PRACTI CAL URORADI OLOGY

Urol Sci 2010;21(2):9698

96 2010 Taiwan Urological Association. Published by Elsevier Taiwan LLC.
Duplex Kidney and Related Abnormalities
Jia-Hwia Wang
1,2
*
1
Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan
2
School of Medicine, National Yang-Ming University, Taipei, Taiwan
*Corresponding author. Department of Radiology, Taipei Veterans General Hospital, 201, Shih Pai Road,
Section 2, Taipei 112, Taiwan.
E-mail: wangjh@vghtpe.gov.tw
A 20-year-old woman felt right flank pain 2 years previ-
ously. At this time, she went to a local medical clinic where
an abnormal urinary tract on the right side was found by
imaging examinations. Surgical intervention was recom-
mended, but she did not want to have surgery at that
time. During those 2 years, intermittent right flank pain
(three times per month) still bothered her. Therefore,
she went to the outpatient department of our hospital.
Sonogra phy of the kidney was carried out, revealing du-
plex bi lateral kidneys with hydronephrosis of the upper
moiety of the right duplex kidney. Abdominal computed
tomo graphy was then carried out, which revealed the
following: (1) a right complete duplex kidney with an
ectopic ureteral orifice and ureterocele, hydronephrosis
and hydroureter of the upper moiety of the right complete
duplex kidney; and (2) a left incomplete duplex kidney
(Figure 1). Right retrograde pyelography (Figure 2) and
cystography (Figure 3) were carried out, which confirmed
the computed tomography findings of the abdomen. Two
right ureteral orifices were found with cystoscopy: one was
near the bladder neck, and the other associated with the
uretero cele was over the trigone. Surgical intervention
was recommended. She was admitted to our hospital for
further management.
Physical examination did not show any abnormalities.
Laboratory examinations showed that all parameters
were within normal limits. Surgical removal of the upper
moiety and its ureter of the right complete duplex kidney
was performed. The postoperative course was unremark-
able. The patient was discharged in a stable condition,
and outpatient department follow-up was recommended.
A duplex (duplicated) kidney is a kidney that has two
pyelocalyceal systems and is associated with a bifid ureter
(incomplete duplication) or two ureters (double ureters)
that empty separately into the bladder (complete dupli-
cation). A duplex kidney occurs in 12% of the population
and may be unilateral or bilateral.
1
Usually the lower
moiety is dominant and drains most of the kidney, and a
large renal pelvis drains the lower moiety via multiple cal-
yces. The upper moiety may have only a single calyx and
Accepted: April 13, 2010
There are 2 CME questions based on this article
Figure 1 Coronal view of contrast-enhanced computed tom-
ography of the abdomen shows (1) ectopic ureteral orifice
with ureterocele over the trigone of the urinary bladder,
hydronephrosis and hydroureter of the upper moiety of a right
complete duplex kidney; (2) downward displacement of the
normal collecting system of the lower moiety of a right com-
plete duplex kidney by hydronephrosis of the upper moiety
of the right complete duplex kidney (drooping lily sign); and
(3) a left incomplete duplex kidney.
Duplex kidney and related abnormalities
Vol. 21, 9698, June 2010 97
a single infundibulum, and no renal pelvis. Each ureter has
its own vesical orifice. The ureter that drains the upper
moiety is ventral to the lower one, but crosses over and
empties into the bladder in a lower, more medial ectopic
location (Weigert-Meyer rule). In males, the ureter may
open into the seminal vesicles, the vas deferens, the ejac-
ulatory duct or the posterior urethra. In females, the ure-
ter may open into the urethra, the lateral vulvar wall, the
uterus, the vagina, or rarely, the rectum.
In patients with complete ureteral duplication, ob-
struction at the ureterovesical junction with hydro-
nephrosis and ectopic ureteral orifice associated with
ureterocele may occur in the upper moiety. Vesico-
ureteral reflux into the lower moiety occurs in 50%
of patients with lower moiety ureteropelvic junction
obstruction.
2,3
The most common symptoms of duplex kidney are
flank pain and hematuria caused by obstruction and in-
fection of the upper moiety of duplex kidney.
4
Ectopic
ureteral orifice is usually associated with urinary inconti-
nence in females, resulting from insertion of the ure-
teral orifice below the urethral sphincter.
The imaging findings of duplex kidney depend on the
degree of obstruction. If both moieties of duplex kidney
remain functioning, the diagnosis is usually achieved
by intravenous urography. However, the upper moiety
commonly becomes obstructed and enlarged, resulting
in a nonfunctioning upper moiety mass effect (hydrone-
phrosis). The opacified calyces of the lower moiety may
be displaced by the large nonfunctioning upper moiety
hydronephrosis (drooping lily sign) (Figure 1).
5
Retrograde
pyelography may confirm the diagnosis.
A B
Figure 2 Right retrograde pyelography shows (A) a right complete duplex kidney with hydronephrosis and hydroureter of its
upper moiety, and (B) a normal collecting system and ureter of its lower moiety.
F
Figure 3 Cystography shows ectopic ureteral orifice with ure-
terocele (arrow) over the trigone of the upper moiety of a
right complete duplex kidney. F = Foleys catheter.
Figure 4 Non-contrast computed tomography of the kidneys
shows faceless kidney of a left incomplete duplex kidney and
hydronephrosis of the upper moiety of a right complete duplex
kidney.
J.H. Wang
98 Vol. 21, 9698, June 2010
Computed tomography is an excellent method for
detecting obstruction in either moiety of a duplex kidney.
6

It can also determine whether the orifice of an obstructed
upper moiety ureter is intravesical or extravesical. Face-
less kidney has been reported as a computed tomogra-
phy sign of duplex kidney (Figure 4).
6
Cystography or
cystoscopy may be needed to locate the ureteral orifices
of duplex kidneys. However, sometimes ectopic ureteral
orifices cannot be located, and one may need to rely on
antegrade pyelography or an operation for identification.
We conclude that complete duplex kidney associated
with ectopic ureteral orifice and hydronephrosis of the
upper moiety should be treated by surgical removal of
the upper moiety and its ureter.
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