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274
a
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
b
c
Fig. 16.70 Posterior cloaca variant. The urogenital sinus is located immediately anterior to the anus, but far away from
the clitoris. ( a ) Diagram of sagittal aspect, ( b ) external appearance of the perineum, ( c ) demonstration of large pubis
by John Duckett and Ballinger. They entitled
their paper “Accessory Phallic Urethra in the
Female Patient,” and actually they described
three cases that are posterior cloacas according
to the pictures that we saw in the paper [ 126 ]. In
1994, Chatterjee published a case described as
clitoromegaly, duplex urethra, and dysplastic
vagina, but the photograph of the patient shows
the 2-orifi ce variant of the posterior cloaca spectrum [ 127 ].
More recently, the term posterior cloaca seemed
to be becoming more popular and we were able to
16.2 Posterior Cloaca and Absent Penis Spectrum
a
c
275
b
Fig. 16.71 Diagrams showing a sagittal view of three different variants of posterior cloaca. ( a ) With hydrocolpos,
( b ) with double vagina and ( c ) with an accessory quasi-atretic urethra opening at the tip of a pseudophallus (clitoris)
fi nd publications referring to this specifi c defect
using the term posterior cloaca [ 128 – 131 ].
as megaureters and hydronephrosis. That should
be treated at birth.
16.2.1.1 Typical Cloaca with a Single
16.2.1 Surgical Repair
Perineal Orifi ce in the Anal
Location
Most of these patients came to us with a colostomy performed at another institution during the
newborn period. Patients born with a posterior
cloaca also may have hydrocolpos with all of the
negative consequences related to this defect such
The patient is placed in the prone position with
the pelvis elevated. A posterior sagittal transanorectal incision is performed. As described in
Chap. 26 , the incision includes the skin, subcuta-
neous tissue, parasagittal fi bers, muscle com-
276
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
plex, posterior rectal wall, anterior rectal wall,
and anterior sphincter mechanism. With the use
of self-retractors, we have beautiful exposure
(Fig. 16.72 ). The urogenital sinus that is
connected to the anterior rectal wall, usually
about 1 or 2 cm from the anal verge, can be seen
as soon as we open the posterior rectal wall.
Multiple 5-0 silk sutures are placed in the circumference of the single orifi ce of the urogenital
sinus, in order to be able to apply uniform traction to facilitate its dissection. The urogenital
sinus is separated from the anterior rectal wall.
The perineum anterior to the anus is also divided
in the midline all the way up to the clitoris. At
this point, we can evaluate and feel if the pubis is
interfering and does not allow us to fi nd space to
place the urethra immediately behind the clitoris
and the vagina behind. If that is the case, in
babies, we can resect more than 50 % of that
very heavy thick cartilage of pubis with needletip cautery, with minimal bleeding. By doing
that, we create enough space to switch forward
and mobilize the urethra and vagina together
(total urogenital mobilization). As previously
described in the Sect.
channel is shorter than 3 cm, the overwhelming
majority of cases can be repaired without opening the abdomen, placing the urethra immediately behind the clitoris, with the vagina behind,
without separating the vagina from the urethra.
Figure 16.72 shows pictures and diagrams of this
procedure.
The urethral opening is sutured with 6-0 longterm absorbable sutures to the tissue immediately
behind the clitoris as described in the repair of
cloacas. The vaginal walls are sutured with interrupted 5-0 long-term absorbable sutures, to the
skin of what is going to be the neolabia. The
divided rectum is already perfectly visible
(Fig. 16.72 ). A meticulous reconstruction is per-
formed, bringing together corresponding portions of the sphincter mechanism anterior to the
anus. The anterior rectal wall and the anal wall
are sutured with two layers of interrupted longterm absorbable sutures. The posterior rectal wall
is reconstructed in the same manner followed by
a meticulous reconstruction of a normal sphincter
mechanism located behind the rectum and anus.
16.1 , if the common
16.2.2 Surgical Repair
of the 2-Perineal-Orifi ce
Variant of the Posterior
Cloacal Spectrum
When patients are born with two orifi ces, one is
the anal opening located in the normal location or
slightly anteriorly and the second one located
anterior to the anus, but far away from the clitoris,
it represents the urogenital sinus (Fig. 16.70a , b ).
In that type of malformation, provided the common channel is not too long, it is conceivable, as
many authors like to say, that we could repair the
urogenital sinus without necessarily dividing the
anorectum. Of course, we agree that if that is feasible, it should be done that way, but also we
believe that we should not compromise the quality
of the repair trying to avoid the opening of the
healthy rectal walls (See Chap.
The urogenital sinus is mobilized as described in
the case of cloacas. When the pubic bone and cartilage represent an obstacle, it should be resected,
part of it, to create the necessary space, and the
reconstruction is done following the same steps
already described.
Our experience with the repair of posterior
cloaca and its variants includes 30 patients [ 132 ].
The results in terms of urinary function, bowel
control, and sexual concerns are very similar to
those found in the repair of cloacas and depend,
like in cloacas, on the length of the common
channel that infl uences mainly the urinary function and the presence, absence, or abnormalities
of the sacrum.
26 , Fig. 26.13a , b ).
16.2.3 Posterior Cloaca
and Absent Penis
The reader must be surprised that we are discussing here a malformation called absent penis in a
chapter dedicated to female patients with posterior cloacal malformations. The reason for this is
that we found interesting striking similarities
between both malformations: posterior cloaca in
females and absent penis in males.
Absent penis through history is a very wellknown malformation. We believe that the defect
16.2 Posterior Cloaca and Absent Penis Spectrum
a
277
b
c
d
Fig. 16.72 Operative pictures of the posterior approach
of a posterior cloaca. ( a ) The rectum has been divided in
the midline, and the urogenital sinus is already mobilized.
( b ) The posterior wall of the urogenital sinus has been
opened. “ V ” is vagina and “ U ” is urethra. ( c ) The urethral
opening is sutured a few millimeters behind the clitoris.
( d ) The lateral walls of the vagina are sutured to the
neolabia
278
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
has been recognized for many years, due to the
fact that it is a very obvious, conspicuous defect. It
is not the purpose of this presentation to review the
historical information related with this defect; we
found references even from the last two centuries.
We included in this review a few references
that we consider important related with this
defect [ 133 – 138 ]. These babies are chromosom-
ally males, have testicles, and are born without a
phallus. The posterior urethra is posteriorly deviated and opens into the anterior rectal wall or
immediately anterior to the anus (Fig. 16.73 ).
Looking carefully to this diagram, one can see
the remarkable similarity with the anatomy of a
posterior cloaca in female patients.
Historically, and occasionally even now, these
babies are raised as females. In other words, they
are subjected to bilateral orchiectomies, the urethra is mobilized to be placed similar to a female
patient, and a neovagina is created [ 139 – 150 ].
There is another very interesting malformation, seen in male patients, that is known with
different names such as “urethral atresia with urethrorectal communication,” “duplication of the
urethra,” “dystopic urethral orifi ce into the anus,”
and “Y-duplication of the male urethra.” All these
names refer to a malformation very similar to the
absent penis, except that the penis is present. In
other words, the posterior urethra is posteriorly
deviated and opens into the anterior rectal wall or
immediately anterior to it; the patient has a very
hypotrophic, usually useless, tiny penile urethra
that opens in the tip of the penis, and that is the
reason why a lot of people call this “double urethra” or “Y urethra” (Fig. 16.74 ). Again, one can
see the striking similarity between this defect and
absent penis and also with the posterior cloaca in
females [ 151 – 154 ].
We have no experience with the treatment of
absent penis. However, we have experience in the
treatment of the variation that we just described,
namely, the posterior urethra deviated to the anterior rectal wall and a quasi-atretic penile urethra
(Fig. 16.74 ). This malformation is repaired by
stages. The fi rst procedure is done with the
patient in prone position, mobilizing the urethra
from the anterior rectal wall or from the very
anterior perineum, forward, to put it in a more
convenient perineal location away from the anus
(Fig. 16.75a ). This operation can be done divid-
ing the anterior rectal wall and the posterior rectal wall or in more simple cases without touching
the rectum. After the operation, basically, the
Fig. 16.73 Diagram showing a sagittal view of an absent
penis. The posterior urethra is connected to the anterior
rectal wall
Fig. 16.74 Diagram showing a sagittal view of a malfor-
mation considered a variant of the absent penis. The posterior urethra is connected to the anterior rectal wall and
the penile urethra is very structured
References
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279
Fig. 16.75 Diagrams showing the repair of the “absent
penis variant.” ( a ) The posterior urethra has been discon-
nected from the anterior rectal wall and switched forward
patient would have the equivalent to a severe perineal hypospadias. The penile urethra is usually
very narrow. Sometimes we see urine coming out
through the tip of the penis, but certainly most of
the urine comes from a wide posterior urethra
originally connected to the anterior rectal wall or
to the perineum. In the subsequent stages, the
penile urethra is reconstructed; the posterior urethra connected to the perineum is switched forward and is connected to the reconstructed penile
urethra (Fig. 16.75b ). This part of the procedure
(urethral reconstruction) must be done by skilled,
dedicated pediatric urologists.
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