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.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Choi JU, Kim DS, Kim EY, Lee KC (1997) Natural history of Moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. Clin Neurol Neurosurg 99(Suppl 2):S11–S18
Fujiwara H, Momoshima S, Kuribayashi S (2005) Leptomeningeal high signal intensity (ivy sign) on fluid-attenuated inversion-recovery (FLAIR) MR images in Moyamoya disease. Eur J Radiol 55:224–230
Fung LW, Thompson D, Ganesan V (2005) Revascularisation surgery for paediatric Moyamoya: a review of the literature. Childs Nerv Syst 21:358–364
Goda M, Isono M, Ishii K, Kamida T, Abe T, Kobayashi H (2004) Long-term effects of indirect bypass surgery on collateral vessel formation in pediatric Moyamoya disease. J Neurosurg 100:156–162
Houkin K, Kuroda S, Ishikawa T, Abe H (2000) Neovascularization (angiogenesis) after revascularization in Moyamoya disease. Which technique is most useful for Moyamoya disease? Acta Neurochir (Wien) 142:269–276
Kim SH, Choi JU, Yang KH, Kim TG, Kim DS (2005) Risk factors for postoperative ischemic complications in patients with Moyamoya disease. J Neurosurg 103(5 Suppl):433–438
Kim SK, Seol HJ, Cho BK, Hwang YS, Lee DS, Wang KC (2004) Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment. Neurosurgery 54:840–846
Matsushima T, Inoue T, Natori Y, Fujii K, Fukui M, Hasuo K, Kuwabara Y (1994) Children with unilateral occlusion or stenosis of the ICA associated with surrounding moyamoya vessels – “unilateral” Moyamoya disease. Acta Neurochir (Wien) 131:196–202
Scott RM, Smith ER (2009) Moyamoya disease and Moyamoya syndrome. N Engl J Med 360:1226–1237
Suzuki J, Takaku A (1969) Cerebrovascular “Moyamoya” disease: disease showing abnormal net-like vessel in base of brain. Arch Neurol 20:288–299
Yilmaz E, Pritz M, Bruno A, Lopez-Nunez A, Biller J (2001) Moyamoya: Indiana University Medical Center experience. Arch Neurol 58:1274–1278
Yonekawa Y, Ogata N, Kaku Y, Taub E, Imhof HG (1997) Moyamoya disease in Europe, past and present status. Clin Neurol Neurosurg 99(Suppl 2):558–560
Conflict of interest statement
We declare that we have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2011 Springer-Verlag/Wien
About this paper
Cite this paper
Ulrich, P.T., Januschek, E. (2011). Revascularisation Surgery and Long-Term Follow-up in Juvenile Moyamoya Syndrome: A Retrospective Analysis. In: Tsukahara, T., Regli, L., Hänggi, D., Turowski, B., Steiger, HJ. (eds) Trends in Neurovascular Surgery. Acta Neurochirurgica Supplementum, vol 112. Springer, Vienna. https://doi.org/10.1007/978-3-7091-0661-7_8
Download citation
DOI: https://doi.org/10.1007/978-3-7091-0661-7_8
Published:
Publisher Name: Springer, Vienna
Print ISBN: 978-3-7091-0660-0
Online ISBN: 978-3-7091-0661-7
eBook Packages: MedicineMedicine (R0)