Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis

R. Michael Scott, Jodi Smith, Richard L. Robertson, Joseph R. Madsen, Sulpicio G. Soriano, Mark A. Rockoff

Research output: Contribution to journalArticle

330 Citations (Scopus)

Abstract

Object. Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral "moyamoya" vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients. Methods. The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status. Conclusions. Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.

Original languageEnglish (US)
Pages (from-to)142-149
Number of pages8
JournalJournal of Neurosurgery
Volume100
Issue number2 SUPPL.
StatePublished - Feb 2004
Externally publishedYes

Fingerprint

Moyamoya Disease
Stroke
Transient Ischemic Attack
Pediatrics
Cranial Irradiation
Neurosurgical Procedures
Neurofibromatoses
Craniotomy
Down Syndrome
Brain Ischemia

Keywords

  • Cerebral arteriopathy
  • Cerebral revascularization
  • Intracranial stenosis
  • Moyamoya disease
  • Pediatric neurosurgery
  • Pial synangiosis
  • Stroke

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Scott, R. M., Smith, J., Robertson, R. L., Madsen, J. R., Soriano, S. G., & Rockoff, M. A. (2004). Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. Journal of Neurosurgery, 100(2 SUPPL.), 142-149.

Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. / Scott, R. Michael; Smith, Jodi; Robertson, Richard L.; Madsen, Joseph R.; Soriano, Sulpicio G.; Rockoff, Mark A.

In: Journal of Neurosurgery, Vol. 100, No. 2 SUPPL., 02.2004, p. 142-149.

Research output: Contribution to journalArticle

Scott, RM, Smith, J, Robertson, RL, Madsen, JR, Soriano, SG & Rockoff, MA 2004, 'Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis', Journal of Neurosurgery, vol. 100, no. 2 SUPPL., pp. 142-149.
Scott RM, Smith J, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. Journal of Neurosurgery. 2004 Feb;100(2 SUPPL.):142-149.
Scott, R. Michael ; Smith, Jodi ; Robertson, Richard L. ; Madsen, Joseph R. ; Soriano, Sulpicio G. ; Rockoff, Mark A. / Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. In: Journal of Neurosurgery. 2004 ; Vol. 100, No. 2 SUPPL. pp. 142-149.
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abstract = "Object. Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral {"}moyamoya{"} vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients. Methods. The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8{\%} of the population and transient ischemic attacks (TIAs) in 43.4{\%} prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7{\%} per patient; 4{\%} per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status. Conclusions. Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.",
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T1 - Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis

AU - Scott, R. Michael

AU - Smith, Jodi

AU - Robertson, Richard L.

AU - Madsen, Joseph R.

AU - Soriano, Sulpicio G.

AU - Rockoff, Mark A.

PY - 2004/2

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N2 - Object. Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral "moyamoya" vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients. Methods. The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status. Conclusions. Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.

AB - Object. Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral "moyamoya" vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients. Methods. The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status. Conclusions. Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.

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KW - Cerebral revascularization

KW - Intracranial stenosis

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KW - Pediatric neurosurgery

KW - Pial synangiosis

KW - Stroke

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