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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
Fanconi anaemia 3
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
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
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:
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
appearance" 1. 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.
marked hydronephrosis of the upper pole moiety may have a mass effect or become infected
overview
Nomenclature
When a single ureteral bud bifurcates before the ampulla bifurcates, a
duplex kidney with a bifid renal pelvis or bifid ureter results. [3] If 2 ureteral
buds arise from the Wolffian duct, a duplex kidney with complete ureteral
duplication ensues. The Committee on Terminology, Nomenclature, and
Classification of the Section on Urology of the American Academy of
Pediatrics suggests the use of the following terms in reference to duplex
collecting systems [4] :
Preferred examination