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13. 2 Mal es
193
delicate and selective ligation of mesenteric vessels, preserving the necessary arcades in order to
preserve the blood supply of the distal rectum.
Laparoscopically, this maneuver is done using
cautery, which is a less than optimal way to
divide vessels, because the extension of the burn
is not controlled accurately and may damage collateral vessels necessary to preserve the blood
supply of the distal rectum. In addition, some of
these patients have a very dilated rectum that
requires tapering. We found diffi cult to perform
the tapering of the bowel laparoscopically, and
therefore we have to open the abdomen. It is
important to remember that unfortunately, these
groups of patients (recto-bladder neck fi stula)
have a very poor functional prognosis. Only
20–25 % of our patients in this category have voluntary bowel movements, and about half of those
suffer from soiling. These patients are born with
serious congenital defi ciencies in their sphincteric mechanism and frequently are associated to
very poor sacra.
Ninety-three cases of our literature review
belong to this category of recto-bladder neck fi stula. We believe that all pediatric surgeons agree
that this malformation represents an excellent
indication for a laparoscopic approach.
Perineal fi stulas are malformations that can
be repaired with an operation that takes less
than 45 min with 100 % chance of bowel control. Therefore, we do not see the reason to
attempt a less invasive procedure than the one
that we do at present time. Yet, we found two
publications that included perineal fi stula
repaired laparoscopically [
2 , 12 ].
Imperforate anus with no fi stula is a malformation in which the rectum is blind; it has no
communication with the urinary tract. Half of
these patients have Down syndrome. Our experience shows that this is a benign condition if we
consider the good functional prognosis. Ninety
percent of our patients without Down syndrome
have bowel control, and 70 % of our Down
syndrome patients also have voluntary bowel
movements.
We found that 17 patients (from our literature
review) with this defect underwent a laparoscopic
repair [ 2 , 7 , 10 , 17 , 22 , 23 , 25 , 34 , 37 ]. We believe
that patients with anorectal malformation without
fi stula should not be approached laparoscopically. The rectum is located very close to the
perineum, and therefore it requires a minimal dissection to be mobilized. A laparoscopic approach
implies in these cases a full dissection of the rectum, which is actually unnecessary. In our series,
the operation takes 2 h, the patients have minimal
pain, the blood loss is negligible, and the patient
can be discharged 24 or 48 h after the operation.
Rectourethral bulbar fi stula is fortunately
the most common defect that we see in male
patients. The rectum is located approximately
2 cm above the skin and connects to the bulbar
urethra at the same level of those cases of imperforate anus with no fi stula. Eighty-fi ve percent
of our patients with normal sacrum have bowel
control, and 94 % of them have urinary control.
The operation takes approximately 2½ h, and the
patients eat the same day, have minimal pain, and
can be discharged the next day. We fi nd it very
diffi cult to have a less invasive procedure than
the one that we perform. Again, trying to repair
these malformations laparoscopically represents
a maximally invasive approach since the entire
rectum has to be dissected from above. When
these malformations are repaired posterior sagittally, we only dissect the distal part of the rectum
as much as necessary to bring the rectum down;
we do not have to go all the way to the peritoneum. As a consequence of attempting the repair
of this malformation laparoscopically, a signifi cant number of patients suffered from recurrent
rectourethral fi stula or a large persistent posterior
urethral diverticulum which represents a piece
of rectum left attached to the urethra [ 53 , 54 ]
(Animation 13.2 ) (Fig. 13.1 ). We believe that
laparoscopically the surgeons found it impossible to reach the bulbar fi stula site and amputated the rectum leaving a piece of rectum that
later gave signifi cant symptoms. We believe that
the laparoscopic approach of this malformation
is contraindicated. Unfortunately, many surgeons
keep trying [ 5 , 6 , 11 – 13 , 17 , 24 – 26 , 30 , 34 , 35 ,
37 – 39 ].
Rectourethral prostatic fi stula is the second
most common malformation that we have seen in
male patients. For us, this malformation
194
a b
Fig. 13.1 Posterior urethral diverticulum. ( a ) Diagram. ( b ) MRI study showing a giant posterior urethral
diverticulum
13 Minimally Invasive Approach to Anorectal Malformations
represents a source of controversy concerning the
best way to approach it. The posterior sagittal
approach in these malformations renders a 66 %
chance of bowel control by the age of three.
Ninety-two percent of the patients have urinary
control. In most of these patients, the posterior
sagittal dissection of the rectum requires to open
the peritoneum. In other words, the dissection is
a complete one, due to the fact that the rectum is
located high in the patient’s pelvis. If a surgeon is
particularly well trained laparoscopically and
prefer to approach this type of malformation laparoscopically, rather than posterior sagittally, we
do not see a real difference between going one
way or the other. In addition, some of the rectoprostatic fi stulas are located a little higher than
others closer to the bladder neck; in those cases,
we have seen complications when inexperienced
surgeons try to repair this malformation via posterior sagittal. Perhaps, if they have good training
in laparoscopic techniques, it will be better for
them to approach those malformations that way.
We were happy to fi nd in our literature review
that 185 out of 357 male cases operated laparoscopically had this kind of defect.
Unfortunately, we found in our literature
review that many surgeons still use the old terminology that we consider inadequate and misleading. They still refer to “high, intermediate, and
low” malformations. Sixteen of the published
cases received the nonspecifi c diagnosis of
“rectourethral fi stula” [ 7 , 10 , 13 , 22 ]. Operating a
male baby with an anorectal malformation
without knowing the detailed anatomy represents
a potential misadventure with serious consequences for the patient.
13.3 Females
Anorectal malformations in females are divided
into the following categories:
• Perineal fi stula
• Vestibular fi stula
• Cloacas
• Complex malformations
Perineal fi stulas in females are repaired with a
minimal posterior sagittal operation that takes
less than an hour; the patients have minimal pain,
100 % of them have bowel control, and therefore
we do not see a justifi cation to do a maximally
invasive operation dissecting the entire rectum
through the abdomen to repair these malformations. Yet, as previously mentioned, some cases
are occasionally done laparoscopically.
Vestibular fi stulas are by far the most common
defects in female patients with anorectal malformations. In our series, 95 % of these patients with
a normal sacrum have bowel control. The operation to repair this defect takes us approximately
2½ h. The patients can eat the same day when
operated with a colostomy and can be discharged
13. 3 Females
195
the next day after surgery. The most important
anatomic feature in these patients is the common
wall existing between the rectum and the vagina
(see Chap. 15 ). The real challenge in the repair of
these malformations is to make two walls out of
one, preserving intact the posterior vaginal wall
and the anterior rectal wall. It is diffi cult for us to
imagine why somebody wants to dissect the
entire rectum through the abdomen, in order to
repair these malformations and still claim that it
is less invasive [ 9 , 17 , 36 , 37 ]. In some of those
patients in whom the surgeons attempted a laparoscopic repair, actually, they left a piece of rectum attached to the vestibule and mobilized more
colon from above, which is against the basic principles of the repair of anorectal malformations.
Cloacal malformations are the second most
common anorectal malformations seen by us in
females. The repair of a cloaca requires separation of the rectum from the urinary tract followed
by the mobilization and repair of the urethra and
vagina. For that, most of the time we use a maneuver called total urogenital mobilization. There are
reports of laparoscopic attempted repairs of cloacas [ 7 , 9 , 10 , 17 , 23 , 29 ]. The authors actually
only repaired the rectal component of the malformation, and then they performed a posterior sagittal approach from below in order to repair the
urogenital component of the malformation. In
other cases the surgeons repaired the rectal component and left the urogenital tract intact! In other
words, we cannot say that the laparoscopic
approach has been used to repair cloacas, but,
rather, we should say that it has been used to
repair the rectal component of cloacal malformations. Yet, in our experience, in over 500 cloacas,
the rectum can be reached posterior sagittally in
the majority of cases; therefore, we do not see a
reason to try to go through the peritoneal cavity to
repair a malformation that can be repaired from
below. Again, in theory, laparoscopy could be
helpful to mobilize a rectum that can only be
mobilized through the abdomen. Yet, the patient
still will require a posterior sagittal approach anyway to repair the urogenital sinus.
Laparoscopy can also be useful instead of a laparotomy for the examination of the intra- abdominal
anatomy in complex anorectal malformations.
We are obviously biased against the laparoscopic approach of cases with rectourethral bulbar fi stula because of the cases that we see at our
center, suffering from a complication consecutive
to the laparoscopic approach, including urethral
injuries and posterior urethral diverticula
[ 53 , 54 ].
We are aware of the fact that these complications are not necessarily a consequence of the
laparoscopy, since we have seen similar complications occurring during the repair of anorectal
malformations with other techniques. However,
these complications occurred mainly in patients
suffering from rectourethral bulbar fi stulas.
In our literature review, we found that 29
patients were operated laparoscopically with the
diagnosis of rectovaginal fi stula [ 2 , 3 , 6 – 10 , 17 ,
23 , 28 , 29 , 34 – 36 ].
We consider that real vaginal fi stula is an
almost nonexistent malformation. In our series
of over 1,000 female patients, we have seen only
seven real rectovaginal fi stula cases (see Chaps.
15 and 16 ). Most of the cases that came to us
with the diagnosis of “rectovaginal fi stula,” in
retrospect, were misdiagnosed cloacas or rectovestibular fi stula. If these authors found 29
vaginal fi stulas out of 45 female cases, we have
reason to suspect that they were not really vaginal fi stulas.
Some authors [ 10 , 17 , 35 ] try to evaluate the
clinical results using an old score that includes
“number of bowel movements” as indicative of
results; “many bowel movements” were considered as bad and “few bowel movements” were
considered “good”!! The number of bowel movements relates to many factors (diet, length of the
colon, infl ammatory factors, degree of dilatation
of the rectosigmoid); none of them related to the
type of repair (laparoscopic or posterior
sagittal).
We were negatively impressed by the fact that
only eight of the papers that we reviewed mentioned the characteristics of the sacrum of the
patients [
5 , 13 , 14 , 17 , 21 , 28 , 30 ]. We think that
it is impossible to discuss functional results in the
treatment of anorectal malformations without
specifying the characteristics of the sacrum of the
patients. The presence or absence of tethered
196
13 Minimally Invasive Approach to Anorectal Malformations
cords was only mentioned by fi ve authors [ 2 , 4 ,
17 , 28 , 40 ]. The presence of this anomaly is rec-
ognized as a negative prognostic factor, mainly
for the urinary function.
One of the rationales to justify the laparoscopic approach in patients with anorectal malformations is the short length of stay. We were
very surprised to fi nd that the length of stay was
only mentioned in three papers [ 22 , 35 , 41 ], and
the authors reported 5–11 days’ range of length
of stay. In our series, 90 % of our male cases did
not require a laparotomy, and they remained in
the hospital 48 h postoperatively. In females,
with the exception of complex cloacas that
required a laparotomy, the length of stay was
similar to the males.
Ten publications [ 6 , 10 , 17 , 22 , 32 – 35 , 37 , 41 ]
compared the functional results obtained with
laparoscopy and the traditional PSARP (posterior
sagittal anorectoplasty). Most of the authors concluded that the differences found were not signifi cant. Others suggested that the results
obtained with the use of laparoscopy are better
[ 17 , 41 ].
Four of the papers [ 6 , 10 , 22 , 35 ] evaluated
their results using rectal manometry, which we
consider a non-reliable method (see Chap. 25 ).
Some authors mentioned the “potential negative effect of the scarring produced by the posterior sagittal approach.” That is a reasonable
theoretical concern; however, we strongly suggest for those authors and the readers to read our
publication [
55 ] describing our experimental
experience (in dogs), comparing different types
of rectal operations and their effect on bowel control. The posterior sagittal incision did not affect
bowel control. In addition, from the clinical point
of view [ 56 ], we have operated multiple cases
with normal rectum that required a complete
division of the rectum and anus, including the
posterior and anterior sphincter mechanism (what
we call trans-anorectal approach), and bowel
control was not affected. All patients have normal bowel control.
We have not found conclusive evidence that
the laparoscopy is less invasive than the posterior
sagittal approach for the repair of anorectal malformations in patients that do not require a
laparotomy. There is no current evidence of better clinical results. There are a signifi cant number
of cases in which laparoscopy has been used with
no real justifi cation.
We are happy to see that many authors recognize the risk of urethral damage and leaving a
posterior urethral diverticulum and are showing a
high degree of creativity, using sophisticated
technology to avoid these complications [ 12 , 24 ,
30 ]. Others are already limiting the use of lapa-
roscopy to cases with bladder neck or prostatic
fi stula [ 37 ].
We strongly suggest to all surgeons interested
in performing laparoscopic repair of anorectal
malformations to follow the following
guidelines:
• Use an adequate terminology; try to be accu-
rate in the type of malformation treated.
• Refer to the characteristics of the sacrum as
well as the presence or absence of tethered
cord, in order to discuss clinical results.
• Avoid confusing terms such as “high,” “inter-
mediate,” and “low.”
• When discussing cloacal approach laparo-
scopically, be specifi c as to “repair of a clo-
aca” or repair of the “rectal component of the
cloaca.” Be specifi c in saying exactly what
was done with the urogenital component,
which represents the real technical challenge.
We fi rmly believe that minimally invasive
techniques will play an increasing positive role in
the management of many congenital anomalies
including of course anorectal malformations.
However, we must be cautious to avoid negative
results as a consequence of excessive enthusiasm
or the desire of notoriety.
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Rectal Atresia
1 4
Rectal atresia is a very unique malformation that
deserves a special description. It happens in our
experience, in about 1 % of all cases of anorectal malformations. In this defect, the anus seems
to be completely normal, including the quality of
the sphincter and the location of the anal orifi ce.
However, deep inside the anus, just at the junction of
the anal canal with the rectum, there is an atresia or
narrowing (stenosis) (Fig. 14.1 ). Occasionally, we
see atresias or stenosis located at a different level.
The space that separates the dilated blind rectum,
from the anal canal, is represented by a septum that
sometimes is extremely thin and can be perforated,
and other times it is very thick. In some unusual
cases, there is a signifi cant separation between the
blind upper rectum and the lower anal canal.
a
Interestingly, the sphincter mechanism is excellent in most cases. There is one particular malformation similar to this one that is represented by a
stricture or by atresia of the rectum, associated to a
presacral mass and a sacral defect (see Chap. 8 ,
Sect. 8.2 ), which is a completely different type of
defect. The only thing they have in common is the
fact that the rectum is narrow or atretic.
We believe that rectal atresia with normal
sacrum and no presacral mass is unique, because
the sphincter mechanism is normal and also
because these patients do not have the typical
association with all the defects that we see in
other anorectal malformations. As a consequence, the prognosis for these patients is excellent, in terms of bowel control. They have a
b
Fig. 14.1 Rectal Atresia. ( a ) Diagram. ( b ) External appearance
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_14, © Springer International Publishing Switzerland 2015
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202
14 Rectal Atresia
signifi cant tendency to suffer from severe constipation because they are born with a blind, very
dilated rectum. These malformations have been
previously described in the literature [ 1 – 5 ].
Rectal atresia has been traditionally described
in the old textbooks. The baby is born with a
normal- looking anus, and the nurse or the pediatrician tries to pass a thermometer through the
anus and fi nds an obstruction. In fact, part of a
routine examination of every “normal” newborn
is to check the patency of the anus, unless the
baby is already passing meconium.
14.1 Treatment
If one could think in an ideal indication for a posterior sagittal approach, this would be the malformation which seems to be more indicated. The
defect is easily repaired through a posterior sagittal incision. In our initial cases, we simply remove
the septum that separates the upper rectum from
the anal canal and created an end-to-end anastomosis (Figs. 14.2 and 14.3 ). Subsequently, we
found some cases in which the size discrepancy
between the upper blind rectum and the small
anal canal was very severe, and in order to expand
the size of the anal canal, we introduced a techni-
cal modifi cation maneuver [ 5 ] (Fig. 14.4 ). Most
of the patients that we operated on came to us
already with a colostomy in place. Since the
patient has a colostomy, one can perform a distal
colostogram and simultaneously introduce a
metallic dilator in the anal canal to have a lateral
image of the atresia and estimate the distance
between the upper pouch and the anal canal. If
we could make the diagnosis early in an otherwise healthy newborn baby, we would recommend to do the operation without a colostomy.
14.2 Surgical Repair
The patient is placed in the prone position and we
approach the malformation posterior sagittally.
We go through the skin, subcutaneous tissue,
parasagittal fi bers, ischiorectal fossa, and the
entire sphincter mechanism to expose and open
completely the anal canal and the upper blind rectum. One can see in most cases the pectinate line,
at the same location as the atresia (Fig. 14.2a ).
Unfortunately, we still see some of these
patients, previously operated in whom the surgeon considered that the little anal canal was useless and therefore decided to resect it and pulled
down the dilated piece of rectum. That is rather
ab
Fig. 14.2 Repair of a Rectal Atresia. ( a ) Incision, exposed defect, open upper rectum, and anal canal. ( b ) Anastomosis
of the upper rectum to anal canal
14.2 Surgical Repair
ab
203
Fig. 14.3 Diagram showing the repair of rectal atresia. ( a ) Rectum repaired, ( b ) Sagittal view of the fi nished
operation
regrettable, because the anal canal, as we know,
represents the area of sensation that will provide
bowel control to these patients. It is, therefore,
very important to preserve that little anal canal.
Sometimes the size of the anal canal is too small.
For that, we introduced a technical modifi cation
[
5 ], consisting in mobilizing the posterior rectal
wall, down to the skin of the anus (Fig. 14.4 ),
enlarging the circumference of the anus. We realize that by doing that, the posterior aspect of the
anus will no longer be a real anal canal, but rather
a rectal wall. However we manage to preserve
most of the circumference of the original anal
canal, which will provide enough sensation to
have bowel control. We must keep in mind that
after we fi nish this procedure, the anastomosis
that we created between the upper dilated rectum
and the anal canal is going to be permanently collapsed by the effect of the sphincter mechanism
that keeps the anal canal closed all the time,
except during defecation; therefore, these babies
must be subjected to the same protocol of anal
dilatations that we already described.
Some surgeons [ 4 ] went as far as to perform a
“laparoscopic transanal approach” to repair this
malformation. To demonstrate that something
can be done does not mean that it must be done.
We cannot justify to change a limited, painless,
bloodless, quick, minimally invasive, nonlaparoscopic procedure for a laparoscopic invasive operation that includes an unnecessary total
rectal dissection .
Our experience includes 11 cases and has
been previously published [ 5 ] .