Introduction: Moyamoya disease is an idiopathic cerebrovascular disease characterized by stenosis culminating in occlusion of the terminal internal carotid artery and its major branches. This in turn causes diminished blood flow to the corresponding cerebral territories which may be appreciated qualitatively and quantitatively by various imaging modalities in order to assess the cerebrovascular reserve.
The aim of revascularization surgeries - the mainstay strategy in the treatment of moyamoya patients, is to increase collateral blood flow to hypoperfused areas of cortex, using the external carotid circulation as a donor supply.
The aim of this study was to evaluate the success rate of indirect revascularization, review the clinical profiles of pediatric and adult moyamoya patients and to assess outcome.
Methods: Patients diagnosed with Moyamoya disease at a tertiary center between 1998 and 2014 were enrolled in this study. All patients where surgically treated with indirect revascularization. The clinical presentations, underlying diseases, concurrent diseases, radiological findings, treatment and outcome of the patients were reviewed in a retrospective fashion.
Results: 64 revascularization operations were performed in thirty-seven patients with angiographically proven Moyamoya disease. The age at diagnosis varied between seven months and forty-three years (mean 20.7 years). Included where eighteen adult patients and 19 pediatric patients with an equal male to female distribution. The mean follow-up period after diagnosis was 81.7 months. In three patients, the initial clinical presentation was intra-cranial hemorrhage. Six pediatric patients harbored moyamoya syndrome as part of a syndromatic presentation. Subsequent to medical and surgical treatment, one patient suffered from a recurrent hemorrhagic event and died. All other patients recovered without sequelae.
Conclusions: Surgical indirect revascularization provides long-term, durable, and marked reduction in stroke risk for children and adults with moyamoya.
Patient Care: Increasing the awarness of physicians to moyamoya disease. Thereby, shortening the latency period between clinical manifestations to diagnosis and treatment.
Learning Objectives: Understanding the different diagnostic and follow-up modalities.
Understanding the variety of surgical methods available in treating moyamoya disease.