Revascularisation surgery and long-term follow-up in juvenile Moyamoya syndrome: a retrospective analysis
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Updated: May 31, 2026
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Published on: October 20, 2017
Peter T Ulrich1, Elke Januschek
1Neurosurgical Department, Klinikum Offenbach GmbH, Starkenburgring 66, D-63069, Offenbach, Germany. peter.ulrich@klinikum-offenbach.de
Insights
Early revascularisation surgery effectively treats juvenile Moyamoya disease, preventing neurological deficits and intellectual decline. This surgical intervention significantly improves outcomes for affected children.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Vascular Neurology
Background:
- Juvenile Moyamoya disease is rare in Western countries, leading to low physician awareness.
- Revascularisation surgery benefits are primarily proven in the juvenile form of the disease.
- Early intervention is crucial to prevent irreversible ischaemic deficits and cognitive impairment in young patients.
Purpose of the Study:
- To evaluate the efficacy and safety of surgical revascularisation for juvenile Moyamoya disease.
- To assess the long-term outcomes of revascularisation in pediatric patients.
- To highlight the importance of early diagnosis and treatment.
Main Methods:
- Retrospective analysis of 19 children with juvenile Moyamoya disease treated between 1984 and 2009.
- Surgical revascularisation procedures were performed.
- Follow-up included clinical assessment and angiography, with a mean duration of over 17 years.
Main Results:
- The leading presenting symptoms were epilepsy, transient ischaemic attacks (TIA)/prolonged reversible ischaemic neurologic deficits (PRIND), and mental retardation.
- Angiography confirmed neovascularisation in most patients post-indirect bypass surgery.
- Revascularisation eliminated ischaemic symptoms in 94% of patients with minimal complications, morbidity, or mortality.
Conclusions:
- Surgical revascularisation is highly effective in treating juvenile Moyamoya disease.
- Early diagnosis and timely surgical intervention are key to maximizing positive outcomes.
- Treatment benefits appear independent of the specific revascularisation technique used.
Abstract:
Due to its low incidence in Western countries, physician awareness of juvenile Moyamoya disease should be improved. The benefits of revascularisation surgery have only been proven in the juvenile version of the disease. Therefore, early revascularisation may prevent irreversible ischaemic deficits and rapidly progressive mental retardation in young patients.From 1984 to 2009, a total of 19 children (mean age 8 years, range 1-18 years, female predominance 2:1, 17/19 European white patients, 2/19 Asian origin of at least one parent) were treated for juvenile Moyamoya disease by surgical revascularisation. The leading symptoms were epilepsy (17/19), followed by transient ischaemic attacks (TIA) or prolonged reversible ischaemic neurologic deficits (PRIND) (15/19) and mental retardation (11/19). Angiography showed a clear neovascularisation in the majority of patients after indirect bypasses after 6 months. The mean follow-up was 17 years and 3 months (maximum 25 years, minimum 2 years). Two patients were lost to follow-up. In accordance with the literature, ischaemic symptoms were eliminated by the revascularisation operation in 94% of our patients with a very low rate of complications, and no lasting morbidity and mortality in any of the patients.Early diagnosis and surgical treatment seem to potentiate the benefits independently of the type of revascularisation procedure.
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