ArticlePediatric nephrology (Berlin, Germany)2018
Imaging studies in pediatric fibromuscular dysplasia (FMD): a single-center experience.
Article in Pediatric nephrology (Berlin, Germany), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 1 of them a synthesis that pooled it.
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Who cites it
23 citing papers in PubMed, 1 synthesis or guideline pooled it, 48 citations in OpenAlex.
- Cardiovascular implications in adolescent and young adult hypertension.Reviews in cardiovascular medicine · 2022Pooled it
- Aorto-Renal Dysplasia in Childhood: The Overlap of Neurofibromatosis Type 1 and Pediatric Renovascular Hypertension.Current hypertension reports · 2026Review
- Artificial Intelligence and Machine Learning Applications in Fibromuscular Dysplasia: Transforming Diagnosis, Risk Stratification, and Clinical Decision-Making.Journal of cardiovascular translational research · 2026Review
- Mid-to-long term outcomes following renal artery angioplasty in children and young adults with renal artery stenosis: a retrospective review.Pediatric nephrology (Berlin, Germany) · 2025Article
- Coiling in Renovascular Hypertension: A Case Report of Fibromuscular Dysplasia and Complex Urinary Tract Abnormalities.The American journal of case reports · 2025Article
- Pediatric Renovascular Hypertension: Diagnosis and Management.Seminars in interventional radiology · 2025Review
- Secondary Hypertension in Children-Identifying and Investigating at Risk Children.Current hypertension reports · 2025Review
- Renal Artery Stenosis and Mid-Aortic Syndrome in Children-A Review.Journal of clinical medicine · 2024Review
- Evidence for routine brain-to-pelvis imaging and antiplatelet therapy in patients diagnosed with fibromuscular dysplasia.Journal of clinical hypertension (Greenwich, Conn.) · 2024Review
- Cerebral arteriopathies of childhood and stroke - A focus on systemic arteriopathies and pediatric fibromuscular dysplasia (FMD).Vascular medicine (London, England) · 2024Review
- Pediatric Renovascular Hypertension: A Pediatric Interventional Radiologist's Perspective.The Indian journal of radiology & imaging · 2023Review
- Intravascular ultrasound provides additional insights in the hypertensive patients with focal renal artery fibromuscular dysplasia.Hypertension research : official journal of the Japanese Society of Hypertension · 2023Observational
- Presentation, treatment, and outcome of renovascular hypertension below 2 years of age.European journal of pediatrics · 2022Article
- The complex genetic basis of fibromuscular dysplasia, a systemic arteriopathy associated with multiple forms of cardiovascular disease.Clinical science (London, England : 1979) · 2022Review
- Renovascular hypertension in pediatric patients: update on diagnosis and management.Pediatric nephrology (Berlin, Germany) · 2021Review
- Validation of computed tomography angiography as a complementary test in the assessment of renal artery stenosis: a comparison with digital subtraction angiography.Pediatric radiology · 2021Article
- Clinical characteristics and long-term outcomes of endovascular treatment of renal artery fibromuscular dysplasia with branch lesions.Pediatric nephrology (Berlin, Germany) · 2021Article
- Hypertension in Children and Adolescents with Turner Syndrome (TS), Neurofibromatosis 1 (NF1), and Williams Syndrome (WS).Current hypertension reports · 2021Review
- Diagnostic sensitivity and specificity of CT angiography for renal artery stenosis in children.Pediatric radiology · 2021Article
- Fibromuscular Dysplasia As a Rare Cause of Ileus and Aneurysm in Childhood.JPGN reports · 2021Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
backgroundFibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Doppler ultrasound (US), magnetic resonance angiography (MRA), computed tomography angiography (CTA), and catheter-based angiography (angiography) are currently used to help make a clinicoradiological diagnosis of FMD. The main aim of the study was to compare the efficacy of imaging modalities which can allow for earlier and improved detection. Furthermore, an anatomical mapping of the location of lesions can help determine the best treatment modalities.
methodsAll patients with non-syndromic non-inflammatory renovascular hypertension were recruited from the Nephrology Department at the Children's Hospital of Philadelphia (CHOP) and enrolled in the U.S. FMD Registry maintained at the University of Michigan. Clinical presentation and imaging findings on US, CT, and MRI of children diagnosed with FMD were evaluated.
resultsMean age at diagnosis was 7 ± 4.9 years (4 months-17 years). Family history of hypertension (HTN) (52%), FMD (8.7%), Caucasian (60%), headache (48%), and HTN (80%) were the most prevalent symptom and sign at presentation. Bruits were 100% specific for renal artery stenosis (RAS) diagnosis but were heard in the minority of patients (3 patients, 12%). FMD was mainly unifocal within a single site (68%) or multiple sites (28%) and involved the main or first order renal branch in about 68% of children. Isolated distal lesions beyond the second order branches were found in about 25% of children. US imaging was significantly less sensitive than angiography (28%, p = 0.003). MRA had a better sensitivity (62.5%, p = 0.3) than US. Overall, CTA had the best sensitivity (84.2%, p = 0.4) compared to angiography; however, only angiography showed distal vessel disease.
conclusionsLimitations of the study include the sample size and biases-only patients diagnosed with FMD were included in this study and most patients were referred to a pediatric nephrologist for unexplained hypertension. Angiography should be performed as part of the initial work-up of any child suspected of having renovascular FMD, regardless of the findings seen on US, MRA, or CTA.
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