title: Multiple encephalogaleoperiosteal synangiosis for bilateral carotid artery stenosis in a 13-year-old girl: a case report.
abstract: BACKGROUND
  Encephalogaleoperiosteal synangiosis (EGS) has been widely used to treat children with moyamoya disease (MMD). We present the first case of successful multiple EGS in a patient with brain ischemic disease who presented with different cerebrovascular findings from MMD.
  
  
  METHODS
  A 13-year-old girl had an increased frequency of transient ischemic attacks that affected her right extremities. Digital subtraction angiography showed tapering of the internal carotid artery (ICA). The anterior cerebral artery (ACA) and middle cerebral artery (MCA) were visible on vertebral angiogram, but not on carotid angiogram. The intact circle of Willis and lack of hypervascularity of the lenticulostriate arteries were observed. Decreased regional cerebral blood flow (CBF) in the bilateral ACA and MCA territories quantified by (123)I-N-isopropyl-p-iodoamphetamine-single photon emission computed tomography indicated the need for extracranial-intracranial bypass surgery. Multiple EGS procedures were performed instead of direct anastomosis, which is the standard procedure for intracranial ICA stenosis, because the space for the craniotomy was limited by transdural anastomosis.
  
  
  RESULTS
  Despite the fact that the diagnosis of MMD was questionable, the hemispheres were well vascularized, and the neurology and CBF improved postoperatively.
  
  
  CONCLUSIONS
  The preserved circle of Willis and lack of moyamoya vessels were inconsistent with the features of MMD. However, childhood onset, bilateralness, chronic intracranial ICA stenosis, and transdural anastomosis indicated the same underling pathogenicity as MMD. It is hypothesized that ICA stenosis occurred immediately proximal to the posterior communicating artery in this case. This would have produced the atypical finding of the remaining circle of Willis without growth of the basal moyamoya vessels.
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Inamura
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan. snomura@yamaguchi-u.ac.jp.
authors.forename: Sadahiro
authors.initials: S
authors.lastname: Nomura
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Sadahiro
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Oku
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ishihara
authors.affiliation: Department of Neurosurgery, Yamaguchi University Graduate School of Medicine, Minamikogushi 1-1-1, Ube, Yamaguchi, Japan.
authors.forename: Michiyasu
authors.initials: M
authors.lastname: Suzuki
mesh_terms: D000293:Adolescent; D015901:Angiography, Digital Subtraction; D016893:Carotid Stenosis / Q000000981:diagnostic imaging / Q000601:surgery*; D002548:Cerebral Revascularization / Q000379:methods*; D002560:Cerebrovascular Circulation; D005260:Female; D006801:Humans; D002546:Ischemic Attack, Transient / Q000000981:diagnostic imaging / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods*; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome
keywords: Indirect anastomosis surgery; Internal carotid artery occlusion; Moyamoya disease; Multiple burr-hole procedures