Seminars in Pediatric Surgery 25 (2016) 82–89

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Early urologic considerations in patients with persistent cloaca

Brian A. VanderBrink, MDn, Pramod P. Reddy, MD
Division of Urology, Cincinnati Childrenʼs Hospital Medical Center, MLC 5037, 3333 Burnet Ave, Cincinnati, Ohio

a r t i c l e in f o abstract

Cloacal malformations represent one of the most complex conditions among anorectal malformations.
Keywords: Urologic conditions occur with an increased frequency in cloaca patients compared to patients with
Cloaca other types of ARM. The morbidity of the upper and lower urinary tract dysfunction/malformations at
Urology times can be severe; manifested by urinary tract infection, lower urinary tract symptoms, urinary
Neurogenic bladder incontinence, chronic kidney disease, and even end stage renal disease. Long-term follow-up of patients
Intermittent catheterization with cloaca has described significant chronic kidney disease and end-stage renal disease. Whether this
rate of chronic kidney disease is a function of intrinsic renal dysplasia or acquired renal injury from
neurogenic bladder is currently unknown. However, it is well known that severe lower urinary tract
dysfunction, no matter the etiology, poses significant risk to the upper tracts when untreated. Neonatal
assessment of the urinary tract accompanied by early identification of abnormal structure and function is
therefore fundamental to minimize the impact of any urologic condition on the childʼs overall health.
Adequate management of any associated bladder dysfunction is essential to preserving renal function,
minimizing risk of urinary tract infection, and potentially avoiding need for future reconstructive surgery.
This article summarizes our institutionʼs approach to the ongoing early urologic management in patients
with cloaca.
& 2016 Elsevier Inc. All rights reserved.

Introduction intrinsic renal dysplasia or acquired renal injury from neurogenic

Cloacal malformations have been reported to occur in one in
50,000 live female births and represent one of the most complex
conditions among anorectal malformations. Multidisciplinary care
among colorectal, urologic, and gynecologic providers is critical to
optimizing patient care and ultimately clinical outcomes. Urologic
conditions occur with an increased frequency in cloaca patients
compared to patients with other types of ARM.1–3 The morbidity of
the upper and lower urinary tract dysfunction/malformations at
times can be severe; manifested by urinary tract infection, lower
urinary tract symptoms, urinary incontinence, chronic kidney
disease, and even end-stage renal disease.
Spinal cord abnormalities such as spinal cord tethering occur in
up to one third of patients with ARM.4,5 This association also
increases the importance of surveillance of bladder function over
time as neurogenic bladder can develop with spinal cord and
vertebral anomalies.6 Long-term follow-up of patients with cloaca
has described significant chronic kidney disease and end stage
renal disease; up to 75% and 17%, respectively in some reports.7–9
Whether this rate of chronic kidney disease is a function of
n
Corresponding author.
E-mail address: brian.vanderbrink@cchmc.org (B.A. VanderBrink).
bladder is currently unknown. However, it is well known that
severe lower urinary tract dysfunction, no matter the etiology,
poses significant risk to the upper tracts when untreated.10,11
Neonatal assessment of the urinary tract accompanied by early
identification of abnormal structure and function is therefore
fundamental to minimize the impact of any urologic condition
on the childʼs overall health. Adequate management of any
associated bladder dysfunction is essential to preserving renal
function, minimizing risk of urinary tract infection, and potentially
avoiding need for future reconstructive surgery. This article sum-
marizes our institutionʼs approach to the ongoing early urologic
management in patients with cloaca.
Newborn radiographic assessment relevant to urinary tract
The diagnosis of cloaca is made on physical examination by
identifying a solitary perineal orifice that communicates with
gastrointestinal, urologic, and gynecologic tracts. Concomitant
abnormalities of the heart, spine, spinal cord, and urinary tract
may be identified by physical examination. Only following stabi-
lization of the infant, any radiographic survey for purposes of
assessing the urinary tract such as plain radiographs and/or ultra-
sound should be performed.

http://dx.doi.org/10.1053/j.sempedsurg.2015.11.005
1055-8586/& 2016 Elsevier Inc. All rights reserved.

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 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
Lumbosacral vertebral segments and spinal cord
Plain radiograph of the abdomen allows for imaging of the
lumbar and sacral spine, which is important as abnormalities of
vertebral segments occur frequently in ARM patients. Measure-
ment of the sacral ratio on anterior–posterior and lateral plain
radiograph has been proposed as prognostic indicator for develop-
ment of future voluntary bowel movements in ARM population.12
Unfortunately, the sacral ratio or sacral anomalies have not been as
reliable for the prediction of urinary continence, development of
neurogenic bladder or need for intermittent catheterization.13,14
Lumbosacral vertebral abnormalities can also be associated
with spinal cord abnormalities. Spinal ultrasound can be a helpful
imaging modality in the newborn as ossification of the bone has
not occurred permitting ultrasound to image the distal spinal cord.
A fatty filum that tethers the spinal cord (anchoring the cord with
no displacement of the cord upon movement from prone to supine
position), lipomatous elements around or within spinal cord has
been identified with ultrasound.15 Magnetic resonance imaging
maybe helpful in confirming the abnormal findings of the screen-
ing ultrasound. Accurate identification of spinal cord abnormalities
can have prognostic and clinical implications for bladder and
bowel function.4,13,16
Vagina
The abdominal radiograph can also identify a soft tissue density
in pelvis that may represent a fluid filled structure such as hydro-
colpos or distended bladder can be visible and have mass effect
pushing intestine superiorly (Figure 1). At times the hydrocolpos
can be palpated or visible on physical examination. Ultrasound is
Fig. 1. Abdominal radiograph demonstrating soft tissue density in pelvis that
represents hydrocolpos with mass effect pushing intestine superiorly.
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83
Fig. 2. Mechanism of obstructive uropathy from hydrocolpos. (Reproduced with
permission from Hendren.20)
helpful in distinguishing whether a pelvic mass is bladder or
vaginal in origin. The urine-filled vagina can also cause compres-
sion of the trigone resulting in hydroureteronephrosis making
diagnosis and subsequent management of hydrocolpos imperative
(Figure 2).
Kidney and bladder
Ultrasound offers the most common modality for widespread,
rapid, and reliable imaging of kidneys and bladder. Sonography
does not expose the patient to ionizing radiation, which is a
particular advantage in prenatal care. Antenatal identification of
cloacal malformation has been described with sonographic find-
ings of a distended cystic structure in the pelvis representing a
distended vagina/hydrocolpos, distended bladder with hydro-
nephrosis, and accompany dilated ureters and dilated bowel.
However, accurate prenatal diagnosis remains a challenge given
the broad differential diagnoses of these radiographic findings.
Abnormalities of kidney number, location, echogenicity, and
rotation have been reported in multiple series of patients with
cloaca.7–9,17 Renography and cystourethrography are typically
utilized to help distinguish whether obstruction or vesicoureteral
reflux is present as possible causes for the hydronephrosis.
Catheterization of the bladder for voiding cystourethrogram can
be difficult given the unrepaired cloacal anatomy. At times, the
catheter may unavoidably be introduced into the vagina due to
angulation or compression of the urethra by hydrocolpos. It is for
this reason; it is our practice that we do not routinely perform
retrograde cystourethrography as part of the initial evaluation of
hydronephrosis in our unrepaired cloaca patients. Regardless
of the timing of voiding cystourethrography, active treatment of
high-grade vesicoureteral reflux is important to mitigate its
potential effects on kidney function that can result from pyelo-
nephritis or reflux occurring as a result of abnormal bladder
storage pressures.7–9
Cloacal common channel
Fluoroscopic evaluation of the cloacal anatomy by insertion of a
catheter into the common channel for instillation of contract can
be helpful for identifying the complex spatial relationships of the
urologic, gynecologic and rectal structures. Insertion of an appro-
priately sized Foley catheter with inflation of the balloon just
proximal to the meatus can allow for instillation of contrast to
opacify the common channel without leakage around the catheter.
Such a “flush genitogram” is also performed with the intent of
84 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
delineating proximal structures and identification of the conflu-
ence of the urinary tract, vagina, and rectum.18 However, opacifi-
cation of both structures with instilled contrasted to obtain
meaningful images to adequately measure and visualize spatial
relationship of this complex malformation is not always achieved
with conventional fluoroscopy. In order to better accomplish the
task of imaging and understanding the associations of confluence
of the rectum, vagina, and urinary tract, our institution has
developed what we refer to as the “cloacogram.”19
Risk factors for intensive urologic management in cloaca at
time of presentation
There have been many retrospective studies performed that
attempt to identify and stratify the factors that can be utilized to
predict the urologic dysfunction in patients with cloaca.7–9,17,20–23
Unfortunately, as with all retrospective studies, limitations such as
recall bias, variability of duration of patient follow-up, heteroge-
neous patient anatomy, and multiple surgeon providers affect our
ability to make firm conclusions based upon the reported data.
Nevertheless, there can be information gleaned from these studies
that aid clinicians in urologic risk stratification for cloaca patients
by recognizing the following shared clinical features among many
of these studies:

Common channel length

Abnormal spinal cord

Solitary functioning kidney
Common channel length and its association with need for CIC
One of the most frequently reported variables in outcome
studies of patients with cloacal anomalies has been length of the
common channel. Peña et al.17 described the utility of this metric in
preparing the surgeon for increased complexity of malformation
and frequency of requiring additional maneuvers beside the poste-
rior sagittal approach for surgical repair. In his series, he divided 339
patients into those with a common channel length o3 cm and
those 43 cm. The need for clean intermittent catheterization (CIC)
differed between the two groups with 78% requiring CIC for
common channel length 43 cm compared to 28% for common
channel length o3 cm (Table). Urinary continence was achieved in
54% of all patients old enough to be assessed in the series.
Other institutions have examined the associated importance of
common channel length and neurogenic bladder. Warne et al.,22
from the Great Ormond Street in London, reviewed bladder
function in a cohort of ten cloaca patients both preoperativey
and postoperatively using urodynamics. The authors described five
patients with common channel o 3 cm and five patients with a
common channel 43 cm along with any baseline urodynamic
abnormality matched to observed changes postoperatively. In all,
Table
Cloaca common channel length and associated urinary continence rates and need for CIC.
Braga et al.8 (n ¼ 12) Peña et al.17 (n ¼ 193) LeClair et al.24 (n ¼ 22)
CC o 3 cm 5 N/A 10
Continent 40% (n ¼ 2) 28% 70% (n ¼ 7)
CIC o 3 cm 20% (n ¼ 1) 28% 44% (n ¼ 4)
CC 4 3 cm 7 N/A 12
Continent 86% (n ¼ 6) 72% 67% (n ¼ 8)
CIC 4 3 cm 71% (n ¼ 5) 78% 50% (n ¼ 4)
CC, cloacal common channel; CIC, clean intermittent catheterization; N/A, information not available from manuscript.
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90% of the preoperative urodynamics were classified as abnormal
and equivalent number was classified as abnormal, postopera-
tively. A change in urodynamic pattern occurred in 50% of all
patients prompting initiation of CIC or surgical diversion. Of the
five patients with deterioration of bladder function four had a
common channel longer than 3 cm and one had a common
channel o 3 cm (Table). Comparably Braga et al.8 from Toronto,
described a series of 12 cloaca patients with seven patients having
common channel Z 3 cm and future need for CIC. Six of the seven
patients (85%) required CIC with four patients having undergone
prior bladder neck closure, augmentation and Mitrofanoff. This
observation was in contrast to two of the five patients who were
continent and voiding having common channel Z 3 cm. Shimada
et al. reported their case series of nine cloaca patients (six with
common channel 4 3 cm), of the nine patients, five required CIC
for bladder management.25 Unfortunately the authors did not
separate these five patients on CIC based upon common channel
length to comment on association but with two-thirds of cohort
have common channel 4 3 cm this represents another study
showing higher rates of bladder dysfunction in cloaca patients
with longer common channel lengths.
Spinal cord and sacral anomalies and their association with
development of neurogenic bladder
Sacral anomalies, as previously discussed, occur in a large
proportion of patients with cloaca and other ARM patients. The
increased observation of sacral anomalies leads to further imaging
and a tethered cord is identified in significant number of ARM
patients with an abnormal sacrum. However, there are many patients
that have an abnormal spinal cord with normal sacrum. A tethered
cord has been shown to be a negative predictor for achievement of
fecal continence in ARM,4 but its exact prognostic role in bladder
function remains unclear. The radiographic diagnosis of a tethered
cord many times leads to further testing and at times urologic
evaluation, including a urodynamic assessment. The clinical signifi-
cance of a tethered cord in an asymptomatic patient is controversial
with varying reported rates of surgical untethering in the literature.26
The proposed pathophysiologic mechanism for urologic dysfunction
in patients with tethered cord syndrome involves stretch mediated
ischemic injury to nerves with vertical growth of the patient.27 As
this is a dynamic process longitudinal follow-up is crucial to identify
any changes that can occur in voiding history, onset of urinary tract
infections or radiographic findings. There is debate over what specific
testing is necessary in urologic follow-up in these patients; however,
most would agree that a comprehensive urologic assessment in
patients suspected of having a clinically significant tethered cord
involves detailed history and physical examination, voiding diary,
renal/bladder ultrasound, voiding cystourethrogram and, most
importantly, urodynamics.
Interpretation of the urodynamics can be challenging if not
properly performed, because if the child is moving during the
study, motion artifact will occur and once again there exists
Versteegh et al.23 (n ¼ 25) Muller et al.21 (n ¼ 23) Totals
19 15 49
68% (n ¼ 13) 53% (n ¼ 8) 61% (n ¼ 30)
21% (n ¼ 4) N/A 18% (n ¼ 9)
6 8 29
83% (n ¼ 5) 50% (n ¼ 4) 79% (n ¼ 23)
50% (n ¼ 3) N/A 41% (n ¼ 12)
 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
anatomic challenges of placement of catheter into bladder if the
cloacal malformation has not yet been repaired. We recommend
avoidance of any type of sedation or anesthesia to eliminate the
possible pharmacologic effects of these agents as well as allowing
for voiding phase to be observed. Nevertheless, there have been
multiple studies examining the effect of spinal cord abnormalities
on lower urinary tract function in patients with ARM.6,13–16,28–30
Different urodynamic patterns (overactive, atonic, dysnergia
between external urethral sphincter and detrusor, elevated leak
point pressures during filling phase) have been observed empha-
sizing the need for an individualized approach to each child. The
radiographic abnormalities of the sacrum or spine do not always
predict urodynamic abnormalities. In a study by Taskinen et al.,30
of 30 male and female ARM patients in whom the spinal cord
imaging was normal, 54% of the patients had abnormal urody-
namic findings; but when the spinal cord imaging was abnormal,
59% had abnormal urodynamics. Stathopoulos et al.13 looked at 80
ARM to determine if a pathological post-reconstruction urody-
namic was associated with abnormal spinal or sacral imaging. Of
the 14 children with pathological urodynamics, no vertebral
anomaly or spinal cord abnormality was found in 50% of them.
Of the 66 children with normal urodynamics, 40 presented with
some type of vertebral or spinal malformation. Therefore, normal
spine or spinal cord imaging in their series did not exclude
neurovesical dysfunction. Conversely, an abnormal spinal cord or
vertebral anomaly did not determine lower urinary tract dysfunc-
tion. Similarly, an abnormal urodynamics will not always be
associated with abnormal radiographic findings of the urinary
tract such as hydronephrosis or continence outcomes, i.e., voiding
and dry, respectively.
A recent article by Muller et al.21 specifically examined the
impact of spinal cord abnormalities on urinary prognosis in cloaca.
When examining the variety of spinal cord dysraphisms such as
fatty filum, low conus, or short spinal cord/caudal regression, the
authors described varying degrees of urinary continence. of the
cohorts, 52% were continent by volitional voiding while 48% were
incontinent and/or on CIC. The authors did not see a difference in
urinary continence rates based upon common channel length
o 3 cm vs. 4 3 cm. A poor urologic prognosis for continence was
associated with short spinal cord where none of these five patients
were continent.21 In stark contrast, eight of the nine patients in the
series with a tethered cord complex were continent with “rigorous
urologic management”; although it is not specified whether such
management included CIC.
In summary, The lower urinary tract can be influenced by an
abnormal spinal cord or vertebral abnormalities and our current
ability to accurately predict the long-term outcome is unreliable.
Solitary kidney and its association with development of chronic
kidney disease
Chronic kidney disease occurs at higher incidence upon long-
term follow-up of cloaca patients compared to the normal pop-
ulation.7 Prior retrospective studies of cloaca patients have
observed chronic kidney disease, defined as glomerular filtration
rate of o 80 mL/min/1.73 m2, and end-stage renal disease of up to
75% and 17%, respectively.7,8 This sobering statistic underscores
the need for close urology surveillance as these children age
because the onset of end stage renal disease may not be entirely
evident at birth. Of the 32 patients in one series who subsequently
had chronic kidney disease, including six with bilateral renal
dysplasia, 21 had normal nadir creatinine in the first year of life.7
A solitary functioning kidney has been associated with increa-
sed potential for development of renal injury in children defined
as hypertension, albuminuria, and chronic kidney disease later in
life regardless of the etiology being congenital or acquired.31
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85
However, in that study the children with congenital anomalies of
the kidney and urinary tract (CAKUT) had a higher risk of renal
injury compared to those children without CAKUT (48% vs 25%) at
mean age of 9.5 years.31 All eight patients with a solitary kidney in
the Great Ormond cloaca series had glomerular filtration rate of
o80 mL/min/1.73 m2.7 Similarly, Rink et al.9 observed an increa-
sed incidence of chronic kidney disease, which they defined as an
abnormal or borderline serum age-adjusted creatinine value,
in two-thirds of their patients with a cloacal malformation and a
solitary kidney.
Urology concerns at time of diverting colostomy
Once the child is stable enough for diverting colostomy follow-
ing thorough cardiopulmonary assessment, a decision can be made
of the appropriateness of endoscopic evaluation of the cloacal
anatomy under the same anesthetic. At our institution we usually
attempt to pass a four or seven French cystoscope through the
common channel in an attempt to measure the common channel
length for future planning and prognostic counseling. However,
endoscopy is not a uniform practice and the primary purpose of
the procedure is to decompress gastrointestinal tract and hydro-
colpos. As previously stated anatomy can be assessed in an
interdisciplinary fashion at later date combined with contrast
studies. Difficult Foley placement can be circumvented by place-
ment of 0.035 in guide wire via the working channel of the
cystoscope under direct vision into the bladder and the Seldinger
technique used to place a Council-tip catheter over the wire.
Additional information regarding gynecologic anatomy such as
single or duplicated vagina is acquired if feasible.
The technique for diverting colostomy can have urologic
implications as well. A diverting colostomy with separated stomas
is preferred, as this separates the fecal and urinary streams
completely given the presence of a rectourinary fistula in cloaca.
A loop colostomy may accomplish this; however, not as reliably as
there stills exists communication for passage of stool distally
which we have seen contribute to urinary tract infections. A left
lower quadrant rather than transverse colostomy is preferred to
minimize the length of distal colon following separation of stomas.
A long colonic segment such as that present with a transverse
colostomy theoretically can allow for more resorption of urine that
can leak through the rectourinary fistula and lead to metabolic
acidosis.32 This has been shown to contribute to the childʼs failure
to thrive. Stomal location can also affect future urologic incisions.
A vesicostomy or ureterostomy may become necessary to surgi-
cally address urologic pathology and a colostomy stoma too medial
would necessitate an appliance covering the midline and make a
vesicostomy stoma in the midline impossible for secure stomal
appliance fit. Any proximal stoma ideally should be located away
from both the umbilicus and the iliac crest, thus surrounded by a
good portion of normal skin so that the stoma bag can be easily
adapted.33
Hydrocolpos must be identified and adequate drainage critical
to minimizing the obstructive and infectious complications of it.
The exact mechanism of how hydrocolpos develops is unclear;
however, the extrinsic compression by the hydrocolpos on the
bladder outlet, trigone and distal ureters resulting in urinary
retention and upper tract dilation is undisputable. Decompression
of the hydrocoplos is our recommended first step to improve the
hydroureteronephrosis that is observed (Figure 3). This maneuver
alone may result in resolution of the dilated vagina and urinary
tract avoiding the need for procedures to empty the bladder or
upper tracts such as vesicostomy or ureterostomy, respectively. We
have routinely preferred a tube vaginostomy at the time of
colostomy creation to drain the hydrocolpos. Care must be
86 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89

Fig. 3. (A) Bilateral hydronephrosis in newborn cloaca as a result of hydrocolpos. (B) Decompression of the hydrocoplos alone resulted in resolution of the
hydroureteronephrosis.

exercised to inspect if a Mullerian duplication exists, as drainage of
a single vagina does not insure complete and sufficient drainage of
the hydrocolpos depending upon vaginal septal anatomy in such
cases.
Alternative ways to drain hydrocolpos have been utilized such
as intermittent catheterization of the common channel. An unre-
paired cloaca will have three systems communicating through the
common channel so that intermittent catheterization of the
common channel will not guarantee that the catheter will enter
the vagina(s) to drain hydrocolpos every time. It is our practice not
to advocate intermittent catheterization as a technique to drain
hydrocolpos, as we have been referred patients where this
technique was being performed but not adequately addressing
the hydrocolpos resulting in complications from the undrained
hydrocolpos, i.e., obstructed urinary tract and urinary stasis with
UTIs. However, a recent report by Chalmers et al.34 compared CIC
to surgical diversion (vesicostomy, vaginostomy, ureterostomy, and
nephrostomy) for genitourinary decompression and preservation
of renal function, regardless of the severity of cloacal anomaly.
A total of 25 patients were identified. Nine (36%) patients under-
went early surgical diversion, but unfortunately the exact proce-
dures performed were not outlined in the manuscript. In all, 16
(64%) managed by CIC prior to formal reconstruction. Seven had
short common channels ( o3 cm) and 18 had long common
channels ( Z3 cm). Hydrocolpos was present in 14 (56%) of the
patients. When comparing the two management groups, there was
no significant difference in hydronephrosis, vesicoureteral reflux
or renal dysplasia. No significant differences were found between
mean baseline GFR for diversion [22.9 mL/min per 1.73 m(2)] vs.
CIC [39.2 mL/min per 1.73 m(2), p ¼ 0.22]. There was no difference
in creatinine trend between the two groups. Regardless of the
manner employed to decompress the genitourinary tract, imaging
post intervention is highly recommended to determine whether
decompression of the hydrocolpos has been reliably achieved, and
to ensure that the urologic complications from it have been
mitigated.
Urology concerns at time of repair of cloaca
Preoperative counseling and education of the parents for
potential lower urinary tract dysfunction is crucial to setting
expectations based upon specific anatomy of the patient. Preparing
for the possible need for intermittent catheterization in the future
management of the patient needs to be in the surgeonʼs thinking
during the reconstruction regardless of whatever surgical techni-
que is utilized. Total urogenital mobilization (TUM) has enabled a
less complicated surgical strategy of cloaca with shorter common
channel which has in turn decreased the incidence of complica-
tions such as urethrovaginal fistula.35 At times following recon-
struction employing TUM the urethral meatus may not be able to
be placed directly underneath the clitoris with the meatus visible
upon separation of the labia. While this is not the ideal situation
we have had frequent success in performing urethral catheter-
ization using a Coude catheter to gain access to the urethra when
intermittent catheterization is necessary.
In the case of the longer common channel, total urogenital
mobilization may be inadequate to result in having the urethra and
vagina reach the perineum. Separation of the vagina from the
urinary tract with subsequent vaginal pull-through will accomplish

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 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
having the vagina reach the perineum and the common channel
is left as the urethra. Ensuring that the prior connection bet-
ween the vagina and common channel is smooth without irregu-
larity is important technical aspect of the separation of the vagina
from common channel. Intraoperative assessment of the ease of
catheterizability of the remnant common channel, now the
urethra, is paramount to avoid postoperative problems. Concern
over the viability of the urethra following extensive dissection and
mobilization of the common channel during cloacal repair should
trigger the surgeon, without reluctance nor hesitation, to place a
suprapubic tube to insure a mechanism to empty bladder once the
urethral catheter is removed. The suprapubic tube will allow for
measurement of post-void residuals without intermittent catheter-
ization of the urethra, provide a means for cystogram and
even urodynamics if desired. This proactive intraoperative maneu-
ver avoids the unanticipated emergent return to operating
room for postoperative urinary retention due to transient reversible
causes of bladder obstruction or urethral atresia due to vascular
insult.
The surgeon will need to be prepared for aberrant ureteral
anatomy that can occur in children with cloacal anomalies.
Ureteral ectopia can occur and will need to be addressed depend-
ent upon the differential function of the renal unit associated with
it. Ureteral reimplantation into the bladder can be performed and
an antirefluxing mechanism is preferred. At times the ureteral
ectopia may drain a poorly functioning or dysplastic kidney and
removal of that kidney or moiety along with the ectopic ureter
may be more suitable. Knowledge of the function of the moiety
associated with the ectopic ureter may not be available and
cutaneous ureterostomy is reasonable surgical option at the time
of cloacal repair. Postoperative testing of the renal function would
then dictate if the ureter is reimplanted or removed along with the
ipsilateral renal unit.
Assessing the competency of the bladder outlet during the
cloacal repair is important but unreliable when attempting to
predict urinary continence. In a retrospective review Rink et al.9
stated the bladder neck appearance was available in 10 of 18 cloaca
patients older than 4 years of age to examine its predictive value.
The endoscopic and radiographic appearance at heterogeneous
time points during follow-up was not a predictor of ultimate
continence, but four of five patients with an open bladder neck
required reconstruction of the bladder and/or bladder neck,
compared with one of five with evidence of a coapting bladder
neck.
The cloacal spectrum can result in a severe malformation where
rectum and vaginas converge onto bladder neck making recon-
struction formidable and possible influence the postoperatively
competency of the bladder neck. We have seen ectopic single
system ureters resulting in hyoplastic bladder and an under-
developed bladder neck in patients with cloacal malformation
(Figure 4). These two scenarios may influence an intraoperative
strategy where the bladder neck is reconstructed or even closed at
time of primary repair given concern over ability to provide
bladder outlet resistance if left alone. Concomitant urinary diver-
sion then becomes mandatory if bladder neck is closed and
surgeonʼs discretion over whether an incontinent diversion, such
as vesicostomy, or continent diversion, such as Mitrofanoff, is
appropriate depends on patient age, social circumstances and
other clinical factors.
Short-term postoperative urologic concerns
By virtue of the fact that urethral reconstruction accompanies
the overwhelming majority of cloacal repairs, the bladder usually
has a catheter left to drain the bladder. Dependent upon the
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87
Fig. 4. Endoscopic view through common channel of unrepaired cloaca in a
9-month old with a single system ectopic single ureter draining distal to bladder
neck into common channel resulting in very small capacity bladder and an
underdeveloped bladder neck. The patient had ureteral reimplant into bladder,
closure of bladder neck, and creation of vesicostomy due to intraoperative
assessment that the bladder outlet was unreconstructable and urinary inconti-
nence due to poor outlet resistance inevitable. Future continent reconstruction
with Mitrofanoff with possible bladder augmentation is planned when the child is
older with concomitant Malone procedure.
complexity of the repair the catheter is left behind for several days
and in our institution typically 2–3 weeks. A voiding trial is
pursued and the majority of these patients will be able to void.
Close observation is mandatory as reports exist of changes in
bladder function in cloaca requiring active management including
clean intermittent catheterizations with or without pharmacologic
therapy, i.e., antibiotic prophylaxis and/or antichiolinergic medi-
cations to protect the kidneys.22,36
We recommend a repeat renal ultrasound 4–6 weeks after
cloacal repair to identify structural changes of bladder and kidney
compared to preoperative imaging studies. The parental observa-
tions of the patientsʼs voiding habits are helpful when prolonged
intervals are elicited, which may be the indicator of voiding
dysfunction; although this would need to be confirmed with
diagnostic testing as indicated. A dilated rectum can negatively
affect bladder function and while the diverting colostomy is in
place we will perform a VCUG as a bladder functional study to
observe bladder emptying, bladder capacity and contour as well as
identify if de novo vesicoureteral reflux is present. At times formal
urodynamics are performed at this time point as well if clinical or
radiographic signs of bladder dysfunction are evident. However,
we typically wait several months from the cloacal repair to allow
sufficient time to pass before obtaining a baseline urodynamic
study in order to minimize any potential confounding transient
effects from a major pelvic reconstructive surgery on bladder
function.
Under the same anesthetic as the proposed colostomy closure, a
multidisciplinary evaluation similar to the preoperative endoscopy
is undertaken. Assessment of urethral meatus, ease of catheter-
izability, bladder neck appearance and, if necessary, exchange of
existing suprapubic tube were performed. We do not usually
combine reconstructive urologic procedures such as antireflux
surgery or bladder neck surgery/Mitrofanoff at this time. We favor
allowing the child to recover and observe with close surveillance
how the bladder functions with spontaneous voiding in most cases
and a more proactive approach in others at deemed at higher risk
for requiring CIC in the future.
As previously discussed, a successful voiding trial may not be
predictive of healthy lower urinary tract in all cloaca patients.
Some of our select patients with combination of clinical factors
(long common channel, chronic kidney disease, solitary kidney,
etc.) and intraoperative findings direct us to take a proactive
approach to bladder management in an effort to prevent or slow
progression of CKD. Our center is a tertiary referral center for
88 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
(-) abnormal
spinal cord
Cloaca
Repair
Baseline
RUS
Serum Cr
(+) abnormal
spinal cord
Fig. 5. Suggested protocol of early urologic follow-up for cloaca. RUS, renal ultrasound; Cr, creatinine for glomerular filtration rate measurement; VCUG, voiding
cystourethrogram; UDS, urodynamic study; surveillance, annual blood pressure measurement, urine analysis and renal bladder ultrasound; active bladder management,
consider clean intermittent catheterization based upon urodynamic results. Consider prophylactic antibiotics if high-grade vesicoureteral reflux. Consider anticholinergic
with/without intermittent catheterization based upon urodynamic result.
complex cloaca both primary and secondary repairs. We examined
our experience with the interdisciplinary care of cloaca patients
since the creation of the Colorectal Center at Cincinnati Childrenʼs
in 2006 until 2012. DeFoor et al.36 retrospectively reviewed the 55
patients referred to our center and excluded 11 patients for lack of
follow-up, unrepaired at time of review or deceased leaving 44
patients for anlysis. The median length of the common channel
was 3.5 cm (IQR ¼ 2.5–5 cm). CKD Stage 1 or 2 existed in 38/44
patients and CKD Stage 3 existed in five patients with the
remaining one patient presenting with CKD Stage 4 at birth. In
this cohort we diagnosed a neurogenic bladder in 68% of the
patients. Of the patients diagnosed with neurogenic bladder, 48%
had common channel length o3.5 cm vs. 89% of those patients
with common channel 4 3.5 cm (p ¼ 0.007) In the median follow-
up period of 5 years, no patients with initial CKD stage 1–3 have
had stage progression. The single patient with CKD Stage 4 had a
renal allograft placed at 34 months of age. Our study demonstrates
that early renal function can be maintained in cloaca patients
despite a high rate of lower urinary tract dysfunction. Further
follow-up through adolescence is needed to determine if further
CKD progression occurs or differs from historical descriptions.
Annual blood pressure measurements along with urine analysis
to detect proteinuria are important adjuncts that assist in discov-
ering renal injury in the patients with cloaca as they grow. Periodic
renal ultrasound will also aid in identifying structural changes that
may prompt earlier investigations of bladder function. A suggested
postoperative schedule for early urologic follow-up for cloaca is
present in Figure 5. Boemers et al.37 have recommended given the
increased incidence of lower urinary tract dysfunction a protocol
based upon abnormalities of screening studies leading to compre-
hensive testing to identify early on bladder dysfunction. The
authors aim of such screening is to minimize the urologic sequelae
of a neurogenic bladder and instituting proactive measures such as
CIC when acceptance by patient and family may be easier than
beginning later in life.
Conclusions
Patients with cloacal malformation represents a group of
individuals that are at an increased risk of urologic dysfunction
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Colostomy
Closure Surveillance
RUS
VCUG
Serum Cr
UDS
Active
RUS Bladder
VCUG Management
UDS
Serum Cr
Surveillance
compared to other ARM populations. We feel that collaborative
patient care combined with aggressive, proactive bladder manage-
ment may help minimize the risk of progressive acquired renal
injury.
References
1. Goossens WJ, de Blaauw I, Wijnen MH, de Gier RP, Kortmann B, Feitz WF.
Urological anomalies in anorectal malformations in The Netherlands: effects of
screening all patients on long-term outcome. Pediatr Surg Int. 2011;27(10):
1091–1097.
2. Metts JC 3rd, Kotkin L, Kasper S, Shyr Y, Adams MC, Brock JW 3rd. Genital
malformations and coexistent urinary tract or spinal anomalies in patients with
imperforate anus. J Urol. 1997;158(3 Pt 2):1298–1300.
3. McLorie GA, Sheldon CA, Fleisher M, Churchill BM. The genitourinary system in
patients with imperforate anus. J Pediatr Surg. 1987;22(12):1100–1104.
4. Levitt MA, Patel M, Rodriguez G, Gaylin DS, Peña A. The tethered spinal cord in
patients with anorectal malformations. J Pediatr Surg. 1997;32(3):462–468.
5. Kim SM, Chang HK, Lee MJ, et al. Spinal dysraphism with anorectal malforma-
tion: lumbosacral magnetic resonance imaging evaluation of 120 patients.
J Pediatr Surg. 2010;45(4):769–776.
6. Mosiello G, Capitanucci ML, Gatti C, et al. How to investigate neurovesical
dysfunction in children with anorectal malformations. J Urol. 2003;170(4 Pt
2):1610–1613.
7. Warne SA, Wilcox DT, Ledermann SE, Ransley PG. Renal outcome in patients
with cloaca. J Urol. 2002;167(6):2548–2551.
8. Braga LH, Lorenzo AJ, Dave S, Del-Valle MH, Khoury AE, Pippi-Salle JL. Long-
term renal function and continence status in patients with cloacal malforma-
tion. Can Urol Assoc J. 2007;1(4):371–376.
9. Rink RC, Herndon CD, Cain MP, et al. Upper and lower urinary tract outcome
after surgical repair of cloacal malformations: a three-decade experience. BJU
Int. 2005;96(1):131–134.
10. Sheldon C, Cormier M, Crone K, Wacksman J. Occult neurovesical dysfunction in
children with imperforate anus and its variants. J Pediatr Surg. 1991;26(1):
49–54.
11. Kasabian NG, Bauer SB, Dyro FM, Colodny AH, Mandell J, Retik AB. The
prophylactic value of clean intermittent catheterization and anticholinergic
medication in newborns and infants with myelodysplasia at risk of developing
urinary tract deterioration. Am J Dis Child. 1992;146(7):840–843.
12. Peña A. Anorectal malformations. Semin Pediatr Surg. 1995;4(1):35–47.
13. Stathopoulos E, Muehlethaler V, Rais M, et al. Preoperative assessment of
neurovesical function in children with anorectal malformation: association
with vertebral and spinal malformations. J Urol. 2012;188(3):943–947.
14. Boemers TM, Beek FJ, van Gool JD, de Jong TP, Bax KM. Urologic problems in
anorectal malformations. Part 1: urodynamic findings and significance of sacral
anomalies. J Pediatr Surg. 1996;31(3):407–410.
15. van den Hondel D, Sloots C, de Jong TH, Lequin M, Wijnen R. Screening and
treatment of tethered spinal cord in anorectal malformation patients. Eur J
Pediatr Surg. 2015 [Epub ahead of print].
 B.A. VanderBrink, P.P. Reddy / Seminars in Pediatric Surgery 25 (2016) 82–89
16. DeGennaro M, Rivosecchi M, Lucchetti MC, Silveri M, Fariello G, Schingo P. The
incidence of occult spinal dysraphism and the onset of neurovesical dysfunc-
tion in children with anorectal anomalies. Eur J Pediatr Surg. 1994;4(Suppl 1):
12–14.
17. Peña A, Levitt MA, Hong A, Midulla P. Surgical management of cloacal
malformations: a review of 339 patients. J Pediatr Surg. 2004;39(3):470–479.
18. Shopfner CE. Genitography in intersexual states. Radiology. 1964;82(4):
664–674.
19. Patel MN, Racadio JM, Levitt MA, Bischoff A, Racadio JM, Peña A. Complex
cloacal malformations: use of rotational fluoroscopy and 3-D reconstruction in
diagnosis and surgical planning. Pediatr Radiol. 2012;42(3):355–363.
20. Hendren WH. Cloaca the most severe degree of imperforate anus: experience
with 195 cases. Ann Surg. 1998;228(3):331–346.
21. Muller CO, Crétolle C, Blanc T, et al. Impact of spinal dysraphism on urinary and
faecal prognosis in 25 cases of cloacal malformation. J Pediatr Urol. 2014;
10(6):1199–1205.
22. Warne SA, Godley ML, Wilcox DT. Surgical reconstruction of cloacal malforma-
tion can alter bladder function: a comparative study with anorectal anomalies.
J Urol. 2004;172(6 Pt 1):2377–2381.
23. Versteegh HP, Sloots CE, Wolffenbuttel KP, et al. Urogenital function after
cloacal reconstruction, two techniques evaluated. J Pediatr Urol. 2014;10(6):
1160–1164.
24. Leclair MD, Gundetti M, Kiely EM, Wilcox DT. The surgical outcome of total
urogenital mobilization for cloacal repair. J Urol. 2007;177(4):1492–1495.
25. Shimada K, Hosokawa S, Matsumoto F, Johnin K, Naitoh Y, Harada Y. Urological
management of cloacal anomalies. Int J Urol. 2001;8(6):282–289.
26. Tuuha SE, Aziz D, Drake J, Wales P, Kim PC. Is surgery necessary for
asymptomatic tethered cord in anorectal malformation patients? J Pediatr Surg.
2004;39(5):773–777.
Downloaded for Nasha Mae Dela Cruz (gatsbynasha@gmail.com) at Saint Louis Universty - Philippines from ClinicalKey.com by Elsevier on July 23, 2018.
For personal use only. No other uses without permission. Copyright ©2018. Elsevier Inc. All rights reserved.
89
27. Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatr
Neurosurg. 2007;43(3):236–248.
28. De Filippo RE, Shaul DB, Harrison EA, Xie HW, Hardy BE. Neurogenic bladder in
infants born with anorectal malformations: comparison with spinal and
urologic status. J Pediatr Surg. 1999;34(5):825–827.
29. Borg H, Holmdahl G, Olsson I, Wiklund LM, Sillén U. Impact of spinal cord
malformation on bladder function in children with anorectal malformations.
J Pediatr Surg. 2009;44(9):1778–1785.
30. Taskinen S, Valanne L, Rintala R. Effect of spinal cord abnormalities on the
function of the lower urinary tract in patients with anorectal abnormalities.
J Urol. 2002;168(3):1147–1149.
31. Westland R, Schreuder MF, Bökenkamp A, Spreeuwenberg MD, van Wijk JA.
Renal injury in children with a solitary functioning kidney—the KIMONO study.
Nephrol Dial Transplant. 2011;26(5):1533–1541.
32. Caldamone AA, Emmens RW, Rabinowitz R. Hyperchloremic acidosis and
imperforate anus. J Urol. 1979;122(6):817–818.
33. Peña A, Migotto-Krieger M, Levitt MA. Colostomy in anorectal malformations:
a procedure with serious but preventable complications. J Pediatr Surg. 2006;
41(4):748–756.
34. Chalmers DJ, Rove KO, Wiedel CA, Tong S, Siparsky GL, Wilcox DT. Clean
intermittent catheterization as an initial management strategy provides for
adequate preservation of renal function in newborns with persistent cloaca.
J Pediatr Urol. 2015;11(4):211.e1-4.
35. Peña A. Total urogenital mobilization—an easier way to repair cloacas. J Pediatr
Surg. 1997;32(2):263–267.
36. DeFoor WR, Bischoff A, Reddy P, et al. Chronic kidney disease stage progression
in patients undergoing repair of persistent cloaca. J Urol. 2015;194(1):190–194.
37. Boemers TM, Beek FJ, Bax NM. Guidelines for the urological screening and
initial management of lower urinary tract dysfunction in children with
anorectal malformations—the ARGUS protocol. BJU Int. 1999;83(6):662–671.