History for Management of Moyamoya Disease in Children

Understanding of Disease

  • Early Case Reports: In 1957 Takeuchi and Shimizu (57) reported a case of ‘hypoplasia of the bilateral internal carotid arteries’ in a Japanese journal and Kudo et al. (33) published a case of ‘occlusion of internal carotid artery’  the same year.
  • The Term ‘Moyamoya’ Disease: The term ‘moyamoya’ is a Japanese word used for description of puffs of smoke. The term ‘moyamoya’ was introduced for the angiographic appearance of fine tangles of basal moyamoya vessels as visualized on arteriogram. Suzuki and Takaku first used the term ‘Moyamoya Disease’ in an article in 1969 and it became the most commonly used name of this entity worldwide(53).

Technological Development

  • Imaging of Moyamoya Disease: For the definitive diagnosis of Moyamoya Disease, cerebral angiography is mandatory. However, advancement of neuroimaging such as MRI, SPECT, and PET allows for an initial diagnosis (without angiography) in addition to a functional evaluation and assessment of the state of cerebral hemodynamics.

Surgical Technique

  • Bypass revascularization: Treatment of Moyamoya is predicated on a surgical revascularization, typically using branches of the external carotid artery to bypass the reduced flow at the stenotic areas of the internal carotids.  Bypass techniques can be indirect (relying on ingrowth of new vessels over time) or direct (involving surgical anastomosis between a donor and recipient vessel).
  • Indirect revascularization:  In 1977 Karasawa et al. first described an indirect revascularization method, encephalomyosynangiosis (EMS)(23). Thereafter, other techniques of indirect revascularization such as  encephaloduroarteriosynangiosis (EDAS) and various modifications or combinations of direct and indirect revascularization methods were suggested(43).