Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

Robberechts, Tom; Devos, Hannes; Tussenbroek, F. Van; Januszewicz, Andrzej; Persu, Alexandre

doi:10.33963/kp.a2021.0040

Cited by 20 publications

(15 citation statements)

References 53 publications

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“…Among stroke patients between 18 to 50 years old, also other vascular diseases, like congenital or iatrogenic artery stenosis, Takayasu disease, fibromuscular dysplasia, premature atherosclerosis, moyamoya disease, cerebral venous thrombosis, and reversible cerebral vasoconstriction syndrome have been more frequently found compared to non-young adults ( 6 , 8 , 9 ).…”

Section: Discussionmentioning

confidence: 99%

Coarctation of the aorta, carotid artery stenosis and aberrant right subclavian artery as a rare cause of cerebral ischemia in a primigravid woman

Wawak,

Pieniążek,

Tekieli

et al. 2024

Quant Imaging Med Surg

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Section: Discussionmentioning

confidence: 99%

Coarctation of the aorta, carotid artery stenosis and aberrant right subclavian artery as a rare cause of cerebral ischemia in a primigravid woman

Wawak,

Pieniążek,

Tekieli

et al. 2024

Quant Imaging Med Surg

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“…2 According to previous literature, patients with focal and multifocal lesions have different baseline characteristics, including age at FMD diagnosis (30 vs 49 years), age at hypertension diagnosis (26 vs 40 years), gender distribution (2:1 vs 5:1), initial blood pressure (157/97 vs 146/88 mmHg), current smoking (50% vs 26%), prevalence of unilateral renal artery lesions (79% vs 38%), and the HTN cure rate in patients who had revascularization (54% vs 26%). 7 Non-white ethnic data are currently underrepresented in the literature. 2 In our case, the patient is non-white and younger in age than recorded worldwide (17 years at HTN diagnosis vs 21 years at intervention) and has focal FMD, bilateral renal arteries FMD, with clinical cure of hypertension as early as the first day after intervention and continuing for 1 year.…”

Section: Discussionmentioning

confidence: 99%

“…5 It is defined as a “string of beads” appearing in angiographic views as a result of post-stenotic aneurysms in multifocal FMD or only narrowing of the renal artery or arteries in focal FMD. 6 Though FMD can affect both genders, occur at any age, and affect mostly white females, it manifests as HTN with a positive family history of hypertension, affecting renal arteries in up to 91% of cases and 57% of cases with multi-vessel affection 7 . However, two entities of FMD are recognized: multifocal and focal FMD.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Focal Renal Fibromuscular Dysplasia Treated With Angioplasty: A Case Report

Al-Falahi,

Al-Aghbari,

Al-Muriesh

2023

Vasc Endovascular Surg

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Background Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic, noninflammatory vascular disease that can lead to arterial stenosis, tortuosity, occlusion, aneurysms, and dissection. Fibromuscular dysplasia is a rare cause of hypertension that can easily be missed. To date, there has been no definitive treatment for fibromuscular dysplasia. Case report In this report, we present an uncommon case of renovascular hypertension in a 21-year-old non-white female with a 3-year history of hypertension secondary to fibromuscular dysplasia involving bilateral renal arteries. Computed tomography angiography during the arterial phase revealed distal focal narrowing of the right main renal artery, distal focal narrowing of the left main renal artery, and proximal focal narrowing of the left accessory lower renal artery. Percutaneous balloon dilatation of the stenotic lesion was performed successfully up to 1 year After the procedure, the arterial blood pressure was within the normal range (110/70 to 125/75 mmHg) without medication. After 1 year of follow-up, CTA revealed re-stenosis in left main renal artery without clinical symptoms and normal blood pressure. Repeated procedure was done successfully. Conclusions This case report highlights the difficulty in the diagnosis and treatment of focal fibromuscular dysplasia in young non-white female patients. Computerized tomographic angiography is a useful tool for identifying the cause and showing the benefit of percutaneous transluminal renal angioplasty treatment for this rare entity, as an early percutaneous angioplasty intervention may have a clinical cure for hypertension.

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“…The pathophysiology of fibromuscular dysplasia remains incompletely understood, and the mechanisms driving its extrarenal involvement, such as in the mesenteric arteries, are particularly enigmatic [ 3 ]. Genetic predisposition, hormonal influences, and mechanical factors have been implicated in fibromuscular dysplasia's etiology, but the specific interplay leading to extrarenal manifestations remains an area of ongoing research.…”

Section: Discussionmentioning

confidence: 99%

A Case Report on Fibromuscular Dysplasia With Extrarenal Involvement

Al Mubarak,

Alshalan,

Alkhunaizi

et al. 2023

Cureus

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Fibromuscular dysplasia is a vascular disorder characterized by nonatherosclerotic, noninflammatory arterial abnormalities, primarily affecting renal arteries. While extrarenal involvement is rare, it poses diagnostic challenges due to diverse clinical presentations. We present the case of a middle-aged female who presented with sudden-onset severe abdominal pain and hypertension. Physical examination revealed tenderness, guarding, and hypertensive urgency. Diagnostic workup, including laboratory investigations and computed tomography, identified multifocal arterial stenosis in both renal and mesenteric arteries, consistent with fibromuscular dysplasia. A multidisciplinary team initiated antihypertensive medications and performed angioplasty to address vascular stenosis. The patient showed significant improvement post intervention, highlighting the efficacy of a comprehensive management strategy. This case underscores the clinical complexities of fibromuscular dysplasia, emphasizing the diagnostic challenges posed by extrarenal manifestations. The successful multidisciplinary intervention highlights the importance of timely and targeted measures in addressing both renal and extrarenal complications.

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Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

Cited by 20 publications

References 53 publications

Coarctation of the aorta, carotid artery stenosis and aberrant right subclavian artery as a rare cause of cerebral ischemia in a primigravid woman

Coarctation of the aorta, carotid artery stenosis and aberrant right subclavian artery as a rare cause of cerebral ischemia in a primigravid woman

A Rare Case of Focal Renal Fibromuscular Dysplasia Treated With Angioplasty: A Case Report

A Case Report on Fibromuscular Dysplasia With Extrarenal Involvement

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