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Ureteral Duplication and Ureteroceles

Ramnath Subramaniam
A duplex (duplicated) system is a kidney with two pelvicaliceal systems. Complete duplication refers to a
kidney with two ureters that drain separately into or below the bladder. Incomplete duplication refers to a kidney
with two ureters that fuse into a unit proximal to the bladder and then drains into the bladder through a single
orifice. A bifid system is a form of incomplete duplication with two renal pelves that join into a single ureter.
The upper or lower pole ureter refers to the ureter draining the upper or lower pole (moiety) of the kidney,
respectively. The upper or lower pole orifice refers to the ureteral orifice associated with the upper or lower pole
ureter, respectively. An ectopic ureter refers to a ureter that drains into an abnormal site. The greater majority of
duplicated systems are incomplete, and these forms rarely give rise to clinical problems. In contrast, complete
duplication anomalies are rare, affecting less than 0.1% of individuals (most females), and are more commonly
of clinical significance.1 Patients with urinary symptoms such as those associated with urinary tract infection
(UTI), have about a 4% incidence of ureteral duplication. 2
Two thirds of children with duplex systems who present with UTI have vesicoureteric reflux (VUR). 3 This is
particularly true of complete duplex systems, which are associated with higher grades of reflux and renal
dysplasia with poor renal function in the affected moieties. 4 A significant familial predisposition exists
between 12.5% and 30% of siblings of affected patients have duplications. 57 It has been suggested that the
mode of inheritance is autosomal dominant with incomplete penetrance. 5,8
Less common types of ureteral duplications are (1) the inverted Y ureter, consisting of a single pelvis and a
proximal ureter that bifurcates distally into two ureters ending with two separate orifices, one of them being
frequently ectopic; and (2) the blind-ending duplication of the ureter, which occurs when one limb of a bifid
ureter does not drain a portion of renal parenchyma. 9,10 A ureterocele is a cystic dilatation of the lower end of the
ureter where it joins the epithelium of the lower urinary tract. An intravesical ureterocele lies entirely within the
bladder, whereas an ectopic ureterocele has a portion that lies below the bladder neck.

Embryology
Ureteral duplication is the consequence of the abnormal development of the ureteral bud from the mesonephric
(wolffian) duct. Premature branching of the bud causes incompletely duplicated ureters. Complete ureteral
duplication occurs when two separate ureteral buds arise independently from the mesonephric bud and induce
the development of a duplex kidney. The cloaca is divided into the urogenital sinus anteriorly and the alimentary
tract behind. Around the eighth week of gestation, the distal end of the mesonephric duct plus a short segment of
this duct above the ureteric bud expands and, by differential growth, is incorporated into the lower end of the
posterior wall of the developing bladder and urogenital sinus. At this stage the mesonephric duct lies below and
medial to the ureteral orifice and is referred to as the wolffian duct. As a result of this migration, the normal
ureteral orifice is situated at the superolateral angle of the bladder trigone (Fig. 115-1, A).11 In the male the
mesonephric duct becomes the epididymis, vas deferens, and seminal vesicle and enters the posterior urethra
above the external urethral sphincter at the verumontanum. The associated ureteric bud must, therefore, also join
the developing bladder and urethra above the external sphincter. The testis that initially arises from a region
medial to the developing kidney becomes attached to the mesonephric duct. As it descends to the deep inguinal
ring, the vas deferens (mesonephric duct) crosses the origin of the ureter superiorly as it arches medially and
inferiorly to the seminal vesicle at the level of the verumontanum. 11 In the female the mesonephric duct becomes
the Gartner duct, which lies close to the lateral wall of the vagina. The paramesonephric ducts (mu llerian
ducts) develop medial to the mesonephric duct. At the caudad end of the embryo, these incorporate the distal
end of the remnants of the mesonephric duct where they penetrate the cloaca near the midline. Thus owing to
differential growth, the ureteric bud may migrate with the associated paramesonephric duct structures to sites
such as the vestibule, vagina, cervix, uterus, and rectum. 11 The induction of the metanephric blastema by the
ureteral bud in a central position is critical to normal renal development. If the ureteral bud meets the
metanephric blastema either too caudal or too cranial along the metanephric blastema, renal dysplasia will
result.12 The more superior of the two ureteric buds may make contact with the blastema of the upper pole of the
kidney too late to induce normal development. In clinical practice, renal dysplasia affecting the upper pole of the
kidney is often encountered in duplex systems (see Fig. 115-1, A and B).
During development of the trigone, the most cranial ureter that drains the upper moiety of the duplex kidney
rotates inward on its long axis and crosses the lower pole ureter. Therefore the upper pole ureter opens in the
urogenital sinus in a more distal and medial position than the lower pole ureter that opens more proximally. This
relationship between the two ureteral orifices is constant and is called the Weigert-Meyer rule (Fig. 115-1, A).
When the two ureters originate close to each other and in a near normal position, both ureteral orifices open in

the trigone (see Fig. 115-1, A). Conversely, when the two ureteral buds originate at widely separate positions on
the mesonephric duct, the upper pole ureter, which is located at a more cranial position on the mesonephric duct,
is incorporated into the urogenital sinus at a later stage of development. As a result, the ureteral orifice is
situated in an ectopic position, inferior to the trigone (see Fig. 115-1, B). In the male in this situation, the
location of the ureteral orifices can be anywhere along the lower mesonephric duct or its derivatives below the
bladder neck in the posterior urethra but always above the external urethral sphincter. Therefore urinary
incontinence due to duplex systems with ectopia of the upper pole ureter does not occur in boys. In girls,
however, the situation is different. During development, the mesonephric duct derivatives can open anywhere
caudally from the developing bladder, commonly into the vault of the vagina.
The lower pole ureter, which joins the mesonephric duct closer to the urogenital sinus, is incorporated into the
developing trigone at an early stage and consequently migrates laterally and cranially, leaving a relatively short
intravesical tunnel (Fig. 115-1, C). VUR into the lower pole ureter may be present because of the short length of
the intramural tunnel. Occasionally, in either complete or incomplete duplication, one of the ureters fails to meet
the developing blastema of the metanephros. In this case, a blind-ending ureter occurs, with no attachment to the
kidney.10 Rarely, an inverted Y abnormality is encountered. It is presumed to be caused by double ureteric buds
arising from the mesonephric duct caudally and fusing cranially before merging above with the blastema of the
metanephros.9 Recently, there has been a shift from classic theories to the cell biology view of congenital
anomalies of the kidney and urinary tract (CAKUT).13 The process of ureteral budding and metanephric
differentiation into the final kidney is under the control of the renin-angiotensin system through the Agtr-2
receptor, which is intensely expressed in the mesenchymal cells surrounding the wolffian duct at the time of the
initial budding of the ureter. Most recently it has been shown that ectopic budding and ureteral duplication may
occur in Agtr2 null mice.14 The formation of a double collecting system has also been observed in another group
of experimental animals that are heterozygous mutants for the bone morphogenetic protein 4 (BMP4). BMP4 is
a member of the transforming growth factor-beta (TGF-b) superfamily and is credited to be part of the
navigating system that allows the budding of the ureter from the mesonephric duct at a precise site. 13
Abnormalities in the number and site of the initial ureteral buds preceding a duplex collecting system and
ectopic ureteral orifice with concurrent renal abnormalities are constantly observed in mice that are homozygous
mutants for the FOXC1 gene, which encodes for a transcription factor playing an essential role in embryonic
development.13

Incomplete Duplicated Systems


Uncomplicated duplex kidneys may be detected incidentally on ultrasound scans done for unrelated reasons.
Bifid pelvis is never responsible for any symptoms. Incompletely duplicated ureters are also often completely
asymptomatic but in rare cases are associated with distinct clinical problems. Three clinical problems may affect
an incompletely duplicated ureter: (1) VUR, (2) ureteropelvic junction obstruction, and (3) retrograde ureteral
peristalsis (yo-yo phenomenon). Treatment depends on the function of the affected renal moiety and on the
length of the lower limb. If the function is poor, a partial lower pole nephroureterectomy is the appropriate
choice, removing the lower moiety and its ureter down to the junction with the upper pole ureter. If the lower
moiety is worth saving, the type of repair depends on the length of the ureter. If the ureter is very short, the
upper and lower pelvis may be joined side by side (pyelopyelostomy) to create a single pelvis drained by the
upper ureter. Conversely, if the lower ureter is long, a conventional dismembered pyeloplasty can be done, but,
if the junction between the two limbs is close to the bladder, a ureteroureterostomy with excision of the distal
ureter is a better solution in order to avoid the possibility of uretero-ureteral reflux.
Even when uretero-ureteral reflux (yo-yo reflux) is demonstrated, it is difficult to relate the frequently vague
complaints of the patient to the peristaltic disorder. Clinical judgment is required before proceeding to a surgical
repair, which may consist of a proximal pyelo-ureteral anastomosis with removal of one ureter or, if the ureteral
junction is close to bladder wall, excision of the common limb and reimplantation of the two ureters into the
bladder side by side.

COMPLETE DUPLICATION ANOMALIES


Vesicoureteric Reflux
The most frequent anomaly detected in completely duplicated ureters is VUR. As a rule, it always affects the
lower pole ureter either independently or with VUR into the upper pole ureter because it opens in the trigone in
a more cranial and lateral position with a shorter submucosal tunnel, causing incompetence of the ureterovesical
junction. When VUR rarely occurs into both moieties, the ureteral orifices are usually close together and in a
lateral ectopic location. The most common presentation is UTI, with more than 60% of children with UTIs and a
duplex kidney having vesicoureteral reflux.15 Therefore when ultrasound reveals a duplex kidney in a child with
a history of a UTI, a micturating cystourethrogram is mandatory.
Cystourethrogram often reveals the pelvis in a lower than normal position with an abnormal renal axis. The
upper calyces are missing, and the lower ones often show signs of chronic reflux nephropathy, resulting in the
appearance of a drooping lily (Fig. 115-2).

The affected lower pole on dimercaptosuccinic acid (DMSA) renal scan may be nonfunctioning or poorly
functioning, indicating severe dysplastic changes or parenchymal scarring due to repeated episodes of UTI. In
this relatively rare case, the treatment of choice is lower pole partial nephrectomy and ureterectomy (Fig. 1153). It is recommended that the lower pole ureter be removed completely down to the bladder wall in order to
avoid leaving a refluxing stump. In most cases, however, the lower pole is worth saving and several options are
available for treatment. Contrary to popular belief, a significant proportion of refluxing units associated with
duplex systems resolve spontaneously with time. This is more likely with grade 1 and 2 VUR (up to 85%) than
with higher grades.1619
Provided the child remains asymptomatic (as with primary VUR), antibiotic prophylaxis is justified initially.
These patients must be followed carefully to assess the kidneys for scarring and its progression. VUR can be
assessed using either indirect MAG3 cystography or a micturating cystourethrogram (MCUG). If progressive
renal scarring with deteriorating renal function is identified, or if symptomatic upper renal UTIs cannot be
controlled by antibiotic prophylaxis, operative intervention is indicated.20,21
Endoscopic treatment of VUR in a duplicated system is not as successful as with a single-unit VUR, with a
permanent resolution rate of only 46%.22 However, it is still worthwhile to consider it as an effective option in
selected cases.17,23 In duplex systems in which the ureteral orifices are close together, the implant is injected
under the upper pole moiety orifice (inferomedial). The needle is advanced below both orifices, and the
injection is commenced under direct vision until both moiety orifices are compressed. When the ureteral orifices
are separated by more than a few millimeters, they can be individually injected; however, caution is required
when placing the injection under the lower pole orifice because the upper pole ureteral lumen may be entered in
error. The procedure of choice, when the refluxing ureter is not excessively dilated, is double barrelled or
common sheath ureteroneocystostomy. When the ureteral orifices are situated
relatively normally on the trigone, both should be mobilized from the bladder base together, even if only the
lower pole ureter is refluxing. This is necessary because the ureters share the same adventitial sheath and blood
supply.24Attempts tomobilize only the affected ureter will result in ischemia of one or both ureters.
Alternatively, both ureters can be mobilized to a level above the common sheath. The ureters can then be
rerouted through the bladderwallusing either an intravesical cross-trigonal technique or an extravesical
approach. The objective in both instances is to create a sufficiently long submucosal tunnel for the ureters
(preferably three times their diameter), in order to create a satisfactory flap valve antireflux mechanism.
When one or both ureters are massively dilated, they can be safely reduced in size, plicating or excising the side
away from the common wall.25 When both ureters need to be reduced in size, excisional tapering is a better
choice in order to avoid an excessive bulk of tissue that may make subsequent ureteroneocystostomy difficult.
Other surgical options, when only one ureter needs to be addressed, include uretero-ureterostomy. Recent
literature indicates it is effective and has a low incidence of complications. 2628 The size of the ureter does not
seem to be a problem with this technique, which has also been performed via an inguinal incision with good
results.29 It is important to make sure there is no reflux in the ureter draining into the bladder. There is a
theoretical risk of inducing yo-yo reflux. Such a risk can be avoided with pyelo-ureterostomy, which is a highly
successful procedure, but a second lower abdominal incision is required to completely remove the refluxing
stump or the stump can be dealt with by endoscopic correction.
Ectopia of the Upper Pole Ureter
Another common anomaly in complete duplications is ectopia of the upper pole ureter.
Approximately 80% of all ectopic ureters occur in duplicated systems. The upper pole drained by an ectopic
ureter commonly has severe dysplasia. In addition, findings consistent with obstructive changes are the rule in
the male, where ectopic ureters are always obstructed. In females, obstruction affects only ureters ending inside
the urinary tract. Ureters ending onthe bladder neck or in theposterior urethra are obstructed when the bladder
neck is closed but may occasionally reflux during micturition; in such casesUTI is common.
In the male, ectopic ureters generally cause infections and are identified during the evaluation for UTI.
Epididymo-orchitis is a frequent presentation when the ureter opens in the genital tract in association with the
vas or seminal vesicle.3032 Many infant girls with ureters opening inside the urinary tract and, as a consequence,
obstructed, present with UTI. Conversely, an ectopic ureter opening in the mu llerian derivatives, having its
orifices outside the realm of the urinary sphincter, causes urinary pseudo incontinence. A constant dribbling of
urine in the interval between normal micturition is frequently observed.33 This symptom is generally overlooked
until the girl is fully toilet trained and has normal voiding habits. If the ectopic upper pole function is poor and
little urine is produced, dribbling may not be observed and the patient may present with constant vaginal
discharge. When the urine pools in a dilated ureter, especially at night when the patient is in a recumbent
position, urinary losses may assume different features and mimic stress or urge incontinence.
Physical examination is often completely normal. Rarely, close and careful scrutiny of the external female
genitalia reveals that the ectopic ureter is located in the urethro-vaginal septum. In addition, a dilated ureter is
occasionally palpated during rectal examination. Diagnosis of a suspected ureteral ectopia is straightforward

when the ectopic ureter is massively dilated and may be easily identified on an abdominal ultrasound or when
the upper pole functions sufficiently to excrete contrast material on intravenous urography (IVU).
Difficulties arise when the upper pole function is so poor that contrast is not excreted and the ureter is not
dilated or when the ectopic ureter is associated with other malformations like massive reflux in the ipsilateral
lower pole ureter or contralateral pathology that confuses the picture. Ultrasound, the first-line investigation,
may reveal a dilated duplex ureter and upper pole or, when the ureter is not dilated, show changes in the
echogenicity of an otherwise normal upper pole that help in defining the correct diagnosis.
MCUG should be included in the diagnostic workup. Associated reflux either in the ipsilateral lower pole ureter
or in the ectopic upper pole ureter may be demonstrated; in the latter case MCUG delineates the anatomy of the
ureter, making more sophisticated imaging studies unnecessary. An ectopic ureter should always be suspected in
cases of isolated reflux into the upper pole of a duplicated system, which most commonly occurs on the voiding
phase of the MCUG. Excretory urography is useful for defining the anatomy in a ureter associated with a
functioning renal unit.34 However, when the affected moiety is nonfunctioning, the findings are more subtle and
changes on ultrasonography, DMSA scans, and excretory urography are easily missed.19,34 Magnetic resonance
imaging (MRI) scanning is particularly useful in these circumstances and provides both anatomic detail and
functional analysis (Fig. 115-4)3538; however, anesthesia is required in children.
The choice between the different treatment options largely depends on the function of the affected segment, on
the coexistence of associated malformations, and, to a lesser degree, on the size of the affected ureter.
In the great majority of cases when the involved upper pole contributes to less than 5% total function, open
surgical, laparoscopic, or, even better, retroperitoneoscopic removal of the upper pole along with its ureteral
segment is the treatment of choice.
Partial nephrectomy involves identifying the demarcation between the normal lower pole and the
nonfunctioning upper pole and excising the ureter of the affected renal unit inferiorly as near the bladder as
possible without compromising the lower pole ureter. The potential problems of excising the lower end of the
affected ureter are well recognized and are discussed in more detail later. The operation may be undertaken by
either open or endoscopic techniques. The important steps are to clearly identify the ureter and pelvis of the
affected upper pole moiety. After dividing the blood supply to the upper pole of the kidney, it is then possible to
identify the limit of the affected upper pole, and a relatively bloodless plane can be developed between the
abnormal and normal renal tissue without entering the caliceal system of the lower pole.39 The ureter can then be
excised down to the pelvic brim.
This upper tract approach lends itself well to minimally invasive surgery because access to the kidney and ease
of defining the vascular anatomy, particularly with the retroperitoneoscopic approach, are excellent.4043
Secondary surgery at the bladder level to remove the stump or to reimplant the refluxing lower pole ureter is
necessary only in a small number of cases.44,45
If the upper pole retains some function and is considered worth saving, then proximal ureteropyelostomy or
distal uretero-ureterostomy is advisable. In a series of 11 infants who underwent ureteropyelostomy for
obstructed duplicated ureters with satisfactory results, only 3 children needed secondary surgery at the bladder
level for persisting reflux in the lower pole ureter.46 When ipsilateral lower pole reflux is present, a doublebarrelled ureteroneocystostomy may allow resolving both obstruction and reflux with a single operation.
It requires a meticulous and tedious extravesical dissection in order to isolate enough length of both ureters and
perform a safe reimplantation. Moreover, both ureters are usually markedly dilated and need tapering before
ureteroneocystostomy. A possible, easier alternative is to perform a distal end-to-side anastomosis of the
obstructed ureter to the refluxing one and to reimplant the lower pole ureter into the bladder.
However, in the great majority of cases an upper tract approach is strongly recommended, either by an upper
pole partial nephrectomy or a ureteropyelostomy. If VUR is present preoperatively or develops postoperatively,
it can be safely managed conservatively with antibiotic prophylaxis or treated endoscopically at a later stage.
With such an approach the number of surgical procedures and complications can be substantially reduced,
allowing, when possible, the preservation of a functioning upper pole.
Ureteropelvic Obstruction
In duplex systems, the obstruction usually affects the lower pole and is more common in boys.47,48 The decision
to operate is guided by the size and function of the renal unit involved. An increase in hydronephrosis or
decreased function of the affected part of the kidney on isotope study is an indication to intervene. However,
these parameters are sometimes difficult to assess objectively in a growing child.
Loin pain in older children, typically preadolescents, associated with objective evidence of ureteropelvic
junction obstruction on MAG3 isotope scan, is a common indication for operation. The surgical options are
pyeloplasty or pyeloureterostomy to the unaffected upper pole ureter, with excision of the distal ureter of the
affected lower pole.4749 Partial nephroureterectomy is undertaken when the function of the affected renal unit is
severely impaired.

Ureteroceles
The term ureterocele describes the cystic dilatation of distal intravesical portion of the ureter. A single-system
ureterocele is associated with a kidney with only one ureter, whereas a duplex system ureterocele is associated
with the upper pole of a kidney with a complete ureteral duplication.
Most frequently the pelvicaliceal system drained by the ureterocele is also obstructed. The malformation may be
further complicated by VUR into the ipsilateral lower pole or into the contralateral ureter or by ureteral
obstruction. The incidence of ureteroceles is approximately 0.02% of individuals with 80% occurring in
females.50,51 A slight majority are left sided and bilateral in 10%.52 Some 80% of ureteroceles are associated with
the upper pole of a complete duplication,53 and in most series 60% to 80% are ectopic.54
Bilateral ureteroceles or those associated with contralateral duplicity occur in 15% of cases.55 Single-system
ureteroceles are uncommon, occur most often in males, and can be associated with other anomalies including
abnormalities of the kidneys such as fusion, ectopia,56 or multicystic dysplasia.57

CLASSIFICATION
The Committee on Terminology of the Urologic section of the American Academy of Pediatrics, proposed a
simple and widely adopted classification, whereby ureteroceles contained entirely within the bladder are named
orthotopic or intravesical and a ureterocele that has a portion permanently located outside the bladder is called
ectopic.58 In 1971 Douglas Stephens classified the ureteroceles associated with ureteral duplication according to
the position and/or presence of intrinsic obstruction of the ureteral orifice as shown in Table 115-1.59
Stenotic ureteroceles are completely located inside the bladder and have a small, stenotic, often pinpoint orifice;
they are usually tense and show a well muscularized wall with predominantly longitudinal muscle fibers. The
nonobstructed variety is rather rare; in such cases the ureterocele is visible only when a peristaltic wave fills it.
In the sphincteric subtypes the ureteral orifice is wide and often gaping, but it is located inside the bladder neck
or urethra and is obstructed by the contraction of sphincteric muscle. These ureteroceles decompress during
voiding. Sphinctero-stenotic ureterocele is similar to the previous one, but the orifice is both ectopic and
stenotic. Because a sphinctero-stenotic ureterocele does not decompress during micturition, it may ball valve
and obstruct the bladder outlet. The meatus of the cecoureterocele is in the bladder, but a
tongue of the ureterocele extends down in the urethra. Ureteroceles are described as blind when no kidney or
upper pole associated with ureterocele can be demonstrated.
In duplex kidneys the upper pole drained by the ureterocele is affected by some degree of dysplasia in 43% to
73% of cases. In 20% the dysplasia is severe, affecting more than 25% of the upper pole.60,61 The contribution of
the affected upper pole to the global renal function ranges from 4% to 8%. Measurable loss after upper pole
partial nephrectomy is, on average, around 1% while functional gain after endoscopic incision is around 2%.62

CLINICAL PRESENTATION
Prenatal diagnosis of ureterocele represents approximately 15% of all antenatal diagnosis of duplex kidneys as
the number of neonates with prenatally detected ureteroceles has increased from 2% to 28% in the past 20
years.63 Prenatal ultrasound screening of the kidneys often identifies some degree of hydronephrosis, which
ismost pronounced with ureteroceles.64 Although there is no evidence that treatment of these prenatally
diagnosed children affects the prognosis for the kidney, symptomatic UTIs can be reduced in frequency. 6567
In the postnatal period, infection is the most common presentation for ureteroceles in both sexes; in infants
symptoms may vary from a life-threatening gram-negative sepsis to a febrile illness with gastrointestinal
symptoms or failure to thrive. In older boys and girls symptoms aremore specific and point to a urinary tract
infection: fever, malaise, dysuria, foul-smelling urine, and back pain.
Other presenting symptoms in both girls and boys may be hematuria after minimal trauma or a palpable
abdominal mass representing the bladder or the obstructed urinary tract. In girls an interlabial mass or acute
urethral obstruction due to the ball valve effect of a ureterocele prolapsing in the urethra is another possible
presentation. Rarely, the most distal portion of a cecoureterocele can protrude from the urethra as a pink,
interlabial mass without obvious urethral obstruction or retention and, consequently, such cases do not
need emergency treatment. Prolapsing ureteroceles are the most common cause of acute urethral obstruction in
girls,68 but this event has been also reported in males,69 even if much less frequently. Patients present in obvious
pain with an acute urinary retention and a palpable bladder. A purple red or frankly necrotic round mass may be
seen protruding from the labia. In such cases emergency treatment to decompress the ureterocele and remove the
bladder outlet obstruction is warranted. In extreme cases, when the ureterocele prolapses and obstructs the
urethra, it may affect the function of all the renal units.69,70

DIAGNOSIS

The diagnostic workup relies on the use of ultrasound, MCUG, and renal isotope scans. Excretory urography,
the mainstay of ureterocele diagnosis until a decade ago, is much less commonly used now. Computed
tomography scan and magnetic resonance urography may be useful, especially the latter, when the urinary tract
anatomy is not clear. Ultrasound of the urinary tract is the first investigation and generally depicts clearly the
ureterocele as a sonolucent round image that sits on the bladder base and occupies a portion of the bladder. One
or more dilated ureters can be seen behind the bladder (Fig. 115-5). Ultrasound gives valuable information on
the presence of unilateral or bilateral renal duplicity and on the dilatation of the collecting systems, and it helps
in identifying which kidney is drained by the ureterocele. Cortical cysts and severe parenchymal thinning, but
not increased echogenicity, suggest renal dysplasia that is confirmed by isotope scan.71
An MCUG is an essential part of a ureterocele evaluation. The ureterocele is seen in the first films as a negative
shadow in the bladder, with a rim of contrast around it. If the ureterocele is not tense, it may be obscured with
the progressive filling of the bladder because it may be compressed or, if it is small, may be obscured by the
contrast medium around it. MCUG may also reveal VUR in the lower pole ureter (50%) or in the contralateral
ureter (25%).53,72,73 In less than 10% of cases, reflux can be identified in the ureterocele if it is ruptured or if it
has a large open meatus placed on the bladder neck that allows reflux during voiding.33,73 The MCUG is also
useful to ascertain the degree of the detrusor backing for the ureterocele. If the detrusor support is poor, the
ureterocele may evert during micturition, mimicking a bladder diverticulum (Fig. 115-6).
During voiding, the ureterocele may also be seen prolapsing through the urethra and obstructing the urinary
flow. IVU was the most important diagnostic step in the past. More recently, however, the progress of
ultrasonography, DMSA, and MRI has made this examination obsolete in most cases. Nevertheless, when the
anatomy is confusing, excretory urography may still play a role.
If the renal parenchyma associated with the ureterocele retains some function, which most commonly occurs in
single systems, a characteristic cobra head or spring onion deformity of the intravesical ureter is produced
due to opacified urine in the ureterocele being surrounded by a radiolucent halo that represents the wall of the
ureter. More commonly, when the ureterocele is associated with the upper pole of a duplex kidney, function is
absent or minimal in 90% of cases; therefore the radiographic signs are mainly negative, reflecting the
displacement of the lower pole renal unit by the hydronephrotic upper pole ureter. The lower pole pelvis is often
laterally and downward displaced, producing the characteristic drooping lily appearance; the number of
calices is reduced; and the upper calices are missing (Fig. 115-7).
At the bladder level a negative shadow may be seen, suggesting the presence of a ureterocele (see Fig. 115-7).
The shadow may vary from a large, tense, round shadow occupying most of the bladder to a minor irregularity
in the base of the bladder. Excretory urogram also shows the condition of the contralateral kidney collecting
system that may be duplex (see Fig. 115-7) or may be obstructed at the bladder level by a large and tense
ureterocele compressing the ureteral orifice. The function of the pole or of the kidney associated with the
ureterocele is best assessed by a DMSA renal scan that shows the quality and quantifies the amount of
functioning renal tissue (Fig. 115-8).

PRINCIPLES OF TREATMENT
The goals of the ureterocele treatment are to preserve renal function by providing unobstructed drainage of all
functioning renal tissue, removing any potential source of infection and treating VUR; prevent bladder outlet
obstruction and/or urinary incontinence; and prevent and treat any bladder wall deficiency (e.g., diverticula,
poor detrusor backing). The surgical strategy should be optimized in order to attain these goals with minimal
surgical morbidity. Few cases of prenatal treatment of a prolapsing obstructing ureterocelewithanhydramnios
have been recently reported with a view to restore amniotic volume.7476 In a small number of prenatally
detected cases of ureteroceles with no obstruction or reflux, a non operative watchful waiting approach has been
attempted successfully.65,77,78
Postnatally, the treatment options are endoscopic incision, upper pole partial nephrectomy (upper tract
approach), complete reconstruction at the bladder level, or nonoperative (conservative) treatment. These
methods cannot be compared easily, and the choice must be appropriately applied to patients with different
clinical presentations. Skill in all methods of management is important, and an understanding of when to apply
these methods is critical because the management of each patient with a ureterocele must be individualized.79
The factors that influence treatment choice are type of presentation (prenatally diagnosed or symptomatic), age
of the patient, type ofureterocele (intravesical versus ectopic), differential function of the renal moieties, and
presence of VUR and UTI.

ENDOSCOPIC DECOMPRESSION
Wide, open deroofing of the ureterocele was performed in the past. The problem with this technique is that if the
ureterocele arises from the urethra, epithelial remnants that have not been completely excised may result in
urethral obstruction owing to the valvelike nature of these folds.80 In addition, with this type of operation,
postoperative VUR is almost inevitable. Therefore deroofing usually requires a subsequent antireflux procedure

that is normally best undertaken as part of the initial surgery. This more aggressive approach is potentially
difficult and is associated with increased morbidity, especially in the very young child with a small bladder.81
Endoscopic techniques have been used for some time to simply incise the ureterocele. At any age endoscopic
incision has the advantage of being a simple, quick, and minimally invasive procedure that can be performed
with a minimal hospital stay. This can take the form of either diathermy incision or simple
puncture of the cyst.8284
During this procedure it is important not to overfill the bladder; otherwise, the ureterocele may be compressed,
making it difficult to identify. If this occurs, loin compression on the affected side may assist in refilling
the ureterocele. The planned decompression point on the ureterocele is important and, if possible, should not
overlie the ureteral lumen where it is attached to the bladder base. Recently a novel technique of fulgurating the
internal layer of the ureterocele has been described to achieve and maintain collapse of the ureterocele.85 This
technique is challenging and tedious, especially when the postoperative outcomes following endoscopic incision
depend on anatomic and functional characteristics rather than the technique used.86
Simple decompression obviates obstruction and allows a better evaluation of the function of the kidney or pole
involved by the ureterocele. It often reduces the size of the ureter so that secondary procedures (such as excision
of the ureterocele and ureteroneocystostomy), if required, are less difficult and may reduce the need for ureteral
tapering. In addition, associated VUR into the lower pole ureter may subside spontaneously. 72
Endoscopic incision of intravesical ureterocele is the definitive treatment in 77% to 93% of cases; therefore it
can be considered to be the initial treatment for this type of ureterocele. 79 A recent meta-analysis looking at
endoscopic management of ureteroceles confirmed what others have suggested that the risk of reoperation after
endoscopic incision of ectopic ureteroceles was significantly higher than with intravesical ureteroceles.73,87
There is no consensus about the effectiveness of endoscopic incision in ectopic ureteroceles because it rarely
represents the definitive treatment modality88,89; seldom ameliorates the function of the involved upper renal
pole; and, if an iatrogenic reflux is created in the ureterocele, it may unnecessarily commit the patient to future
lower tract surgery. Alternative treatment options are represented by the upper tract approach, which includes
either an open or retroperitoneoscopic upper pole partial nephrectomy and ureterectomy, leaving the upper
ureteral stump open in order to decompress the ureterocele, or, if the upper pole is considered worth being kept,
a ureteropyelostomy or uretero-ureterostomy, joining the upper pole ureter to the lower one.
The preservation of the upper pole affected by an ectopic ureterocele is rarely warranted because its function is
often minimal, the severity of renal dysplasia being directly proportional to the degree of ectopia of the
ureterocele.62 There is little hope of recovery of function after relief of obstruction in most cases because the
upper pole is often dysplastic with irreversible changes, neither progressive nor amenable to any type of
treatment.
The upper tract approach may be recommended when there is no associated VUR and the choice between
removing and keeping the upper pole is dictated by its relative function. With this approach, the rate of
secondary procedures on the bladder ranges from 15% to 20% if preoperative VUR was absent.90,91
When VUR is associated with an extravesical ureterocele, no matter which approach is used first, a second stage
at the bladder level to remove the ureterocele and reimplant the ureters is necessary in the majority of cases. As
the number of renal units affected by VUR in a duplex system with an ectopic ureterocele increases, the higher
the incidence of surgery at the bladder level.61,92 The reoperation rate in patients with duplex system ectopic
ureterocele and VUR varies from approximately 50% to 100% after endoscopic incision93 and from 84% to 90%
after upper pole partial nephrectomy.90

BLADDER BASE RECONSTRUCTION


As mentioned earlier, bladder base reconstruction including excision of ureterocele and ureteroneocystostomy
may be required. This usually produces a significant defect that necessitates a precise anatomic reconstruction;
otherwise, disruption of the bladder neck and posterior urethra could lead to incontinence. Fortunately, it is
unusual for the ureter to open directly into the urethra; this most often occurs at a level at or just above the
bladder neck. Recently, it has been suggested that the ureterocele can be excised or marsupialized without much
effect on clinical outcome.94 The ureterocele, however, often involves the urethra, requiring that the epithelial
edges of the cyst be excised completely and then meticulously repaired. Otherwise, obstruction of the urethra
may result from either the residual valvular remnants of the ureterocele or stricturing due to excessive
scarring.80 Therefore it is usually necessary to mobilize the unaffected lower pole ureter with that associated
with the ureterocele. If the upper pole ureter is to be excised, mobilization of both ureters should be extended
above the common adventitial sheath. Alternatively, the two ureters can be separated by preferentially taking the
adventitia of the affected ureter, thus preserving the blood supply to the normal ureter. In any case the defect
in the bladder base and outlet must be repaired without narrowing the outlet. The remaining lower pole ureter
can then be reimplanted using either a cross-trigonal or an extravesical method as described earlier.
Proponents of complete primary lower urinary tract reconstruction early in the neonatal period or infancy argue
that the need for secondary surgery in patients affected by duplex system ectopic ureteroceles with preoperative

VUR ranges from 0% to 32%.9597 They have reported that this approach in patients with ectopic ureterocele
with VUR appears to have better results than a staged approach with initial endoscopic treatment. Moreover,
they conclude and have been supported by others that extensive reconstructive bladder surgery in neonates and
infants does not lead to bladder function deterioration at a later age.98 However, severe bladder dysfunction was
observed in 7 of 10 girls who underwent bilateral ureterocele repair in their first 2 years of life,81 suggesting that
due to the gross abnormality of the bladder base in duplex systems, there is a significant risk of damaging the
bladder outlet in the course of mobilizing the ureters. The difficulty is in determining whether these childrens
problems are a primary abnormality of bladder function or congenital and unrelated to surgical intervention.99,100

Summary
Ureteral duplications and ureteroceles represent a spectrumof often overlapping embryologic and anatomic
abnormalities. Most unobstructed or nonrefluxing duplications are of no clinical significance along with
incomplete duplications; others are associated with significant morbidity. Complete duplications are often
asymptomatic but can be associated with VUR or ureteroceles with a predominance in girls. VUR is the most
common anomaly of duplications, occurring in about 65% of those with symptomatic UTIs; the reflux is usually
into the lower pole ureter. Ectopia of the ureter is not uncommon, and a detailed history is often useful in
pointing toward the cause of incontinence. Specialized imaging techniques are often required, however, to
identify the precise anatomic abnormality; these include ultrasonography, isotope scanning, MCUG, excretory
urography, and MRI. On the basis of these data, a planned, individualized approach to each case can be taken,
with an emphasis on preventing deterioration in renal function, controlling symptoms, and avoiding surgical
interventions that could risk bladder function.