Abstract: FR-PO550

Radiological Imaging in Pediatric Renovascular Fibromuscular Dysplasia (FMD)

Session Information

Category: Hypertension

  • 1106 Hypertension: Clinical and Translational - Secondary Causes

Authors

  • Louis, Robert Justin, The Children Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Cahill, Anne marie, The Children Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Meyers, Kevin E.C., The Children Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania, United States
Background

FMD is a non-inflammatory vascular disease that in children unlike adults has no sex-predilection. FMD is under-recognized, underdiagnosed, with unclear pathogenesis. Doppler Ultrasounds (US), Magnetic Resonance Angiography (MRA), Computed Tomography Angiography (CTA), and Catheter Based Angiography (Ang) are used to make a presumptive diagnosis of FMD.

Methods

We did a retrospective analysis of the clinical features and radiological findings in 26 children diagnosed with FMD at the Children’s Hospital of Philadelphia (CHOP), all are entered into the national FMDSA database and have institutional IRB consent

Results

Mean age at diagnosis was 7.4 ± 4.7 yr (4m – 17yr). Family history HTN (54.2%) of FMD (8.3%), Caucasian (61.5%). Headache (46%) and HTN (89.7%) were the most prevalent symptoms and signs at presentation. FMD was unifocal single site 17/25 (68%) or unifocal multiple sites 7/25 (28%) and involved the main or first order renal branch in 17/25 (68%). Isolated deep vessel lesions beyond the 2nd order branches were found in 7/25 (28%) children (Table). Only Ang showed deep vessel disease. US imaging was significantly less sensitive (28% US vs 100% Ang, p = 0.003) and was the least sensitive imaging technique. The NPV of US (41.9%) was also less than that of Ang (100%). US had an equivalent specificity and PPV to that of Ang. This was true for the specificity and PPV of MRA imaging as well, but MRA imaging showed a lower NPV (40%) when compared with Ang. This was the lowest NPV of the imaging modalities. MRA had a better sensitivity (62.5%) than US. Overall, 3D CTA had the best sensitivity (84.2%) and NPV (70%) compared with Ang; but still had much lower specificity (70%) and PPV (84.2%) when compared with Ang.

Conclusion

Only Ang showed deep renal vascular disease. Ang should be done as part of the initial work-up of any child suspected of having renovascular FMD, no matter what the findings are on US, MRA, or 3D CTA.

Peripheral site of renal vasculature narrowing in 7/25 (28%) (more than 1 site often involved)
LocationTotal stenoses (%)Unifocal single siteUnifocal multiple sitesMultifocalPatients n=25 (%)
2nd order6 (9.4)1324 (16)
3rd order5 (7.8)2124 (16)
4th order3 (4.7)1022 (8)
Periphery1 (1.6)0011 (4)

Funding

  • Private Foundation Support