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Paeds Casesnephrology-urology-fluids-and-electrolytes

Paeds Cases · nephrology-urology-fluids-and-electrolytes

Congenital anomalies of the kidney and urinary tract: Case

Clinical case of an infant with a unilateral multicystic dysplastic kidney and contralateral vesicoureteral reflux, covering the antenatal detection, the postnatal investigation pathway, the conservative management and surveillance, and the long-term risk of hyperfiltration injury and chronic kidney disease.

nephrology long case3 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 6-month-old infant is reviewed in the paediatric nephrology clinic following an antenatal finding of a right-sided cystic renal mass. The postnatal ultrasound confirmed a right multicystic dysplastic kidney with a normal-appearing left kidney. The MCUG showed grade 3 vesicoureteral reflux into the left kidney. The infant has had one febrile urinary tract infection at 3 months of age, caused by Escherichia coli. The blood pressure is normal, the growth is on the 25th centile, and the serum creatinine is 42 micromoles per litre. The DMSA scan shows a non-functioning right kidney and a left kidney with 100 percent of the total function and no cortical defects.

This infant has a right multicystic dysplastic kidney with a non-functioning right kidney on the DMSA scan and a grade 3 vesicoureteral reflux into the left, the solitary functioning kidney. The right multicystic dysplastic kidney is a parenchymal CAKUT, and the contralateral reflux into the left kidney is a common and important association, because the left kidney carries the entire renal function. The febrile urinary tract infection at 3 months reflects the reflux into the left kidney, and the prevention of further infection is the priority. [6]

Clinical findings

The key clinical findings are the non-functioning right multicystic dysplastic kidney, the grade 3 vesicoureteral reflux into the left kidney, the history of one febrile urinary tract infection, the normal blood pressure, the growth on the 25th centile, and the serum creatinine of 42 micromoles per litre. The DMSA scan confirms that the right kidney is non-functional and the left kidney has 100 percent of the total function with no cortical scarring, which is reassuring but places the entire renal burden on the refluxing left kidney. [5]

The differential for the contralateral anomaly in a child with a multicystic dysplastic kidney includes vesicoureteral reflux, which is the most common, a ureteropelvic junction obstruction, and renal hypoplasia. The MCUG has confirmed the grade 3 reflux, and the DMSA has shown no scarring in the left kidney. The serum creatinine is normal for age, but the long-term risk to the solitary functioning kidney is the central concern. [6]

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References4ShowHide
  1. [1]Murugapoopathy V, Gupta IR A Primer on Congenital Anomalies of the Kidneys and Urinary Tracts (CAKUT). Clin J Am Soc Nephrol, 2020.PMID 32188635
  2. [5]Matsell DG, Bao C, Po White T, et al Outcomes of solitary functioning kidneys-renal agenesis is different than multicystic dysplastic kidney disease. Pediatr Nephrol, 2021.PMID 33954810
  3. [6]Aslam M, Watson AR Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child, 2006.PMID 16754654
  4. [10]Harambat J, van Stralen KJ, Kim JJ, Tizard EJ Epidemiology of chronic kidney disease in children. Pediatr Nephrol, 2012.PMID 21713524
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