What is the role of surgery in the treatment of moyamoya disease?

Updated: Nov 09, 2018
  • Author: Roy Sucholeiki, MD; Chief Editor: Amy Kao, MD  more...
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Answer

Various surgical procedures have been used in the treatment of moyamoya disease, with the goal of revascularizing the ischemic hemisphere, including the following [12] :

  • Superficial temporal artery–middle cerebral artery (STA-MCA) anastomosis

  • Encephaloduroarteriosynangiosis (EDAS) [13]

  • Encephaloduroarteriomyosynangiosis (EDAMS)

  • Pial synangiosis

  • Omental transplantation

These procedures can be divided into 2 groups depending on whether they involve direct or indirect anastomosis. The STA-MCA anastomosis is a direct revascularization procedure, whereas indirect procedures depend on the subsequent formation of collateral vessels to increase blood delivery. Which of these procedures is most effective remains controversial. Sufficient evidence suggests that surgical revascularization procedures result in some symptomatic benefits along with demonstration of improved blood flow. Direct and/or combined procedures provide improved vascularization. However, data proving sustained or improved long-term outcomes are insufficient. [14, 15]

STA-MCA anastomosis is very difficult in children younger than 2 years because of the small diameter of the STA. In these cases, EDAS may be more suitable. This procedure sometimes has failed because of poor revascularization. Hoffman suggested that this is due to the presence of atrophy and a layer of spinal fluid between the pia and the arachnoid tissue. [16] Division of the arachnoid membrane and placement of the STA directly on the pial membrane help to avoid the problem. In cases of EDAS failure, EDAMS can be considered.


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