588 Surg Neurol 1984;21:588-92 Moyamoya Disease Associated with Craniopharyngioma Naoki Tsuji, M.D., Tsuyoshi Kuriyama, M.D., Munehisa Iwamoto, M.D., and Kohzo Shizuki, M.D. Departments of Neurosurgery and Pathology, Wakayama Red Cross Hospital, Komatsubara, Japan Tsuji N, Kuriyama T, Iwamoto M, Shizuki K. Moyamoya disease associated with craniopharyngioma. Surg Neurol 1984;21:588-92. A case of coexisting moyamoya disease and craniopharyngioma in an 8-year-old boy is reported. The patient had been suffering from recurrence of transient right hemiparesis with headache and vomiting during the three years before admission. After the diagnosis of both moyamoya disease and craniopharyngioma, evacuation of the cystic fluid of the craniopharyngioma, encephalomyosynangiosis, and internal irradiation through an Ommaya drainage system were carried out. The symptoms gradually improved after these procedures. The relation of moyamoya disease to craniopharyngioma is discussed. We consider that internal irradiation is one of the best means of treatment for a cystic craniopharyngioma associated with moyamoya disease. KEY WORDS: Moyamoya disease; Craniopharyngioma; Internal irradiation; Intracranial occlusive disease M o y a m o y a or "puff of smoke" [15] is a term derived from the appearance of the cerebral angiographic picture. Typically, there is an abnormal vascular network at the base of the brain associated with an occlusion of the circle of Willis [14]. T h e etiology of m o y a m o y a disease, however, still remains controversial. Though this is generally believed to be an acquired process, familial cases have occasionally been reported [11,17,18]. T h e r e are also some other cases associated with tumor [5,8,9], irradiation [14,16], neurocutaneous syndrome [6], and arteriosclerosis. In these, angiographic findings of m o y a m o y a vessels are the same as those without other accompanying diseases. W e report a case of m o y a m o y a disease accompanied by craniopharyngioma, and we present a review of the literature. Address reprint requeJts to: Tsuyoshi Kuriyama, M.D., Wakayama Red Cross Hospital, Komatsubara, Wakayama-shi, 640 Japan. @ 1984 by Elsevier Science Publishing Co., Inc. Case R e p o r t An 8-year-old boy was admitted to Wakayama Red Cross Hospital with hemiconvulsion on the right side on January 30, 1982. In the 3 years before admission, he had suffered from recurrent episodes of headache with vomiting and convulsions of the right hand, and also attacks of transient hemiparesis when he cried out. Examination Neurological examination on admission showed disturbance of the visual acuity o f the right eye with optic atrophy, hemiparesis of the right side with hyperreflexia and pathological reflexes. Plain cranial x-ray examination disclosed a saucerlike sella turcica with suprasellar calcification (Figure 1). An electroencephalogram showed diffuse continuous high voltage delta waves, especially over the right hemisphere. Plain c o m p u t e r tomography (CT) showed a mild atrophy of the brain. T h e r e was a round low-density area circumscribed by a high-density zone in the sellar to suprasellar region (Figure 2). The suprasellar lesion did not show a definite contrast enhancement. N e i t h e r of the intracranial internal carotid arteries nor the middle cerebral arteries were enhanced in the suprasellar cistern or in the sylvian cisterns. An angiogram of the left cerebral artery revealed a network of numerous fine vessels (moyamoya vessels) with nonfilling of the A~ segment of the anterior cerebral artery and poor filling of the M~ segment of the middle cerebral artery. The left internal carotid artery was narrowed and shifted laterally probably by the t u m o r (Figure 3). The findings in the angiogram of the right carotid artery were similar to those of the left side (Figure 4). Collateral vessels from the external carotid arteries to the intracranial region were not visualized. Endocrinologic examination revealed panhypopituitarism. M o y a m o y a disease associated with a craniopharyngioma was suggested by these findings. 0090-3019/84/$3.00 Moyamoya Disease and Craniopharyngioma Surg Neurol 1984;21:588-92 589 Figure 1. Plain x-ray film of the skull (lateral view) showing a saucerlike sella with suprasellar calcification. A Operation Biopsy of the cyst wall and evacuation o f the cyst fluid were carried out through a frontotemporal craniotomy on February 10, 1982. After the frontal lobe was retracted, we saw that the right internal carotid artery was narrowed and displaced laterally by a cystic tumor. T h e right optic nerve was also compressed by the tumor. T h e r e were many tortuous fine vessels in the basal part o f the frontal lobe. T h e content o f the cyst was a gritty motor-oil-like fluid. T h e cyst was drained into the subgaleal space of the temporal region using an O m m a y a drainage system. Because the superficial temporal artery was too small, superficial temporal a r t e r y - m i d d l e cerebral artery anastomosis was not performed. Instead, an encephalomyosynangiosis was carried out using the temporal muscle in order to increase blood flow and prevent ischemia o f the brain [3]. Postoperative Course After the operation, the symptoms improved gradually. H o w e v e r , frequent taps o f the O m m a y a reservoir were necessary to treat overretention of fluid causing headache and vomiting. On February 22, cystograms with metrizamide instilled into the O m m a y a reservoir revealed an irregular shaped cyst (2.2 cm x 3.4 cm) in the suprasellar region (Figure 5). T h e volume of the cyst was calculated to be about 12 cm 3, and no leakage of the dye was seen. Because the patient's liver function was abnormal as a side effect of anesthesia at this time, the internal irradiation was postponed until his liver function returned to normal. On May 11, he was given B Figure 2. (A) A C T scan demonstrating mild atrophy of the brain. (B) Note a round low-density area circumscribed by a high-density zone in the suprasellar region. Neither the suprasellar lesion nor the Syh,ian cisterns are enhanced by contrast medium. intracystic administration o f 1.0 mg of bleomycin daily for 5 days. On J u n e 5, internal irradiation was p e r f o r m e d by the injection of 10 mCi of ~)~Au-colloid. Secretion of the fluid stopped 2 weeks after this injection. T h e r e were no side effects. The cyst did not change its size, probably because of calcification of the tumor's capsule. The patient was discharged from the hospital on J u n e 29, 1982, having only a mild paresis of the right hand 590 Surg Neurol 1984;21:588-92 A Figure 3. Angiogram of the left carotid artery, anteroposterior (A) and lateral (B) vieu's, showing a fine vascular network (moyamoya vessels) with nonfilling of the A, segment of the anterior cerebral artery and poor filk'ng of the M 1 segment of the middle cerebral artery. and disturbance of vision in the right eye. The patient has been healthy without aspiration of the cyst fluid to the time of writing this report, March 1983. Discussion The etiology of m o y a m o y a disease has not yet been established [9]. An acquired etiology is plausible, but there are also a few reports of familial occurrence, which may suggest a congenital etiology [14]. There are some Tsuji et al A Figure 4. Right carotid angiogram of the right carotid artery, anteroposterior (A) and lateral (B) views, demonstrating similar findings to those in the left side. Note the internal carotid artery to he compressed and displaced by the tumor. other cases suggesting a development of collateral channels after the occlusion of the internal carotid arteries secondary to intracranial tumors, arteriosclerosis, and so on, in which angiographic findings are the same as those in so-called moyamoya disease of unknown etiology [2]. Takeuchi [19] advocates that an abnormal vascular network secondary to the known etiology (moyamoya phenomenon) should be differentiated from an idiopathic moyamoya disease. We presented here a case of moyamoya disease associated with craniopharyngioma. The association of these Moyamoya Disease and Craniopharyngioma Figure 5. Cystograms showing an irregularly shaped cyst in the suprasellar region. two lesions may be explained by coincidence or by dev e l o p m e n t o f collaterals secondary to stenosis of the internal carotid artery by the tumor. Only two cases of coexistence o f m o y a m o y a disease and craniopharyngioma have been reported in the literature [2,9]. Stenosis or occlusion of the internal carotid artery is a rare p h e n o m e n o n in cases of intracranial tumors except for meningiomas [8] and pituitary adenomas. Such a vascular change associated with craniopharyngiomas has been reported only in three cases [5,9]. In two of these three cases, radiation therapy had been given before the appearance o f the vascular change. In the other case, the m o y a m o y a change o f vessels was thought to be attributable to enlarged collateral vessels that were secondary to stenosis o f the internal carotid artery due to compression by the craniopharyngioma. Mori et al [9] presented Surg Neurol 1984 ;21:588-92 591 4 cases of m o y a m o y a vessels associated with brain tumor, and concluded that " m o y a m o y a " disease was not an independent disease entity, but a neuroradiological syndrome caused by an occlusion of the circle of Willis and might result from a variety of causes. T h e y also described four possible situations in which m o y a m o y a changes might occur: compression of the carotid bifurcation by a slowly growing basal tumor; secondary arterial changes by radiotherapy of a basal tumor; in association with a neurocutaneous syndrome; and coincidence. Kojima and Waga [5] reported a case of stenosis of the internal carotid artery due to combined effects of direct compression by craniopharyngioma and radiation o f the arterial wall. Kaku et al and Mizukawa et al [8] reported m o y a m o y a disease associated with a meningioma. A case of m o y a m o y a disease associated with glioma was reported by Pecker et al [13]. In these cases, the association was thought to be coincidence. Ishikawa et al [2] reported m o y a m o y a vessels associated with craniopharyngioma and suggested that compression o f the internal carotid artery by the craniopharyngioma was closely related to the d e v e l o p m e n t of collateral circulation, namely, m o y a m o y a vessels. We suppose that the m o y a m o y a vessels and craniopharyngioma in our case had developed independently, because the M1 segm e n t o f the middle cerebral artery was occluded even though it was away from the t u m o r and received no direct compression by the tumor. H o w e v e r , compression by the t u m o r might have contributed to the dev e l o p m e n t of m o y a m o y a vessels in our case. Kobayashi et al [4] reported that internal irradiation [1,4,7,10] was effective for cystic craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to r e m o v e completely, for recurrent cysts resistant to previous treatment, or for multiple cysts. A safe and adequate dose to the cyst wall ranges from 9 0 0 0 - 3 0 , 0 0 0 fads for craniopharyngiomas [4]. Retraction of the brain during an operation may injure blood vessels. M o y a m o y a vessels may be m o r e vulnerable to irradiation than normal vessels. Therefore, internal irradiation should be applied to cystic craniopharyngioma associated with m o y a m o y a disease to damage m o y a m o y a vessels as little as possible. T h e O m m a y a drainage system was effective in aspirating cystic fluid in the present case. This system is r e c o m m e n d e d in the treatment of a cystic craniopharyngioma associated with m o y a m o y a disease because it is convenient for the internal irradiation and intracystic administration of bleomycin [12]. We wish to express our thanks to Dr. Norihiko Komai, Professor and Chairman, and to Dr. Seiji Hayashi, Associate Professor, both of the Department of Neurosurgery of WakayamaMedical College, for their constant guidance and kind advice. 592 Surg N e u r o l 1984;21:588-92 References 1. Cabezudo JM, Vaquero J, Areitio E, Martinez R, De Sola RG, Bravo G. Craniopharyngioma: a critical approach to treatment. J Neurosurg 1981 ;55:371-5. 2. Ishikawa M, Handa H, Mori K, Matsuda I. "Moyamoya" vessels on the tumor in the sellar region. ArchJpn Chir 1979;48:639-44. 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 1977;17:(Part l), 29-37. 4. Kobayashi T, Kageyama N, Ohara K. lnternal irradiation for cystic craniopharyngioma. J Neurosurg 1981 ;55:896-903. 5. Kojima T, Waga S. Stenosis of the intracranial internal carotid artery by a craniopharyngioma: report of a case. Noshinkeigeka 1982;10:777-82. 6. Lamas E, Lobato RD, Cabello A, Abad JM. Multiple intracranial arterial occlusions (moyamoya disease) in patients with neurofibromatosis. Acta Neurochir 1978;45:133-45. 7. Matsukado Y, Kodama T, Marubayashi T. Follow-up studies of the craniopharyngiomas treated by combined surgery and intracystic 19SAu irradiation therapy. Shoni No Noshinkei 1979; 2:115-23. 8. Mizukawa N, Iwatsuki K. A case of cerebral basal rete mirabile combined with brain tumor. Rinsho Shinkei 1973;13:650-7. Tsuji et al 9. Mori K, Takeuchi J, lshikawa M, Handa H, Toyama M, Yamaki T. Occlusive arteriopathy and brain tumor, J Neurosurg 1978;49:22-35. 10. Nakayama T, Kodama T, Matsukado Y. Treatment of inoperable craniopharyngioma with radioactive gold. No To Shinkei 1971;23:509-13. 11. Nishimoto A, Takeuchi S. Abnormal cerebrovascular netwnrk related to the internal carotid arteries. J Neurosurg 1968;29:255-60. 12. Ogashiwa M, Takeichi K. Intracystic treatment of bleomycin for craniopharyngioma. No To Hattatsu 1976;8:229-35. 13. Pecker J, Simon J, Guy G, Herry JF, Nishimoto's disease: significance of its angiographic appearances. Neuroradiol 1973; 5:223-30. 14. Rajakulasingan K, Cerullo LJ, Raimondi AJ. Childhood moyamoya syndrome. Child's Brain 1979;5:467-75. 15. Raimondi AJ. Pediatric neuroradiology. Philadelphia: Saunders, 1972:654. 16. Servo A, Puramen M. Moyamoya syndrome as a complication of radiation therapy. J Neurosurg 1978;48:1026-9. 17. Suzuki J, Takaku A. Cerebrovascutar "moyamoya" disease. Arch Neurol 1969;20:288-99. 18. Suzuki J, Takaku A. The disease showing the abnormal vascular network at the base of brain particularly found in Japan. No To Shinkei 1968;20:35-40. 19. Takeuchi K. Moyamoya phenomenon and moyamoya disease. No To Shinkei 1978;30:1183-91.