Moyamoya disease is a chronic occlusive cerebrovascular disease of unknown etiology for which no effective treatment has been found. The authors report the result of 23 superficial temporal-middle cerebral artery (STA-MCA) anastomoses and seven encephalomyosynangioses, performed on 13 cases with moyamoya disease and on four additional atypical cases. There were 10 children and seven adults in this study. The follow-up period ranged from 1 year and 4 months to 4 years and 1 month postoperatively; nine patients had excellent results, five good, and one fair; two patients were unchanged. The anastomotic procedure was most effective for transient ischemic attacks, reversible ischemic neurological deficits, and even minor or moderate neurological symptoms. The STA-MCA anastomosis appears to be an effective treatment for moyamoya disease.
Between May, 1974, and March, 1991, 104 patients with moyamoya disease, all under 16 years old at the time of first surgery, underwent superficial temporal-to-middle cerebral artery anastomosis and/or encephalomyosynangiosis. The mean follow-up period was 9.6 years (range 4.8 to 16.0 years). Hemiplegia was the most frequent symptom before the first operation. Transient ischemic attacks (TIA's) were noted in 57 patients and minor stroke with hemiplegia in 44. The most frequent type of cortical dysfunction was aphasia (21 cases). Postoperatively, the incidence of TIA's and/or completed stroke with motor weakness of the extremities was markedly decreased, but visual disturbance progressed and major or minor stroke with visual disturbance was found in two cases. In patients under the age of 3 years, a major stroke prior to surgery resulted in a poor outcome in 36% of cases. Preoperative major stroke in patients between the ages of 3 and 7 years was less frequent, and poor outcomes were seen in 17% of this group. There were no major preoperative strokes in patients with surgery after the age of 7 years, and no poor outcomes were recorded in this group. A major preoperative stroke prior to surgery had adverse impact on the ultimate patient intelligence quotient (IQ) following surgery. All patients operated on after the age of 7 years had a normal or borderline IQ at follow-up examination.
A follow-up angiography study must be performed during the early stage (within approximately 3 weeks after onset of symptoms) to confirm the formation or enlargement of an aneurysm, because such conditions may be amenable to surgical treatment. Unruptured VA dissection could otherwise be treated and followed conservatively. Although the majority of dissected lesions seem likely to stabilize within a few months, as evidenced on angiography, in some cases a longer observation period is required.
SummaryAn operation to place the temporal muscles on the brain surface, namely Encephalo Myo Synangiosis, was carried out on 10 patients with "Moyamoya" disease lacking the middle cerebral artery on the brain surface for anastomosis.Postoperative superselective deep temporal angiogram proved the development of rich vascular supply from the temporal muscle to the brain surface. Satisfactory clinical improvements were seen in nine patients. The remaining one child with transient ischemic attacks (TIA) and major neurological deficits showed slight improvement in spite of being unable to take care of himself. The other nine patients also showed improved electro encephalographic findings. Significant blood supply through the external carotid system to the operated area of the brain was shown in some cases by appearance of diffuse slow activity when the common carotid artery on the same side was compressed.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.