An 8-month-old boy with congenital fibromuscular dysplasia presenting with shock caused by sudden renal hemorrhage.
Fibromuscular dysplasia (FMD) is a non-atheromatous, non-inflammatory, multifocal segmental angiopathy. FMD is the most common cause of pediatric renovascular hypertension. Aneurysmal formation of the main renal artery and distal branches is a rare complication of FMD in infancy. We report an 8-month-old boy with FMD presenting with shock caused by sudden renal hemorrhage that necessitated removal of one kidney. A diagnosis of renovascular hypertension resulting from intimal type FMD with aneurysmal formation was made on the basis of the presence of hypertension, elevation of PRA and aldosterone activity, pathological findings and the results of renal angiography. Our findings suggest that it is therefore necessary to consider FMD with aneurysmal formation as a possible cause of hypertension and renal hemorrhage in infants.
Takano
K
K
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
Kawasaki
Y
Y
Imaizumi
T
T
Ohara
S
S
Takeyama
A
A
Suyama
K
K
Hashimoto
K
K
Hosoya
M
M
D000450:Aldosterone / Q000097:blood; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D000959:Antihypertensive Agents / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D005352:Fibromuscular Dysplasia / Q000151:congenital* / Q000000981:diagnostic imaging / Q000628:therapy; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D006801:Humans; D006978:Hypertension, Renovascular / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D007223:Infant; D007668:Kidney / Q000098:blood supply* / Q000473:pathology / Q000601:surgery; D007674:Kidney Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D008297:Male; D009392:Nephrectomy; D011859:Radiography; D012083:Renin / Q000097:blood; D012771:Shock, Hemorrhagic / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy; D016896:Treatment Outcome; D015854:Up-Regulation
D000959:Antihypertensive Agents; D015415:Biomarkers; D000450:Aldosterone; D012083:Renin