Asymptomatic renal infarction, due to fibromuscular dysplasia, in a young woman with 11 years of follow-up

We report a 27-year-old woman with renovascular hypertension, renal infarction, and hepatic artery aneurysm due to fibromuscular dysplasia. The patient was first noted to have renal artery aneurysm and hepatic artery aneurysm at the age of 17. The renal infarction was asymptomatic and was incidentally detected by magnetic resonance imaging (MRI) examination. Because of the rather peripheral location of the aneurysms, percutaneous transluminal renal artery angioplasty was considered inappropriate. This case suggests the need for long-term and periodical follow-up of patients with fibromuscular dysplasia.     

Rapid Progression of Native Renal Artery Fibromuscular Dysplasia Following Kidney Donation

Fibromuscular dysplasia is the second commonest anatomical abnormality apart from multiple renal arteries in the potential live donors. Pretransplant evaluation of the donors may include an angiography to evaluate the renal arteries, and failure to recognize renal arterial stenosis, particularly fibromuscular dysplasia, by noninvasive methods may eventually lead to hypertension and ischemic renal failure. We report a case of fibromuscular dysplasia that was undetected by computed tomographic angiography prior to donation. One year after kidney donation, it rapidly progressed to severe symptomatic stenosis with hypertension and acute renal failure. Following renal artery angioplasty, her blood pressure normalized over a period of 2 weeks without any need for antihypertensive medications and the serum creatinine returned to her baseline. The acceptability of renal donors with fibromuscular dysplasia depends on the age, race and the availability of the other suitable donors. Mild fibromuscular dysplasia in a normotensive potential renal donor cannot be considered a benign condition. Such donors need regular follow-up postdonation for timely detection and treatment.     

Renal fibromuscular dysplasia

Renal artery fibromuscular dysplasia is a noninflammatory, nonatherosclerotic vasculopathy that can affect renal arteries at various degrees with different severity. The etiology is still unknown, but there is a strong belief that a genetic disorder is the main cause for the pathogenesis of this disease. The main presentation is a sudden onset of recalcitrant hypertension at a young age, which is usually resistant to medical treatment. Once renal artery fibromuscular dysplasia is suspected, several diagnostic tools are available to make an accurate diagnosis. The advent of minimally invasive interventions has revolutionized the options for treatment. This update should provide the clinician with a base understanding of available evidence for diagnosing and treating renal artery fibromuscular dysplasia.     

Renal arteriovenous fistula associated with fibromuscular dysplasia

A case is reported of a woman with long-standing progressive hypertension and an abdominal bruit who on angiography demonstrated fibromuscular dysplasia of the right renal artery and an arteriovenous fistula in the upper pole of the right kidney. The various causes of renal arteriovenous fistula are reviewed. The relationship of mural aneurysms seen in fibromuscular dysplasia and the formation of arteriovenous fistulas by rupture of these aneurysms is discussed. Review of the English literature indicates a probable causal relationship between fibromuscular dysplasia of the renal arteries and intrarenal arteriovenous fistulas.     

Prognostic significance of intrarenal resistance indices in patients with renal artery interventions: a preliminary duplex sonographic study

No non-invasive test can predict the clinical outcome of renal revascularization procedures. Because duplex sonographic measurements of intrarenal flow patterns reflect the resistance to flow within the kidney, the prognostic value of the cortical end-diastolic to peak systolic (d/s) velocity ratio was investigated in patients undergoing intervention for renal artery stenosis. The clinical and duplex sonographic data on 32 patients with 35 interventions (30 percutaneous transluminal angioplasties and five operations) on 42 renal artery sides were analysed. Twenty-three patients had atherosclerotic renal artery stenosis and nine patients had fibromuscular dysplasia resulting in ≥60% renal artery stenosis. Measurements of the renal to aortic velocity ratio and cortical d/s ratio were performed before and after intervention. In the atherosclerotic patients, three interventions were clinically and technically successful, eight were technically successful but clinical failures, and 14 were clinically and technically unsuccessful. In the fibromuscular dysplasia patients, eight interventions were clinically and technically successful, and two were clinically and technically unsuccessful. The difference between the corresponding d/s ratios for atherosclerotic and fibromuscular dysplasia sides was significant on both the treated and not-treated sides (P < 0.02, two-tailed unpaired t-test). None of the 11 clinically successful procedures had a d/s ratio below 0.3, compared with seven values below 0.3 in the 24 clinically unsuccessful interventions (P = 0.05, one-tailed Fisher's exact test). It is concluded that: (1), a d/s ratio below 0.3 correlates with clinical failure in subsequent treatment of hypertension by renal revascularization, while a value above 0.3 has no prognostic significance; (2), despite technical success, not all atherosclerotic patients have clinical success from renal artery interventions; (3), in fibromuscular dysplasia patients, all clinical failures of renal artery interventions are associated with technical failures; and (4), the difference in d/s ratio between atherosclerotic and fibromuscular dysplasia patients may be a consequence of the more advanced age, longer duration of hypertension and additional risk factors in atherosclerotic patients.     

Percutaneous transluminal angioplasty for renovascular hypertension in children

Percutaneous transluminal angioplasty (PTA) has been developed over the past 8 years as an alternative to reconstructive surgery for renovascular hypertension. We report three cases and review the use of PTA in children with renal artery stenosis. At least 37 cases of PTA have been reported in patients whose ages ranged from 1.3 to 17 years (mean 10 years). Of these, 10 had fibromuscular dysplasia; 13 unspecified unilateral renal artery stenosis; 4 bilateral stenosis; 4 neurofibromatosis; 4 renal transplant; 1 atherosclerosis; and 1 postsurgical stenosis. Nine of 10 patients with fibromuscular dysplasia were cured and 3 of 4 with renal transplant arterial stenosis were cured or improved. There were 11 failures of PTA, including all 4 patients with neurofibromatosis and 1 with transplant arterial stenosis. We conclude that PTA is the treatment of choice for children with hypertension due to fibromuscular dysplasia and should be attempted for stenosis of the transplanted renal artery. Other lesions resulting in renal artery stenosis may not be as amenable to dilation and should be considered on an individual basis.R. L. Chevalier is an Established Investigator of the American Heart Association     

Calcified renal artery aneurism and high blood pressure. A case report and review of the literature

Renal artery aneurysm is a rare disease in children and usually is due to fibromuscular dysplasia. Clinical symptoms are frequently high blood pressure, abdominal pain, and hematuria. Diagnosis is carried out by means of angiography. We report the case of a 13-year-old male patient who had renovascular hypertension due to calcification and aneurysm of fibromuscular dysplasia-associated renal artery. We carried out total nephrectomy to resolve high blood pressure. We suggested that presence of discreet calcification in region of renal artery in a boy with renovascular high blood pressure should guide us toward diagnosis of fibromuscular dysplasia-related renal artery aneurysm.     

Ask-Upmark kidney with contralateral renal artery fibromuscular dysplasia

A case of Ask-Upmark kidney with coexistent contralateral renal artery fibromuscular dysplasia is presented with detailed light and electron microscopic findings. Both lesions are known to produce renin-mediated hypertension which was corrected in this patient by nephrectomy and contralateral renal artery bypass. Ask-Upmark kidney is briefly reviewed.     

Multivisceral Fibromuscular Dysplasia in Childhood: Case Report and Review of the Literature

We report here a 9-year-old girl with fibromuscular dysplasia of many muscular arteries including both renal and internal carotid arteries, the celiac artery, superior mesenteric artery, and one external carotid artery. She suffered from severe renovascular hypertension with beginning secondary cardiac decompensation, typical angina abdominalis, and neurological signs, including severe headaches and hemianopsia. Surgery was performed for all major vessels and the outcome is good 2.5 years after the operation. The clinical presentation, differential diagnosis, and treatment options of fibromuscular dysplasia in childhood are discussed and the literature is reviewed.     

Renovascular hypertension: a case of intimal fibroplasia with congenital anomaly of the renal artery

We report a case of renovascular hypertension caused by fibromuscular dysplasia (intimal fibroplasia) with congenital anomaly of the renal artery. Arteriography revealed complete occlusion from the root to the middle of the right renal artery and a collateral artery from the right lumbar artery. Histological examination showed intimal fibroplasia and three arterioles in the intima of the renal artery.     

Successful revascularization of an occluded renal artery after prolonged anuria

Renal atherosclerosis and fibromuscular dysplasia are the most common causes of curable human renovascular hypertension and renal failure. Vascular reconstruction often preserves renal function, but renal failure is rarely reversed, especially after days of anuria. We report a case of a 23-year-old woman who as a child underwent a nephrectomy for congenital hydroureter and renal hypoplasia. She later experienced fibromuscular dysplasia of the remaining renal artery, which ultimately progressed to a complete occlusion and 31 days of total anuria. The patient was revascularized, and within 2 months renal function returned with a blood urea nitrogen and creatinine of 9.0 and 1.0 mg/dl, respectively. After a follow-up of 6 months the patient's blood pressure remained 120/80 to 130/80 mm Hg without administration of hypertension medication. In this report we emphasize that under selected circumstances a kidney can survive prolonged ischemia and that delayed revascularization may reestablish renal function.     

Autogenous internal iliac artery bypass in the surgical treatment of renovascular hypertension: A case report

A 24 year old female with severe renovascular hypertension resulting from bilateral fibromuscular dysplasia was successfully treated surgically. The internal iliac artery was used for a left aortorenal grafts. The right renal artery was dilated through the amputated left renal stump. Although saphenous vein graft is most commonly used, the autogenous internal iliac artery is considered a satisfactory graft in aortorenal reconstruction, especially for a young patient.     

Autogenous internal iliac artery bypass in the surgical treatment of renovascular hypertension: a case report

A 24 year old female with severe renovascular hypertension resulting from bilateral fibromuscular dysplasia was successfully treated surgically. The internal iliac artery was used for a left aortorenal grafts. The right renal artery was dilated through the amputated left renal stump. Although saphenous vein graft is most commonly used, the autogenous internal iliac artery is considered a satisfactory graft in aortorenal reconstruction, especially for a young patient.     

Fibromuscular dysplasia of renal arteries

This case reports a young child having uncontrolled hypertension, resulting from bilateral renal artery stenosis due to fibromuscular dysplasia presenting with abdominal pain, headache and visual disturbance. Diagnostic features and management is discussed.     

Fibromuscular dysplasia of the renal arteries: a radiological review

Fibromuscular dysplasia is the most common cause of renovascular hypertension in young patients. Digital subtraction angiography is still the best investigation used to determine the location, extent and complication of renal artery involvement. String of beads appearance (reflecting multiple stenoses), aneurysms, focal or tubular stenosis are classic angiographic appearances. The aim of this pictorial essay is to illustrate the various imaging findings of renal artery fibromuscular dysplasia.     

Cutting balloon angioplasty of resistant renal artery stenosis caused by fibromuscular dysplasia

Balloon angioplasty is an established intervention to treat renovascular hypertension. Conventional balloon angioplasty is usually effective in cases of renovascular hypertension caused by fibromuscular dysplasia. In the present report, we describe two cases of renovascular hypertension caused by fibromuscular dysplasia in which stenotic lesions were resistant to conventional balloon angioplasty but were successfully managed by a cutting balloon. After cutting balloon angioplasty, systemic blood pressure was normalized in both patients, without the use of antihypertensive agents. Angioplasty by using a cutting balloon is a new therapeutic choice against renal artery stenosis that is resistant to dilation by a conventional balloon.     

The role of arterial reconstruction in spontaneous renal artery dissection

Spontaneous renal artery dissection is an uncommon cause of renovascular hypertension, usually associated with fibromuscular dysplasia. Among reported nonautopsy cases (N = 80), arterial reconstruction has seldom been attempted (N = 21) and the outcome has frequently been poor (48% clinical failure rate). This is attributed in part to the frequent involvement of renal artery branches by the dissection. Furthermore, the report of spontaneous reversion to normotension among patients treated medically has also clouded the role of surgery in this disease. Since progress in the technique of renal artery repair now allows successful treatment of anatomically complex lesions, we reviewed our experience with arterial reconstruction in the management of spontaneous renal artery dissection to determine the frequency of and factors correlating with cure after operative repair. Ten patients (eight men, two women; mean age, 39.3 +/- 5.9 years) were admitted with severe hypertension (10/10), often associated with neurologic symptoms, hematuria, or flank pain (8/10). Serum creatinine was elevated in only two patients. Angiography demonstrated changes consistent with fibromuscular dysplasia in 7 of 10 patients and evidence of dissection in 6 of 10. Bilateral disease was present in three patients. Only five patients had a single renal artery on the involved side. The dissection extended into the primary branches in 8 of 10 patients and involved both renal arteries in four of the five patients with two arteries. Histologic study confirmed fibromuscular dysplasia in six and intramural dissection in all operative specimens. Five patients underwent revascularization (in one case requiring the ex vivo technique), with use of hypogastric artery as a conduit in four of five or resection and primary reanastomosis in one of five. Three patients became normotensive, and two returned to their previous level of blood pressure control. Follow-up averaged 14.5 years. Two patients underwent nephrectomy after exploration demonstrated nonreconstructible vessels, and two underwent nephrectomy when intraoperative assessment of the kidney showed that revascularization had failed to adequately reverse extensive renal ischemia. After a mean follow-up of 14.6 years these patients remain normotensive, although two require antihypertensive medications. One patient was treated medically and is currently hypertensive off all medications. Nine of 10 patients have maintained a normal serum creatinine during follow-up. We conclude that renal revascularization is frequently successful in spontaneous renal artery dissection (five of seven, 71.4%) and results in sustained relief of hypertension with maximal conservation of renal tissue. This is important because of the young age at onset and the not infrequent occurrence of bilateral fibromuscular dysplasia, and even of dissection.(ABSTRACT TRUNCATED AT 400 WORDS)     

经皮血管成形术治疗肌纤维发育不良型肾动脉狭窄的临床疗效

目的评价经皮血管腔内血管成形术(PTA)治疗肾动脉肌纤维性发育不良的疗效及安全性。方法选择肌纤维发育不良型肾动脉狭窄患者32例(均合并2~3级高血压或难治性高血压),行经皮肾动脉成形术治疗。术后随访观察患者血压、药物治疗种类、术后再狭窄、生存率和并发症等。结果 PTA技术成功率93.94%(31/33)。术前、术后收缩压分别为(189.6±26.0)mmHg、(136.6±8.0)mmHg(t=9.117,P0.001),舒张压分别为(121.6±21.7)mmHg、(81.1±11.5)mmHg(t=7.745,P0.001)。无肾动脉破裂、夹层、分支堵塞及血栓形成等相关并发症和术后不良事件发生。术后随访时间5~100个月,平均(40.4±26.1)个月,患者生存率100%(32/32)。结论经皮血管成形术治疗肌纤维发育不良肾动脉狭窄安全、有效。     

Simultaneous bilateral renal artery reconstruction and intraoperative renal protection. A case report

A 22-year-old Japanese man with bilateral renal artery stenosis associated with hypertension underwent successful surgery of simultaneous bilateral renal artery reconstruction under conditions of intraoperative renal perfusion with St. Thomas Hospital solution which is used for cardioplegia in open heart surgery. Circulation in the left kidney was interrupted for 58 minutes and that of the right kidney for 35 minutes. The patient fully recovered with no serious impairment of renal function. In addition to these stenotic lesions of the renal artery, there were medial necrosis of the aorta and fibromuscular dysplasia of the superior mesenteric artery. Administration of SQ14,225, an angiotensin I converting enzyme inhibitor, was effective in controlling hypertension during the preoperative period.     

Simultaneous bilateral renal artery reconstruction and intraoperative renal protection

A 22-year-old Japanese man with bilateral renal artery stenosis associated with hypertension underwent successfull surgery of simultaneous bilateral renal artery reconstruction under conditions of intraoperative renal perfusion with St. Thomas Hospital solution which is used for cardioplegia in open heart surgery. Circulation in the left kidney was interrupted for 58 minutes and that of the right kidney for 35 minutes. The patient fully recovered with no serious impairment of renal function. In addition to these stenotic lesions of the renal artery, there were medial necrosis of the aorta and fibromuscular dysplasia of the superior mesenteric artery. Administration of SQ14,225, an angiotensin I converting enzyme inhibitor, was effective in controlling hypertension during the preoperative period.