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.
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Abbreviations
- FMD :
-
fibromuscular dysplasia
- MMD :
-
Moyamoya disease
- RVH :
-
renovascular hypertension
- US :
-
ultrasound
References
Choi Y, Kang BC, Kim KJ, Cheong HI, Hwang YS, Wang KC, Kim IO (1997) Renovascular hypertension in children with moyamoya disease. J Pediatr 131: 258–263
Currie AD, Bentley CR, Bloom PA (2001) Retinal haemorrhage and fatal stroke in an infant with fibromuscular dysplasia. Arch Dis Child 84: 263–264
Dillon MJ, Ryness JM (1975) Plasma renin activity and aldosterone concentration in children. BMJ 4: 316–319
Halonen H, Halonen V, Donner M, Iivanainen M, Vuolio M, Makinen J (1973) Occlusive disease of intracranial main arteries with collateral networks in children. Neuropädiatrie 4: 187–206
Ikeda E (1991) Systemic vascular changes in spontaneous occlusion of the circle of Willis. Stroke 22: 1358–1362
Jansen JN, Donker AJ, Luth WJ, Smit LM (1990) Moyamoya disease associated with renovascular hypertension. Neuropediatrics 21: 44–47
Kaneko K, Shimazaki S, Ino T, Yabuta K, Nakazawa T, Takahashi H, Kaneko K (1994) Severe hyponatremia in a patient with renovascular hypertension: case report. Nephron 68: 252–255
Lee EK, Hecht ST, Lie JT (1998) Multiple intracranial and systemic aneurysms associated with infantile-onset arterial fibromuscular dysplasia. Neurology 50: 828–829
Lindner V (2001) Vascular repair processes mediated by transforming growth factor-beta. Z Kardiol 90[Suppl 3]: 17–22
Maresi E, Becchina G, Ottoveggio G, Orlando E, Midulla R, Passantino R (2001) Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta, and its branches. Cardiovasc Pathol 10: 43–48
Nakano T, Azuma E, Ido M, Itoh M, Sakurai M, Suga S, Kawaguchi H (1993) Moyamoya disease associated with bilateral renal artery stenosis. Acta Paediatr Jpn 35: 354–357
Pesonen E, Koskimies O, Rapola J, Jaakelainen J (1980) Fibromuscular dysplasia in a child: a generalized arterial disease. Acta Paediatr Scand 69: 563–536
Pilz P, Hartjes HJ (1976) Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries. A further cause of moyamoya syndrome. Stroke 7: 393–398
Price RA, Vawter GF (1972) Arterial fibromuscular dysplasia in infancy and childhood. Arch Pathol 93: 419–426
Rushton AR (1980) The genetics of fibromuscular dysplasia. Arch Intern Med 140: 233–236
Suzuki J, Kodama N (1983) Moyamoya disease–a review. Stroke 14: 104–109
Yamada I, Himeno Y, Matsushima Y, Shibuya H (2000) Renal artery lesions in patients with moyamoya disease: angiographic findings. Stroke 31: 733–737
Yamashita M, Tanaka K, Kishikawa T, Yokota K (1984) Moyamoya disease associated with renovascular hypertension. Hum Pathol 15: 191–193
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Kaneko, K., Someya, T., Ohtaki, R. et al. Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome. Eur J Pediatr 163, 241–244 (2004). https://doi.org/10.1007/s00431-003-1312-x
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DOI: https://doi.org/10.1007/s00431-003-1312-x