pelviureteric junction obstruction

Pelviureteric junction (PUJ) obstruction/stenosis, also known as ureteropelvic junction (UPJ) obstruction/stenosis, can be one of the causes of obstructive uropathy. It can be congenital or acquired with a congenital pelviureteric junction obstruction being one of the commonest causes of antenatal hydronephrosis.

Epidemiology

It may present in both pediatric and adult populations although they tend to have differing etiology. The estimated incidence in pediatric populations is ~1 per 1,000-2,000 newborns, and there is a male predominance (M:F = 2:1).

Clinical presentation

Many cases are asymptomatic and identified incidentally when the renal tract is imaged for other reasons. When symptomatic, symptoms include recurrent urinary tract infections, stone formation and even a palpable flank mass. They are also at high risk of renal injury even by minor trauma.

Classically intermittent pain after drinking large volumes of fluid with a diuretic effect is described, due to the reduced outflow from the renal pelvis into the ureter .

Pathology

Pelviureteric junction obstruction is most commonly unilateral but is reported to be bilateral in ~30% (range 10-49%) of cases . There is a recognized predilection towards the left side (~67% of cases).

During embryogenesis, the pelviureteric junction forms usually around the fifth week and the initial tubular lumen of the ureteric bud become recanalized by ~10-12 weeks. The pelviureteric junction area is the last to recanalize. Inadequate canalization is thought to be the main embryological explanation of a pelviureteric junction obstruction. Extrinsic obstructions secondary to bands, kinks, and aberrant vessels also are commonly encountered.

Interestingly, research has failed to identify any anatomically-discrete pelviureteric junction although physiologically there is evidence of a sphincter-like action in this region .

Etiology
  • congenital (neonatal)
    • idiopathic and often unknown; proposed causes include
      • abnormal muscle arrangement at the pelviureteric junction
      • anomalous collagen collar at pelviureteric junction
      • ischemic insult to pelviureteric junction region
      • urothelial ureteral fold
    • extrinsic ureter compression or encasement
      • crossing vessel (at pelviureteric junction)
  • adult
Associations

Radiographic features

Fluoroscopy/IVU

Traditionally intravenous urography/pyelography (IVP/IVU) has been performed for assessing for pelviureteric junction obstruction. The administration of furosemide may be used to assist in confirming the diagnosis, in particular, to exclude a dilated non-obstructed upper collecting system (so-called 'baggy pelvis').

Ultrasound
  • will often show a dilated renal pelvis with a collapsed proximal ureter
  • Doppler sonography: the obstructed kidneys may show higher resistive indices
CT

May show evidence of hydronephrosis +/- caliectasis with collapsed ureters. Useful for assessing crossing vessels at the pelviureteric junction, especially when surgical intervention is planned .

Renal scintigraphy

Renal scintigraphy can quantitate the degree of obstruction:

  • Tc-99m-MAG3: Agent of choice due to a high extraction rate, which may be necessary for an obstructed system. Diuretic (furosemide) renogram is performed to evaluate between obstructive vs. nonobstructive hydronephrosis. The non-obstructive hydronephrosis will demonstrate excretion (downward slope on renogram) after administration of diuretic from the collecting system. Whereas mechanical obstructive hydronephrosis will show no downward slope on renogram, with retained tracer in the collecting system.
  • Tc-99m-DTPA: Not the agent of choice because of predominantly glomerular filtration of Tc-99m-DTPA. Glomerular function declines earlier and more rapidly than does tubular function in obstructive uropathy. Tc-99m-DTPA may be used if the kidney is known to have good function.
  • Treatment and prognosis

    Treatment depends on the underlying cause. In a majority of congenital cases, the condition is benign, and usually, no intervention is required. However, when there is a definitive structural obstruction (commonly adult cases), surgical intervention with pyeloplasty or stenting may be necessary.

    Differential diagnosis

    General imaging differential considerations include:

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