Surgical treatment and perioperative management of moyamoya disease associated with glycogen storage disease Type 1a: Case report

Yusuke Egashira, Jun C. Takahashi, Hiroyuki Ohnishi, Yukako Kawasaki, Masamune Higashigawa, Koji Iihara, Susumu Miyamoto

Research output: Contribution to journalArticle

Abstract

The authors report a case of concurrent moyamoya disease and glycogen storage disease Type 1a that was successfully managed with bypass surgery. This 7-year-old Japanese girl, diagnosed with glycogen storage disease Type 1a at the age of 2 years, presented with repeated transient ischemic attacks. Cerebral angiography revealed severe stenosis at the terminal portions of the bilateral internal carotid arteries, with typical moyamoya vessels. The patient underwent superficial temporal artery-middle cerebral artery anastomosis and encephalomyosynangiosis bilaterally, in 2 staged procedures at an interval of 4 months. Despite perioperative administration of glucose, hypoglycemia and metabolic acidosis occurred after both surgeries. The symptoms were milder after the second surgery, in which an increased dose of glucose was used. The patient tolerated the perioperative conditions well under intensified medical treatment, and no further ischemic symptoms occurred.

Original languageEnglish
Pages (from-to)11-14
Number of pages4
JournalJournal of Neurosurgery: Pediatrics
Volume7
Issue number1
DOIs
Publication statusPublished - Jan 1 2011
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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