Systemic Arterial Involvement in Moyamoya Disease

  • Hae Il Cheong
  • Yong Choi


Moyamoya disease is a rare cerebrovascular occlusive disorder that is characterized by stenosis or occlusion of the distal internal carotid or proximal anterior or middle cerebral arteries, which causes the formation of multiple tiny collateral vascular networks (moyamoya vessels) at the base of 1the brain [1, 2, 3]. While this disorder commonly occurs alone (moyamoya disease), it occasionally occurs with well-recognized associated conditions including sickle cell disease, neurofibromatosis type I, cranial therapeutic irradiation, and Down syndrome (moyamoya syndrome) [4].


Renal Artery Down Syndrome Balloon Angioplasty Renal Artery Stenosis Moyamoya Disease 
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  1. 1.
    Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya”disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299PubMedCrossRefGoogle Scholar
  2. 2.
    Suzuki J, Kodama N (1983) Moyamoya disease: a review. Stroke 14:104–109PubMedCrossRefGoogle Scholar
  3. 3.
    Nishimoto A, Takeuchi S (1968) Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29:255–260PubMedCrossRefGoogle Scholar
  4. 4.
    Scott RM, Smith ER (2009) Moyamoya disease and moyamoya syndrome. N Engl J Med 360:1226–1237PubMedCrossRefGoogle Scholar
  5. 5.
    Kapusta L, Daniëls O, Renier WO (1990) Moyamoya syndrome and primary pulmonary hypertension in childhood. Neuropediatrics 21:162–163PubMedCrossRefGoogle Scholar
  6. 6.
    Goldenberg HJ (1974) “Moyamoya”associated with peripheral vascular occlusive disease. Arch Dis Child 49:964–966PubMedCrossRefGoogle Scholar
  7. 7.
    Ikeda E (1991) Systemic vascular changes in spontaneous occlusion of the circle of Willis. Stroke 22:1358–1362PubMedCrossRefGoogle Scholar
  8. 8.
    De Vries RR, Nikkels PG, van der Laag J et al (2003) Moyamoya and extracranial vascular involvement: fibromuscular dysplasia? A report of two children. Neuropediatrics 34:318–321Google Scholar
  9. 9.
    Weber C, Tatò F, Brandl T et al (2001) Adult moyamoya disease with peripheral artery involvement. J Vasc Surg 34:943–946PubMedCrossRefGoogle Scholar
  10. 10.
    Komiyama M, Nishikawa M, Yasui T et al (2001) Moyamoya disease and coronary artery disease – case report. Neurol Med Chir (Tokyo) 41:37–41CrossRefGoogle Scholar
  11. 11.
    Ou P, Dupont P, Bonnet D (2006) Fibromuscular dysplasia as the substrate for systemic and pulmonary hypertension in the setting of Moya-Moya disease. Cardiol Young 16:495–497PubMedCrossRefGoogle Scholar
  12. 12.
    Godin M, Helias A, Tadie M et al (1978) Moyamoya syndrome and renal artery stenosis. Kidney Int 15:450Google Scholar
  13. 13.
    Ellison PH, Largent JA, Popp AJ (1981) Moya-moya disease associated with renal artery stenosis. Arch Neurol 38:467PubMedCrossRefGoogle Scholar
  14. 14.
    Terasawa K, Yamaguchi Y, Ishihara O et al (1983) Moya-moya disease associated with fibromuscular dysplasia of renal artery. No To Hattatsu 15:350–355PubMedGoogle Scholar
  15. 15.
    Yamashita M, Tanaka K, Kishikawa T et al (1984) Moyamoya disease associated with renovascular hypertension. Hum Pathol 15:191–193PubMedCrossRefGoogle Scholar
  16. 16.
    Halley SE, White WB, Ramsby GR et al (1988) Renovascular hypertension in moyamoya syndrome. Therapeutic response to percutaneous transluminal angioplasty. Am J Hypertens 1:348–352PubMedCrossRefGoogle Scholar
  17. 17.
    Jansen JN, Donker AJ, Luth WJ et al (1990) Moyamoya disease associated with renovascular hypertension. Neuropediatrics 21:44–47PubMedCrossRefGoogle Scholar
  18. 18.
    Rupprecht T, Wenzel D, Schmitzer E et al (1992) Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography. Pediatr Radiol 22:527–528PubMedCrossRefGoogle Scholar
  19. 19.
    Nakano T, Azuma E, Ido M et al (1993) Moyamoya disease associated with bilateral renal artery stenosis. Acta Paediatr Jpn 35:354–357PubMedCrossRefGoogle Scholar
  20. 20.
    Van der Vliet JA, Zeilstra DJ, Van Roye SF et al (1994) Renal artery stenosis in moyamoya syndrome. J Cardiovasc Surg (Torino) 35:441–443Google Scholar
  21. 21.
    Shoskes DA, Novick AC (1995) Surgical treatment of renovascular hypertension in moyamoya disease: case report and review of the literature. J Urol 153:450–452PubMedCrossRefGoogle Scholar
  22. 22.
    T akagi Y, Hashimoto N, Goto Y (1997) Haemodynamic ischaemia in paediatric moyamoya disease associated with renovascular hypertension. Acta Neurochir (Wien) 139:257–258CrossRefGoogle Scholar
  23. 23.
    Akasaki T, Kagiyama S, Omae T et al (1998) Asymptomatic moyamoya disease associated with coronary and renal artery stenosis – a case report. Jpn Circ J 62:136–138PubMedCrossRefGoogle Scholar
  24. 24.
    Kuwayama F, Hamasaki Y, Shinagawa T et al (2001) Moyamoya disease complicated with renal artery stenosis and nephrotic syndrome: reversal of nephrotic syndrome after nephrectomy. J Pediatr 138:418–420PubMedCrossRefGoogle Scholar
  25. 25.
    Caldarelli M, Di Rocco C, Gaglini P (2001) Surgical treatment of moyamoya disease in pediatric age. J Neurosurg Sci 45:83–91PubMedGoogle Scholar
  26. 26.
    Hoshino Y, Nakano A, Oguri M et al (2001) Intravascular ultrasound detects coarctation of the renal artery in a patient with Moyamoya disease. Hypertens Res 24:283–287PubMedCrossRefGoogle Scholar
  27. 27.
    Fuchs FD, Francesconi CR, Caramori PR et al (2001) Moyamoya disease associated with renovascular disease in a young African-Brazilian patient. J Hum Hypertens 15:499–501PubMedCrossRefGoogle Scholar
  28. 28.
    Kaczorowska M, Józwiak S, Litwin M et al (2005) Moyamoya disease associated with stenosis of extracranial arteries: a case report and review of the literature. Neurol Neurochir Pol 39:242–246PubMedGoogle Scholar
  29. 29.
    Choi Y, Kang BC, Kim KJ et al (1997) Renovascular hypertension in children with moyamoya disease. J Pediatr 131:258–263PubMedCrossRefGoogle Scholar
  30. 30.
    Yamada I, Himeno Y, Matsushima Y et al (2000) Renal artery lesions in patients with moyamoya disease. Angiographic findings. Stroke 31:733–737CrossRefGoogle Scholar
  31. 31.
    Togao O, Mihara F, Yoshiura T et al (2004) Prevalence of stenoocclusive lesions in the renal and abdominal arteries in moyamoya disease. Am J Radiol 183:119–122Google Scholar
  32. 32.
    Safian RD, Textor SC (2001) Renal artery stenosis. N Engl J Med 344:431–442PubMedCrossRefGoogle Scholar
  33. 33.
    Castellote E, Romero R, Bonet J et al (1995) Takayasu's arteritis as a cause of renovascular hypertension in a non-Asian population. J Hum Hypertens 9:841–845PubMedGoogle Scholar
  34. 34.
    Hosoda Y (1984) Pathology of so-called “spontaneous occlusion of the circle of Willis.”Pathol Annu 2:221-244Google Scholar
  35. 35.
    Deal JE, Snell MF, Barratt TM et al (1992) Renovascular disease in childhood. J Pediatr 121:378–384PubMedCrossRefGoogle Scholar
  36. 36.
    Ellis D, Shapiro R, Scantleburg VP et al (1995) Evaluation and management of bilateral renal artery stenosis in children: a case series and review. Pediatr Nephrol 9:259–267PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  • Hae Il Cheong
    • 1
    • 2
    • 3
  • Yong Choi
    • 1
    • 2
  1. 1.Department of PediatricsSeoul National University Children's HospitalJongno-guRepublic of Korea
  2. 2.Kidney Research InstituteMedical Research Center, Seoul National University College of MedicineJongno-guRepublic of Korea
  3. 3.Research Center for Rare DiseaseSeoul National University HospitalJongno-guRepublic of Korea

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