Moyamoya Disease in Adults: The Role of Cerebral Revascularization

Gregory J. Zipfel; Douglas J. Fox; Dennis J. Rivet

doi:10.1055/s-2005-868161

Skull Base, Table of Contents

Skull Base 2005; 15(1): 27-41
DOI: 10.1055/s-2005-868161

Moyamoya Disease in Adults: The Role of Cerebral Revascularization

Gregory J. Zipfel¹ ^, ² , Douglas J. Fox¹ Jr. , Dennis J. Rivet¹ ^, ³

¹Departments of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri
²Departments of Neurology, Washington University School of Medicine, St. Louis, Missouri
³Departments of Radiology, Washington University School of Medicine, St. Louis, Missouri

Abstract

ABSTRACT

Moyamoya disease is a disorder characterized by bilateral progressive steno-occlusion of the terminal internal carotid arteries with associated development of a fragile network of basal collateral vessels. It most commonly presents in children, but is also frequently seen in adults, especially in the third or fourth decade of life. Adults afflicted with this disease have very different clinical characteristics as compared with children. For example, adults more commonly present with hemorrhage than cerebral ischemia, while children present with cerebral ischemia nearly 75% of the time and very rarely present with hemorrhage. This significantly impacts treatment considerations for the adult-onset moyamoya patient, as cerebral revascularization, though well accepted in the context of cerebral ischemia, is relatively controversial for the prevention of rehemorrhage. The purpose of this article is to review the pertinent general features of moyamoya disease, examine the clinical characteristics associated with the adult-onset form of this disease, and provide a detailed discussion regarding the indications, operative techniques, and outcomes of direct and indirect revascularization surgical procedures.

KEYWORDS

Moyamoya disease - hemorrhage - ischemia - revascularization - adult

Full Text

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Gregory J ZipfelM.D.

Washington University School of Medicine, Department of Neurological Surgery, 660 S. Euclid Ave.

Campus Box 8057, St. Louis, MO 63110

Email: zipfelg@nsurg.wustl.edu