We found a case of hypoplasia of vertebral artery with fibromuscular dysplasia in an 82-yr-old Korean female cadaver during a routine dissection course. In the present case, intracranial hypoplasia in left vertebral artery and bilateral origin of posterior inferior cerebellar artery at the vertebrobasilar junction were recognized. Histopathologically, left vertebral artery showed intimal type of fibromuscular dysplasia both in its extracranial and intracranial courses. These results indicate that the association of fibromuscular dysplasia and hypoplasia does exist in the vertebral artery, although the etiologies are not verified yet.
Aged, 80 and over ; Female ; Fibromuscular Dysplasia/*pathology ; Heart Ventricles/abnormalities/*pathology ; Humans ; Republic of Korea ; Vertebral Artery/*pathology

Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.
Adult ; Arterial Occlusive Diseases/*pathology ; Child ; Female ; Fibromuscular Dysplasia/complications/epidemiology/*pathology ; Humans ; Hypertension, Renovascular/*etiology/surgery ; Korea/epidemiology ; Renal Artery/*pathology/surgery

OBJECTIVETo characterize the clinical and angiographic features in children with renovascular hypertension.

METHODClinical data of 14 children (7 male, 7 female; age 0.8-14 years, mean 8.7 years), who were diagnosed with renovascular hypertension by renal angiography in our institute from January 2005 to December 2012 were collected and retrospectively analyzed.

RESULTThe mean blood pressure at the diagnosis was 187/127 mm Hg. Chief complaints of symptomatic patients were headache (29%, 4/14), hypertensive encephalopathy (36%, 5/14), signs of congestive heart failure (14%, 2/14) and hematemesis (7%, 1/14). Renovascular hypertension was found incidentally in 14% (2/14) of patients who were asymptomatic. Conventional renal angiography elucidated the anatomical distribution of lesions in the renal arterial system. It was found that 14% (2/14) of patients had bilateral disease, 50% (7/14) had single stenosis at main or accessory renal artery, while multiple stenoses was seen in 43% (6/14) of children, with involvement of segmental renal artery and small interlobar or arcuate vessels. Compared with catheter angiography, 50% (7/14) of patients with renovascular hypertension, especially intrarenal arterial disease, were missed on computed tomography angiography or magnetic resonance angiography.

CONCLUSIONIt is mandatory to emphasize blood pressure measurement in pediatric clinical practice for early recognition of renovascular hypertension. As children with renovascular hypertension display involvement of multiple arteries, including in smaller intrarenal arteries, digital subtraction angiography is the only method that can reliably diagnose pediatric renovascular hypertension.

Adolescent ; Angiography, Digital Subtraction ; Child ; Child, Preschool ; Female ; Fibromuscular Dysplasia ; diagnosis ; diagnostic imaging ; Humans ; Hypertension, Renovascular ; diagnosis ; diagnostic imaging ; Infant ; Kidney ; diagnostic imaging ; pathology ; Magnetic Resonance Angiography ; Male ; Renal Artery ; diagnostic imaging ; pathology ; Renal Artery Obstruction ; diagnosis ; diagnostic imaging ; Retrospective Studies ; Sensitivity and Specificity ; Tomography, X-Ray Computed