International Urogynecology Journal

https://doi.org/10.1007/s00192-020-04587-9

REVIEW ARTICLE

Embryology of the urogenital tract; a practical overview

for urogynecologic surgeons
Tiffanie Tam 1 & Rachel N. Pauls 1

Received: 5 August 2020 / Accepted: 22 October 2020

# The International Urogynecological Association 2020

Abstract
Introduction and hypothesis Urinary tract anomalies are one of the most common birth defects. Nevertheless, they prove
challenging to diagnose as a result of variable presenting symptoms. We aimed to perform a review of urogenital tract develop-
ment, highlight common congenital upper urinary tract anomalies encountered by urogynecologists and tools to facilitate
diagnosis.
Methods Multiple searches were performed utilizing resources such as PubMed and the TriHealth library database to access
publications related to embryology of the urinary tract and urinary tract anomalies. Each citation was reviewed.
Results Congenital urinary tract anomalies account for up to 20% of all birth defects and occur more often in females. The true
incidence of these malformations is unknown as some can remain clinically insignificant throughout life. In addition, patients
may present with non-specific complaints such as urinary tract infections, nephrolithiasis or urinary incontinence. Therefore,
unsuspected anomalies pose a risk of delayed diagnosis and potential injury during urogynecologic surgery. Imaging modalities
such as computed tomography or magnetic resonance imaging are the most common diagnostic tests. Management and treatment
options range from observation to surgical resection with the goal of optimizing long-term functionality and prevention of
chronic sequelae.
Conclusion Patients with urinary tract anomalies can present with vague complaints often encountered by urogynecologists. It is
crucial to understand the embryologic development of urinary tract anomalies to help facilitate diagnosis and guide care within
the office and operating room setting.

Keywords Embryology of the urinary tract . Ectopic ureter . Pelvic kidney . Ureteral duplication . Ureterocele . Urinary tract
anomalies

Introduction Embryology of the urinary system

Congenital anomalies of the urogenital tract often have variable
presentations and may be challenging to diagnose. Since many
of these conditions are asymptomatic, pelvic surgeons should
have an index of suspicion to avoid complications. This review
provides an overview of urogenital tract development as well as
detailed descriptions of the congenital upper urinary tract anom-
alies that may be encountered by urogynecologists in order to
facilitate diagnosis and management.
* Tiffanie Tam
Tiffanie_Tam@TriHealth.com
1
TriHealth, Division of FPMRS, 9489 Chardon Cir, Apt 111, West
Chest, OH 45069, USA
A critical period of embryologic development, known as or-
ganogenesis, occurs between weeks 4 through 8 after fertili-
zation. At that time, the cells of the developing embryo be-
come ‘multipotent’ and form three germ cell layers: the endo-
derm, mesoderm and ectoderm [1]. It is during this period that
the upper and lower urinary tract system and genital system
are formed simultaneously [2].
The upper urinary tract develops in a rostro-caudal se-
quence and begins with the pronephros and mesonephros [2,
3]. The pronephros is a temporary structure that regresses,
while the mesonephros, located inferiorly, forms a series of
tubules and ducts, which are paired along the dorsal aspect of
the embryo. These function as an interim site for waste filtra-
tion. Lastly, through cell signaling pathways, the mesonephric
duct triggers the formation of the metanephros, which marks

the final stage of development of the ureters and adult kidney
[3–5]. The mesonephric duct is also known as the Wolffian
duct, whereas a pair of ducts that lay lateral to it are termed the
paramesonephric or Mullerian ducts. Both Wolffian and
Mullerian ducts are crucial to later development of the male
and female genital tracts, respectively [2, 3, 5].
Lower urinary tract
The cloaca is an endodermal lined structure that guides the
development of the lower urinary tract, genital tract and rec-
tum. The urinary (allantois) and digestive (hindgut) systems
terminate caudally into this single cloacal cavity [3]. At week
4 of intrauterine development, the cloaca is divided into two
cavities, the urogenital sinus ventrally and the rectal cavity
dorsally [2, 3, 5]. The urogenital sinus will further mature into
the lower vagina, urethra and bladder. The allantois, which
remains fused to the urogenital sinus, matures into the
urachus. This acts as a connection point between the bladder
and yolk sac along the umbilical cord and functions for fetal
waste excretion until birth [4].
Upper urinary tract
By week 5 of intrauterine development, the caudal aspect of
the mesonephric duct fuses with the cloaca [5]. This action
stimulates a cluster of adjacent intermediate mesoderm to
form a structure called the metanephric blastema, which sub-
sequently triggers the mesonephric duct to generate a second-
ary outgrowth called the ureteric bud. These structures com-
prise the metanephros, which will in turn develop into the
renal calyx, pelvis, ureters and kidney [3–5]. As the kidney
and ureters complete their development, they will migrate
cephalad, lateralize and rotate from a sacral location to rest
in the T12-L3 vertebral region [3, 6–9]. Once the metanephros
becomes functional, the mesonephros regresses in females.
Genital Differentiation
During the first 6 weeks of intrauterine development, the em-
bryo is sexually indifferent and retains the ability to develop
into either male or female. However, by 8 weeks of intrauter-
ine development, gonadal differentiation will follow the re-
spective pathway based on chromosomal composition [10].
Female sexual differentiation depends on two factors: the Y
chromosome with functioning sex-determining region Y
(SRY) and anti-Mullerian hormone (AMH). The lack of both
of these factors results in gonadal differentiation into ovaries
and regression of the Wolffian duct, respectively, thus
resulting in a female phenotype [3–5].
The remaining paired paramesonephric ducts migrate me-
dially and fuse at the midline. Resorption of the intervening
septum between the two ducts results in the uterine body and
Int Urogynecol J
upper portion of the vagina [11]. The remaining cranial as-
pects of the paramesonephric ducts remain separate and form
the fallopian tubes.
External genitalia differentiation occurs in a different man-
ner and is largely based on the influence of estradiol. Prior to
6 weeks, both male and female embryos develop a genital
tubercle, which is surrounded by paired genital folds and
swellings. These folds surround the urogenital sinus at the
location of the embryonal surface. At 9 weeks of intrauterine
development, the functional ovaries secrete estradiol, which
affects the urogenital sinus, triggering differentiation of the
external genitalia and development of the remaining lower
urinary tract. The genital tubercle forms the glans clitoris;
the genital folds and swelling become the labia minora and
majora, respectively [10, 12, 13]. Lastly, the paramesonephric
duct and urogenital sinus fuse, and the intervening transverse
septum resorbs to generate a single vaginal canal.
Urinary tract anomalies
The true incidence of congenital urinary tract malformations
remains unknown but has been reported in up to 10% of the
population and represents > 20% of all birth defects [14, 15]. A
duplicated collecting system is one of the most common con-
genital urinary tract abnormalities and is found in up to 0.8% of
the population [16–18]. Although these malformations may
lead to symptomatic consequences after birth, some remain
asymptomatic and clinically insignificant throughout life.
Incidentally, urinary tract anomalies are found more commonly
in females and are often associated with genital tract anomalies
[19, 20].
This review will highlight upper urinary tract
malformations most commonly encountered by
urogynecologists. Clinical presentation, implications and po-
tential management are outlined and summarized in Table 1.
Ureteral anomalies
Ureteral duplication
Ureteral duplication is the most commonly reported ureteral
anomaly and is twice as common in females [16, 17, 19–21].
It is often associated with a duplicated renal collecting duct
system and classified as either partial or complete. Partial du-
plication occurs three times more often and results in a bifid
ureter with a single common orifice [16, 17, 19–22]. The
abnormal branching can occur anywhere along the native ure-
ter or at the site of metanephric fusion, but the most common
manifestation is both ureteric branches fused to separate poles
of the kidney (upper and lower) with the upper pole often
drained by the accessory ureter and lower pole fused with
the native ureter. Due to its bifid nature, if obstruction or reflux
Int Urogynecol J

Surgical resection to prevent kidney

VUR: vesico-ureteral efflux, UTI: urinary tract infection, CT: computed tomography, MRI: magnetic resonance imaging, IVP: Intravenous pyelography, US: ultrasound, VCUG: voiding cystourethrogram
Observation, urologic referral if risk

Observation, excision if at risk for

occurs at the site of the common ureter, both upper and lower

Observation, urologic referral for

Treatment recommendations poles are equally affected.

of chronic kidney injury

resection depending on
Complete duplication occurs when two separate ureteric

Surveillance for sequelae

Excision of urachal tract
buds are formed and enter the metanephric blastema separate-
ly, resulting in two ureters that connect the kidney and bladder

malignancy
[16, 22]. Similar to partial duplication, the native ureter often

symptoms
injury drains the lower pole of the kidney and has a normally located
orifice at the bladder trigone. The accessory ureter often drains
the upper pole, travels posteriorly to the native ureter and
inserts inferiorly and medially to the trigone [22, 23]. This is
Clinical diagnosis, CT,

Clinical diagnosis, CT,

Asymptomatic, VUR, UTI, palpable Clinical diagnosis, CT,

Cystoscopy, CT, MRI,

Cystoscopy, CT, MRI,

MRI, Pyridium pad

MRI, IVP, VCUG

described as the Weigert-Meyer rule, which characterizes the
Diagnostic tests

relationship of duplicated collecting systems and their corre-

Asymptomatic, palpable abdominal CT, MRI, US sponding ureteral paths (Fig. 1) [22–25]. Less commonly, the
IVP, US

accessory ureter fails to fuse with the renal pole leading to a

MRI
IVP
test

blind ending pouch, while its distal aspect fuses with the
abdominal mass, nephrolithiasis bladder.
nephrolithiasis, hydronephrosis
Common presenting symptoms

Asymptomatic, persistent urinary

Failure to obliterate the urachus into Extraperitoneal along anterior abdominal Urinary leakage from umbilicus,
leakage, UTI, hydronephrosis

Asymptomatic, hydronephrosis,

Clinical implications Symptoms associated with ureteral

Commonly drains upper pole of kidney Asymptomatic, VUR, UTI,

anomalies vary depending on the location of the duplication

urinary obstruction,

and the ureteral orifices. The most common complication of

UTI, hematuria

complete duplication is vesico-ureteral reflux (VUR), often

mass, infection
nephrolithiasis

affecting the native ureter and consequently the lower pole

of the kidney [19]. There are many factors that are believed
to protect against VUR, such as total ureteral length, ureteral
length within the bladder wall, pathway to the bladder and
and inserts inferior medial into bladder

location of insertion into the trigone [26–28]. In the setting

Below aortic bifurcation and pelvic brim
Extraperitoneal along median umbilical
wall between umbilicus and bladder
Anywhere along ureter, commonly at

of ureteral duplication, the accessory ureter, located medially

Bladder neck, upper urethra, vaginal

and inferior to the trigone, may displace the native ureteral

orifice superiorly and lateral from the trigone, thus predispos-
vestibule, cervix, uterus

ing the native ureter to VUR [19, 28]. As a result, recurrent

urinary tract infections (UTIs) or persistent hydronephrosis
Anatomic location

can occur, with sequelae of renal scarring and chronic kidney

distal aspect

disease. When patients are diagnosed with ureteral duplica-

ligament
Summary of urinary tract anomalies—diagnosis and management

tion, they should be counseled on the possible sequelae such

as VUR, UTI, and subsequent kidney disease. Until symptom-
atic, observation is appropriate, and patients may not require
urologic referral.
Ureteric bud implantation beyond

Dilation of an aspect of the ureter

the median umbilical ligament

Failure of complete ascension of

Surgical treatment in the setting of ureteral duplication pre-

median umbilical ligament
Patent intervening portion of
Duplication of ureteric buds

sents unique challenges. In general, ureteral injury during be-

nign gynecologic surgery is reported in up to 2.5% of cases
[29]. In the setting of ureteral duplication, the incidence is
the bladder

unreported, but is thought to be higher. In fact, several case

reports document challenges with recognition despite having
kidney
Etiology

prior computed tomography (CT) scans [16, 19, 30–32].

Davis described a higher likelihood of injury in the setting
of vaginal hysterectomy for prolapse. He postulated that
Complete or partial ureteral

downward displacement of the duplicated ureter from pro-

Ectopic ureteral orifice

lapse places it at increased risk of injury [30]. If undetected,

Ureteral anomalies

Urachal anomalies

patients may present postoperatively with symptoms of flank

Persistent urachus

Renal anomalies

pain, abdominal pain, oliguria, hematuria, vaginal leakage or

duplication

Pelvic kidney
Urachal cysts
Ureterocele

ileus from urine ascites [30–32].

Anomaly
Table 1

Meticulous dissection and anatomical exploration are sur-

gical techniques that may assist in prevention of ureteric injury
 Int Urogynecol J

Ectopic ureteral orifice

An ectopic ureter is defined as an accessory ureter that drains

the upper pole of the kidney but terminates in a location other
than the bladder. The accessory ureteric bud can form any-
where along the mesonephric duct, and the further it develops
from the native ureteric bud, the more likely its corresponding
ureter will be ectopic [22, 23, 25]. Because the mesonephric
duct regresses in females, the ectopic ureteral orifice will often
terminate where mesonephric remnants are found [19, 25].
Reported locations include the bladder neck and upper urethra
in 33%, vaginal vestibule between vaginal and urethral open-
ing in 33%, vagina (25%) and less commonly the cervix or
uterus [37–39]. Up to 80% of ectopic ureters are associated
with ureteral duplication [40].

Clinical implications Symptoms of an ectopic ureter include

constant vaginal discharge or urinary incontinence if the ori-
fice is located beyond the urethral sphincter complex [19, 22,
Fig. 1 CT urogram depicting bilateral ureteral duplication
37]. The abnormal location of the orifice is prone to stenosis;
therefore, ectopic ureters are frequently associated with dys-
plasia of their corresponding renal segment [19, 22]. This may
in the setting of a known duplication [33]. It is pertinent that lead to diagnostic challenges, particularly if the drainage is
surgeons who perform procedures such as hysterectomy or minimal or renal scarring has occurred resulting in a non-
sacrocolpopexy, which involve retroperitoneal or parametrial functioning renal unit.
dissection, utilize their knowledge of the ureteral tract to rec- Persistent urinary leakage in an infant is frequently the first
ognize an unsuspected duplication. Intraoperative cystoscopy presenting symptom for an ectopic ureter. Although most
may also be useful as it has 80–90% sensitivity for detection
of ureteral trauma [34]. The utility of preoperative imaging or
cystoscopy has not been researched among patients with
suspected duplication, and these modalities remain at the dis-
cretion of the surgeon.
If an injury to a duplicated ureter is suspected, determining
if there is a complete or partial duplication is the first step in
management. Utilizing a guidewire, ureteral stent and imaging
via retrograde pyelogram may assist with detection. CT
urogram is superior to ultrasound (US) and intravenous
pyelography (IVP) to delineate the duplication pattern in ad-
dition to locating an area of injury [35]. Furthermore, obstruc-
tion of an accessory ureter may fail to demonstrate the corre-
sponding upper pole of the kidney during IVP thus highlight-
ing only the lower pole. This is known as the ‘drooping lily
sign’ (Fig. 2) [36].
Ureteral repair is guided by the location of the injury. In
the case of a transected blind ending accessory ureter, con-
tinuous drainage via urethral catheter allows the distal end
of the accessory ureter to epithelialize without need of fur-
ther intervention after follow-up voiding cystourethrogram
(VCUG) confirms resolution [30]. Intraoperative usage of
a pediatric Foley or guidewire can also be a useful tool to
further characterize the transection site via retrograde Fig. 2 ‘Drooping lily sign’ seen on the right kidney after IVP. Case
pyelogram [29]. In the case of complex ureteral injury, courtesy of Dr. Mohammad Taghi Niknejad, Radiopaedia.org, rID:
urologic referral is necessary. 61705
Int Urogynecol J
ectopic ureters are repaired at a young age, diagnosis can be
delayed until adolescence and even adulthood because of the
varying degrees of incontinence. More common diagnoses for
urinary incontinence, such as fistula, detrusor overactivity and
obstruction, can also delay treatment [41]. A high clinical sus-
picion for ectopic ureter should be maintained for women who
complain of continuous vaginal discharge, vaginitis, perineal
irritation and recurrent UTIs. Voiding patterns can vary with
some reports documenting normal voiding while others dem-
onstrate obstructive voiding during urodynamic evaluation
[41–43].
A close vaginal inspection is warranted, and Pyridium pad
testing or backfill instillation of methylene blue in the bladder
may assist in diagnosis [41, 43]. The degree of obstruction of
the ectopic ureteral orifice may also cause a vaginal
outpouching, which can be mistaken for an anterior vaginal
wall prolapse or a Gartner’s duct cyst [42, 43]. If the results
remain unclear, imaging is often indicated for diagnosis and
surgical planning. Although CT and US may detect obstruc-
tion, or upper tract duplication, vaginal and perineal structures
are best visualized via magnetic resonance imaging (MRI).
Therefore, MRI urography may be the best imaging modality
to detect ectopic ureteral orifices located within the vagina or
perineum [42, 44]. In addition, cystoscopy or vaginoscopy can
assist in visualization of a ureteral opening along the vaginal
wall and conducting a retrograde pyelogram by stenting the
orifice may assist in upper tract mapping and screen for ure-
teral duplication [43].
Surgical resection of an ectopic ureteral orifice is often
recommended because of the risk of injury of its correspond-
ing renal segment in addition to symptom improvement.
Although resection of the distal aspect of the ureter can be
performed vaginally, urologic referral may be necessary for
surgical planning and assistance with ureteral re-implantation
of the proximal segment or resection of a dysplastic renal
segment [42].
Ureterocele
A ureterocele is a congenital dilation of the distal-most portion
of the ureter. They are frequently associated with ectopic ure-
ters in 75% of cases, but also can be found in patients without
ureteral duplication [45, 46]. They are 4–7 times more com-
mon in females and are often found on the left [20]. Various
theories suggest this dilation is due an abnormal development
between the accessory ureteral bud and urogenital sinus or a
defect in the muscular wall of the ureter [20, 45, 47, 48].
Clinical implications Symptoms and consequences of
ureteroceles are based on size and location. Large ureteroceles
can cause obstruction and affect the contralateral ureter and the
ipsilateral native ureter in the case of duplication [22, 45, 47]. A
ureterocele found at the vesico-ureteral junction can prolapse
into the bladder neck and cause outlet obstruction that can fur-
ther extend through the urethral meatus presenting as a painful
vaginal mass [45, 47, 49–52]. In addition to obstructive urinary
symptoms, patients may experience hematuria and dysuria with
UTI being the most common complaint [50].
Acute urinary obstruction from large prolapsing
ureteroceles have been reported [49, 50, 52]. Although pro-
lapse accounts for < 5% of cases, acute urinary obstruction
should be immediately addressed [50]. Manual reduction of
the ureterocele with placement of a urethral catheter is recom-
mended. However, if reduction is not possible because of size
or edema, proceeding to the operating room for decompres-
sion is necessary. At times, a vaginal incision may be needed
to assist with reduction [51, 52]. Cystoscopy at the time of
reduction is useful to determine the side of ureterocele and
relative location to the other orifices [52]. Once the acute
obstruction is alleviated, imaging should be conducted to fur-
ther delineate the location as well as presence of additional
upper urologic tract anomalies prior to urologic referral for
definitive resection.
Although US and VCUG are useful in detecting
hydronephrosis and VUR, CT urogram or MR urography are
better equipped for detecting the ureterocele and additional uro-
logic anomalies, such as ureteral duplication, which are often
associated [49]. IVP has been commonly used in the past for its
ability to diagnosis ureteroceles by demonstrating a filling de-
fect. This is often referred to as the ‘cobra-head sign’ where a
radiolucent halo within the bladder demonstrates the ureterocele
wall in addition to a dilated distal ureter (Fig. 3) [49, 51].
However, due to the possible production of a stone within the
ureterocele, IVP may miss the diagnosis [45, 47, 49–51].
Ureteroceles found among asymptomatic patients can be
managed conservatively. Patients should be counseled on
the risk of bladder outlet obstruction, calculi formation and
recurrent UTI [49]. US is a cost-effective modality that may
be useful in this setting to help detect ureteral duplication and
hydronephrosis to assist with counseling. Referral for trans-
urethral resection is indicated for patients who have experi-
enced bladder outlet obstruction, hydronephrosis from VUR
and recurrent UTI and/or who are at risk for renal injury [49,
50, 52].
Urachal anomalies
Persistent urachus
At birth, the urachus undergoes fibrosis and forms the median
umbilical ligament [53, 54]. Congenital persistent urachus oc-
curs when the process of obliteration fails. This is the most
common urachal anomaly and is often found incidentally. It
occurs in 1:5000 adults, with a higher incidence in men [53].
Urachal cysts are the second most common urachal anomaly
and are fluid-filled structures that persist along the embryonal

urachal tract. They are most often found along the lower third
of the obliterated tract and occur when the umbilical and ves-
ical ends of the urachus are closed with an intervening portion
that remains patent [53, 55, 56]. Other less common variants
of urachal anomalies include urachal sinus (18%) and vesico-
urachal diverticulum (3%) [57–59].
Clinical implications Congenital persistent urachus will often
present in children with the complaint of constant leakage of
urine from the umbilicus. Other urachal anomalies can at pres-
ent at various ages with signs of infection of a palpable abdom-
inal mass in addition to peri-umbilical erythema, hematuria or
dysuria [57, 60]. Although rare, malignancy of the urachal rem-
nant has been documented and accounts for up to 1% of all
bladder cancers [61]. Predictors of malignancy are patients be-
ing > 55 years of age and having symptoms of hematuria [62].
Urachal injury during surgery has also been documented.
Due to the increasing trend of minimally invasive surgery,
injury from laparoscopic port placement can occur. Injury most
commonly occurs if a suprapubic port or umbilical port is
placed through a persistent urachus. In those cases, patients
have symptoms similar to those from a sustained bladder injury
[63–65]. Postoperative drainage from the umbilicus, low urine
output and peritonitis from urine ascites are possible presenta-
tions [63, 64]. CT and cystogram are useful modalities to con-
firm this injury by visualizing urine extravasation at the com-
munication site between the urachus and bladder.
Fig. 3 “Cobra-head sign” demonstrating a left ureterocele after IVP. Case
courtesy of Dr. Mohammad Taghi Niknejad, Radiopaedia.org, rID:
61872
Int Urogynecol J
Depending on patient symptomatology and the suspected
diagnosis, the approach to urachal anomalies varies.
Diagnostic imaging such as an US, CT or MRI may be help-
ful, with US being successful in detection in up to 100% of
cases because of the extraperitoneal nature of the condition
[53, 57]. CT assists with clarification and with detection of
lesions such as stones or malignancy. Due to its close prox-
imity along the midline, urachal cysts can be confused with an
ovarian mass, and if surgical resection is planned, inspection
of the surrounding anatomy should be performed [64, 66].
Due to the possibility of regression with age, immediate
surgical excision is often delayed when diagnosed at birth.
In the case of a patent urachus, excision of the urachal tract
is often recommended with closure of the bladder communi-
cation [53, 67, 68]. Due to the higher risk of malignancy with
age, referral for surgical excision in the adult population
should be considered [60].
Renal anomalies
Renal ectopy
If the developing kidney fails to ascend to its final resting
location, it results in renal ectopy. Below the aortic bifurcation
and pelvic brim, it is further characterized as a pelvic kidney
(Fig. 4) [69]. Due to its largely asymptomatic nature, the in-
cidence of renal ectopy varies. It has been documented to
occur in 1:900 of autopsies but in a smaller number of clinical
cases (1:12,000) [70, 71]. Cross renal ectopy is a variant
where the ectopic kidney is located on the contralateral side;
85% of these times the ectopic kidney is fused with the ipsi-
lateral native kidney [72].
Clinical implications In asymptomatic patients, renal ectopy is
often an incidental finding or can present as a palpable mass
on abdominal examination [70]. Those that present with
symptoms may exhibit recurrent UTIs, nephrolithiasis or se-
quelae from abnormal ureteral termination. In 30% of ectopic
kidneys and 70% of pelvic kidneys, VUR is present [70, 73,
74]. Furthermore, 43% of patients with renal ectopy have
other associated urologic and genitourinary malformations in-
cluding vaginal atresia [69, 75]. Because of its aberrant loca-
tion, infection of the kidney can present similarly to appendi-
citis or pelvic inflammatory disease [76, 77]. Furthermore,
with increased risk of hydronephrosis and cystic dilation or
stone formation, the pelvic kidney can give an appearance
similar to an ovarian cyst or solid mass [78].
If planning surgery within the pelvic region, imaging such
as CT scan, IVP and VCUG may be sufficient to help delin-
eate the renal parenchyma and screen for additional anomalies
in the remaining urogenital system [69, 79]. Angiogram may
also be useful in mapping vascular variations.
Int Urogynecol J
Management primarily focuses on symptoms and conse-
quences of the abnormally placed kidney. Ectopic kidneys
are often smaller and dysplastic from VUR and therefore
should be referred to urology for consistent monitoring.
Surgical procedures such as a retropubic sling place pa-
tients with a pelvic kidney at higher risk for injury. In addition
to kidney damage, these patients tend to have venous and
arterial variation [80]. Therefore, other routes should be pri-
oritized prior to consideration of a retropubic approach to
avoid pelvic entry [81].
Discussion
Diagnosis of congenital urogenital anomalies is significantly
challenging because of the variability in presentation.
Anomalies are often discovered after a surgical procedure or
Fig. 4 CT urogram demonstrating a right pelvic kidney
incidentally on imaging. Therefore, understanding normal and
disrupted urogenital tract development facilitates diagnosis
and treatment planning. The urinary and Mullerian tracts are
unique such that anomalies are often closely related. Because
of this, when managing patients with urinary anomalies, it is
wise to maintain a high index of suspicion for anomalies with-
in the genital system. The field of urogynecology is unique in
its ability to care for patients affected by both systems.
When a congenital urinary anomaly is suspected, symp-
toms, renal function, patient’s age and quality of life should
guide management. Beyond an accurate medical history and
physical examination, imaging studies are mandatory to con-
firm diagnosis. CT and MRI are often modalities of choice for
demonstrating duplicated collecting duct systems and ureteral
anomalies. Despite this, there is no consensus regarding pre-
operative imaging in asymptomatic individuals. We suggest
preoperative imaging be considered if there is strong clinical

suspicion for a urinary tract abnormality, or in those who have
a known Mullerian anomaly, to diagnose pertinent urinary
tract findings prior to surgery.
We present various urologic anomalies and consequences
that can present in our patient population. Patient counseling is
of the utmost importance when discussing risks, benefits and
outcomes prior to proceeding with intervention, especially in
patients who are asymptomatic. Lastly, surgery and urologic
referral should be based on symptom improvement and pre-
vention of consequences, with the goal of optimizing long-
term functionality.
Compliance with ethical standards
Conflict of interest None.
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