Duplex collecting system

Dr Henry Knipe and Dr Jeremy Jones et al.

A duplex collecting system, or duplicated collecting system, is one of the most common congenital renal tract abnormalities. It is characterised by an incomplete fusion of upper and lower pole moieties resulting in a variety of complete or incomplete duplications of the collecting system. While considered an anatomical variant, duplex collecting systems may be complicated by vesicoureteric reflux, obstruction or ureterocoele. 

Epidemiology

Duplex collecting systems are seen in 0.7% of the healthy adult population and 2-4% of patients investigated for urinary tract symptoms 7.

Clinical presentation

Most duplicated systems asymptomatic and diagnosed incidentally. However, where symptoms do occur (infection, reflux or obstruction), the patient is likely to have completely duplicated ureters. Occasionally, hydronephrosis can be severe enough to result in flank discomfort or even a palpable mass. 

Pathology

Embryologically, duplication occurs when two separate ureteric buds arise from a single Wolffian duct. Interestingly, and explaining the Weigert-Meyer rule, the future lower pole ureter separates from Wolffian duct earlier and thus migrates superiorly and laterally as the urogenital sinus grows.

Duplication can be variable. At one end of the spectrum, there is merely a duplication of the renal pelvis, draining via a single ureter. At the other extreme, two separate collecting systems drain independently into the bladder or ectopically (see below)

Duplex systems may be unilateral or bilateral and can be associated with a variety of other congenital abnormalities of the urinary tract, e.g. ureterocoele

Associations
Classification

Duplex collecting system or duplex kidney anomalies can be classified into the following categories depending on the level or lack of fusion 9-10:

  • duplex kidney: two separate pelvicalyceal systems draining a single renal parenchyma
  • duplex collecting system: a duplex kidney draining into:
    • single ureter: i.e. duplex kidney's duplication pelvicalyceal systems uniting at the pelviureteric junction (PUJ)
    • bifid ureter (ureter fissus): two ureters that unite before emptying into the bladder
    • double ureter (complete duplication)
  • bifid collecting system: refers to a duplex kidney with the two separate pelvicalyceal collecting systems uniting at the PUJ or as bifid ureters
  • double/duplicated ureters (or collecting system): two ureters that drain separately into the bladder or genital tract

Radiographic features

As the abnormality is an anatomic alteration, all modalities able to image the renal tract may be able to visualise the typical features. 

General features include:

  • duplicated ureters extending a variable distance down to the bladder
  • obstruction of the upper pole moiety down to the bladder, often with a ureterocoele
  • vesicoureteric reflux into the lower pole moiety, often due to distortion in its insertion by the aforementioned ureterocoele
  • ectopic insertion of the upper pole moiety e.g. into the prostatic urethra in males or vaginal vault in females

Additionally, if reflux is significant, evidence of reflux nephropathy may be evident. 

Fluoroscopy
Excretory urography (IVP)

Although able to elegantly image both collecting systems, one should be aware that a poorly functioning system may not excrete contrast. In such a situation, the functioning lower pole moiety will be inferiorly displaced, taking on the so-called "drooping lily appearance1. The differential for such an appearance is that of an upper pole mass or cyst. 

Ultrasound

Ultrasound, when no obstruction/hydronephrosis is present can be suboptimal in the detection of a duplicated system and will especially struggle to distinguish between partial and complete duplication. It is certainly able to detect ureterocoeles, if present.

It provides excellent anatomic information but does not necessarily differentiate a bifid renal pelvis from a bifid ureter or two complete ureters. 

CT

CT can delineate all abnormalities essentially, especially when performed during the excretory (IVP) phase (contrast outlining the collecting systems). CT reconstruction software can produce striking single images of the collecting systems. In an unobstructed system, the diagnosis can be difficult. A duplicated renal collecting system can be suspected by identifying the so-called faceless kidney

Nuclear medicine

Renal scintigraphy is significantly impaired in its ability to identify non-obstructed systems, as the spacial resolution is poor. However, it is able to evaluate renal function and is particularly useful in planning corrective surgery. 

Treatment and prognosis

A duplex kidney usually does not require any treatment per se, however, complications may necessitate intervention:


Abdominal and pelvic anatomy
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Article Information

rID: 5700
System: Urogenital
Section: Anatomy
Synonyms or Alternate Spellings:
  • Duplex system
  • Duplicate collecting system
  • Duplicated collecting system
  • Duplex kidney
  • Duplex ureter
  • Duplex renal collecting system
  • Duplicted renal collecting system

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    Figure 1: illustration
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    Case 1: with ureterocoele
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    Case 2: ureteral duplication (CT)
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    Case 3: duplex renal system
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    Duplex left kidney
    Case 4: duplex left kidney
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    MCU

Grade 5 refl...
    Case 5: duplex left kidney in five month old
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    Case 6: with drooping lilly sign
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    Case 7: with unusual obstructed lower pole moiety
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    Case 9: with drooping lily sign
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    Faceless kidney
    Case 10: with faceless kidney
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    Case 11: duplex collecting system in six week old
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    In the same patie...
    Case 12: complete duplication
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    Case 13: with ureterocele
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Incomplete righ...
    Case 14: duplicated collecting system (IVP)
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    Right kidney
    Case 15: on ultrasound
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    Case 16: bilateral
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    Case 17: bilateral with ureterocoele
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    Case 18: bilateral
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    Case 19: bifid ureter
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    Case 20: on left
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    A post compressio...
    Case 21
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    Case 22: bilateral
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    Case 23
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    Case 24: with contralateral PUJ obstruction
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    Case 25
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    Case 26: with malrotation
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