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Congenital anomalies of the ureter and bladder

  • Lesion

Developmental errors of the ureters and bladder are common; the ones that matter block urine, let it flow backwards or leave open channels.

Updated

What it is

Anomalies of the ureter appear in a few percent of autopsies, most without symptoms. Ureteropelvic junction obstruction is the leading cause of hydronephrosis in infants and children. Duplicated and bifid ureters, ureteroceles and ectopic openings favour obstruction and reflux. In the bladder, diverticula, exstrophy and remnants of the urachus are the main lesions; vesicoureteral reflux, from a short intravesical course of the ureter, is the most important for kidney damage.

Structure

  • UPJ obstruction: dilated pelvis with a narrow junction; the muscle is disorganized and replaced by collagen.
  • Diverticulum: pouch of mucosa through the muscle layer, with thin wall, often inflamed.
  • Exstrophy: exposed bladder mucosa that becomes inflamed, ulcerated and metaplastic.
  • Urachal remnant: a channel between the bladder dome and the umbilicus, lined by urothelium or glandular epithelium.

Congenital anomalies of the ureter and bladder

AnomalyProblemConsequence
Ureteropelvic junction obstructionNarrow, poorly propulsive junctionHydronephrosis in infants; in adults more often in women, unilateral
Duplicated or bifid ureterTwo ureters from one kidneyObstruction of the upper moiety, reflux into the lower
Vesicoureteral refluxShort intravesical tunnelPyelonephritis and reflux nephropathy
Bladder exstrophyOpen anterior bladder and abdominal wallInfection, metaplasia, adenocarcinoma
Urachal anomaliesPersistent connection to the umbilicusUmbilical leak, cyst infection, adenocarcinoma at the dome

Clinical notes

  • Prenatal ultrasound often shows hydronephrosis; many cases of mild reflux resolve as the child grows.
  • A cyst or mass at the bladder dome of an adult suggests a urachal lesion.

Test yourself

Think of the answer, then tap the question to see it.

What is the commonest cause of hydronephrosis in infants and children?

Ureteropelvic junction obstruction.

Which cancer arises in urachal remnants and exstrophic bladders?

Adenocarcinoma.

Why does a short intravesical ureter cause reflux?

The bladder muscle cannot compress the ureter during voiding, so urine flows back toward the kidney.

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: Vesicoureteral Reflux (VUR) (NIDDK (National Institutes of Health), 2024); Embryology, Kidney, Bladder, and Ureter - StatPearls - NCBI Bookshelf (StatPearls Publishing (NCBI Bookshelf), 2023).

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Representative model, for educational purposes only.