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Paeds Vivasnephrology-urology-fluids-and-electrolytes

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

Congenital anomalies of the kidney and urinary tract: Viva

Branching clinical structured oral on congenital anomalies of the kidney and urinary tract covering the classification and embryology, the antenatal hydronephrosis investigation pathway, the management of posterior urethral valves and multicystic dysplastic kidney, and the long-term CKD surveillance.

branching clinical structured oral2 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A 2-week-old infant is reviewed in the outpatient clinic following an antenatal finding of left-sided hydronephrosis. The postnatal ultrasound confirms moderate left hydronephrosis with a normal right kidney. The examiner asks how you would classify the CAKUT spectrum, what investigations you would arrange, how you would manage the child, and what the long-term implications are.

Branch 1: Classification and embryology

The candidate should define CAKUT as the spectrum of congenital structural malformations of the kidney and urinary tract that arise from errors in embryological development. A strong candidate classifies the spectrum into three anatomical groups: the kidney and parenchymal anomalies including renal agenesis, hypoplasia, dysplasia, and the multicystic dysplastic kidney, the ureteric and collecting system anomalies including vesicoureteral reflux, megaureter, ureterocele, and duplex systems, and the bladder and urethral anomalies including posterior urethral valves, prune belly syndrome, and neurogenic bladder. [1]

If the examiner presses on the embryology, the candidate should describe the ureteric bud and the metanephric mesenchyme interaction. The ureteric bud grows from the Wolffian duct and invades the metanephric mesenchyme, branching to form the collecting system while the mesenchyme forms the nephron. The GDNF-RET pathway is the master regulator, and disruption produces agenesis, dysplasia, or ureteric anomalies depending on the stage and the nature of the disruption. A strong candidate names the Weigert-Meyer rule for duplex systems, in which the upper-pole ureter inserts ectopically and distally. [1]

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References3ShowHide
  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. [6]Aslam M, Watson AR Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child, 2006.PMID 16754654
  3. [7]Lee RS, Cendron M, Kinnamon DD, et al Antenatal hydronephrosis as a predictor of postnatal outcome: a meta-analysis. Pediatrics, 2006.PMID 16882811
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