Congenital fibromuscular dysplasia involving multivessels in an infant with fatal outcome.
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.


CONCLUSIONS
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.
Department of Paediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, 113-8421 Tokyo, Japan. knkkznr@juntendo-urayasu.jp
Kazunari
K
Kaneko
Tomonosuke
T
Someya
Risako
R
Ohtaki
Yuichiro
Y
Yamashiro
Atsuyuki
A
Yamataka
Yuo
Y
Iizuka
Yuki
Y
Fukumura
Kohichi
K
Suda
D000792:Angiography; D001808:Blood Vessels / Q000473:pathology*; D003331:Coronary Vessels / Q000473:pathology; D017809:Fatal Outcome; D005352:Fibromuscular Dysplasia / Q000150:complications* / Q000151:congenital; D006801:Humans; D007223:Infant; D008297:Male; D012171:Retinal Vessels / Q000473:pathology; D014652:Vascular Diseases / Q000209:etiology