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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

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

duplex collecting system: a duplex kidney draining into:


o

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
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.

Treatment and prognosis


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

vesicoureteric reflux into lower pole moiety

marked hydronephrosis of the upper pole moiety may have a mass effect or become infected

overview

Duplicated collecting systems (also known as duplex collecting systems)


can be defined as renal units containing 2 pyelocaliceal systems that are
associated with a single ureter or with double ureters. The 2 ureters
empty separately into the bladder or fuse to form a single ureteral orifice.
See the images of duplicated collecting system below.

Line diagram shows


development of complete ureteral duplication.
View Media Gallery

Excretory urography in an adult


patient with bilateral complete ureteral duplication.

View Media Gallery

Ultrasonographic scan of the


bladder of a child with bilateral ureteral duplication demonstrates bilateral
ureteroceles.
View Media Gallery
Duplex collecting systems can be unilateral or bilateral and can be
associated with a variety of congenital genitourinary tract abnormalities.
Most patients are asymptomatic, with genitourinary tract abnormalities
being detected incidentally on imaging studies performed for other
reasons. Symptomatic patients usually have complete ureteric
duplication in which the ureters are prone to developing obstruction,
reflux, and infection. Ureteropelvic obstruction is more common when a
duplex kidney exists and can be inherited in an autosomal dominant
pattern. [1]
Congenital renal anomalies in patients with classic bladder exstrophy
occur in 2.8% of patients. The most common anomaly is a duplicated
collecting system, which occurs in approximately 1.3% of patients.

Although uncommon, diagnosis is important for operative planning and


long-term follow up. [2]
Transitional cell carcinoma has been reported in patients with duplex
collecting systems.

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] :

Duplex kidney - The duplex kidney has a single renal parenchyma


that is drained by 2 pyelocaliceal systems.
Upper or lower pole - The poles represent one component of a
duplex kidney.
Duplex system - The kidney has 2 pyelocaliceal systems and is
associated with a single ureter or with a bifid ureter (a partial
duplication) or, in the case of a complete duplication, with 2 ureters
(double ureters) that drain separately into the urinary bladder.
Bifid system - Two pyelocaliceal systems join at the ureteropelvic
junction (bifid pelvis), or 2 ureters join before draining into the urinary
bladder (bifid ureters).
Double ureters - Two ureters open separately into the renal pelvis
superiorly and drain separately into the bladder or genital tract.
Upper and lower pole ureters - Upper and lower pole ureters drain a
duplex kidney's upper and lower poles, respectively.

Preferred examination

A patient's duplex kidney is almost always more elongated than his/her


nonduplex kidney. The kidney may be enlarged when hydronephrotic and

can be associated with rotational anomalies. Excretory urographic


findings are almost always diagnostic. Difficulty may arise when function
is poor or absent in one of the moieties. [5]
Magnetic resonance (MR) urography may be used as the primary
diagnostic method for assessing a duplex ectopic ureter, as well as the
complications associated with duplex kidneys. [6, 7] Spatial resolution is a
limiting factor. The availability of magnetic resonance imaging (MRI) is
limited, the procedure is expensive, and it requires the sedation of
patients with claustrophobia. However, MR urography is an extremely
useful technique in patients who have the probability of an adverse
reaction to radiopaque contrast media. [6]
If vesico-ureteral reflux exists, the presence of an ectopic ureter in a
nonfunctioning moiety can best be demonstrated using a voiding
cystourethrogram. [8, 9]
Antegrade pyelography is useful in patients with hydronephrosis, to
demonstrate the presence of a second ureter and to determine the level
of ureteric termination.
Computed tomography (CT) scanning with contrast is superior to
ultrasonography and excretory urography in diagnosing the nubbin. [10] On
excretory urography, an obstructed, nonfunctioning upper or lower pole
may mimic a renal mass. On a CT scan, a large column of Bertin can
mimic a duplex collecting system. However, CT scanning is valuable in
the evaluation of an intravesical ureterocele, either orthotopic or
ectopic. [11, 12]
Ultrasonography is a noninvasive and extremely useful examination,
particularly in children. The ultrasonographic appearance of a duplex
kidney is specific but not sensitive. [13] Ultrasonographic findings provide

excellent anatomic information but do not necessarily differentiate a bifid


renal pelvis from a bifid ureter or from 2 complete ureters.
Scintigraphy is useful in the assessment of relative renal function and in
the detection of renal scars. [14] Scintigraphy can reveal differential
functioning. However, if the functioning is markedly depressed, imaging
is limited. [15]
Plain radiography makes no major contribution to diagnosis. Plain
radiographs can demonstrate a renal mass, which is a nonspecific
finding. However, because a patient's duplex kidney is almost always
longer than his/her nonduplex kidney, a renal mass may be apparent on
a plain radiograph. Hydronephrotic upper or lower pole moiety in a
duplex collecting system also can be observed as a renal mass.
Arteriography is an invasive procedure and is no longer used to diagnose
duplex collecting systems. However, it may occasionally be useful in
planning nephron-sparing surgery. [16]