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346
21 Operations for the Administration of Antegrade Enemas
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24. Kaye JD, Jafri SM, Cuda SP, Kalisvaart JF, Cerwinka
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25. Mitrofanoff P (1980) Trans-appendicular continent
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Stevens PS, Mesrobian HG (1996) The Malone antegrade colonic enema enhances the quality of life in
children undergoing urological incontinence procedures. J Urol 155(4):1416–1418
27. Mor Y, Quinn FM, Carr B, Mouriquand PD, Duffy
PG, Ransley PG (1997) Combined Mitrofanoff and
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29. English SF, Pisters LL, McGuire EJ (1998) The use
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31. Bau MO, Younes S, Aupy A, Bernuy M, Rouffet MJ,
Yepremian D, Lottmann HB (2001) The Malone
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urological incontinence procedures: evaluation from
patient point of view. J Urol 165(6 Pt 2):2399–2403
32. Kajbafzadeh AM, Chubak N (2001) Simultaneous
Malone antegrade continent enema and Mitrofanoff
principle using the divided appendix: report of a
new technique for prevention of stoma complications.
J Urol 165(6 Pt 2):2404–2409
33. Clark T, Pope JC 4th, Adams mC, Wells N, Brock JW 3rd
(2002) Factors that infl uence outcomes of the Mitrofanoff
and Malone antegrade continence enema reconstructive
procedures in children. J Urol 168(4 Pt 1):1537–1540
34. Boemers T, Schimke C, Ardelean M, Ludwikowski B
(2005) Evaluation of urinary and faecal continent stomas. J Pediatr Urol 1(2):85–88. doi:
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35. Hoy NY, Metcalfe P, Kiddoo DA (2013) Outcomes
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36. Yerkes EB, Cain MP, King S, Brei T, Kaefer M,
Casale AJ, Rink RC (2003) The Malone antegrade
continence enema procedure: quality of life and family perspective. J Urol 169(1):320–323
37. Herndon CD, Rink RC, Cain MP, Lerner M, Kaefer
M, Yerkes E, Casale AJ (2004) In situ Malone antegrade continence enema in 127 patients: a 6-year
experience. J Urol 172(4 Pt 2):1689–1691
38. Bani-Hani AH, Cain MP, Kaefer M, Meldrum KK,
King S, Johnson CS, Rink RC (2008) The Malone
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39. Bani-Hani AH, Cain MP, King S, Rink RC (2008) Tap
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discussion 1760
40. VanderBrink BA, Cain MP, Kaefer M, Meldrum KK,
Misseri R, Rink RC (2013) Outcomes following
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10.1016/j.jpedsurg.2013.05.010
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41. Koyle MA, Kaji DM, Duque M, Wild J, Galansky SH
(1995) The Malone antegrade continence enema for
neurogenic and structural fecal incontinence and constipation. J Urol 154(2 Pt 2):759–761
42. Stock JA, Hanna MK (1996) Appendiceal cecoplication: a modifi cation of the Malone antegrade colonic
enema procedure. Tech Urol 2(1):40–42
43. Wilcox DT, Kiely EM (1998) The Malone (antegrade
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51. Hirst GR, Arumugam PJ, Watkins AJ, Mackey P,
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Reoperations
2 2
22.1 Introduction
Anorectal malformations are represented by a
wide spectrum of defects. On the good side of
the spectrum, that includes, malformations that
can be repaired with a relatively easy technique
and can obtain excellent functional results, making the patient basically a normal individual that
can enjoy a normal life. On the other hand, on the
“bad side” of the spectrum, one can fi nd complex
defects, associated to very signifi cant anatomic
defi ciencies that make the functional prognosis rather somber. In these serious and complex
defects, it is almost impossible to restore normal bowel function, urinary function, or sexual function. Many of these patients are born
without the necessary nerves and muscles that
represent the mechanism of bowel and urinary
control. In the middle of these two extremes of
the spectrum, there are many types of malformation with variable anatomic setups and different
prognoses. We look forward to the design and/
or discovery of therapeutic methods that allow
obtaining better functional results for patients
that are currently born in the “bad side” of the
spectrum.
However, it must be considered unacceptable
to see a patient born with a malformation considered benign, in other words, in the “good side” of
the spectrum to receive a technically defi cient
surgical procedure that destroys important structures and mechanisms of bowel and urinary control and ends up with a patient that belongs to the
“bad side” of the spectrum. This is preventable
and totally unacceptable. Unfortunately, it is
something that happens more often than desired.
We do not know if there is a problem related
with our perception, the fact that we feel that
more morbidity occurs during the attempt to
repair anorectal malformations than the one that
occurs in other congenital defects. The fact is that
at our center, from a total of 2,032 cases of anorectal malformations operated by the authors over
the previous 30 years, 478 are reoperations. One
hundred and fi fty-three of those were done in an
attempt to regain bowel control. Three hundred
and twenty-fi ve were done in cases that underwent
an attempted failed repair at another hospital and
suffered serious complications. We are aware of
the fact that we are a referral center, and therefore
the proportion of cases that we see, that had failed
attempted repairs, is not representative of the proportion seen in the general population. Yet, we
still consider this number extremely high. From
a total of 909 male cases we had operated, 223
are reoperations, 93 of them were performed in an
attempt to improve bowel control, and 130 were
done to repair sequelae from injuries provoked by
previous surgical misadventures. Between 1,123
female cases, 60 were reoperated in an attempt to
regain bowel control and 195 for other reasons.
This takes us into a controversial issue of
“reproducible” versus “non-reproducible” operations in surgery. We are convinced that some
operations are highly reproducible, such as pyloromyotomy, hernia repair, cholecystectomy, and
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_22, © Springer International Publishing Switzerland 2015
349
350
22 Reoperations
other similar procedures. Those operations have
demonstrated the effi ciency and effi cacy through
many years, performed by all kinds of surgeons.
Unfortunately, we believe that the operations
designed to repair anorectal malformations are
not very reproducible. This means that to repair
anorectal malformations successfully, the surgeon must be familiar with an anatomic territory
that was not well known until recently. For that,
he must be open-minded enough to forget many
of the traditional anatomic concepts and become
familiar with the anatomic spectrum seen in this
complex part of the body, in patients with anorectal malformations. In addition, the surgeon in
charge of repairing these defects must be very
meticulous, careful, and delicate. There are, we
are convinced, many surgeons who are very good
to repair certain types of defects, but not for others. Many surgeons have a tendency to be in a
hurry and do not have tolerance or patience to
slow down when dealing with delicate tissues
and complex anatomic arrangements. Anorectal
malformation patients are born with a rectum,
sometimes a vagina, and sometimes a urethra
located in rather unusual places. These three
structures are frequently abnormally attached one
to another, and they share common walls without
a plane of dissection. The separation of these
structures is a mandatory step, in order to reconstruct the anatomy of these unfortunate patients.
The separation of those structures represents a
technical challenge. Until now, the only way to
do it is observing a very meticulous, delicate dissection. This mandatory step (the separation of
the structures) has not been facilitated by new
technologic advances, such as laparoscopic
approach or robotic approach. In fact, we have
seen more complications when such separation
has been attempted through a laparoscope. We
are sure that new advances in the technology will
result in fi ner, most likely digital instruments that
will allow to perform complex reconstructions
with minimally invasive type of procedures.
Professors of pediatric surgery, all over the
world, are responsible for the surgical training of
the young generation of pediatric surgeons that
will be operating on thousands of babies born
with these defects. We look forward to the time
when all patients born with “benign” malformations undergo impeccable operations to repair the
defect and enjoy a normal life.
Perhaps, part of the problem to explain why
these operations are not as reproducible as others
is the fact that the wide spectrum of anatomic
variations found in these patients is not well
known by the majority of pediatric surgeons. In
addition, during the adult general surgical training of most surgeons, they learn the traditional
anatomic concepts from adult general surgical
textbooks, which are not representative of the
anatomic variations seen in patients with anorectal malformations.
22.2 Reoperations to Improve
Bowel Control
During our early experience with the posterior
sagittal approach [ 1 ], we were extremely opti-
mistic and believed that we would be able to
restore the anatomy of many patients that have
been operated with old techniques and suffered
from fecal incontinence. We assumed that since
most of the operations used before 1980 were
performed, at least in part, blindly, one would
expect that the surgeons may have positioned the
rectum in the wrong location and not in the center
of the sphincter mechanism. Based on that, we
thought that by repositioning the rectum within
the limits of the sphincter, the patient may gain
bowel control. Consequently, we accepted to surgically reexplore all patients who underwent a
previous repair and suffered from fecal incontinence. Our initial experience included eight
patients [
common mislocation of the rectum was an anterior one (Fig. 22.1 ). These mislocations could be
total or partial. The second most common
mislocation of the rectum was a posterior one
(Fig. 22.2 ). In general, the cases of posterior mis-
located rectum were the patients operated at a
time before the Stephens contribution, when surgeons believed that the rectum should be pulled
down as close as possible to the sacrum to avoid
damage to the urinary tract. The cases in whom
the rectum was located too anterior, in general,
1 ]. In these, we found that the most
22.2 Reoperations to Improve Bowel Control
a
c
351
b
Fig. 22.1 Anterior mislocation of the rectum. ( a ) Diagram. ( b ) Perineum. ( c ) MRI. S sphincter, A anal opening
belonged to the era when Dr. Stephens suggested
that the rectum should be pulled down, as close
as possible to the rectum, in order to preserve the
“puborectalis sling” and give the patient the possibility of bowel control.
A third type of rectal mislocation was a lateral
one that happened to be very unusual (Fig. 22.3 ).
The operation consists in opening posterior
sagittally (Fig. 22.4 ). Multiple silk stitches are
placed at the mucocutaneous junction of the anal
opening in order to apply uniform traction to
facilitate this redissection of the rectum. The
incision is performed exactly in the midline,
dividing all sphincter mechanisms found, posterior to the rectal wall. The incision continues
until we identify the posterior rectal wall. Many
times, what we really fi nd is the colon. In other
words, the surgeons who performed the fi rst
352
22 Reoperations
a
b
c
Fig. 22.2 Posterior mislocation of the rectum. ( a ) Diagram. ( b ) Perineum. S sphincter, A anal opening. ( c ) MRI
operation resected the rectum and pulled down
colon from inside the abdomen. We must be prepared to be able to identify whether the patient
has a colon or rectum.
Originally, we were concerned and thought
that this type of reoperation could be
extremely diffi cult. Actually, it turned out that
the reoperation was easier to perform than the
primary procedures. Since most of these patients
did not suffer from infections, retractions, and
abscesses (catastrophes), the surgical planes and
the anatomic features were easily recognized. In
addition, it was not necessary to separate the
rectum from the urogenital structures, which is,
22.2 Reoperations to Improve Bowel Control
a
Fig. 22.3 Lateral mislocation of the rectum. ( a ) Diagram. ( b ) Perineum
Fig. 22.4 Opening
incision. ( a ) Diagram. ( b )
Operative
a
b
353
b
as we know, the most important challenge in the
treatment of anorectal malformations. Also, there
was no fi stula, and fi nally, there was not a problem of bowel length, because the bowel was
already connected to the perineum, so it turned
out to be a rather quick and easy procedure.
Once we identify the posterior bowel wall, the
dissection must be extended to the lateral walls of
the bowel and eventually to the distal end at the
skin. The posterior sagittal incision is continued
in a circumferential manner, peripheral to the silk
stitches to mobilize the entire rectum (Fig. 22.5 ).
Once the rectum has been mobilized, the limits of the sphincter are electrically determined
(Fig. 22.6 ). Our fi ndings in this type of operation
include patients who had the rectum completely
mislocated and an intact sphincter mechanism. In
those procedures, it was extremely satisfactory
to mobilize the rectum and place it within the
limits of the sphincter. We fi nished the operation
354
22 Reoperations
Fig. 22.5 Diagram showing the dissection of the rectum
with the impression that we really benefi ted the
patient. Other times, to our dismay, we found
either that the patient was born with no sphincters and the rectum was surrounded by fat tissue
or we found that the sphincter had been destroyed
during the previous operation and the bowel was
surrounded by scar tissue only. In those cases, we
fi nished the procedure, feeling that we did not
help the child.
The rectum then is relocated and placed within
the limits of the sphincter mechanism. The previous location of the rectum is obliterated and
repaired with long-term absorbable sutures. The
rectum is anchored to the sphincter mechanism
as in the primary procedures, and the anoplasty is
done with circumferential multiple, fi ne, longterm, absorbable sutures (Fig. 22.7 ).
The fi rst retrospective review of our experience
with this type of operations showed us that fi ve of
our eight cases had a signifi cant improvement in
terms of bowel control [
1 ]. In other words, they
recovered voluntary bowel movements. We then
analyzed the characteristics of the group of
patients that improved with the operation and
compared with the group that did not improve and
found that, as expected, those patients who
improved were the ones that had a completely
mislocated rectum, a preserved rectum, and a
good sacrum and were born with a malformation
that belongs to the “good side” of the spectrum.
Based on that experience, we changed the indications for this type of procedure, and ever since
that time, we only reoperate, with the expectation
to improve bowel control, on those patients that
have a good sacrum (sacral ratio between 0.6 and
0.8), no evidence of tethered cord, and a completely mislocated rectum with an intact sphincter; they still have their original rectum (was not
resected), and they were born with a malformation that we consider a “benign” type. The results
of those reoperations with the new indications
were not as good as we expected; only about 2/3
of our 77 patients improved signifi cantly in terms
of bowel control [ 2 , 3 ]. Yet, 85 % of them still soil
the underwear signifi cantly.
The review of the history of the reoperations
designed to improve bowel control, in patients
suffering from fecal incontinence, is a demonstration of the ingenuity, imagination, and creativity of surgeons in general. However, the
results have been in general less than optimal.
Those surgeons who believed in the existence
of the puborectalis, soon enough, designed an
operation to recover the “missed puborectalis”
[
4 , 5 ]. Others [ 6 – 10 ] believed that they could
improve bowel control by plicating, tightening,
or releasing the levator mechanism from its posterior attachments. These types of procedures
were known as “levatorplasties.”
A few surgeons embraced the idea of using the
posterior sagittal approach to perform a relocation
of the rectum or to perform a “levatorplasty” [ 11 –
16 ], most of them with encouraging results and
others with bad results [ 12 ]. One author described
an “anterior sagittal approach” to reoperate incontinent patients [ 17 ]. A careful reading of the article
showed that the procedure was actually a posterior
approach performed in lithotomy position.
22.2 Reoperations to Improve Bowel Control
a
355
b
Fig. 22.6 Limits of the sphincter. ( a ) Diagram. L limits of the sphincter. ( b ) Operative view of one side of the incision,
showing the limits of the sphincter and the original anterior mislocation of the anus
a b
Fig. 22.7 Anoplasty. ( a ) Diagram. ( b ) Perineum
356
22 Reoperations
Following the original idea of Pickrell [ 18 ],
many surgeons tried the “gracilis sling” operation, with and without electrical stimulation, with
questionable results [ 19 – 36 ]. The gluteus muscle
was also used to create a voluntary sphincter [ 37 –
41 ] with variable results.
The possibility of using an artifi cial sphincter,
capable of giving bowel control, has always been
in the mind of surgeons [ 42 – 52 ]. It has been tried
only in adult patients. The morbidity of this procedure is high, including infection, rectal stricture, and mechanical failure of the device. In
1988, we implanted in pigs Silastic, hydraulic
devices normally used in the urinary tract (unpublished data). We were able to avoid bowel movements when the cuffs were infl ated, but we were
not able to produce bowel movements when the
device was deactivated. That experience contributes to make us believe that we will not be able to
produce bowel control, unless we fi nd the way to
manipulate the rectosigmoid motility.
In 1975, Hakelius from Sweden suggested
that we could produce bowel control with free
autogenous muscle transplantation [ 53 , 54 ].
Several surgeons followed his idea, with variable
results [ 55 – 59 ].
Surgeons who believed in the existence of the
“internal sphincter” and its importance for bowel
control devised ingenious procedures to create a
structure similar to the “internal sphincter” [ 60 –
63 ]. The lack of long-term results makes us to
suspect that the results have not been good.
Based on the idea that the contraction of the
sphincter mechanism is a response to electric- like
stimuli, transmitted by a nerve, some surgeons
have been trying different modalities of electrical,
magnetic, or temperature-controlled radiofrequency stimulation to produce bowel control. The
reports describe mainly adult patients, and the
results are rather controversial [
The extreme example of the rather simplistic
and naïve idea of the mechanisms of bowel control
is the use of a tampon-like device [ 75 ] or by the
injection of bulking agents in the anus [ 76 , 77 ].
In summary, we believe that a rational
approach to the problem of fecal incontinence
in children must consist in the regulation of
colonic motility in patients with borderline fecal
64 – 74 ].
incontinence. In other words, patients with mild
degree of incontinence may benefi t by the use of
medication and/or diet to slow down the colon,
in cases with tendency to diarrhea and the use
of laxatives in those patients who suffer from
constipation.
In some cases with total fecal incontinence
consecutive to a previously repaired anorectal
malformation, we offer a reoperation to those
patients that have the following characteristics:
A. Completely mislocated rectum
B. Have normal sacrum
C. No evidence of tethered cord
D. Were born with good prognosis type of ano-
rectal malformation (perineal fi stula, vestibular fi stula, bulbar fi stula, and absent fi stula)
In all other cases, or those who did not respond
to our procedure, we offer them our bowel management program (see Chap. 20 ).
22.3 Reoperations Performed
After Failed Attempted
Repair (Catastrophes) Males
We have done approximately 130 cases of male
patients born with an anorectal malformation,
who underwent an attempted failed repair. These
patients suffered from postoperative acquired
rectal atresia, anorectal stenosis, dehiscence,
retractions, abscess, infections, and persistent,
recurrent, or acquired rectourethral fi stulas.
Interestingly, in all these cases, we found that
the common denominator to explain the failed
operation was the lack of a preoperative highpressure distal colostogram or a technically
defi cient one. The surgeons did not have accurate anatomic information, which resulted in a
defi cient surgical technique.
The lack of a high-pressure distal colostogram
frequently induced the surgeons to look for the
rectum in the wrong place, damaging other
important structures, including the urethra, vas
deferens, and seminal vesicles, or provoking
nerve damage that resulted in neurogenic bladder
and/or lack of erections (impotence). The most
common scenario was a patient with anorectal
malformation with a recto-bladder neck fi stula or