: a review of 42 cases. Neuroophthalmol 6:51-60, 1972 Zfllch KJ, Dreesbach HA, Eschbach O: Occlusion of the middle cerebral artery with the formation of an abnormal arterial collateral system - - moyamoya type - - 23 months later. Neuroradiology 7:19-24, 1974

Address reprint requests to: Antti Servo, M.D., Roentgen Division, T8815 Hospital, Topeliuksenkatu 5, 00260 Helsinki 26, Finland. 1029

Moyamoya syndrome as a complication of radiation therapy. Case report.

J Neurosurg 48:1026-1029, 1978 M oyamoya syndrome as a complication of radiation therapy Case report ANITI SERVO, M . D . , AND MATTI PURANEN, M . D...
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