Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome
| Autor: | Risako Ohtaki, Kazunari Kaneko, Yuki Fukumura, Yuichiro Yamashiro, Y. Iizuka, Kohichi Suda, Tomonosuke Someya, Atsuyuki Yamataka |
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| Rok vydání: | 2003 |
| Předmět: |
Male
medicine.medical_specialty Fibromuscular dysplasia Renovascular hypertension Fatal Outcome medicine.artery Medicine Fibromuscular Dysplasia Humans cardiovascular diseases Moyamoya disease Right Renal Artery Vascular Diseases business.industry Vascular disease Abdominal aorta Angiography Infant Retinal Vessels medicine.disease Coronary Vessels Surgery Stenosis Dysplasia Pediatrics Perinatology and Child Health Blood Vessels Radiology business |
| Zdroj: | European journal of pediatrics. 163(4-5) |
| ISSN: | 0340-6199 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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