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Neuropediatrics 2003; 34(6): 318-321
DOI: 10.1055/s-2003-44664
Short Communication

Georg Thieme Verlag Stuttgart · New York

Moyamoya and Extracranial Vascular Involvement: Fibromuscular Dysplasia? A Report of Two Children

R. R. de Vries 1 , P. G. J. Nikkels 2 , J. van der Laag 3 , G. Broere 4 , K. P. J. Braun 1
  • 1Department of Child Neurology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
  • 2Department of Pathology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
  • 3Department of Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
  • 4Department of Neurosurgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
Further Information

Publication History

Received: July 23, 2003

Accepted after Revision: September 9, 2003

Publication Date:
18 December 2003 (online)

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Abstract

We present two patients with moyamoya syndrome and the unusual involvement of extracranial vessels. The first case illustrates the rare association between moyamoya and primary pulmonary hypertension. In the second patient, moyamoya was complicated by stenoses of vertebral, renal, and mesenteric arteries. In both cases, a systemic intima-proliferative disease, such as fibromuscular dysplasia (FMD), was suspected to be the cause of both intracranial and extracranial arterial disease.

Key words

Moyamoya - fibromuscular dysplasia - pulmonary hypertension

References

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M. D., Ph.D. K. P. J. Braun

Department of Child Neurology
Wilhelmina Children's Hospital
University Medical Center Utrecht

P.O. Box 85090

3508 AB Utrecht

The Netherlands

Email: K.Braun@wkz.azu.nl