Experience of treatment of moyamoya disease at the Clinic of Neurosurgery of Kaunas University of Medicine.

BACKGROUND: Moyamoya disease was first described in Japan and represents characteristic appearance on cerebral angiography an abnormal network ("puff of smoke") of collaterals around stenotic arteries. This disease is characterized by progressive intracranial vascular obliterations of the circle of Willis, resulting in successive ischemic or hemorrhagic events. Moyamoya disease primarily occurs among orient people (Japanese, Koreans, Caucasians) and is very rare in Lithuania. AIM OF THE STUDY: To evaluate long-term effectiveness of cerebral revascularization in order to prevent cerebral ischemic and hemorrhagic events in patients with moyamoya disease. MATERIAL AND METHODS: Since 1995, 14 patients with moyamoya disease have been treated at the Clinic of Neurosurgery of Kaunas University of Medicine. All patients underwent neurological examination, computed tomography or/and magnetic resonance imaging, transcranial Doppler sonography, single photon emission computed tomography and four vessels cerebral angiography investigations. The diagnosis of moyamoya disease was confirmed by cerebral angiography. Cerebral ischemia was observed in 7 cases and cerebral hemorrhage was detected in the other 7 moyamoya disease patients. Neurological disability was evaluated using the Scandinavian Stroke Scale, and performance in the active daily life using the Barthel Index. The degree of handicap was assessed with the Rankin Scale. Different surgical procedures (superficial temporal artery to middle cerebral artery anastomosis, encephalosynangiosis, bifrontal free omental flap) were applied for cerebral revascularization of moyamoya disease patients. RESULTS: The diagnosis of moyamoya disease was confirmed by cerebral angiography in all our patients. Ten patients were selected for surgical brain revascularization. The main criterion for selection of patients for surgery was hypoperfusion of the brain on single photon emission computed tomography. During follow-up period (mean follow-up period was 36 months) there were no rebleeding or ischemic events in both surgical groups. CONCLUSIONS: Cerebral angiography is the main diagnostic procedure which confirms the diagnosis of moyamoya disease. Cerebral hypoperfusion on single photon emission computed tomography is the main criterion for selection of patients for cerebral revascularization. Extra-intracranial anastomosis is an effective procedure for preventing both ischemic and hemorrhagic events in moyamoya patients.