Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome
- 1 April 2004
- journal article
- case report
- Published by Springer Science and Business Media LLC in European Journal of Pediatrics
- Vol. 163 (4-5), 241-244
- https://doi.org/10.1007/s00431-003-1312-x
Abstract
We report the unusual case of a 2-month-old boy with systemic fibromuscular dysplasia (FMD). He presented with congenital renovascular hypertension due to stenosis of the right renal artery, and later developed renal infarction on the contralateral side resulting in renal failure. The boy subsequently died of intracranial haemorrhage at the age of 14 months. During the course, hemiconvulsion caused by a Moyamoya disease-like vascular lesion was noted. Stenotic lesions of both the abdominal aorta and its branches were also revealed by angiography. Post-mortem examination confirmed that the coronary, splenic and mesenteric arteries were also affected and their histological findings were compatible with FMD. To our knowledge, this is the first congenital case of FMD demonstrating a rapidly progressive course resulting in a fatal outcome. In this case, multivessels in both intracranial and extracranial arteries were involved. Conclusion:our case suggests that the nature of fibromuscular dysplasia is congenital in origin and its aetiology, at least in some cases, is a systemic abnormality of vascular development.Keywords
This publication has 17 references indexed in Scilit:
- Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta, and its branchesCardiovascular Pathology, 2001
- Multiple intracranial and systemic aneurysms associated with infantile-onset arterial fibromuscular dysplasiaNeurology, 1998
- Severe Hyponatremia in a Patient with Renovascular Hypertension: Case ReportNephron, 1994
- Systemic vascular changes in spontaneous occlusion of the circle of Willis.Stroke, 1991
- Moyamoya Disease Associated with Renovascular HypertensionNeuropediatrics, 1990
- Moyamoya disease associated with renovascular hypertensionHuman Pathology, 1984
- Moyamoya disease--a review.Stroke, 1983
- Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries. A further cause of Moyamoya syndrome.Stroke, 1976
- Occlusive Disease of Intracranial Main Arteries with Collateral Networks in Children1Neuropediatrics, 1973