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Published on: February 3, 2012
A spectrum of segmental multicystic renal dysplasia
1Department of Radiology, Janeway Child Health Centre, St. John's, Newfoundland, Canada.
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.
Background:
Multicystic renal dysplasia (MCDK) is a common anomaly well described in the literature, but less well described when involving only a portion of a kidney.
Objective:
To present the imaging spectrum, natural history and associated anomalies of six kidneys with segmental MCDK.
Materials And Methods:
Five children with segmental MCDK (one with bilateral segmental MCDK) referred to our hospital between 1989 and 1996 were reviewed. All had at least one ultrasound examination. Four had a voiding cystogram and three had renal scintigraphy.
Results:
Four children had antenatal diagnosis of cystic renal abnormality. In two, with obvious duplex kidneys and associated ureteroceles, the diagnosis of upper moiety MCDK was obvious either antenatally or immediately postnatally. In the other three there were diagnostic difficulties. One patient had bilateral widespread cysts obscuring the functioning renal portions. Another presented in utero with a large ureterocele and a cystic upper pole that had involuted by birth. The fifth had a nephrectomy at 3 years for a multiloculated cystic mass. Varying degrees of involution occurred in the five kidneys seen early. Reflux was demonstrated into the ipsilateral functioning lower moiety and midpole.
Conclusion:
In these children as in other studies, the commonest presentation of segmental MCDK is in the upper pole of a duplex kidney associated with a ureterocele at the end of the atretic ureter. Atypical segmental MCDK may present a diagnostic dilemma and should be included in the differential diagnosis of multiloculated cystic masses and cystic kidneys.
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