Moyamoya disease in a hispanic child: a case report

• Published in: Surgical neurology 2002, Vol.57 (1), p.55-62
• Main Authors: Knierim, David S, Woodward, Meredith V.(Olds)
• Format: Article
• Language: English
• Subjects: Brain - blood supply; Brain - pathology; Brain - surgery; Carotid Stenosis - diagnosis; Carotid Stenosis - surgery; cerebral revascularization; Child; EC-IC bypass; Female; Hispanic Americans; Humans; Magnetic Resonance Angiography; Moyamoya disease; Moyamoya Disease - diagnosis; Moyamoya Disease - surgery; Neurosurgical Procedures - methods; stroke; Tomography, Emission-Computed, Single-Photon; transient ischemic attacks
• ISSN: 0090-3019; 1879-3339
• DOI: 10.1016/S0090-3019(01)00637-1

BACKGROUND Moyamoya disease was initially described by Suzuki and Takaku in 1963 as a radiographic phenomenon relating to the tiny collateral vessels characteristic of the disease that resemble a cloud or puff of smoke [13,16,17]. The disease is rare and initially it was believed that the disease was confined to the Japanese population. It consists of occlusive vascular disease at the Circle of Willis with a tendency toward multiple ischemic neurological events and small strokes. In older populations it can often be associated with further vascular degeneration and intracerebral hemorrhage. This paper discusses the diagnosis, treatment, and management of moyamoya disease in a Hispanic child. CASE DESCRIPTION The case of a Hispanic child who presented with transient ischemic attacks over a period of 1 year is reported. Magnetic resonance imaging (MRI) revealed occlusive vascular disease in the posterior Circle of Willis. Digital subtraction cerebral angiography showed vascular occlusion at the base of the skull with collateral leptomeningeal and posterior circulation contribution in a pattern typical of moyamoya disease. Technetium was injected for a SPECT study demonstrating less uptake in the left frontal and left parieto-occipital regions. The patient underwent a left superficial temporal-to-middle cerebral artery anastamosis followed by a right-sided anastamosis in a second operation. The patient tolerated the cerebral revascularization and was symptom-free at 6-month follow-up. Cerebral angiography demonstrated improved perfusion in both cerebral hemispheres postoperatively. CONCLUSION This article reports the occurrence of moyamoya in a Hispanic child. It illustrates the improved perfusion postoperatively as seen on digital subtraction cerebral angiography. Direct revascularization is felt to be difficult in children and alternatives such as encephaloduroarteriosynangiosis have been advocated. Direct revascularization was effective in treating moyamoya disease in this instance. Most of the discussion of moyamoya disease has been focused on the Japanese and Far East population. This report confirms the entity as occurring in a Hispanic individual in the United States with no known Japanese ancestry.