Journal of Pediatric Urology (2011) 7, 484e487

CASE REPORT

Ureteral triplication: A rare anomaly with a variety

of presentations
N. Kokabi a,b, N. Price a,b, G.H.H. Smith b, P.J. Gibbons c, A.J.A. Holland a,*

a
Douglas Cohen Department of Paediatric Surgery, The Children’s Hospital at Westmead, Sydney Medical School,
The University of Sydney, New South Wales, Australia
b
Department of Paediatric Urology, The Children’s Hospital at Westmead, Sydney Medical School,
The University of Sydney, New South Wales, Australia
c
Department of Orthopaedics, The Children’s Hospital at Westmead, Sydney Medical School, The University of Sydney,
New South Wales, Australia

Received 14 July 2010; accepted 7 December 2010

Available online 8 January 2011

KEYWORDS Abstract Ureteral triplication remains a very rare congenital malformation of the urinary
Triplication; tract with a wide spectrum of presentation. The sporadic nature of this condition and its asso-
Ureter; ciation with other anomalies makes evidence-based management difficult. We report two
VACTERL association; cases of triplication in association with the VACTERL syndrome, one developing pelvi-ureteric
Vesico-ureteric reflux; junction obstruction and the other vesico-ureteric reflux.
Pelvi-ureteric junction Crown Copyright ª 2010 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology
obstruction Company. All rights reserved.

Introduction part of the VACTERL syndrome. Or inherited as a component

Ureteric triplication, whilst rare compared to duplication,
shares a similar female predominance [1]. Although first
reported in 1927 by Perrin, it was classified into four types by
Smith [2] in his review of 11 cases in 1946, with over 100 cases
subsequently reported [1e3]. Triplication may occur in
isolation, in conjunction with other urological anomalies, as
* Corresponding author. Tel.: þ61 2 9845 1908; fax: þ61 2 9845
3346.
E-mail address: andrewh3@chw.edu.au (A.J.A. Holland).
of an autosomal dominant condition with amastia [1,3,4].
Pelvi-ureteric junction (PUJ) obstruction remains a common
cause of hydronephrosis in the paediatric population. Gener-
ally, the upper pole moieties do not form true renal pelvises but
often obstruct in association with ectopia and/or uretrocele. In
contrast, obstruction at the PUJ has been reported only
rarely [5].
We present two cases of ureteric triplication associated
with the VACTERL syndrome: one with an upper pole PUJ
obstruction and another in conjunction with a complex
ipsilateral lower limb anomaly.

1477-5131/$36 Crown Copyright ª 2010 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. All rights reserved.
doi:10.1016/j.jpurol.2010.12.006
Ureteral triplication: A rare anomaly
Figure 1 MRU revealing dilated, hydronephrotic upper pole
moiety of left kidney.
Figure 2 RPG indicating Smith type III left ureteral triplica-
tion. Note tapered appearance of upper pole moiety, with an
absence of filling, as a result of the PUJ obstruction.
485
Figure 3 Diagram illustrating anatomical configuration
following upper pole pyeloplasty.
Case report
Case 1
A term male infant was diagnosed with the VACTERL
syndrome with an anorectal malformation (ARM), a ventric-
ular septal defect and left-sided hydronephrosis initially
diagnosed by abdominal ultrasound. A micturating cystour-
ethrogram (MCUG) revealed bilateral VUR for which he was
commenced on prophylactic antibiotics. The ARM was initially
treated with a colostomy and subsequent anorectoplasty at 3
months of age and the ventricular septal defect repaired at 7
months of age.
Although the patient remained infection free during
regular follow up, he was noted to have a duplex left kidney
(not seen on initial ultrasound) and increasing hydro-
nephrosis of the upper pole on subsequent ultrasound
between 2 and 3 years of age. Intravenous pyelography
revealed a probable bifid left renal system with obstruction
of the upper pole component.
To delineate the anomalous anatomy, MR urography (MRU)
was obtained (Fig. 1), revealing a hydronephrotic upper pole
moiety. Retrograde pyelography (RPG) confirmed that the
ureter underwent fusiform dilatation at the level of the lower
pole of the kidney and then trifurcated (Fig. 2, Smith type III).
At subsequent open operation the findings were confirmed,
with the attenuated upper ureter found to attach in a non-
dependent fashion to a grossly dilated renal pelvis in a typical
PUJ configuration. A dismembered pyeloplasty was per-
formed, via a standard approach, with the anastomosis ante-
rior to the renal pedicle (Fig. 3). Performing a dismembered
486
Figure 4 MCUG revealing bilateral grade III VUR with a right-
sided Smith type III ureteral triplication.
pyeloplasty was straightforward once the abnormality had
been clearly identified, including the upper, middle and lower
segment vessels with all three ureters located posteriorly. The
anastomosis was placed anteriorly to avoid any future vascular
compression.
Figure 5 RPG illustrating lower and middle pole moieties of
Smith type III right ureteral triplication.
N. Kokabi et al.
The patient made an uneventful recovery and a MAG3
Lasix scan three months later was normal with diuretic half
clearance time (t1/2) of 2.1 min in both right and left
kidneys (normal < 10 min).
Case 2
A term female infant was noted at birth to have an ARM and
a complex deformity of the right foot with nine meta-
tarsals, each having corresponding digits. Whilst a DMSA
scan revealed equal renal function with no evidence of
scarring, cystoscopy revealed that the single right ureteric
opening was located slightly laterally. MCUG revealed
bilateral grade III VUR (Fig. 4), with a triplicated system on
the right confirmed on subsequent RPG (Fig. 5, Smith type
III), ipsilateral to her lower limb deformity. She commenced
on prophylactic antibiotics and has remained infection free
for 2 years, with equal renal function and no hydro-
nephrosis. MRU was not performed since, based on our
anecdotal experience, it should be utilized in cases where
anomalous anatomy is difficult to determine.
Her ARM was treated with an anorectoplasty at 4 months
of age, complicated subsequently by mucosal prolapse
requiring sclerotherapy. The lower limb deformity was
treated with a Syme amputation through the ankle which
facilitated adequate flap coverage and early mobilization
with the aid of a prosthesis.
Discussion
Ureteral triplication is presumed to arise either as a result of
splitting of the ureteric bud or from formation of an acces-
sory ureteric outgrowth of the Wolffian duct at around the
fifth week of gestation [1]. The association with VACTERL
syndrome might therefore be expected based on a defect in
mesodermal differentiation early in the first trimester [1].
Triplication may remain asymptomatic (8% of cases) or
present with recurrent UTIs, urinary incontinence or renal
colic [2,3]. It appears frequently associated with other
urological anomalies such as ureteral ectopia, contralateral
ureteric duplication, VUR and renal dysplasia [1e3]. There
appears to have been only one previous case of ureteral trip-
lication associated with PUJ obstruction, albeit in the middle
pole, with none reported involving the upper pole [6]. In this
rare setting, MRU should be considered to assist in defining the
anatomical configuration prior to corrective surgery.
In triplication with associated persistent UTIs, hemi-
nephrectomy with removal of the supernumerary ureters has
been shown to be effective [7]. Although ureteroureterostomy
may be beneficial, when incontinence has been the presenting
complaint, the ectopic ureter should be removed [8]. As
elaborated in the budding theory of Mackie and Stephens, the
more ectopic a ureter, the more dysplastic the segment it
drains [9]. In general, therefore, an ectopic segment would
not be expected to make a useful contribution to function.
Our second patient’s complex lower limb anomaly, occur-
ring on the same side as her ureteral triplication, appears not
to have been previously reported. In general, lower limb
anomalies appear much less common than those involving the
upper limb, and when they do occur typically involve a tibial
field defect rather than partial forefoot duplication [10].
Ureteral triplication: A rare anomaly
Ethics statement
This study was approved by the Ethics Committee of our
institution.
Conflict of interest statement
The authors have no conflict of interest to report.
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