A spectrum of segmental multicystic renal dysplasia

A Jeon1, B C Cramer, E Walsh

  • 1Department of Radiology, Janeway Child Health Centre, St. John's, Newfoundland, Canada.

Pediatric Radiology
|June 26, 1999
PubMed

Insights

Segmental multicystic renal dysplasia (MCDK) often affects the upper pole of duplex kidneys, commonly presenting with ureteroceles. Atypical presentations can mimic other cystic renal masses, posing diagnostic challenges.

Area of Science:

  • Pediatric Radiology
  • Urology
  • Medical Imaging

Background:

  • Multicystic renal dysplasia (MCDK) is a common congenital kidney anomaly.
  • Segmental MCDK, affecting only a portion of the kidney, is less frequently described.
  • Understanding its imaging features and natural history is crucial for diagnosis.

Purpose of the Study:

  • To delineate the imaging spectrum of segmental MCDK.
  • To describe the natural history and associated anomalies in affected kidneys.
  • To aid in the diagnosis of this uncommon renal malformation.

Main Methods:

  • Retrospective review of five children with segmental MCDK (six affected kidneys).
  • Analysis of imaging studies including ultrasound, voiding cystography, and renal scintigraphy.
  • Correlation of imaging findings with clinical presentation and outcomes.

Main Results:

  • Antenatal diagnosis of cystic renal abnormality was noted in four children.
  • Segmental MCDK was often associated with duplex kidneys and ureteroceles.
  • Diagnostic difficulties arose in atypical cases, including widespread cysts and involution of lesions.
  • Reflux was observed into the ipsilateral functioning renal moieties.

Conclusions:

  • Segmental MCDK most commonly presents as upper pole involvement in duplex kidneys with ureteroceles.
  • Atypical presentations can be challenging to diagnose and may resemble other cystic renal masses.
  • Inclusion of segmental MCDK in the differential diagnosis is recommended for complex cystic kidney lesions.
Abstract

Related Concept Videos