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Paediatric multicystic dysplastic kidney disease in Cape Town, South Africa
Datonye Christopher Briggs1,2, Khanyisile Hlongwa3, Mignon McCulloch4
1Department of Paediatrics and Child Health, University of Cape Town/ Red Cross War Memorial Children's Hospital, Cape Town, South Africa. datonye.briggs1@ust.edu.ng.
Insights
Multicystic dysplastic kidney disease (MCDK) in South African children is often unilateral with duplex kidney as the most common contralateral abnormality. Smaller initial kidney size predicts involution, with rare chronic kidney disease progression.
Area of Science:
- Pediatric Nephrology
- Congenital Anomalies
- Urology
Background:
- Multicystic dysplastic kidney disease (MCDK) is a significant congenital kidney anomaly.
- Data on MCDK in sub-Saharan Africa are limited.
- This study addresses the pattern, predictors of involution, and outcomes of MCDK in South Africa.
Purpose of the Study:
- To examine the pattern of MCDK in South African children.
- To identify associated contralateral kidney abnormalities.
- To determine predictors of MCDK involution and assess short-term outcomes.
Main Methods:
- Retrospective study of children under 13 with unilateral MCDK.
- Data collected via ultrasound and [99mTc]Tc-MAG3 scans.
- Log-rank and Cox regression analyses used to find involution predictors.
Main Results:
- 98 cases (6.2%) of unilateral MCDK identified; 57.1% left-sided.
- Contralateral abnormalities in 17.3% (duplex kidney, UPJ obstruction).
- 69.1% showed involution; smaller initial kidney size (≤5.0 cm) predicted it (HR 2.42).
- CKD developed in 2.5%; 18.5% had UTIs. No malignancies or deaths.
Conclusions:
- Unilateral MCDK is common, with duplex kidney as a frequent contralateral anomaly.
- Contralateral hypertrophy is common; CKD progression is rare without contralateral anomalies.
- Adolescent follow-up may be beneficial; multicenter studies are needed for guidelines.
Introduction:
Multicystic dysplastic kidney disease (MCDK) is a notable congenital anomaly of the kidney and urinary tract, with potential risk for chronic kidney disease, yet data from sub-Saharan Africa remain scarce. This study examined the pattern of MCDK, associated contralateral kidney abnormalities, determined the predictors of MCDK involution and assessed short-term outcomes in children followed beyond one year in South Africa.
Method:
This retrospective study involved children under 13 years of age with suspected unilateral MCDK, confirmed on kidney ultrasound and [99mTc]Tc-MAG3 scans at the Red Cross War Memorial Children's Hospital between January 1, 2014, and December 31, 2023. Demographic, clinical, and radiologic data were obtained. The Log-rank test and Cox Proportional Hazards regression analyses were used to identify predictors of MCDK involution.
Results:
Among 1,581 new cases, 98 (6.2%) had unilateral MCDK. 50% were male, and 57.1% had left-sided involvement. Median follow-up was 60 months (IQR: 12-72). Contralateral kidney abnormalities occurred in 17 (17.3%), most commonly duplex kidney (35.3%) and ureteropelvic junction obstruction (29.4%), but no vesicoureteric reflux was noted. Of 81 children followed beyond a year, 80.2% demonstrated contralateral hypertrophy, and 69.1% exhibited involution of the affected kidney. Initial kidney size ≤ 5.0 cm was the sole predictor of involution (Hazard Ratio: 2.42, 95% CI: 1.31-4.48). Urinary tract infections occurred in 18.5%, proteinuria in 2.5%, hypertension in 1.2%, and 2.5% developed chronic kidney disease related to contralateral dysplasia. One nephrectomy was performed, and no malignancies or deaths. At last follow-up, 28.4% were lost to follow-up, and 12.3% had transitioned to adolescent clinics.
Conclusion:
All MCDK cases were unilateral, with duplex kidney being the most common contralateral abnormality, a distinctive finding previously unreported. Follow-up into adolescence may be beneficial, as progression of chronic kidney disease is rare in those without contralateral anomalies. Multicentre long-term studies are needed to provide standardised follow-up guidelines.
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