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Delayed posterior circulation insufficiency in pediatric moyamoya disease

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Abstract

Approximately 30 % of patients with moyamoya disease (MMD) have presented with involvement of the posterior circulation, mainly the posterior cerebral artery (PCA). Diagnosis of delayed progression of PCA stenosis in MMD may be difficult due to the diversity in clinical features. The goal of this study was to evaluate pediatric MMD patients with delayed PCA involvement after completion of revascularization of the anterior circulation. Forty-one pediatric MMD patients who underwent revascularization of the PCA territory due to delayed posterior circulation insufficiency MMD from 2006 to 2011 were retrospectively reviewed. The average interval between the initial operation and the occipital artery (OA) procedure was 5.0 years. Common symptoms were headaches and transient visual symptoms. The decision to operate was made based on a combination of diagnostic tools. The results obtained with perfusion MRI, SPECT, MR angiography, and EEG supported posterior circulation insufficiency in 78, 41, 73, and 71 % of patients, respectively. Encephaloduroarteriosynangiosis (EDAS) using the OA was performed in 15 patients, and 26 patients received multiple burr hole trephination of the occipital area. All patients showed clinical improvement. Clinicians should be aware of the possibility of delayed involvement of the PCA in pediatric MMD patients. The clinical decision regarding treatment should be based on a combination of symptomatology and the results obtained with various tools to assess whether the blood flow in the PCA territory is insufficient. Surgical treatment using indirect revascularization appears to be effective for patients with delayed PCA involvement.

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References

  1. Cho A, Chae JH, Kim HM, Lim BC, Hwang H, Hwang YS, Phi JH, Kim SK, Wang KC, Cho BK, Kim KJ (2014) Electroencephalography in pediatric moyamoya disease: reappraisal of clinical value. Childs Nerv Syst 30:449–459

    Article  PubMed  Google Scholar 

  2. Choi IJ, Hong SH, Cho BK, Wang KC, Kim SK (2005) Encephalo-duro-arterio-synangiosis (EDAS) using occipital artery in children with moyamoya disease. J Korean Neurosurg Soc 38:413–418

    Google Scholar 

  3. Endo M, Kawano N, Miyaska Y, Yada K (1989) Cranial burr hole for revascularization in moyamoya disease. J Neurosurg 71:180–185

    Article  CAS  PubMed  Google Scholar 

  4. Fukui M (1997) Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘moyamoya’ disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 99(Suppl 2):S238–S240

    Article  PubMed  Google Scholar 

  5. Huang AP, Liu HM, Lai DM, Yang CC, Tsai YH, Wang KC, Yang SH, Kuo MF, Tu YK (2009) Clinical significance of posterior circulation changes after revascularization in patients with moyamoya disease. Cerebrovasc Dis 28:247–257

    Article  PubMed  Google Scholar 

  6. Imaizumi T, Hayashi K, Saito K, Osawa M, Fukuyama Y (1998) Long-term outcomes of pediatric moyamoya disease monitored to adulthood. Pediatr Neurol 18:321–325

    Article  CAS  PubMed  Google Scholar 

  7. Kawaguchi T, Fujita S, Hosoda K, Shose Y, Hamano S, Iwakura M, Tamaki N (1996) Multiple burr-hole operation for adult moyamoya disease. J Neurosurg 84:468–476

    Article  CAS  PubMed  Google Scholar 

  8. Kim SK, Cho BK, Phi JH, Lee JY, Chae JH, Kim KJ, Hwang YS, Kim IO, Lee DS, Lee J, Wang KC (2010) Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol 68:92–101

    Article  PubMed  Google Scholar 

  9. Kim SK, Wang KC, Kim IO, Lee DS, Cho BK (2002) Combined encephaloduroarteriosynangiosis and bifrontal encephalogaleo (periosteal) synangiosis in pediatric moyamoya disease. Neurosurgery 50:88–96

    PubMed  Google Scholar 

  10. Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y (2005) Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 36:2148–2153

    Article  PubMed  Google Scholar 

  11. Lee JY, Kim SK, Cheon JE, Choi JW, Phi JH, Kim IO, Cho BK, Wang KC (2013) Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery. Childs Nerv Syst 29(12):2236–2239

    Article  Google Scholar 

  12. Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Ikota T, Takeuchi S (1984) Study of the posterior circulation in moyamoya disease. Clinical and neuroradiological evaluation. J Neurosurg 61:1032–1037

    Article  CAS  PubMed  Google Scholar 

  13. Moen M, Levine SR, Newman DS, Dull-Baird A, Brown GG, Welch KM (1988) Bilateral posterior cerebral artery strokes in a young migraine sufferer. Stroke 19:525–528

    Article  CAS  PubMed  Google Scholar 

  14. Mugikura S, Higano S, Shirane R, Fujimura M, Shimanuki Y, Takahashi S (2011) Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with moyamoya disease: evidence of angiography-based differences by age at diagnosis. Am J Neuroradiol 32:192–198

    CAS  PubMed  Google Scholar 

  15. Mugikura S, Takahashi S, Higano S, Shirane R, Sakurai Y, Yamada S (2002) Predominant involvement of ipsilateral anterior and posterior circulations in moyamoya disease. Stroke 33:1497–1500

    Article  PubMed  Google Scholar 

  16. Okada Y, Kawamata T, Kawashima A, Yamaguchi K, Ono Y, Hori T (2012) The efficacy of superficial temporal artery-middle cerebral artery anastomosis in patients with moyamoya disease complaining of severe headache. J Neurosurg 116:672–679

    Article  PubMed  Google Scholar 

  17. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK (2005) Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg 103:439–442

    Article  PubMed  Google Scholar 

  18. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299

    Article  CAS  PubMed  Google Scholar 

  19. Yamada I, Himeno Y, Suzuki S, Matsushima Y (1995) Posterior circulation in moyamoya disease: angiographic study. Radiology 197:239–246

    Article  CAS  PubMed  Google Scholar 

  20. Zach V, Bezov D, Lipton RB, Ashina S (2010) Headache associated with moyamoya disease: a case story and literature review. J Headache Pain 11:79–82

    Article  PubMed Central  PubMed  Google Scholar 

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Acknowledgments

This study was supported by a grant of the Korea Healthcare Technology R&D Project, Ministry for Health, Welfare & Family Affairs, Republic of Korea (A120099).

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standard

This study was conducted in accordance with the ethical standards of 1964 Declaration of Helsinki and its later amendments.

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea

    Ji Yeoun Lee, Ji Hoon Phi, Kyu-Chang Wang & Seung-Ki Kim

  2. Division of Pediatric Radiology, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea

    Young-Hun Choi & Jung-Eun Cheon

  3. Departments of Nuclear Medicine and Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea

    Jin Chul Paeng

  4. Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, 110-744, Seoul, Republic of Korea

    Hye Won Ryu, Ki Joong Kim & Jong-Hee Chae

  5. Department of Neurosurgery, The Armed Forces Capital Hospital, Seongnam, Republic of Korea

    Byung-Kyu Cho

Authors
  1. Ji Yeoun Lee
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  2. Young-Hun Choi
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  3. Jung-Eun Cheon
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  4. Jin Chul Paeng
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  6. Ki Joong Kim
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  7. Ji Hoon Phi
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  8. Kyu-Chang Wang
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  9. Byung-Kyu Cho
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  10. Jong-Hee Chae
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  11. Seung-Ki Kim
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Corresponding authors

Correspondence to Jong-Hee Chae or Seung-Ki Kim.

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Lee, J.Y., Choi, YH., Cheon, JE. et al. Delayed posterior circulation insufficiency in pediatric moyamoya disease. J Neurol 261, 2305–2313 (2014). https://doi.org/10.1007/s00415-014-7484-7

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  • DOI: https://doi.org/10.1007/s00415-014-7484-7

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