Article
Current management of Moyamoya disease: Outcome of direct / indirect revascularization surgery for 150 affected hemispheres
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Published: | June 4, 2012 |
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Objective: Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Recent advances in molecular biology and genetic research provided better understanding on the pathophysiology of moyamoya disease, while surgical revascularization still remains the preferred procedure for this entity. We sought to review the efficacy and safety of this procedure for moyamoya disease with ischemmic symptoms.
Methods: The present study investigated the clinical course of 106 consecutive patients with moyamoya disease operated on 150 hemispheres by superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis with indirect pial synangiosis.
Results: The outcome of surgery on the operated hemisphere was favorable; no cerebrovascular events during outpatient follow-up (mean 58.4 months) in 89.3% (134 / 150). Two patients manifested as hemorrhagic event on the operated hemisphere during the follow-up period (2 / 150; 1.33%), in one of these patients the neurologic status deteriorated after hemorrhage. Despite the favorable long-term outcome, the incidence of temporary neurologic deterioration due to cerebral hyperperfusion was evident in 18.0% (27 / 150), while no patients suffered permanent neurologic deterioration due to hyperperfusion.
Conclusions: Direct/indirect revascularization surgery is a safe and effective treatment for moyamoya disease, although the issue of bleeding/re-bleeding remained to be solved in the future investigation. Postoperative cerebral hyperperfusion and peri-operative infarction are potential complications of this procedure, thus we recommend intensive postoperative care and cerebral blood flow measurement in the acute stage, because the management of hyperperfusion is contradictory to that of ischemia.