Bilateral Fibromuscular Dysplasia Research Articles

Renovascular Hypertension with Progressive Atherosclerotic Renal Artery Stenosis in an Elderly Patient with Known Fibromuscular Dysplasia: A Case Report.

BACKGROUND Atherosclerotic renal artery stenosis (ARAS) and renovascular fibromuscular dysplasia (FMD) are 2 of the most common etiologies of renovascular hypertension. They have different pathophysiologies, risk factors, presentations, and treatment options. However, as our population ages, it can become increasingly common to see patients who previously had FMD who develop ARAS at an advanced age, indicated by recurrent renovascular hypertension. CASE REPORT We present a case of a 66-year-old female patient who, in 2007, had presented with uncontrolled hypertension. She underwent magnetic resonance angiography and was found to have bilateral FMD, for which she received balloon angioplasty to a severe lesion on the mid-right renal artery and subsequently had normalization of blood pressures and resolution of symptoms. In 2021 she returned with uncontrolled hypertension while being treated with 3 antihypertensives. Bilateral renal arteriography revealed new severe ostial stenosis of the left renal artery and a patent right renal artery in which balloon angioplasty was performed 14 years ago. Based on the angiographic pattern of this new left RAS, we concluded this lesion was caused by atherosclerosis. The left ostial lesion was treated with a bare-metal stent and the patient was continued on antihypertensive medication and statin; at follow-up 4 months later, her blood pressures had normalized. CONCLUSIONS This case features a patient who developed severe ARAS with underlying history of bilateral renal artery FMD. Clinicians need to be aware that in patients with FMD, worsening renovascular hypertension at an advanced age could indicate the development of new hemodynamically significant ARAS. These patients need repeat diagnostic testing and treatment with medial optimization with or without endovascular revascularization in the appropriate clinical setting.

Renal Artery Fibromuscular Dysplasia in 2,640 Renal Donor Subjects: A CT Angiography Analysis

PurposeTo present the incidence, demographics, and clinical presentation of patients diagnosed with renal fibromuscular dysplasia (FMD) who underwent computed tomography (CT) angiography for evaluation of living renal donor protocol. Methods and MaterialsA retrospective review was performed from January 1, 2000, to December 31, 2011 on patients who underwent CT angiography for evaluation of living renal donor protocol. Of 2,640 patients identified, only patients with a diagnosis of FMD by the radiology report underwent independent reader evaluation of CT angiography for the presence of FMD and associated characteristics. The demographics, medical history, and presentation were reviewed using the electronic medical chart. ResultsThe independent readers who evaluated CT angiography identified a diagnosis of FMD in 68 patients (2.6%; 59 female) with an average age of 52 years ± 10. Unilateral FMD was observed in 46 patients (68%), and bilateral FMD was observed in 22 patients (32%). Three patients had aneurysms of the renal artery. Comorbidities included hypertension (n = 21, 31%), dyslipidemia, (n = 13, 19%), history of migraines (n = 3, 4%), and history of smoking (n = 14, 21%). No patients had diabetes mellitus, coronary artery disease, or family history of FMD. Of 21 patients (31%) with a history of hypertension, 13 patients (62%) were treated with antihypertensive medications. ConclusionsThe incidence of FMD in patients who underwent CT angiography for evaluation of living renal donor protocol is 2.6%.

Fibromuscular dysplasia presenting with asymptomatic bilateral renal infarctions

Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic vascular disease. It is the second cause of renovascular hypertension after atherosclerosis. Although FMD usually has a good prognosis, renal infarctions and artery dissections have been described. We present the case of a 38-year-old woman with hypertension and asymptomatic bilateral renal infarctions. Bilateral FMD of segmental branches of the renal arteries was diagnosed by digital subtraction angiography after an exhaustive study. Previous intake of nonsteroidal anti-inflammatory drugs may also have played a significant role in the development of renal infarctions. To our knowledge, bilateral renal infarctions complicating FMD have been reported in only four previous cases; only in one of those cases, renal infarctions were asymptomatic.

Listening to the diagnosis: a physician’s account of spontaneous carotid artery dissection

A healthy 52-year-old male (J.L.A.) experienced a right pulsatile tinnitus (PT). It was synchronous with cardiac systole, was noticed initially at night, and became objectively audible. Valsalva’s maneuver markedly diminished its intensity. There were no previous trauma, otalgia, hypoacusis, fever, nasal congestion, cough, headache or any neurological symptom. Arterial pressure, ECG, chest X-ray, neuro-otological examination, otoscopy, audiometric testing, tympanometry and cerebral magnetic resonance (MR) imaging were unrevealing. A cerebral magnetic resonance angiography (MRA) raised the suspicion of a possible dissection of an extracranial right internal carotid artery (ICA) near the skull base. Subsequently, a cervical CT angiography (CTA) and a cervical MR angiography (MRA) yielded the diagnosis of right steno-occlusive (less than 50% luminal stenosis) ICA dissection, extending from 1 cm above the carotid bulb to the cranial base, ending just proximal to the petrous carotid canal. The dissection was associated with a medium-sized saccular pseudoaneurysm located near the skull base—C1-C2 level. Digital subtraction angiography (DSA) confirmed the diagnosis and showed bilateral fibromuscular dysplasia of carotid arteries as the cause of the dissection (Fig. 1). No intravascular intervention (i.e., stenting) was considered necessary due to the high location of the pseudoaneurysm and the lack of neurological symptoms. Treatment with intravenous heparin was followed by aspirin. Tinnitus gradually disappeared in 4 days. At follow-up, CT demonstrated resolution of the luminal stenosis and thrombosis of the false lumen, although the pseudoaneurysm remained unchanged.

A rare case of familial carotid body tumor in a patient with bilateral fibromuscular dysplasia

Carotid body tumors (CBTs) are neuroendocrine tumors that arise due to mutations of respiratory cycle enzymes. Fibromuscular dysplasia (FMD) is a disease that causes narrowing of medium-sized arteries. There is no documented link between CBT and FMD. In this article, we report a case of a patient with bilateral carotid FMD and familial CBT, including one in an identical twin who underwent successful surgical excision of the CBT. We describe specific considerations in the management of CBT in patients with concomitant carotid FMD. Also, we review the literature about the genetics of familial CBT and its possible relationship to the etiology of FMD.

Possible familial origin of multifocal renal artery fibromuscular dysplasia

To describe phenotypes and estimate the prevalence of familial cases of renal artery fibromuscular dysplasia (FMD). One hundred and four unrelated hypertensive patients (94 women) with renal artery fibromuscular dysplasia documented on angiography and classified as having multifocal or unifocal lesions. Familial cases were defined as those with angiographic evidence of renal artery FMD in at least one sibling. Eighty-one patients had multifocal and 16 had unifocal FMD. Both types of stenosis were present in seven patients. Fifty-four patients had bilateral FMD, including the seven patients with both unifocal and multifocal lesions. The 16 patients with unifocal FMD were younger, more likely to be men, and more commonly had unilateral stenoses, stenoses exceeding 75% and a small ischemic kidney than the 81 patients with multifocal lesions. Eleven cases were identified as familial on the basis of FMD having been documented in at least one sibling (eight sibling pairs and three trios). All probands were women and exhibited multifocal lesions. FMD was more often bilateral in familial than it was in apparently sporadic cases. Multifocal FMD was mostly found in women and unifocal FMD in young men with more severe stenosis and kidney ischemia. The documented prevalence of familial cases was 11% in this series, the true prevalence being probably higher because only a few siblings were examined by angiography. Familial cases all exhibited the multifocal type and were more commonly bilateral than were sporadic cases.

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 (1010), often associated with neurologic symptoms, hematuria, or flank pain (810). 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. Successful reconstruction is facilitated by early treatment, before the dissection extends into the secondary renal artery branches. Because extension of the dissection is a continued threat and because the hypertension associated with this event is usually severe and difficult to control, we do not think that patients should uniformly be treated medically, waiting to see if spontaneous reversion to normotension will occur.

Percutaneous transluminal renal angioplasty: Initial results and long-term follow-up in 202 patients

Percutaneous transluminal renal angioplasty was performed in 202 patients with 250 stenoses. The procedure was successful in 201 of 250 (83%). Results were better for postostial atherosclerotic lesions (94%), fibromuscular lesions (83%), and transplant kidneys (71%) than for ostial atherosclerotic lesions (29%). Of all the patients, 61% had reduced blood pressures following the procedure, with cure (diastolic blood pressure less than or equal to 90 mm Hg) in 31% of the patients. Cure rate with a mean follow-up of 25.8 +/- 19.4 months was 21% in bilateral atheromatous lesions, 30% in unilateral atheromatosis, 65% in unilateral fibromuscular disease, and 40% in bilateral fibromuscular dysplasia. Of the transplanted patients, 60% were cured. Complications occurred in 23 (11%) of the patients. Recurrence of stenoses occurred in 16 lesions (8%), 80% within the first year after the procedure.

Fibromuscular dysplasia of the extracranial internal carotid arteries associated with an ulcerative plaque.

Fibromuscular dysplasia (FMD) of the extracranial internal carotid arteries is a relatively rare angiographical finding which has been reported as an incidental finding during angiography, as well as in association with a variety of different clinical conditions. A case is reported of transient attacks of amaurosis fugax and hemiparesis. Angiography in this patient revealed bilateral FMD of the internal carotids as well as a small ulcerative plaque involving the right internal carotid artery at the region of carotid bifurcation. Surgical treatment directed toward the ulcerative plaque and not the FMD of the carotid arteries has rendered the patient asymptomatic. Lack of symptoms, despite the persistence of FMD, suggests that FMD is of doubtful pathogenic significance. A small, surgically correctable ulcerative plaque should not be overlooked in the presence of FMD of the internal carotid arteries. The significance of the rare finding of FMD of the internal carotid arteries is not clear.