Fibromuscular dysplasia (FMD) is a rare non-inflammatory, non-atherosclerotic arterial diseasecharacterized by abnormal cellular proliferation and distorted architecture. It commonly affects therenal and extracranial carotid and vertebral arteries, but nearly all arterial beds may be affected,and multivessel involvement is common. We report a rare case of a 31-year-old Filipino femalewho presented with a rapidly enlarging right anterior axillary mass. Initial consideration was ableeding soft tissue sarcoma as seen on chest CT scan, while whole abdominal CT scan withIV contrast revealed incidental findings of multiple aneurysms in the superior mesenteric artery,both renal arteries and right common iliac artery, suggestive of fibromuscular dysplasia. Furthervascular imaging studies showed a looped left internal carotid, and a tortuous left brachial arterywith beading pattern.

Human ; Female ; Adult: 25-44 Yrs Old ; Fibromuscular Dysplasia

Infant, Newborn ; Humans ; Fibromuscular Dysplasia/complications* ; Renal Artery ; Hypertension, Renovascular/etiology* ; Renal Artery Obstruction/complications* ; Hypertension/complications*

Fibromuscular Dysplasia/diagnostic imaging* ; Humans ; Hypertension/complications* ; Hypertension, Renovascular/etiology* ; Kidney ; Renal Artery/diagnostic imaging*

Renal artery stenosis (RAS) is one of the most common primary diseases of the renal arteries. It is associated with hypertension as well as ischemic renal disease. Its concomitant presence constitutes a high cardiovascular risk, thus timely diagnosis is important. This study aims to determine the occurrence of RAS in patients undergoing abdominal CT angiography and determines other factors that predict the highest-risk subset who have signifi cant renal artery narrowing. A retrospective review of records of 470 patients who underwent CT angiography of the whole abdomen was done. RAS occurs in about 9.57% of patients who had a CT scan of the whole abdomen. Older patients, patients with heart disease and patients with more atherosclerotic vessels in the abdomen increases the risk for signifi cant RAS (p values of 0.005, 0.008 and <0.001, respectively). Diabetes, hypertension as well as other imaging fi ndings such as the presence of aneurysm elsewhere seen in the abdominal scans and renal artery duplication do not statistically correlate with the increased risk for RAS in this study
Renal Artery Obstruction ; Computed Tomography Angiography ; Atherosclerosis ; Fibromuscular Dysplasia ; Hypertension

No abstract available.
Aneurysm ; Carotid Artery, Internal ; Fibromuscular Dysplasia ; Oculomotor Nerve Diseases ; Oculomotor Nerve

Fibromuscular dysplasia (FMD) of the coronary artery is a rare cause of sudden cardiac death; however, its prevalence and fatality may have been overlooked so far. A 47-year-old man complained of pain in his back and shoulder and became unconscious. Despite resuscitation, he died 3 hours after symptom onset. The heart weight was in the normal range; however, all three major coronary arteries showed intimal thickening without atherosclerosis or inflammatory cell infiltration. Fragmentations and duplications of the internal elastic lamina which are histologic features of intimal fibroplasia, a focal-type FMD, were observed. The prevalence of coronary FMD remains unknown, although it may be related to spontaneous coronary artery dissection and sudden death. The histopathologic confirmation of coronary FMD and exclusion of other possible coronary diseases through autopsy are essential to reveal the nature of the disease and therefore apply the information in dealing with legal problems after death.
Atherosclerosis ; Autopsy ; Cause of Death ; Coronary Disease ; Coronary Vessels ; Death, Sudden ; Death, Sudden, Cardiac ; Fibromuscular Dysplasia ; Heart ; Humans ; Middle Aged ; Prevalence ; Reference Values ; Resuscitation ; Shoulder ; Tunica Intima

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.
Angiography ; Arteritis ; Connective Tissue ; Ehlers-Danlos Syndrome* ; Fibromuscular Dysplasia ; Flank Pain ; Hand Joints ; Joints ; Korea ; Marfan Syndrome ; Neurofibromatoses ; Polyarteritis Nodosa ; Renal Artery* ; Skin ; Tuberculosis

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.
Child* ; Child, Preschool ; Diuretics ; Female ; Femoral Artery ; Fibromuscular Dysplasia ; Glomerulosclerosis, Focal Segmental* ; Heparin ; Humans ; Iliac Artery ; Incidence ; Leg ; Mycoplasma pneumoniae ; Nephrotic Syndrome ; Osteochondroma ; Pneumonia, Mycoplasma ; Popliteal Artery ; Renal Veins ; Steroids ; Thrombectomy ; Thrombosis* ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior ; Warfarin

No abstract available.
Adult ; Angiography, Digital Subtraction ; Angioplasty, Balloon ; *Blood Pressure ; Female ; Fibromuscular Dysplasia/*complications/diagnosis ; Humans ; Hypertension, Renovascular/diagnosis/*etiology/physiopathology/therapy ; Renal Artery Obstruction/diagnosis/*etiology/physiopathology/therapy ; Treatment Outcome

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