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- •Foreword I
- •Foreword II
- •Preface
- •Acknowledgments
- •Contents
- •1: History of the Treatment of Anorectal Malformations
- •1.1 Introduction
- •1.2 The Early Times
- •References
- •2: Basic Anatomy and Physiology of Bowel Control
- •2.1 Internal Sphincter
- •2.2 General Anatomic Principles in Anorectal Malformations
- •2.3 Nerves
- •2.4 Blood Supply
- •2.5 Basic Physiology Principles of Bowel Control
- •References
- •3: Prenatal Diagnosis
- •3.1 Male Fetuses
- •3.1.1 Abnormal Sacrum (Fig. 3.3)
- •3.1.2 Tethered Cord
- •3.1.3 Absent Kidney (Fig. 3.5)
- •3.1.4 Vertebral Anomalies
- •3.1.5 Hydronephrosis (Fig. 3.6)
- •3.2 Female Fetuses
- •3.2.2 Pelvic Cystic Mass
- •3.2.3 Cloacal Exstrophy
- •References
- •4: Neonatal Management
- •4.1 Introduction
- •4.2 Most Common Scenario
- •4.4 Physical Examination
- •4.4.1 Male Patients
- •4.5 Female Babies
- •4.6 Neonatal Management
- •4.7 Cloacal Exstrophy
- •References
- •5: Colostomy
- •5.1 Introduction
- •5.2 Stoma Locations
- •5.3 Ileostomies
- •5.4 To Divert or Not to Divert, That Is the Question
- •5.5 Recommended Types of Colostomies
- •5.5.1 Newborn Babies with Anorectal Malformations
- •5.6 Left Transverse Colostomy
- •5.7 Cecostomies
- •5.8 Creation of a Colostomy
- •5.8.1 Surgical Technique
- •5.9 Colostomy in Cases of Cloaca with Hydrocolpos
- •5.10 Other Types of Colostomies
- •5.11 Colostomy Care
- •5.12 Colostomy Closure
- •5.13 Surgical Technique
- •5.14 Errors and Complications in Colostomies
- •5.16 Prolapse
- •5.17 Surgical Treatment for Prolapse
- •5.18 Malposition of the Stomas
- •References
- •6: Imaging
- •6.1 Introduction
- •6.2 Prenatal Diagnosis
- •6.3 Neonatal Imaging
- •6.4.1 Anatomic Facts and Timing
- •6.5 The Old Invertogram
- •6.6 High-Pressure Distal Colostogram
- •6.7 Technique
- •6.8 Most Common Errors
- •6.9 Not Showing the Coccyx and the Sacrum During the Fluoroscopy Studies
- •6.11 Distal Colostogram in Cloacas
- •6.12 Monitoring Constipation
- •6.13 Radiology During the Bowel Management Program
- •6.14 Monitoring the Urinary Tract
- •References
- •7: Bowel Preparation in Pediatric Colorectal Surgery
- •7.1 Major Procedures
- •7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period
- •7.3 Primary Pull-Through in Newborn Patients with Hirschsprung’s Disease
- •7.4 Patients with Hirschsprung’s Disease with Enterocolitis After the Neonatal Period
- •7.5 Patients with Hirschsprung’s Disease Beyond the Neonatal Period, Without Enterocolitis
- •7.6 Colostomy Closures
- •References
- •8: Recto-perineal Fistula
- •8.2 Associated Defects
- •8.3 Diagnosis
- •8.3.1 Female Patients
- •8.3.2 Male Patients
- •8.4 Management
- •8.5 Dilatations
- •8.6 Cutback Operation
- •8.7 Minimal Posterior Sagittal Anoplasty
- •8.7.1 Male Patients
- •8.7.2 Surgical Technique
- •8.7.3 Female Patients
- •8.8 Postoperative Care
- •References
- •9: Rectourethral Bulbar Fistula
- •Introduction
- •Associated Defects
- •Posterior Sagittal Anorectoplasty
- •Surgical Technique
- •Functional Results
- •References
- •10: Rectourethral Prostatic Fistula
- •10.1 Introduction
- •10.2 Associated Defects
- •10.3 Surgical Repair
- •References
- •11: Recto-bladder Neck Fistula
- •11.2 Associated Defects
- •11.2.1 Sacral Defects
- •11.2.2 Spinal-Associated Defects
- •11.2.3 Urologic-Associated Defects
- •11.2.5 Neurosurgical-Associated Defects
- •11.2.6 Cardiovascular-Associated Defects
- •11.2.7 Other Associated Defects
- •11.3 Diagnosis
- •11.4 Treatment
- •11.4.1 Colostomy
- •11.4.2 Main Repair
- •11.4.3 Laparotomy
- •11.4.4 Laparoscopy
- •11.5 Special Problems
- •11.6 Functional Results
- •11.6.1 Fecal Control
- •11.6.2 Urinary Control
- •References
- •12: Imperforate Anus Without Fistula in Males and Females
- •12.1 Introduction
- •12.2 Anatomic Characteristics
- •12.3 Main Repair
- •12.4 Function and Results
- •References
- •13: Minimally Invasive Approach to Anorectal Malformations
- •13.1 Introduction
- •13.2 Males
- •13.3 Females
- •References
- •14: Rectal Atresia
- •14.1 Treatment
- •14.2 Surgical Repair
- •References
- •15: Rectovestibular Fistula
- •15.2 Associated Defects
- •15.2.1 Sacral
- •15.2.2 Spinal
- •15.2.3 Urologic
- •15.2.4 Gynecologic
- •15.2.5 Gastrointestinal
- •15.2.6 Tethered Cord
- •15.2.7 Cardiovascular
- •15.3 Diagnosis
- •15.4 Treatment
- •15.4.1 Colostomy or No Colostomy
- •15.5 Main Repair (Animation 15.1)
- •15.6 Complications
- •15.7 Functional Results
- •15.9 Surgical Technique
- •References
- •16: Cloaca, Posterior Cloaca and Absent Penis Spectrum
- •16.1 Cloaca
- •16.1.1.1 Associated Defects
- •16.1.1.2 Goals of Treatment
- •16.1.1.3 Neonatal Management
- •16.1.1.4 Main Repair
- •Cloacas with a Common Channel of Less Than 1 cm
- •Cloacas with a 1–3 cm Common Channel
- •Cloacas with a 3- to 5-cm Common Channel (Animation 16.3)
- •Carving of the Pubic Cartilage Maneuver
- •Separations of Vagina(s) from the Urinary Tract (Animation 16.3)
- •Vaginal Switch
- •Vaginal Replacement
- •Vaginal Replacement with Rectum
- •Vaginal Replacement with Colon
- •Vaginal Replacement with Small Bowel
- •Cloacas with Extremely Long Common Channels
- •16.1.1.5 Postoperative Care
- •16.1.2 Urologic Concerns
- •16.1.3 Gynecologic Concerns
- •16.1.4 Reoperations
- •16.1.4.1 Persistent Urogenital Sinus
- •16.1.4.3 Acquired Urethral Atresia or Stricture
- •16.1.4.4 Sequelae from Catastrophic Complications
- •16.1.5 Transpubic Approach
- •16.2 Posterior Cloaca and Absent Penis Spectrum
- •16.2.1 Surgical Repair
- •References
- •17: Cloacal Exstrophy and Covered Cloacal Exstrophy
- •17.1 Neonatal Approach
- •17.2 Pull-Through or “Permanent Stoma”
- •17.3 Covered Cloacal Exstrophy
- •References
- •18: General Principles for the Postoperative Management of Patients with Anorectal Malformations
- •18.1 General Care
- •18.2 Local Care
- •18.3 Anal Dilatations
- •18.4 Avoiding Constipation
- •18.5 Toilet Training
- •19: Postoperative Evaluation
- •References
- •20: Bowel Management for the Treatment of Fecal Incontinence
- •20.1 Introduction
- •20.2 Goals of the Bowel Management Program
- •20.3 Evaluation of the Patient for Bowel Management
- •20.5 Laxative Trial
- •20.6 About Our Program
- •20.7 Content of the Enema
- •20.8 Rationale to Change the Type of Enema
- •20.9 Bowel Management for the Treatment of Severe Diaper Rash
- •20.10 Bowel Management Through a Stoma
- •References
- •21: Operations for the Administration of Antegrade Enemas
- •21.1 Introduction
- •21.2 Our Preferred Technique
- •21.4 Continent Neo-appendicostomy
- •References
- •22: Reoperations
- •22.1 Introduction
- •22.4.1 Recurrent Fistula (17 Cases)
- •22.4.2 Persistent Rectourethral Fistula (24 Cases)
- •22.4.3 Acquired Fistula (9 Cases)
- •22.5 Posterior Urethral Diverticulum (32 Cases)
- •22.6 Acquired Rectal Atresia or Stenosis (83 Cases)
- •22.7 Presacral Masses
- •22.9 Prolapse
- •References
- •23: Urologic Problems in Anorectal Malformations
- •23.1 Introduction
- •23.2 Neonatal Approach
- •23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
- •23.4.1 Absent Kidney
- •23.4.2 Urethral Problems
- •23.6 Hypospadias
- •23.7 Ectopic Ureters in Males
- •23.8 Ectopic Ureters in Females
- •23.9 Ectopic Vas Deferens
- •23.10 Ectopic Verumontanum
- •23.11 Megalourethra
- •23.13 Neurogenic Bladder
- •23.14 Postoperative Problems
- •23.16 Sexual Problems
- •23.17 Tethered Cord
- •23.18 The Ultimate Concern, Kidney Function
- •References
- •24: Hirschsprung’s Disease
- •24.1 Introduction
- •24.2 Historical Review
- •24.3 Incidence, Inheritance, and Associated Anomalies
- •24.4 Pathogenesis
- •24.5 Genetics
- •24.6 Clinical Manifestations and Differential Diagnosis
- •24.7 Histologic Diagnosis
- •24.8 Differential Diagnosis
- •24.9 Early Management
- •24.10 Surgical Treatment
- •24.10.1 The Authors’ Approach
- •24.11 Total Colonic Aganglionosis
- •24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
- •24.13.1.1 Fecal Incontinence
- •24.13.2 Non-preventable Complications
- •24.13.3 Partially Preventable Complications
- •References
- •25: Idiopathic Constipation and Other Motility Disorders
- •25.2 Incidence, Social Impact, and Relevance
- •25.3 Etiology
- •25.3.2 Rectal Manometry
- •25.3.5 Botulinum Toxin Injection
- •25.4 Pathogenesis
- •25.5 Natural History and Clinical Manifestations
- •25.6 Diagnosis
- •25.6.1 Colonic Transit Time
- •25.6.2 The Evaluation of Severity: Search for Objective “Instruments”
- •25.7 Management
- •25.7.3 Electric Stimulation
- •25.8 Surgical Treatment
- •25.8.2 Colonic Resection
- •References
- •26: Posterior Sagittal Approach for the Treatment of Other Conditions
- •26.1 The Kraske Operation
- •26.2 Urogenital Sinus with Normal Rectum
- •26.3 Urogenital Sinus with Normal Rectum and Adrenal Hyperplasia
- •26.4 Acquired Urethral Atresia
- •26.5 Acquired Rectourethral Fistula
- •26.6 Giant Seminal Vesicle
- •26.7 Urethral Tumors
- •26.8 Acquired Rectovaginal Fistula
- •26.9 Rectal Tumors
- •26.10 Presacral Masses
- •26.11 Surgical Technique
- •26.12 Posterior Sagittal Approach, Its Application in Cases with Hirschsprung’s Disease
- •26.13 Vaginal Atresia with Normal Rectum
- •References
- •27: Miscellaneous Conditions
- •27.1 Part I: Perianal Abscess and Fistula

306
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
urinary control. Patients who have a large bladder, rather atonic, diffi cult to empty, benefi t from
intermittent catheterization and, due to the fact
that the bladder is hypotonic, they remain dry in
between catheterizations. Patients that have a
hypertonic bladder and a poor bladder neck are
more diffi cult to manage. Our urologist colleagues would indicate intermittent catheterization not only to keep the patient dry but also for
reasons such as to protect the upper urinary tract
and kidneys, particularly when the patient has
neurogenic bladder and refl ux.

Postoperative Evaluation
1 9
It is very obvious that every day we feel the need
and the pressure to evaluate the results of our
operations in the most objective possible way.
One of the biggest problems of medicine and
surgery through history is the diffi culty to measure, if possible with numbers, the quality of our
results.
In an effort to be objective in the measurement of the bowel control, different surgeons
created scores. Unfortunately, all of these scores
that have been published until now are basically
defi cient, because they include very subjective
issues. Some of them [ 1 ] try to give points to
subjective and abstract concepts such as “extra
underpants for liners needed,” “social problems,”
“activity restrictions,” and “rashes.” Obviously,
those concepts do not refl ect the real result of an
operation. Others [ 2 ] include concepts such as
“infl uence in lifestyle,” “need to wear pad,” “taking constipation medicine,” and “lack of activity”
to “defer defecation for 15 min.” Other scores
[
3 ] are extremely extensive, sophisticated, and
complicated and therefore cannot be used on an
everyday basis; in addition, they are full of subjective concepts not necessarily relevant to bowel
control such as “in the last week has your child
experienced constipation?” “In the last week,
has your child experience pain in the abdomen?”
Even when some of these scores have been “validated,” we considered them highly inadequate.
Other scores [ 4 ] include a very controversial con-
cept such as “frequency of defecation” that is not
necessarily related to bowel control. We can say
the same about “stool consistency,” “stool odor,”
“constipation,” and “modifi cations of the diet.”
Other highly controversial concepts included in
these scores are, for instance [
leak so that you have to change your underwear?”
“Does bowel or stool leakage cause you to alter
your lifestyle?” One of the fi rst scores created
to try to measure bowel control in an anorectal
malformation was designed by Kelly [ 6 ] but,
unfortunately, includes very subjective concepts,
such as “strength of puborectalis muscle action
on digital examination.”
Another very serious problem that we found
in all of our literature review is that when discussing results, in terms of bowel control, unfortunately, most of the times, the surgeons continue
using the old, archaic, misleading classifi cation that divides the malformations into “high,”
“intermediate,” and “low.” In addition, most of
those papers do not include a description of the
characteristics of the sacrum and/or the presence
or absence of tethered cord. Those big defi ciencies obviously disqualify many papers.
We propose that in the future, when we discuss results in terms of bowel control, we describe
malformation per malformation, in other words,
bladder neck fi stula patients, prostatic fi stula,
bulbar fi stula, etc., since each one of those has a
different prognosis. In addition, we must subdivide them into those with normal sacrum and
those with a very defi cient one. A very defi cient
sacrum is one with a sacral ratio lower than 0.4.
In addition, we must indicate if the patient has
5 ]: “Does stool
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_19, © Springer International Publishing Switzerland 2015
307

308
19 Postoperative Evaluation
tethered cord or not. We will be comparing rectal
prostatic fi stula with rectal prostatic fi stula, bulbar with bulbar, perineal with perineal, etc.
Frequency of bowel movements is not necessarily related to the potential for bowel control
or the quality of the procedure that was used to
repair a malformation. The frequency of bowel
movements depends on the length of the colon
and/or the possibility of the patient’s suffering
from some sort of irritation or infl ammation of
the colon. Likewise, the presence or absence of
constipation does not necessarily relate to the
quality of the operation or the capacity of the
patient to have bowel control. Constipation represents rather a hypomotility disorder that, as we
discussed in this textbook, is aggravated by many
other factors. In other words, there are patients
with severe constipation but continent; and there
are other patients that have no constipation and
they are incontinent. We should discuss separately the results in terms of constipation and the
results in terms of bowel control.
In summary, we do not use any scores in the
evaluation of our patients. The optimal best result
in the repair of an anorectal malformation is to
have a patient that behaves like a normal individual, in other words, a person that has voluntary
bowel movements and does not soil or smear the
underwear. That is what we call totally continent
patients. Another category of patients are those
who have voluntary bowel movements. In other
words, the patient tells the parents that he/she
wants to use the toilet, voluntarily; he/she goes
there and has a bowel movement; occasionally,
the mother sees the underwear with smears or
soiling. The third category includes patients that
are totally incontinent; because they do not have
voluntary bowel movements, they simply pass
stool in the underwear. We realized that we are
still far away from being able to quantify with
numbers our results, but we believe that this is
a better way to evaluate patients with anorectal
malformations.
Efforts have been made in the past to evaluate
bowel control, using radiology. Justin Kelly published some of the fi rst studies on defecograms in
anorectal malformations [
7 ]. He emphasized the
importance of the rectal angle, as a manifestation
of the action of the “puborectalis muscle.” Yet,
the studies were diffi cult to interpret, basically
because in those years, anorectal malformations
were still classifi ed as “high,” “intermediate,”
and “low.” The fi ndings of those studies demonstrated that the presence of that angle, in general,
correlates with good clinic results but not 100 %.
The concept was reintroduced by Yagi, doing
what he calls “postoperative fecal fl ow metric
analysis” in patients with anorectal malforma-
8 ]. It is a very sophisticated study, but it
tions [
does not tell us anything new and does not help
us in the management of our patients. The same
concept of the anorectal angle was again used
to compare the results between posterior sagittal anorectoplasty and the laparoscopic approach,
but unfortunately, the authors still refer to the
malformations in terms of “high” and “intermediate” [ 9 ].
Early in our practice, we performed defecograms in all of our patients, and in general, a
good angle was more frequently associated with
patients with bowel control, but not necessarily in
all cases. Very soon we learned that bowel control was something much more complex to
evaluate.
Rectal manometry has been a very popular
study. We exposed our methodological doubts
about the validity of that study (see Chap. 25 ,
Sect. 25.3.2 ). Again, the authors keep referring to
the anorectal malformations as “high” and “low”;
they even claim that they can manometrically
evaluate the quality of the “internal sphincter”
[
10 – 16 ]. The quality of the sacrum as well as the
presence or absence of tethered cord is not mentioned in those papers.
CT scan evaluation of anorectal malformations
has frequently been used. Obviously, the images
have better defi nition than the previous radiologic studies [ 17 ]. Unfortunately, CT scans show
only transverse sections of the pelvis; therefore,
when the rectum is completely anterior or posteriorly mislocated, the study is not good enough.
On the other hand, when the rectum is placed into
the ischiorectal fossa, the study is valid and may
guide us to make a decision about repositioning
of the rectum within the limits of the sphincter
(see Chap. 22 ). Unfortunately, sagittal views are

References
309
not seen in this kind of study, and we consider
that an important defi ciency. We were ecstatic and
happily surprised to read in one of the papers that
the authors no longer refer to the puborectalis and
internal or external sphincter, but rather mention
the “muscle complex” [ 18 ]. We were also very
happy to see that some of the publications related
with computed tomography in anorectal malformations were very honest papers, in which the
authors mentioned that the clinical application of
those imaging fi ndings were not applicable in the
everyday practice [ 19 – 21 ].
Electromyography has also been used to evaluate the sphincter mechanism in patients with
anorectal malformations [ 22 – 24 ]. The informa-
tion obtained with electromyography does not
help us to make any decision preoperative or
postoperative. We believe that there is no need to
use an electromyographic study to see a sphincter
that we already saw directly during an operation.
We have the feeling that sometimes the doctors
who perform sophisticated studies do it with a specifi c goal in mind, to fi nd the preconceived structures that they learned, such as “external sphincter,”
“puborectalis,” and “internal sphincter.”
We are extremely enthusiastic about the MRI
technology to evaluate the anatomy of the pelvis,
particularly in anorectal malformations. We
believe that the MRI studies provide the best
images that refl ect the real anatomy of the patient.
In fact, we feel that the MRI studies confi rm our
operative fi ndings and our concepts, related with
the anatomy of the sphincter mechanism in a normal individual and in patients with anorectal malformations (see Chap.
2 ). We use MRI studies
when we deal with complex malformations and
particularly in patients that already had a previous operation and suffer from fecal incontinence.
The MRI study allows us to determine exactly the
position of the rectum as related with the sphincter mechanism. This study provides beautiful
sagittal images as well as transverse sections. We
perform the study introducing a large rubber tube
into the rectum to be able to see exactly what is
rectum and what is sphincter mechanism [ 25 ].
We have been very impressed by the fact that in
spite of the beautiful, realistic images, provided
by the MRI study, some authors insist in being
able to see a distinct “puborectalis muscle,” as
well as an “internal sphincter” with this study;
and yet, when we look at the images that they
provide, we only see a continuum of muscle and
no separation between those structures [ 26 – 29 ].
Some authors claim not only to be able to see the
“internal sphincter” in the MRI study, but they
evaluate the sphincter, creating what they call an
“internal sphincter score” [ 30 ]. The MRI study
has been used by others to “compare differences
between patients with constipation and fecal
incontinence” [ 31 ]. We do not believe that this
excellent imaging study is necessary to differentiate those two conditions.
In summary, we only use MRI studies in
patients that were already operated on in the past,
and we want to know the specifi c location of the
rectum as related with the sphincter mechanism.
Preoperatively, we fi nd diffi cult to do the MRI,
logistically speaking, in little babies, because
they need heavy sedation or anesthesia.
Endosonography has been used mainly in
adults to evaluate the sphincter mechanism preoperatively and postoperatively. We feel that the
images provided by that study are not nearly as
accurate as an MRI study. In addition, there is no
study that tells us exactly at what levels of the
anorectum are the images taken from [ 32 ].
The reading about the literature related with
imaging technology confi rms the old saying:
“our eyes see only what we want to see.”
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10.1055/s-2008-1059722
10.1007/

Bowel Management for the Treatment of Fecal Incontinence
2 0
20.1 Introduction
We use the term “bowel management for the
treatment of fecal incontinence” to refer to a program implemented at our institution which is
designed to keep patients who suffer from fecal
incontinence artifi cially clean in the underwear
[ 1 – 3 ]. The management basically consists of the
administration of an individually designed enema
that is given once a day, which allows the patient
to remain completely clean in the underwear for
24 h. In a number of cases, the program includes
the medical manipulation of the colonic motility
with a specifi c diet and/or medication such as
loperamide. 1
Enemas have been used for the management
of fecal incontinence for many years with variable results [ 4 – 12 ]. When the patients come to
our clinic and their parents learn about what our
bowel management program is all about, it is not
unusual for us to perceive their disappointment.
They frequently explain to us that they expected a
more sophisticated management of fecal incontinence; in fact, they frequently say that their child
1
Loperamide HCl 2 mg simethicone 125 mg (in each
caplet) slows the rate at which the stomach and intestines
move. It also increases the density of stools and reduces
the amount of fl uid in the stool.
previously received enemas that resulted in no
improvement. Some even say that enemas actually make their son or daughter worse in terms
of bowel control. That parent’s reaction is understandable. The bowel management program that
we implement in our clinic includes therapeutic
elements (enemas, constipating diet, and medications to slow down the colonic motility) that have
been widely used in the past in the management
of fecal incontinence. However, we like to say
that we use the same therapeutic elements, but
with a specifi c, different rationale that allows us
to have a high degree of success. We explain this
to the parents and ask them for patience and tolerance, so we can demonstrate that the same therapeutic elements (enemas, diet, and medication)
when used following a systematic rationale may
have much better results.
The basic principles of our program are:
• Every patient needs a different type of enema
because each one has a different type of colon
(size and motility).
• The only way to monitor the effi ciency and
effectiveness of an enema is by taking an
abdominal x-ray fi lm to determine the amount
and distribution of stool in the colon
before and after the enema. Every day (during
1 week), we readjust the volume, concentration,
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_20 .
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_20, © Springer International Publishing Switzerland 2015
311

312
20 Bowel Management for the Treatment of Fecal Incontinence
and content of the enema, according to the
specifi c patient’s reaction and the radiologic
image of his or her abdomen.
• It is extremely important, as a fi rst step, to
determine the patient’s specifi c type of colonic
motility in order to be successful. We infer
this from a contrast enema.
The bowel management program at our insti-
tution was created by trial and error, out of our
desperation, provoked by the follow-up of many
patients who suffered from this devastating problem (fecal incontinence). During this long process (30 years), we learned many lessons, and we
believe that we now have reached an important
degree of expertise that allows us to have very
good results, and with that, we have improved the
quality of life of many children [ 1 – 3 ].
In dealing with anorectal malformations,
clearly, a perfect initial anatomic reconstruction
is only part of the job. After the operation, we
are obligated to continue following our patients
in order to manage the expected functional
sequelae; the ultimate goal being a child with
clean and dry underwear.
It is unacceptable to operate on a patient and
let other professionals, not knowledgeable about
our procedures, take care of the functional
sequelae related to our operations.
In retrospect, from our own experience in the
surgical treatment of anorectal malformations,
we have learned that roughly 75 % of our patients
have voluntary bowel movements [ 13 ]. This
means the child is capable of verbalizing his or
her desire to use the toilet voluntarily and successfully. Unfortunately, half of that 75 % group
still soils the underwear occasionally. Usually
those episodes of soiling are a manifestation of a
degree of fecal impaction as a consequence of a
mistreated problem of constipation. Once we
readjust the amount of laxatives, the patient usually responds very well, and the soiling disappears. That leaves us with approximately 25 % of
patients who suffer from total fecal incontinence.
It is this group of patients for whom we feel morally obligated to get them clean and from whom
we got the motivation to create, step by step, a
series of principles and maneuvers that we now
call the “bowel management program.” Soon
enough, we started seeing patients not operated
on by us, suffering from fecal incontinence. In
fact, nowadays, most of the patients that we take
care of in our bowel management clinic are
patients operated on at other institutions. What
we convey here is the result of an accumulated
experience with the management of approximately 800 patients suffering from different types
of fecal incontinence.
The bowel management program is only a
medical and not a surgical treatment. Yet, most
pediatricians and gastroenterologists are not
familiar with this kind of management, and surgeons are usually “too busy” to perform medical
treatments. As a consequence, many fecally
incontinent patients remain rather abandoned,
looking for centers where they can receive the
benefi t of a well-integrated, comprehensive,
bowel management program.
We have found that bowel management programs are not popular. There are several reasons
that may explain this. Hospitals like to advertise
what they do, but usually they advertise “elegant”
conditions and procedures. The public relations
departments of hospitals like to advertise, for
example, when the institution performs the fi rst
cardiac transplant, when they inaugurate a department of fetal surgery, or when they make an
advance in the management of cancer. In addition, generally speaking, the media does not like
to talk about fecal incontinence, stool, urine, and
sexual problems. To advertise the opening of a
department for bowel management is “not elegant.” In fact, we found that many doctors in the
United States or in other countries perform an
operation to repair an anorectal malformation,
and then, when the patient goes back to their
clinic suffering from fecal incontinence, the surgeons refer the patient to us, for the bowel management, and sometimes they use derogatory
terms such as “go to that clinic to learn how to
give enemas.” It is a very common misconception
to believe that bowel management is equivalent
to giving enemas. In this chapter, we try to show
that the bowel management program is much
more than giving an enema.
We also believe that the bowel management
program is not popular because it does not pay

20.1 Introduction
313
well. Most insurance companies do not even
know what a bowel management program is. As
the reader will be able to learn from this chapter, it takes a signifi cant amount of time and
effort from surgeons and nurses to implement
the bowel management in a single child, and
insurance companies do not compensate for all
this. It is rather ironic that, at the present time,
a surgeon can charge about ten times more
money for a 30-min operation than he can for a
1-week treatment that requires a lot of dedication
and work with a child with fecal incontinence.
Yet, the bowel management program allowed us
to improve the quality of life, more than with any
operation that we have done and in many more
children.
In addition, there is certain reluctance by parents to accept the bowel management program.
This is based on certain misconceptions. These
include the idea that the enemas may produce
malnutrition in children because some parents
think that the enemas wash out nutrients from the
bowel. We have to go through a long explanation, including showing diagrams, to explain that
enemas only remove the waste material from the
colon and not the nutrients from the small bowel.
In addition, we have never seen a patient suffering from malnutrition related to the administration of enemas.
Another frequent misconception of many parents is the idea that once they start the bowel
management, it is going to be for life. This concept is partly true. Many patients, of course, were
born with severe anatomic defects that allow us
to predict that most likely they will never have
bowel control, and therefore we may reasonably
believe that the bowel management will be necessary for life, unless a new scientifi c advance
allows us to offer them something better.
However, there are many other patients that have
a borderline kind of bowel control, who may benefi t from a temporary bowel management and
who later in life develop bowel control.
Another misconception is the idea that the frequent administration of enemas will make a male
patient a homosexual. There is no evidence that
this could happen. Homosexuality is not more
common among patients who received bowel
management, and the overwhelming majority of
homosexuals have never received bowel
management.
Finally, many parents believe that subjecting their child to a bowel management program
may interfere with the natural toilet training process. This is false. In fact, we are convinced that
the bowel management may help the patient to
become toilet trained. A temporary bowel management in a patient that has borderline bowel
control allows the patient to gain self-confi dence
by feeling clean, not smelly, attend school, and
play with other children without being worried
about having “accidents” in the underwear. If
the patient has some potential for bowel control,
the bowel management is considered temporary
and gives the patient the opportunity of being
absolutely clean. The toilet training process can
subsequently be attempted during the summer
vacations, having more chance of success, particularly when the child already experienced being
clean and odorless. It will be easy for a child that
has been clean for several months to perceive
when he is soiled with stool. A child that grows
up with diapers and stool in the underwear all the
time becomes accustomed to that and sometimes
is more diffi cult to train.
Once we are successful with the bowel management regimen and keep the patients completely clean, provided the patients are old enough
to understand what an operation is all about, we
discuss with the parents and the patients the possibility of performing an operation that will allow
the patient to receive enemas in an antegrade manner. This is through a small orifi ce or an artifi cial
device, located in the abdominal wall, connected
with the colon of the patient, frequently through
the cecal appendix (see Chap.
21 ). This has been
called Malone [ 14 ] or ACE procedure (antegrade
continent enema). There are many techniques and
different ways to do it. There is no question that
these antegrade enema procedures, or techniques,
are benefi cial and contribute to improve the quality of life of many patients. However, we fi rmly
believe that these procedures are only indicated
when the surgeon has demonstrated that the bowel
management is successful. We have seen a signifi cant number of patients that were operated on at

314
20 Bowel Management for the Treatment of Fecal Incontinence
other institutions, undergoing different types of
antegrade enema procedures; in whom the procedures were successful, but the patients were still
dirty with stool in the underwear, simply because
the surgeon never implemented a good bowel
management program. We consider it highly inadequate to offer an antegrade enema operation to a
patient in whom the surgeon never proved or demonstrated that the bowel management worked. If
enemas given through the rectum fail to keep the
patient clean, most likely they will be equally ineffi cient when given in an antegrade fashion.
20.2 Goals of the Bowel Management Program
The bowel management program was designed to
take care of patients who suffer from fecal incontinence, from different origins, not only anorectal
malformations. Our goal is to keep the patient artifi cially completely clean 24 h per day, so the patient
can be socially accepted, attend school, play, and
become psychologically adjusted to society.
The majority of fecally incontinent patients
that we treat are patients that were born with
anorectal malformations, some of them operated
on by us, but the majority of them were repaired
in other centers. Another group of fecally incontinent patients were born with Hirschsprung’s
disease; they were operated on and subsequently suffer from fecal incontinence. This is
very unfortunate because theoretically patients
with Hirschsprung’s disease who are born with
an intact continence mechanism, which receive
a technically correct operation, should not suffer from fecal incontinence; yet, we have treated
many such patients (see Chap.
routine evaluation of patients with Hirschsprung’s
disease, who suffer from fecal incontinence,
includes an examination under anesthesia to
determine the integrity of the anal canal. In a
technically correct operation for Hirschsprung’s,
the patient’s anal canal and the dentate line should
have been preserved intact. Having an intact anal
canal means that the sensation (indispensable
to have bowel control) most likely is preserved
and also that the voluntary sphincter mechanism
24 ). Part of our
Fig. 20.1 Destroyed anal canal
is most likely preserved. Unfortunately, we
see many patients who had an operation that
destroyed the anal canal (Fig.
20.1 ); the surgeon
resected it during the dissection and anastomosed
the normoganglionic bowel to the perianal skin,
leaving no trace of anal canal, which most likely
will make that patient fecally incontinent for life.
Another group of patients that suffer from
fecal incontinence are those who are born with
myelomeningocele and spina bifi da. This group
represents a population of patients much larger
than the population of anorectal malformations
and Hirschsprung’s disease [ 15 ]. We have not
been actively advertising our program in that
population, because we do not have the logistic
capacity to take care of so many patients, particularly with the limitations that were already mentioned, in terms of reimbursement, time, and
personnel. However, we have treated a signifi cant
number of these patients who defi nitely benefi ted
from our bowel management program.
Other patients were born with sacrococcygeal
teratomas or other kinds of tumors in the pelvis.
The tumors or the resection of those tumors damaged the structures that are important for bowel
control and led the patient to suffer from fecal
incontinence. Finally, patients who suffered from
severe pelvic trauma that damaged the mechanism of continence may also benefi t from this
program. Occasionally, we take care of patients
born with sacral agenesis without an anorectal
malformation.

20.3 Evaluation of the Patient for Bowel Management
315
20.3 Evaluation of the Patient
for Bowel Management
Characteristically, we receive letters, phone calls,
or e-mails of families of patients who hear about
us, from pediatricians or pediatric surgeons, or
they learn about our center through the Internet.
They send a letter or an e-mail asking for help.
We ask them to send us copies of the operative
reports of their child and request several studies
that can be done at home and sent to us, or alternatively, the patient and the family may come to
our center and have the studies done here. These
studies include:
• X-ray fi lms of the sacrum and lumbar spine in
AP and lateral positions to evaluate for scoliosis and spinal hemivertebrae and to assess the
development of the sacrum from which we
can partially infer the functional prognosis of
the specifi c malformation
• Kidney ultrasound and voiding cystourethro-
gram to evaluate for associated urologic
problems
• Contrast enema with water-soluble contrast
material and without bowel preparation
• MRI of the pelvis (Peña/Patel protocol) 2 –
mainly in patients born with complex
malformations
• MRI of the spine – to rule out the presence of
tethered cord or other associated spinal and
cord problems
• A voiding cystourethrogram in cases with an
abnormal kidney ultrasound or urinary
symptoms
The purposes of performing all these studies
in all patients that come to our clinic suffering
from fecal incontinence include:
• First, we want to fi nd out the specifi c type of
malformation that they were born with and
their associated malformations (operative
reports, x-rays of the sacrum and lumbar
2
MRI Peña/Patel protocol: The purpose of this study is to
determine the position of the rectum in relationship to the
sphincteric mechanism. We try to see if the rectum is anteriorly, posteriorly, or laterally mislocated, in relation to
the sphincter mechanism. In addition, we look for other
abnormalities such as posterior urethral diverticulum frequently found in these patients.
spine, and MRI of the spine to rule out tethered
cord).
Learning about these allows us to predict
whether or not the bowel management will be
given on a permanent basis or temporarily
(depends on the functional prognosis of the original malformation and the associated problems).
Also, this will help us to detect a very special
and interesting group of patients that were born
with a “good prognosis” type of defect. They
underwent a technically correct operation, they
never received adequate treatment for their
constipation, and they suffer from overfl ow
pseudoincontinence. They only require laxatives and no enemas!!
• Second, we want to learn about the type of
colonic motility that the patient has, which is
the key for success. (For that we use the contrast enema.)
• Third, we want to fi nd out untreated or poorly
treated associated defects (mainly urologic).
This will be discussed separately due to its
importance. This is the reason to request a kidney ultrasound, voiding cystourethrogram,
and MRI of the spine and pelvis. We also want
to know whether or not the rectum following
the pull-through is located within the limits
of the sphincter; the MRI done with a special
technique (Peña/Patel protocol) 3 is the best
way to determine this.
We evaluate all those studies, elaborate a man-
agement plan, and then give the patient an
appointment to come to our clinic.
A few years ago, we decided to run our bowel
management program only during one specifi c
week every month rather than daily. During
that particular week, we see between 15 and 40
patients, all gathered for evaluation and management of fecal incontinence. We start the fi rst day
with a conference from one of us (surgeons) to
welcome the parents and to explain generalities
about the bowel management program. That is
followed by a lecture by one of our nurses to talk
about different types of enemas and techniques
of enema administration. Then, in our clinic we
see each one of the patients to discuss, on an
3
See footnote 2.
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