The Corresponding Author:
Seyed Mohammadreza Rabani; Associate Professor of Urology and
Renal TX
Beheshti Teaching Hospital; Yasuj University of Medical Sciences; Yusuj;
Iran
smrrabani@gmail.com; +989177411389, fax no: +987433235153
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Key Clinical Message
Although the urinary tract is a site for common anomalies, but gathering
all of the anomalies such as UPJO, UVJO, Ectopia, double collecting
system, and reflex of lower moiety to upper one, reported in our
patient, may be rare congenital anomalies
Unilateral multiple Renal Congenital Anomalies: Ectopia; Double
Collecting system; uretero-pelvic junction obstruction; uretero- vesical
junction obstruction, Lower Moiety Ureter to Upper Moiety Ureter Reflux;
A Case Report
Abstract
Urinary tract is a site for common anomalies, but gathering all of the
anomalies reported in our patient, and also being unilaterally, may be a
rare entity. Hereby we report a case with such a rare condition.
Key words: Renal congenital anomalies, renal ectopia, congenital
anomalies of the kidneys, urinary tract system
Introduction
Congenital anomalies of the urinary tract have a wide spectrum including
upper and lower urinary tract anomalies such as renal hypoplasia,
dysplasia, aplasia, ectopia, supernumerary, duplication, uretero-pelvic
junction obstruction (UPJO), uretero-vesical junction obstruction
(UVJO), vesico-ureteral reflux (VUR), ureterocele, posterior urethral
valve, bladder exstrophy, and many other anomalies. The causes for these
wide ranges of urinary tract anomalies are related to abnormal
embryologic development of the urinary tract (1, 2). The urinary tract
system is substantially derived from the intermediate mesoderm (3, 4),
and base on the underlying defect and the disturbances in the
developmental process, different types of congenital anomalies may
develop in the urinary system.
Case Presentation
A 19-year-old army male presented with left lower quadrant (LLQ)
abdominal pain after a blunt abdominal trauma, accompanied with gross
hematuria. He was healthy and asymptomatic until that during a sport
activity has a blunt abdominal trauma and developed pain and gross
hematuria. On arrival to emergency room, physical examination revealed
LLQ tenderness with stable vital signs. Primary laboratory data showed
normal renal function tests with gross hematuria. Ultrasonography
suggested ectopic pelvic left Kidney with hydronephrosis and UPJO with
distal ureteral dilatation. Intravenous pyelography (Figure 1) confirmed
the same findings. Renal DTPA scanning (Figure 2) was done that
suggested ectopia, double collecting system, lower moiety uretero-pelvic
junction obstruction and a common distal ureter with UVJO. Cystoscopy
and try to perform ureteroscopy were failed to pass a catheter into the
left ureter that was a plan for retrograde uretero-pyelography, because
the UVJ area was very narrow. Exploration was done through a Gibson’s
retroperitoneal incision. The upper moiety ureter was significantly
dilated, but the lower moiety was thin and narrow, both of them were
ended into a tapered segment typical for adynamic segment and UVJO.
Pyeloplasty and ureteroneocystostomy were carried out simultaneously. 3
months and 6 months Follow-up visits revealed no relevant complaint and
the patient was asymptomatic, but denied any study for Follow-up due to
COVID 19 situation. The patient has an uneventful post-operative 18
months follow up.
Discussion
Development of the urinary tract is a very complex process and depends
on regular and precise events. Normal Development of the urinary system
also needs integration of a variety of cell populations that have
different embryologic origins (1-3). The final products of these events
are the functional and the conduit part of the urinary system. The
evolution of our knowledge in genetics and molecular biology has greatly
advanced our understanding about pathophysiology of urinary tract
congenital anomalies (5,6 ). Although anomalies of the urinary
tract are among the most common anomalies of the body, there are also a
great number of varieties of anomalies in upper and lower urinary tract
system.
In this patient we have accidentally encountered Unilateral multiple
Renal Congenital Anomalies: Ectopia; Double Collecting system; UPJO, and
UVJO. Each of these has its own pathophysiology and clinical
presentation that will be discussed briefly.
Renal Ectopia
An ectopic kidney is a kidney that isn’t placed in its normal flank
fossa position. It has 2 kinds: cross ectopia in which one of the
kidneys crosses the midline and both kidneys are located in one side of
the body, and simple ectopia in which the kidney may be ipsilaterally
located thoracic, cephalad, lower abdominal, or in the pelvis like our
patient. The cause of this anomaly may be a defect in ascent process of
the kidney or abnormal ureteric bud. An ectopic kidney may be
asymptomatic lifelong, or may be associated with other abnormalities
such as UPJO, because of high inserted ureter, malrotated kidney, and
aberrant vessels.
Duplicated System
Duplicated system may be complete or partial. It is the result of
unusual development of ureteral bud. In complete duplication, two
ureteral buds emerge from the wolfian duct and results in two complete
sets of kidneys and ureters that may insert into the bladder separately.
Partial duplication can be a result of ureteral bud stalk bifurcation
before invading or prior to initial branching of the primary ureteral
bud into the metanephric mesenchyme (7). In complete duplicated system,
the Wiegert-Meyer rule can be seen in 95% of the cases with
consequences of vesico-ureteral reflux and ureterocle or ectopic ureter,
but in our case UVJO was seen. Duplication system is more common in
women than in men with a ratio of 2:1 (7).
Ureteropelvic junction obstruction (UPJO)
UPJO is an obstructive uropathy at the level of junction between renal
pelvis and the origin of the ureter and may be intrinsic or extrinsic in
nature. It accounts for about 50% of all intrauterine detectable
hydronephrosis and it is really the most common cause of congenital
obstructive nephropathy (8,9). It occurs more frequently in males than
in females (5:2) and more frequently on the left side. In our patient
UPJO was in the lower moiety of a duplicated system, that is a rare type
of this anomaly.
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Uretero-vesical junction obstruction (UVJO)
UVJO is an obstruction at the level of the end of ureter where it enters
the bladder. It is the result of an anatomic or a functional abnormality
in the distal segment of the ureter (10). The usual cause for UVJO is a
defect in the peristalsis of distal part of the ureter (an adynamic
segment), that acts as obstruction and causes backward pressure to the
kidney and finally presents with hydroureteronephrosis or megaureter. In
our patient the UVJ was narrow, so that any catheter could not be passed
for performing retrograde pyelography, but during exploration typical
tapering of the common ureter was seen while the lower moiety ureter was
thin and narrow. I think the lower moiety ureter was refluxing into the
dilated upper moiety ureter. The patient underwent simultaneous
pyeloplasty and anti-refluxing ureteroneocystostomy.