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Paeds Vivasnephrology-urology-fluids-and-electrolytes

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

Posterior urethral valves: Viva

Branching clinical structured oral on posterior urethral valves covering the Young classification and the pathophysiology cascade, the MCUG as the diagnostic gold standard, the immediate catheter decompression and endoscopic valve ablation, the valve-bladder management, and the long-term chronic kidney disease surveillance driven by the nadir creatinine.

branching clinical structured oral3 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A male neonate is reviewed in the nursery following a pregnancy complicated by antenatal oligohydramnios and bilateral hydronephrosis. He is tachypnoeic with a palpable distended bladder and a poor urinary stream, and a urethral catheter has been passed. The examiner asks how you classify the valves, what the pathophysiology is, what investigations confirm the diagnosis, how you manage the child, and what the long-term implications are.

Branch 1: Classification and pathophysiology

The candidate should define posterior urethral valves as congenital membrane-like folds in the male posterior urethra that obstruct the urinary outflow, and should state that they are the most common cause of lower urinary tract obstruction in male infants, occurring only in boys at approximately 1 in 4000 to 8000 live male births. A strong candidate classifies the valves by the Young system: the Type I valve, two leaflets fusing in the midline from the verumontanum, is by far the most common, the Type II valve is now regarded as non-obstructing and rare, and the Type III valve is a distal diaphragm with a central pinhole. [1]

If the examiner presses on the pathophysiology, the candidate should trace the urine. The valve blocks the outflow, the bladder thickens into a hard muscular wall, and the back-pressure travels up the ureters to cause bilateral hydronephrosis and renal dysplasia. The reduced fetal urine output causes oligohydramnios, which in the most severe cases produces the Potter sequence with pulmonary hypoplasia. The candidate should name pulmonary hypoplasia as reason worst-affected boys die of respiratory failure at birth, and should explain that the valve bladder persists after ablation. [1]

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References3ShowHide
  1. [1]Caione P, Nappo SG Posterior urethral valves: long-term outcome. Pediatr Surg Int, 2011.PMID 21748651
  2. [8]Coleman R, King T, Nicoara CD, et al Nadir creatinine in posterior urethral valves: How high is low enough? J Pediatr Urol, 2015.PMID 26292912
  3. [9]Khondker A, Chelliahpillai Y, Machado M, et al External validation and reliability assessment of posterior urethral morphology on initial voiding cystourethrogram as a predictor for infants with posterior urethral valves. J Pediatr Urol, 2024.PMID 38129272
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