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Imaging studies in pediatric fibromuscular dysplasia (FMD): a single-center experience

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Abstract

Background

Fibromuscular 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.

Methods

All 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.

Results

Mean 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.

Conclusions

Limitations 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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References

  1. Kurian J, Epelman M, Darge K, Meyers K, Nijs E, Hellinger JC (2013) Role of CT angiography in the evaluation of pediatric renovascular hypertension. Pediatr Radiol 43(4):490–501

    Article  PubMed  Google Scholar 

  2. Zhu G, He F, Gu Y, Yu H, Chen B, Hu Z, Liang W, Wang Z (2014) Angioplasty for pediatric renovascular hypertension: a 13-year experience. Diagn Interv Radiol 20(3):285–292

    Article  PubMed  PubMed Central  Google Scholar 

  3. Meyers KE, Cahill AM, Sethna C (2014) Interventions for pediatric renovascular hypertension. Curr Hypertens Rep 16:422

    Article  PubMed  CAS  Google Scholar 

  4. Tullus K, Brennan E, Hamilton G, Lord R, McLaren CA, Marks SD, Roebuck DJ (2008) Renovascular hypertension in children. Lancet 371(9622):1453–1463

    Article  PubMed  CAS  Google Scholar 

  5. Srinivasan A, Krishnamurthy G, Fontalvo-Herazo L, Nijs E, Meyers K, Kaplan B, Cahill AM (2011) Spectrum of renal findings in pediatric fibromuscular dysplasia and neurofibromatosis type 1. Pediatr Radiol 41(3):308–316

    Article  PubMed  Google Scholar 

  6. Olin JW, Gornik HL, Bacharach JM (2014) Fibromuscular dysplasia: state of science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation 129(9):1048–1078

    Article  PubMed  Google Scholar 

  7. Hendricks NJ, Matsumoto AH, Angle JF, Baheti A, Sabri SS, Park AW, Stone JR, Patrie JT, Dworkin L, Cooper CJ, Murphy TP, Cutlip DE (2014) Is fibromuscular dysplasia underdiagnosed? A comparison of the prevalence of FMD seen in CORAL trial participants versus a single institution population of renal donor candidates. Vasc Med 19(5):363–367

    Article  PubMed  Google Scholar 

  8. Kadian-Dodov D, Gornik HL, Gu X, Froehlich J, Bacharach JM, Chi YW, Gray BH, Jaff MR, Kim ES, Mace P, Sharma A, Kline-Rogers E, White C, Olin JW (2016) Dissection and aneurysm in patients with fibromuscular dysplasia: Findings from the US Registry for FMD. J Am Coll Cardiol 68(2):176–185

    Article  PubMed  Google Scholar 

  9. Ruzicka M, Kucharski SE, Hiremath S (2017) Balancing overscreening and under-diagnosis in secondary hypertension: The Case of Fibromuscular Dysplasia. Cardiol Clin 35(2):247–254

    Article  PubMed  Google Scholar 

  10. Brinza E, Gornik HL (2016) Fibromuscular dysplasia: advances in understanding and management. Cleve Clin J Med 83(11):S45–S51

    Article  PubMed  Google Scholar 

  11. Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin JW, Gornik HL, Cahill AM, Meyers KE (2016) Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry. Pediatr Nephrol 31(4):641–650

    Article  PubMed  Google Scholar 

  12. Lewis S, Kadian-Dodov D, Bansal A, Lookstein RA (2016) Multimodality imaging of fibromuscular dysplasia. Abdom Radio 41(10):2048–2060

    Article  Google Scholar 

  13. Illivitzki A, Glozman L, Lopez Alfonso R, Ofer A, Beck Razi N, Rotman Shapira M (2017) Sonographic evaluation of renovascular hypertension in the pediatric population: state-of-the-art. J Clin Ultrasound 45(5):282–292

    Article  Google Scholar 

  14. National Heart, Lung, and Blood Institute (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114(2):555–576

    Article  Google Scholar 

  15. Parikh R, Mathai A, Parikh S, Sekhar GC, Thomas R (2008) Understanding and using sensitivity, specificity and predictive values. Indian J Ophthalmol 56(1):45–50

    Article  PubMed  PubMed Central  Google Scholar 

  16. Olin JW, Froehlich J, Bacharach JM, Eagle K, Gray BH, Jaff MR, Kim ES, Mace P, Matsumoto AH, McBane RD, Kline-Rogers E, White CJ, Gornik HL (2012) The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation 125(25):3182–3190

    Article  PubMed  Google Scholar 

  17. Kirton A, Crone M, Benseler S, Mineyko A, Armstrong D, Wade A, Sebire G, Crous-Tsanaclis AM, deVeber G (2013) Fibromuscular dysplasia and childhood stroke. Brain 136(6):1846–1856

    Article  PubMed  Google Scholar 

  18. National Center for Biotechnology Information. PHACTR1 phophatase and actin regulator 1 [Homo sapiens (human)]. https://www.ncbi.nlm.nih.gov/gene/221692 Accessed December 1, 2017

  19. Kiando SR, Tucker NR, Castro-Vega LJ, Katz A, D’Escamard V, Treard C, Fraher D, Albuisson J, Kadian-Dodov D, Ye A, Austin E, Yang ML, Hunker K, Barlassina C, Cusi D, Galan P, Empana JP, Jouven X, Gimenez-Roqueplo AP, Bruneval P, Hyun Kim ES, Olin JW, Gornik HL, Azizi M2, Plouin PF, Ellinor PT, Kullo IJ, Milan DJ, Ganesh SK, Boutouyrie P, Kovacic JC, Jeunemaitre X, Bouatia-Naji N (2016) PHACTR1 is a genetic susceptibility locus for fibromuscular dysplasia supporting its complex genetic pattern of inheritance. PLoS Genet 12(10):e1006367

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  20. Trautmann A, Roebuck DJ, McLaren CA, Brennan E, Marks SD, Tullus K (2017) Non-invasive imaging cannot replace formal angiography in the diagnosis of renovascular hypertension. Pediatr Nephrol 32(3):495–502

    Article  PubMed  Google Scholar 

  21. Rountas C, Vlychou M, Vassiou K, Liakopoulos V, Kapsalaki E, Koukoulis G, Fezoulidis IV, Stefanidis I (2007) Imaging modalities for renal artery stenosis in suspected renovascular hypertension: prospective intra-individual comparison of color Doppler US, CT angiography, GD-enhanced MR angiography, and digital subtraction angiography. Ren Fail 29(3):295–302

    Article  PubMed  CAS  Google Scholar 

  22. Eklöf H, Ahlström H, Magnusson A, Andersson LG, Andrén B, Hägg A, Bergqvist D, Nyman R (2006) A prospective comparison of duplex ultrasonography, captopril renography, MRA and CTA in assessing renal artery stenosis. Acta Radiol 47(8):764–774

    Article  PubMed  Google Scholar 

  23. Sabharwal R, Vladica P, Coleman P (2007) Multidetector spiral CT renal angiography in the diagnosis of renal artery fibromuscular dysplasia. Eur J Radiol 61(3):520–527

    Article  PubMed  Google Scholar 

  24. Tan KT, van Beek EJ, Brown PW, van Delden OM, Tijssen J, Ramsay LE (2002) Magnetic resonance angiography for the diagnosis of renal artery stenosis: a meta-analysis. Clin Radiol 57(7):617–624

    Article  PubMed  CAS  Google Scholar 

  25. Hansen NJ, Kaza RK, Maturen KE, Liu PS, Platt JF (2014) Evaluation of low-dose CT angiography with model-based iterative reconstruction after endovascular aneurysm repair of a thoracic or abdominal aortic aneurysm. Am J Roentgenol 202(3):648–655

    Article  Google Scholar 

  26. Noda Y, Goshima S, Koyasu H, Shigeyama S, Miyoshi T, Kawada H, Kawai N, Matsuo M (2017) Renovascular CT: comparison between adaptive statistical iterative reconstruction and model-based iterative reconstruction. Clin Radiol 72(10):901 e13–901.e19

    Article  Google Scholar 

  27. Schäberle W, Leyerer L, Schierling W, Pfister K (2016) Ultrasound diagnostics of renal artery stenosis: stenosis criteria, CEUS, and recurrent in-stent stenosis. Gefassschirugie 21:4–13

    Article  Google Scholar 

  28. Vo NJ, Hammelman BD, Racadio JM, Strife CF, Johnson ND, Racadio JM (2006) Anatomic distribution of renal artery stenosis in children: implications for imaging. Pediatr Radiol 10:1032–1036

    Article  Google Scholar 

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Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Robert Louis, Daniella Levy-Erez & Kevin E. Meyers

  2. University of Pennsylvania, Philadelphia, PA, USA

    Robert Louis, Daniella Levy-Erez, Anne Marie Cahill & Kevin E. Meyers

  3. Interventional Radiology, Department of Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Anne Marie Cahill

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  1. Robert Louis
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  2. Daniella Levy-Erez
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Correspondence to Daniella Levy-Erez.

Ethics declarations

Written consent was obtained, and the study was approved by the CHOP Internal Review Board (IRB).

Conflict of interest

The authors declare that they have no conflict of interest.

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Louis, R., Levy-Erez, D., Cahill, A.M. et al. Imaging studies in pediatric fibromuscular dysplasia (FMD): a single-center experience. Pediatr Nephrol 33, 1593–1599 (2018). https://doi.org/10.1007/s00467-018-3983-6

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  • DOI: https://doi.org/10.1007/s00467-018-3983-6

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