AbouZeid et al.

Annals of Pediatric Surgery (2020) 16:3 Annals of Pediatric Surgery

https://doi.org/10.1186/s43159-019-0010-z

ORIGINAL RESEARCH Open Access

Persistent cloaca: persistence of the

challenge
Amr Abdelhamid AbouZeid* , Ahmed Bassiouny Radwan, Mohamed Eldebeiky and Sameh Abdel Hay

Abstract
Background: Persistent cloaca or cloacal anomalies represent a special category of anorectal anomalies affecting
the female sex with a reported incidence of about one in 25,000 live birth.
The study included 34 cases of cloaca that were managed at our unit between 2003 through 2017. We
retrospectively reviewed patients’ records that included clinical presentation, investigations, operative data, and
follow-up notes.
Anatomically, we stratified cloaca into three types according to the level of urogenital confluence. A low
confluence (type 1) was defined by being at or below the level of the lower border of pubic symphysis with
a short common channel (11 cases). A high confluence (type 3) was defined by being at or above the level
of the upper border of pubic symphysis (9 cases). Between the low and high types, we defined an
intermediate type (type 2) where the urogenital confluence was behind the mid-portion of pubic symphysis
(14 cases).
Results: Renal anomalies were common association: solitary kidney in seven, pelvic kidney in two, and urinary
tract dilatation (hydroureteronephrosis) in 12 cases. At follow-up, chronic renal insufficiency was detected in
seven cases
The prognosis for urinary continence was excellent in low confluence (type 1) cloaca. On the other hand,
urinary incontinence was common among type 3 (high confluence) cloaca (62.5%).
Conclusion: Renal anomalies represent a common association with cloaca and a major cause of morbidity.
Efforts should be directed to preserve renal function during the initial management, and to preserve the
continence potential following the definitive repair.
Level of evidence: This is a case series with no comparison group (level IV).
Keywords: Cloaca, MRI, Surgery, Outcome, Classification

Background Hendren reporting his surgical experience in the

Persistent cloaca or cloacal anomalies represent a spe- management of a wide spectrum of complex cases of
cial category of anorectal anomalies (ARA) affecting cloaca [4, 5]. The introduction of the posterior sagit-
the female sex with a reported incidence of about one tal approach gained wide-spread acceptance and in-
in 25,000 live birth [1]. The combination of urogenital creased our understanding about the surgical anatomy
and anorectal anomalies in the same subject compli- of the anal sphincters [6]. This has been reflected on
cates the clinical picture that it may be considered improving the outcome of repairing the anorectal
one of the most challenging situations in paediatric component of the anomaly. However, separation of
surgery [2, 3]. the vagina from the common urogenital sinus
Searching through the literature, one can find the remained a real challenge. The concept of urogenital
detailed descriptions and illustrations of Hardy sinus mobilization then appeared [7] aiming to save
operative time and blood loss that may result from a
* Correspondence: [email protected] difficult vaginal separation [3]; but still the technique
Pediatric Surgery Department, Faculty of Medicine, Ain-Shams University,
Lotefy El-Sayed street, 9 Ain-Shams University buildings, Abbassia, Cairo
11657, Egypt

© The Author(s). 2020 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
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AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 2 of 7

has its limitations that it cannot be applied in all situ- (vaginostomy and/or vesicostomy; five and four cases
ations [8]. respectively).
Here, we are reporting our experience in the man- It may be generally accepted to classify cloaca accord-
agement of a group of patients with cloaca over the ing to the length of the common channel into either
last 15 years. Studying the surgical outcomes in cloaca short or long channel cloaca: the longer the common
was quite challenging for the rarity of cases being op- channel, the higher the confluence [9,10]. However, we
erated over a long period of time with multiple oper- have found it difficult to apply the proposed cut-off
ating surgeons and evolution of surgical techniques. value of 3 cm to differentiate between the two types
However, we have tried to highlight the difficulties (short or long) [11] for the following reasons:
that we have faced during management of these cases,
and the lessons we have learned to overcome these – It does not consider the age variation at time of
difficulties. repair.
– Applying measurements in preoperative
Methods contrast X-ray studies is liable for magnification
The study included cases of cloaca that were managed errors [12].
at our unit between 2003 through 2017. We excluded – In infancy, both the urethra and the common
cases of posterior cloaca from this study as they rep- channel are fine structures showing narrow variation
resent a separate entity (with different plan of man- in length that should be perceived in millimetres.
agement). We retrospectively reviewed patients’ Approximating the measurements to centimetres
records that included clinical presentation, investiga- during endoscopy may be rather subjective and non-
tions, operative data, and follow-up notes. Available reproducible.
data included DICOM images for investigations (con-
trast studies, MRI), recorded videos of endoscopy, and We have tried to overcome some of the above-
operative photos. mentioned limitations concerning the way of measuring
Usually the diagnosis was made in the neonatal the common channel and urethra by using MRI rou-
period based on the external appearance (female with tinely in the preoperative assessment of cases of cloaca.
imperforate anus and a single opening in the peri- However, discussing these diagnostic challenges is be-
neum; Fig. 1). In few cases, the diagnosis was sus- yond our scope in this report and has been studied ex-
pected ante-Nataly (presence of hydrocolpos), while tensively in a previous one [13].
one case presented later during infancy at the age of Anatomically, we stratified cloaca into three types ac-
6 months. Faecal diversion (colostomy) was performed cording to the level of urogenital confluence in relation
initially, while the definitive ‘corrective’ surgery was to the pubic symphysis (Fig. 2). This was defined pre-
delayed after the age of 3 months. Decompression of operatively by MRI [13] and confirmed at cystoscopy. A
the urogenital tract was required in eight cases low confluence (type 1) is defined by being at or below

Fig. 1 The external appearance (female with imperforate anus and a single opening in the perineum) in 3 different cases of cloaca. a Hypoplastic
external genitalia. b, c Abnormal skin protrusion around the common perineal orifice. Note: the external appearance in b and c was associated
with a long common channel (high type)
AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 3 of 7

Fig. 2 New classification proposed for stratifying cloaca anatomically according to the level of urogenital confluence in relation to the
pubic symphysis. The upper row is a diagrammatic representation for the new classification, while the lower row represents real MRI
images for cases of cloaca (mid-sagittal T2WI). P, pubic symphysis; UB, urinary bladder; v, vagina; R, rectum; the transverse white arrow in
the lower row points to the lower end of the vagina that corresponds to the level of urogenital confluence. Type 1 (low confluence),
urogenital confluence at or below the level of the lower border of pubic symphysis (P); type 2 (intermediate), urogenital confluence at
the level of the mid-portion of pubic symphysis; type 3 (high confluence), urogenital confluence at or above the level of the upper
border of pubic symphysis

the level of the lower border of pubic symphysis with a (anaesthetic problem during a lengthy reconstructive
short common channel (about 1 cm). A high confluence procedure).
(type 3) is close to the bladder neck (few mm) and is de- Renal anomalies were common association: solitary
fined by being at or above the level of the upper border kidney in seven (20.6%), pelvic kidney in two, and
of pubic symphysis. Between the low and high types, we urinary tract dilatation (hydroureteronephrosis) in 12
defined an intermediate type (type 2) where the urogeni- cases (37.5%). At follow-up, chronic renal insuffi-
tal confluence was behind the mid-portion of pubic sym- ciency (persistent elevated serum creatinine) was
physis [13]. detected in seven cases (four of them had a single
kidney). Associated spinal anomalies included caudal
Results regression (high spinal cord termination) in one, teth-
The study included 34 cases of cloaca. Mean follow- ered cord in three cases, and another case of
up ranged from 1 to 15 years (mean 6.8 years, median syringomyelia.
6.5 years). Unfortunately, we had two mortalities in During the period of the study, there has been evolu-
this case series: one case died before definitive repair tion of the surgical techniques starting by the posterior
because of complications of associated cardiac anom- sagittal ano-rectovaginoplasty (PSARVP), partial/total
alies, and another case died during operation urogenital sinus mobilization (PUM/TUM), abdominal/
AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 4 of 7

Table 1 Procedures used for anorectoplasty in cases of cloaca, and the related functional outcome (bowel control)
Procedure for anorectoplasty Number of Voluntary bowel Regular laxatives Regular enemas Less than 3 years or
cases control for evacuation lost to follow-up
Perineal ‘sagittal’ anorectoplasty 18 6 (33.3%) 3 (16.6%) 3 (16.6%) 6 (33.3%)
Abdomino-perineal pull-through 10 2 (20%) 2 (20%) 4 (40%) 2 (20%)
[1 caudal regression + 1 tethered cord]
Laparoscopic assisted 4 1 (25%) -- 1 (25%) 2 (50%)
(poor sacrum)
Total number 32 9 (28.1%) 5 (15.6%) 8 (25%) 10 (31.3%)

laparoscopic-assisted rectal pull-through, vaginal pull- cloaca based on the level of urogenital confluence.
through, and other advanced vaginoplasties (ileo/colo- Regarding the operative technique, urinary incontin-
vaginoplasty). The evolution of the surgical technique ence was high following vaginal pull-through and
also included staging of the corrective surgery in cer- ileo/colo-vaginoplasties (75% and 50% respectively)
tain cases to decrease the surgical stress and hazards of compared with other perineal approaches (Table 2).
lengthy reconstructive operations in infancy. This has Disorders of bowel control were managed either by
been encouraged after the introduction of laparoscopy, laxatives or enemas through bowel management pro-
where the anorectoplasty is performed first, to be gram at the incontinence clinic (Table 1). Urinary incon-
followed by the repair of the common urogenital sinus tinence was often more distressing and required
at a second stage. Tables 1 and 2 summarize the recon- different surgical procedures: Mitrofanoff procedure for
structive procedures performed during the period of clean intermittent catheterization (3 cases), bladder aug-
the study and their related functional outcome at mentation (1 case), and continent urinary diversion (3
follow-up. Figures 3, 4, 5, and 6) illustrate the examples cases).
of the different surgical approaches used for the repair
of common urogenital sinus abnormality depending on Discussion
the level of the urogenital confluence (low, intermedi- Persistent cloaca comprises two principle abnormal-
ate, and high confluence). ities namely the anorectal anomaly and the common
The prognosis for urinary continence was excellent urogenital sinus, which are not necessarily affected to
in low confluence (type 1) cloaca; all except one (with the same extent in the same subject [10]. A low uro-
caudal regression) were continent (spontaneous void- genital confluence may be associated with a high rec-
ing). On the other hand, urinary incontinence (usually tum [9] and vice versa. Therefore, it may be more
in the form of poor evacuation of the bladder) was practical to discuss (plan) the management of each
common among type 3 (high confluence) cloaca. component separately.
Urinary incontinence was 62% among type 3 com- In contrast to the urogenital abnormality, the man-
pared with 14% and 10% among type 2 and type 1, agement of the anorectal component of cloaca is usu-
respectively. Table 3 summarizes the functional out- ally more straightforward. Mobilization of the rectum
come (urinary continence) in relation to the type of is relatively easier based on its robust submucosal

Table 2 Procedures used for correction of common urogenital sinus in cases of cloaca and the related functional outcome (urinary
control)
Procedure Number Voiding spontaneously Urinary incontinence Less than 3 years or lost to follow-up
Interoitoplasty 6 2 (33.3%) 1 (16.6%) 3 (50%)
(caudal regression)
PUM 9 7 (77.8%) -- 2 (22.2%)
TUM 7 1 (14.3%) 2 (28.6%) 4 (57.1%)
PSARVP 2 1 (50%) -- 1 (50%)
Vaginal pull-through 4 1 (25%) 3 (75%) --
Ileo/colo-vaginoplasty 4 2 (50%) 2 (50%) --
(1 poor sacrum + 1 tethered cord)
Total 32 14 (43.7%) 8 (25%) 10 (31.3%)
PUM partial urogenital sinus mobilization, TUM total urogenital sinus mobilization, PSARVP posterior sagittal anorectovagino-plasty
AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 5 of 7

Fig. 3 Type 1 (low urogenital confluence) cloaca. The rectum (R) is separated and mobilized from the urogenital tract. A urinary catheter is seen
inside the urethra at the bottom (patient is in prone position). a Septated vagina (arrow) located between rectum and urethra. b Partial
mobilization of the vagina by limited dissection on dorsal and lateral aspects without extending the dissection to the urethra; note: the vaginal
septum has been divided

blood supply. A perineal (posterior sagittal) approach At both ends of the spectrum, the management of
is usually sufficient for the mobilization of the rectum persistent urogenital sinus appears to be less contro-
reaching below the mid-sacral piece (S3); otherwise, versial. A low confluence is managed by PUM (or
abdominal or laparoscopic assistance may turn to be even just introitoplasty) with excellent functional and
necessary [10]. On the other hand, vaginal reconstruc- cosmetic outcome [9, 10]. On the other end of the
tion is often the most challenging step in cloaca [3]. spectrum, a high confluence is managed by keeping
This is because of the difficulty frequently encoun- the common channel to act as a urethra, while the
tered during vaginal mobilization due to its intimate vagina is separated and brought down to the peri-
adherence to the urethra and unreliable submucosal neum (if its length allows for a direct vaginal pull-
blood supply [3, 9, 10, 14]. through); otherwise, a piece of bowel is used to

Fig. 4 Type 1 (low urogenital confluence) cloaca. Partial urogenital sinus mobilization with the patient in the supine position (note: the
anorectoplasty was done at a previous separate stage): a, b Dissection and mobilization of the common urogenital sinus. c Suturing the vagina
and urethra to the vestibule after excision of the short common sinus
AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 6 of 7

Fig. 5 Type 2 (intermediate urogenital confluence) cloaca. Total urogenital sinus mobilization (TUM procedure): a With the patient in the prone
position, dissection, and mobilization of the common urogenital sinus (UGS). b The rectum (R) is separated and mobilized from the urogenital
tract, incision through the mobilized urogenital sinus (UGS) to expose the vaginal introitus. c The vagina (V) is seen with a longitudinal septum,
and a catheter is seen within the urethra (U)

traverse the gap down to the perineum (ileo/colo-va- urogenital sinus mobilization is more liable for tissue is-
ginoplasty) [10]. chaemia especially with a combined abdomino-perineal
The separation of the vagina from the urinary tract dissection [8], and still its long-term effect on the con-
seems to be most difficult in the intermediate types of tinence mechanism is questionable [3, 16, 17].
cloaca. Separation is difficult from below (perineal pos- In this report, we presented an overview on the surgi-
terior sagittal approach) as well as from the abdomen cal management of a rare disease in paediatric surgery.
[3]. The concept of urogenital sinus mobilization has The report also included the outcome of the different
been proposed to overcome this difficult separation [7]; surgical procedures, while referring to a new proposal
however, the results are not always reproducible [9, 10, for stratifying cloaca [13]. We did not assess gynaeco-
15]. Partial urogenital sinus mobilization is not suitable logical and sexual aspects as these will need longer
for all cases and may end with a high vagina. Total follow-up and involvement of other specialities as well.

Fig. 6 Type 3 (high urogenital confluence) cloaca. The patient was initially managed in the neonatal period by a colostomy and a vaginostomy
(black arrow points to site of vaginostomy on the skin). Vaginal pull-through: a the vaginostomy (V) is detached from the abdominal wall (UB,
urinary bladder). b The vagina is separated from the back of the bladder and reconstructed to form a tube that is ready for a
pull-through procedure
AbouZeid et al. Annals of Pediatric Surgery (2020) 16:3 Page 7 of 7

Table 3 Correlating the functional outcome (urinary control) to the type of cloaca based on the level of urogenital confluence
Urogenital confluence Number Voiding spontaneously Urinary incontinence Less than 3 years or lost to follow-up
Low confluence (type 1) 10 5 (50%) 1 (10%) 4 (40%)
(caudal regression)
Intermediate confluence (type 2) 14 7 (50%) 2 (14.3%) 5 (35.7%)
High confluence (type 3) 8 2 (25%) 5 (62.5%) 1 (12.5%)
(1 poor sacrum + 1 tethered cord)
Total 32 14 (43.7%) 8 (25%) 10 (31.3%)

Another limitation was the subjective evaluation for con- Competing interests
tinence, as objective assessment scores were not avail- The authors declare that they have no competing interest.

able. We believe the outcome is still less satisfactory Received: 1 October 2019 Accepted: 12 November 2019
even in highly specialized centres all over the world [1].
The great diversity and complexity of the cloacal
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Ethics approval and consent to participate anomalies: a necessity or an overdoing? J Genital Surg 2019; DOI: https://
A written consent was taken before operation in all cases. Owing to the doi.org/10.21608/JGS.2019.7646.1015
retrospective nature of the study, an IRB number was not required, and the
study was approved through expedited review by the scientific/ethical
committee of the Surgery Department (Faculty of Medicine, Ain-Shams
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