1/45. Solitary renal myofibromatosis: an unusual cause of infantile hypertension.INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4 protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/45. Staged thoracic and abdominal aortic aneurysm repair using stent graft technology and surgery in a patient with acute renal failure.A 52-year-old male presented with severe hypertension and acute renal failure. carbon dioxide (CO(2)) angiography identified a saccular thoracic aortic aneurysm, right renal artery stenosis, left renal artery occlusion, an infrarenal aortic aneurysm, celiac artery, and inferior mesenteric artery (IMA) orificial stenoses. Via an anterior retroperitoneal approach, bilateral renal artery thromboendarterectomy, infrarenal aortic aneurysmectomy, and IMA reimplantation were performed. The patient's tortuous iliac arteries were straightened to permit future passage of a thoracic stent graft by mobilizing the aortic bifurcation and anastomosing it to a Dacron graft within 4 cm of the renal vessels. Two weeks later, a stent graft was placed via a femoral incision utilizing CO(2) angiography, successfully excluding the saccular thoracic aneurysm. Recovery from both procedures was quick, with rapid return of renal function, and alleviation of the hypertension. At 8 months follow-up, his renal arteries and aorta are patent.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
3/45. fibromuscular dysplasia: a rare cause of cilioretinal artery occlusion in childhood.OBJECTIVE: To report a case of cilioretinal artery occlusion with angiographic findings characteristic of the "string of beads" associated with renovascular hypertension secondary to fibromuscular dysplasia of the renal artery in a child. DESIGN: Case report. INTERVENTION: The patient underwent ex vivo renal artery reconstruction with saphenous vein graft and reimplantation. MAIN OUTCOME MEASURES: The main clinical outcomes were control of severe hypertension, reversible hypertensive retinopathy, and improvement of vision. RESULTS: Revascularization of the kidney improved renal function, and renovascular hypertension was clearly improved. visual acuity improved to 20/200. CONCLUSIONS: A child with hypertensive retinopathy and arterial occlusion in the retina should undergo investigation to rule out a surgically curable hypertension. magnetic resonance angiography of extrarenal vessels may reveal other sites of involvement of fibromuscular dysplasia. Evaluation and early diagnosis of renovascular hypertension will prevent severe end-organ damage.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/45. magnetic resonance imaging for early detection of takayasu arteritis.The patient was a 15-year-old girl with suspected takayasu arteritis. Magnetic resonance (MR) imaging, using the fast low angle shot (FLASH) technique, revealed aneurysmal dilation of the aortic root and irregular thickening of the aortic wall. Three-dimensional (3-D) contrast-enhanced MR angiography successfully demonstrated narrowing of the left carotid and left subclavian arteries. In addition, 2-dimensional (2-D) FLASH MR imaging clearly visualized narrowing of two right renal arteries and the left renal artery. These were angiographically evident. Thus, MR imaging is particularly useful for early detection of subtle arteritic changes and involvement of stenotic lesions in branch vessels in the early phase of takayasu arteritis.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
5/45. moyamoya disease associated with renovascular disease in a young African-Brazilian patient.Moyamoya is a rare disease characterized by fibrous dysplasia of the internal carotid and proximal cerebral arteries, which has been described mainly in young Japanese. We present a case of moyamoya disease with renal artery involvement in a young male patient with an African origin. A 15-year-old boy was referred to our hospital due to uncontrolled blood pressure, headache, somnolence, cognitive deficit and multiple lacunar infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular anatomy at the level of the circle of willis. The intracranial vessels presented severe stenosis or were occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriography showed a tight stenosis of the ostium and proximal segment of right main artery, which was only partially relieved by balloon angioplasty. A saphenous bypass from aorta to the right renal artery re-established the renal blood flow. blood pressure dropped after surgery and was controlled with low doses of diuretic and beta-blocker. After arteriography he presented right hemiplegia, with partial recovering in the following months. In conclusion, we described the first case of moyamoya disease with concomitant renovascular disease in a young adult of African origin, an association that may be more frequent than usually suspected in clinical practice.- - - - - - - - - - ranking = 0.66666666666667keywords = vessel (Clic here for more details about this article) |
6/45. role of the captopril test in renovascular hypertension: a case report.This article reports the case of a rapidly severe stenosis of the right renal artery, causing uncontrolled hypertension. After failure of a percutaneous transluminal renal angioplasty, which provoked the thrombosis of the vessel, a surgical revascularization was performed after /- eighteen hours of renal ischemia. blood pressure, blood urea nitrogen and serum creatinine returned to normal values. A dramatic improvement of the right renal function was attested at the hippuran scintigraphy after a dose test of captopril. The results of renographic studies obtained in this clinical case underline the role of the captopril radionuclide test in detection and follow-up after treatment of renovascular hypertension.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
7/45. renal artery aneurysm: successful exclusion with a stent graft.renal artery aneurysms are uncommon. They are either incidentally discovered or identified during workup for secondary hypertension. An association with rupture at sizes greater than 1.5 cm exists. In this case report, the exclusion of a renal aneurysm with a covered stent is described. In spite of compromise of a side-branch vessel, there were no deleterious effects on renal function at 6-month follow-up.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
8/45. Post-stenotic dilatation: challenge in renal ostial stenting--a simple solution.Stenting is the treatment of choice for treating stenotic renal ostial lesions. During the stenting of an ostial lesion in a renal artery with post-stenotic dilatation, we were faced with the problems of unavailability of a balloon of appropriate length and diameter, and determining the real reference vessel diameter. The problem was solved by a simple technique.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
9/45. Report of a case with surprising etiology of renovascular hypertension.takayasu arteritis is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. Epidemiologically, it is found mostly in female patients and is more prevalent in Asian and Latin American countries. Disease may be heterogeneous in presentation. In this report, we present a different clinical expression of takayasu arteritis in a young man who had hypertension as the sole manifestation of multiple critical arterial involvement with elevated inflammation markers but no other symptoms. A 28-year-old man was admitted with hypertension. There was no evidence for systemic vasculitis by history, serologic studies or other laboratory data. The acute-phase reactants were elevated with an erythrocyte sedimentation rate of 55 mm/h, and a c-reactive protein value of 22 mg/dl. Digital subtraction angiography showed multiple severe stenoses or occlusions of the branches of the abdominal aorta and arcus aortae together with bilateral renal artery involvement. The etiology of renovascular hypertension was found to be takayasu arteritis with the presence of at least three criteria, as outlined by the American College of rheumatology in 1990. patients with takayasu arteritis may have atypical clinical expression of the disease, and a diagnosis of takayasu arteritis should be kept in mind in the differential diagnosis of renovascular hypertension in young subjects, even if they do not have associated symptoms of multiple arterial involvement.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
10/45. Severe renovascular hypertension in an infant with congenital solitary pelvic kidney.renal artery stenosis (RAS) is one of the most common causes of severe arterial hypertension in infants. Its management is very difficult, especially when present in a single kidney. We report a case of severe hypertension caused by RAS of congenital single pelvic kidney in a 4-month-old boy. The patient presented with cardiorespiratory insufficiency that was first treated as acute fulminate myocarditis. Medical treatment of arterial hypertension was disappointing, as it had to be balanced between congestive cardiac failure and acute renal failure. Percutaneous transluminal angioplasty (PTA) done by coronary balloon dilatation catheters through the left axillary access was successful. Following dilatation of the renal artery, blood pressure decreased and its good control was possible by only one drug. With improved medical blood pressure control and normal growth development, the reassessment of clinical therapy options adjusted to a larger vessel size would be possible. Renovascular hypertension due to RAS in infants with a solitary kidney is difficult to control by medical treatment alone. PTA should be considered as a viable option in infants with refractory hypertension due to renal artery stenosis in a solitary kidney, since it has the potential of improving hypertension while preserving renal function.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
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