ИСТИНА

OBJECTIVE: Moyamoya disease is a rare cerebrovascular pathology and its prevalence in Russia is unknown. However Burdenko Neurosurgical Institute has the largest series of observations of MMD in Russia. OBJECT: Between 2008 to 2017 in BNI 71 patients with MMD were observed. Among them there were 14 adults (aged 19-37 years, mean age 29) and 50 children (aged 1,2-17, mean age 7,4). Male/female ratio was 1:1,8. The clinical manifestations included those of transient ischemic attacks (74%), ischemic strokes (50%), epilepsy (24%), intracranial hemorrhage (8%), hyperkinesis (5%) and cephalgia (55%). Diagnostics were performed by subtraction angiography, MR-angiography and CT-angiography. The aim of the diagnostics was to determine the stage of the disease, to assess the angiographic characteristics of the donor and recipient vessels, the state of collateral circulation and the degree of cerebrovascular insufficiency. METHODS: Surgical treatment was performed in 13 adults (17 sides) and 33 children (52 sides). Total number of operations was 69. Indirect revascularization was performed in 12 patients (22 sides), direct revascularization was performed in 11 patients (15 sides), and combined revascularization was performed in 23 patients (32 sides). Indirect revascularization was performed only in children of younger age (up to 10 age). Among them there were 10 bilateral revascularizations. Direct revascularization was performed in 11 adults, 4 – bilateral revascularization. Combined revascularization was performed in 21 children (2 – 18 age) and 2 adults (19 and 35 age), 9 – bilateral revascularization. Follow-up period was up to 9 years. RESULTS: In group of indirect revascularization improvement of the symptoms was seen in 77% of cases, in group of direct revascularization – in 91%, and in group of combined revascularization there was the improvement in 100% of cases. The complications were seen more often in group of indirect revascularization and were associated with the perioperative ischemic disorders. CONCLUSION: Based on our experience we conclude that the neurosurgeon should choose the variant of the surgical revascularization individually and this choice should be based on the age of the patient, the clinical severity of the disease, angiographic characteristics of the donor and recipient vessels, the state of collateral circulation and the degree of cerebrovascular insufficiency.