title: Surgical treatment and perioperative management of moyamoya disease associated with glycogen storage disease Type 1a.
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.
authors.affiliation: Department of Neurosurgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan. egashi@gifu-u.ac.jp
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Egashira
authors.forename: Jun C
authors.initials: JC
authors.lastname: Takahashi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ohnishi
authors.forename: Yukako
authors.initials: Y
authors.lastname: Kawasaki
authors.forename: Masamune
authors.initials: M
authors.lastname: Higashigawa
authors.forename: Koji
authors.initials: K
authors.lastname: Iihara
authors.forename: Susumu
authors.initials: S
authors.lastname: Miyamoto
mesh_terms: D000140:Acidosis, Lactic / Q000209:etiology*; D000714:Anastomosis, Surgical; D015415:Biomarkers / Q000097:blood; D016893:Carotid Stenosis / Q000150:complications / Q000000981:diagnostic imaging; D002533:Cerebral Angiography; D002648:Child; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000150:complications*; D006801:Humans; D002546:Ischemic Attack, Transient / Q000209:etiology / Q000517:prevention & control; D008279:Magnetic Resonance Imaging; D020768:Middle Cerebral Artery / Q000601:surgery; D009072:Moyamoya Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D019990:Perioperative Care / Q000379:methods*; D012086:Reoperation; D013699:Temporal Arteries / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers