Skip to main content

Advertisement

Log in

Magnetic resonance urography of congenital abnormalities — what the radiologist needs to know

  • Pictorial Essay
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Congenital abnormalities of the kidney and urinary tract include a wide range of malformations ranging from asymptomatic to life-threatening conditions. Although pediatric urogenital system imaging is based on the use of US (pre- and postnatal), voiding cystourethrography and scintigraphic study, magnetic resonance (MR) urography plays a fundamental role in the classification and management of congenital abnormalities of the kidney and urinary tract, giving an overview of the different clinical pictures, thanks to its panoramicity and high anatomical detail. In fact the anomalies of the urinary tract are phenotypically variable because they can affect simultaneously several segments of different embryonic derivation, with complex clinical pictures; they can appear both as isolated phenotypes or as complex malformative conditions, involving renal parenchyma, collecting system and bladder. A deep knowledge of this complex embryogenesis and its possible phenotypic patterns allows a correct interpretation of MR urography images. We describe the embryology and pathophysiology of congenital abnormalities of the kidney and urinary tract as well as MR urography technique and findings. Congenital abnormalities of the kidney and urinary tract are classified into four groups: (1) obstruction (proximal, middle and distal), (2) budding with respect to the Wolffian duct (site and number of ureter), (3) ascent and rotation (ectopia, malrotation and fusion of kidney) and (4) anomaly of metanephric differentiation (dysplasia, megapolicalycosis).

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16
Fig. 17
Fig. 18
Fig. 19
Fig. 20

Similar content being viewed by others

References

  1. Guay-Woodford LM (2011) Hereditary nephropathies and developmental abnormalities of the urinary tract. In: Goldman L, Schafer AI (eds) Goldaman’s cecil medicine, 24th edn. Elsevier, Philadelphia, pp 800–805

    Google Scholar 

  2. Dickerson EC, Dillman JR, Smith EA et al (2015) Pediatric MR urography: indications, techniques, and approach to review. Radiographics 35:1208–1230

    Article  PubMed  Google Scholar 

  3. Surabhi VR, Menias CO, George V et al (2015) MDCT and MR urogram spectrum of congenital anomalies of the kidney and urinary tract diagnosed in adulthood. AJR Am J Roentgenol 205:W294–W304

    Article  PubMed  Google Scholar 

  4. Toka HR, Toka O, Hariri A et al (2010) Congenital anomalies of kidney and urinary tract. Semin Nephrol 30:374–386

    Article  PubMed  Google Scholar 

  5. Berrocal T, López-Pereira P, Arjonilla A et al (2002) Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics 22:1139–1164

    Article  PubMed  Google Scholar 

  6. Türkvatan A, Olçer T, Cumhur T (2009) Multidetector CT urography of renal fusion anomalies. Diagn Interv Radiol 15:127–134

    PubMed  Google Scholar 

  7. Jain S, Chen F (2018) Developmental pathology of congenital kidney and urinary tract anomalies. Clin Kidney J 12:382–399

    Article  PubMed  PubMed Central  Google Scholar 

  8. Riccabona M, Avni FE, Dacher J et al (2010) ESPR uroradiology task force and ESUR paediatric working group: imaging and procedural recommendations in paediatric uroradiology, part III. Minutes of the ESPR uroradiology task force minisymposium on intravenous urography, uro-CT and MR-urography in childhood. Pediatr Radiol 40:1315–1320

    Article  PubMed  Google Scholar 

  9. Antonov NK, Ruzal-Shapiro CB, Morel KD et al (2016) Feed and wrap MRI technique in infants. Clin Pediatr 56:1095–1103

    Article  Google Scholar 

  10. Vivier PH, Dolores M, Taylor M et al (2010) MR urography in children. Part 1: how we do the F0 technique. Pediatr Radiol 40:732–738

    Article  PubMed  Google Scholar 

  11. Parikh KR, Hammer MR, Kraft KH et al (2015) Pediatric ureteropelvic junction obstruction: can magnetic resonance urography identify crossing vessels? Pediatr Radiol 45:1788–1795

    Article  PubMed  Google Scholar 

  12. Mitsumori A, Yasui K, Akaki S et al (2000) Evaluation of crossing vessels in patients with ureteropelvic junction obstruction by means of helical CT. Radiographics 20:1383–1395

    Article  CAS  PubMed  Google Scholar 

  13. Yiee JH, Johnson-Welch S, Baker LA et al (2010) Histologic differences between extrinsic and intrinsic ureteropelvic junction obstruction. Urology 76:181–184

    Article  PubMed  Google Scholar 

  14. Krajewski W, Wojciechowska J, Dembowski J et al (2017) Hydronephrosis in the course of ureteropelvic junction obstruction: an underestimated problem? Current opinions on the pathogenesis, diagnosis and treatment. Adv Clin Exp Med 26:857–864

    Article  PubMed  Google Scholar 

  15. Docimo SG, Lebowitz RL, Retik AB et al (1989) Congenital midureteral obstruction. Urol Radiol 11:156–160

    Article  CAS  PubMed  Google Scholar 

  16. Farrugia MK, Hitchcock R, Radford A et al (2014) British Association of Paediatric Urologists consensus statement on the management of the primary obstructive megaureter. J Pediatr Urol 10:26–33

    Article  PubMed  Google Scholar 

  17. Shokeir AA, Nijman RJ (2000) Primary megaureter: current trends in diagnosis and treatment. BJU Int 86:861–868

    Article  CAS  PubMed  Google Scholar 

  18. Tokunaka S, Gotoh T, Koyanagi T et al (1984) Muscle dysplasia in megaureters. J Urol 131:383–390

    Article  CAS  PubMed  Google Scholar 

  19. Berdon WE, Baker DH, Becker JA (1968) Ectopic ureterocele. Radiol Clin N Am 6:205–214

    Article  CAS  PubMed  Google Scholar 

  20. Bisset GS 3rd, Strife JL (1987) The duplex collecting system in girls with urinary tract infection: prevalence and significance. AJR Am J Roentgenol 148:497–500

    Article  PubMed  Google Scholar 

  21. Nordmark B (1948) Double formations of the pelves of the kidneys and the ureters. Embryology, occurrence and clinical significance. Acta Radiol 30:4–5, 267–278

    Article  Google Scholar 

  22. Tam T, Pauls RN (2021) Embryology of the urogenital tract; a practical overview for urogynecologic surgeons. Int Urogynecol J 32:239–247

    Article  PubMed  Google Scholar 

  23. Valerius MT, Patterson LT, Witte DP et al (2002) Microarray analysis of novel cell lines representing two stages of metanephric mesenchyme differentiation. Mech Dev 110:151–164

    Article  CAS  PubMed  Google Scholar 

  24. Saxén L, Sariola H (1987) Early organogenesis of the kidney. Pediatr Nephrol 1:385–392

    Article  PubMed  Google Scholar 

  25. Fernbach SK, Feinstein KA, Spencer K et al (1997) Ureteral duplication and its complications. Radiographics 17:109–127

    Article  CAS  PubMed  Google Scholar 

  26. Adeb M, Darge K, Dillman JR et al (2013) Magnetic resonance urography in evaluation of duplicated renal collecting systems. Magn Reson Imaging Clin N Am 21:717–730

    Article  PubMed  Google Scholar 

  27. Inamoto K, Tanaka S, Takemura K et al (1983) Duplication of the renal pelvis and ureter: associated anomalies and pathological conditions. Radiat Med 1:55–64

    CAS  PubMed  Google Scholar 

  28. Carrico C, Lebowitz RL (1998) Incontinence due to an infrasphincteric ectopic ureter: why the delay in diagnosis and what the radiologist can do about it. Pediatr Radiol 28:942–949

    Article  CAS  PubMed  Google Scholar 

  29. Privett JT, Jeans WD, Roylance J (1976) The incidence and importance of renal duplication. Clin Radiol 27:521–530

    Article  CAS  PubMed  Google Scholar 

  30. Ibrahimi A, Hosni A, Ziani I et al (2020) Zinner’s syndrome: a rare diagnosis of dysuria based on imaging. Case Rep Urol 2020:8826664

    PubMed  PubMed Central  Google Scholar 

  31. Livingston L, Larsen CR (2000) Seminal vesicle cyst with ipsilateral renal agenesis. AJR Am J Roentgenol 175:177–180

    Article  CAS  PubMed  Google Scholar 

  32. Levisay GL, Holder J, Weigel JW (1975) Ureteral ectopia associated with seminal vesicle cyst and ipsilateral renal agenesis. Radiology 114:575–576

    Article  CAS  PubMed  Google Scholar 

  33. Sheih CP, Hung CS, Wei CF, Lin CY (1990) Cystic dilatations within the pelvis in patients with ipsilateral renal agenesis or dysplasia. J Urol 144:324–327

    Article  CAS  PubMed  Google Scholar 

  34. Van den Ouden D, Blom JH, Bangma C et al (1998) Diagnosis and management of seminal vesicle cysts associated with ipsilateral renal agenesis: a pooled analysis of 52 cases. Eur Urol 33:433–440

    Article  PubMed  Google Scholar 

  35. Barakat AJ, Drougas JG (1991) Occurrence of congenital abnormalities of kidney and urinary tract in 13,775 autopsies. Urology 38:347–350

    Article  CAS  PubMed  Google Scholar 

  36. Rinat C, Farkas A, Frishberg Y (2001) Familial inheritance of crossed fused renal ectopia. Pediatr Nephrol 16:269–270

    Article  CAS  PubMed  Google Scholar 

  37. O’Brien J, Buckley O, Doody O et al (2008) Imaging of horseshoe kidneys and their complications. J Med Imaging Radiat Oncol 52:216–226

    Article  CAS  PubMed  Google Scholar 

  38. Winyard P, Chitty LS (2008) Dysplastic kidneys. Semin Fetal Neonatal Med 13:142–151

    Article  PubMed  Google Scholar 

  39. Clark AT, Bertram JF (1999) Molecular regulation of nephron endowment. Am J Physiol 276:F485–F497

    CAS  PubMed  Google Scholar 

  40. Grattan-Smith JD, Little SB, Jones RA (2008) Evaluation of reflux nephropathy, pyelonephritis and renal dysplasia. Pediatr Radiol 38:S83–S105

    Article  PubMed  Google Scholar 

  41. Bekele W, Sanchez TR (2010) Congenital megacalyces presenting as neonatal hydronephrosis. Pediatr Radiol 40:1579

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

Our sincere thanks to Valentina Sanclemente (Velenia) for the original and exclusive drawings.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Irene Campo.

Ethics declarations

Conflicts of interest

None

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Campo, I., Sertorio, F., Wong, M. et al. Magnetic resonance urography of congenital abnormalities — what the radiologist needs to know. Pediatr Radiol 52, 985–997 (2022). https://doi.org/10.1007/s00247-021-05233-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-021-05233-2

Keywords

Navigation