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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_697_Библиотеки_им_академика_М_И_Перельмана.pdf
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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

326
a
20 Bowel Management for the Treatment of Fecal Incontinence
b
Fig. 20.10 Effi cient (adequate) enema. ( a ) High colonic enema. ( b ) Enema given taking advantage of gravity
the patient still does not respond (meaning it
takes a long time to pass stool or does not empty),
and then we add soap in addition to the glycerin.
On the other hand, if the nurses give us information provided by the parents, indicating that
the child reacted with vagal types of symptoms,
we try to reduce the amount of Fleet or the
amount of glycerin; we try only saline solution
and see how the patient responds.
There are patients that show an x-ray image of a
completely clean colon (Fig.
20.12 ) from day one,
and yet they still pass stool in the underwear in
between enemas. That happens in patients who suffer from colon hypermotility. The liquid stool that
runs fast through the colon is not seen on a plain
abdominal x-ray fi lm. These patients are the most
diffi cult to manage. In fact, most of the 5 % group
of patients that did not respond to our bowel management are patients who suffer from severe hypermotility. The colon was very clean from day one,
To slow down the colon in hypermotility
patients, we have the following therapeutic
elements:
(a) Constipating diet (Fig. 20.4 )
(b) Loperamide 13
(c) Pectin
(d) Eliminating the irritating factor when known
(e.g., lactose intolerance, food allergy, phosphate enema, infl ammatory bowel disease)
(see Animation 20.3 ).
The constipating diet we use is a very radical
one. Every patient has a different susceptibility or
idiosyncrasy to different types of foods. In other
words, some patients have severe diarrhea when
they ingest prunes, and in some other patients,
prunes have no effect whatsoever. Since we are
limited by time (1 week), we prescribe for our
patients with colonic hypermotility a diet that
has proved to be extremely constipating in most
patients. Once we are successful with that diet,
after the administration of a small saline enema, yet
there was no way to slow down the colon.
13
See footnote 1.

20.8 Rationale to Change the Type of Enema
327
Fig. 20.11 Foley balloon technique
we advise the parents to try to individualize and
liberalize the diet, trying to fi nd out in the patients’
diet the really offending types of food that produce diarrhea. Our constipating diet is shown in
Fig.
20.4 ; it basically consists of the elimination
of milk and milk products, elimination of fats, as
well as all kinds of fried foods, all fruits except
apple and banana, and all cereals except rice. The
patients can eat all kinds of meat that is broiled
or boiled but never fried. They are allowed to eat
white bread, and they can drink artifi cially sweetened soft drinks, but no fruit juices. It has been
our experience that this diet is constipating for
the majority of the patients that we see. Once the
patient comes to our clinic smiling because he/she
has been completely clean in the underwear for
24 h, we know that with that specifi c constipating diet and that specifi c enema, he/she is completely clean, and that is our baseline or point of
Fig. 20.12 Abdominal x-ray fi lm showing a clean colon
reference. Then we tell the parents that during the
following weeks, they will be able to try to slowly
liberalize the diet. Typically, we ask the patient
what type of food he or she misses the most. The
answer is usually ice cream, pizza, or French fries,
so we tell the parent to give one of those types of
food for three consecutive days, in addition to the
constipating diet. If the patient still remains clean,
this means that the type of food that he/she loves
will now be a part of his regular diet. We ask the
patient again what is the next type of food that he
or she wants, and the answer may be ice cream.
Now we instruct the mother to give ice cream,
in addition to the same constipating diet, and see
what the effect is. If the patient is clean, that is
good news for him/her. Now he/she will be able to
eat the constipating diet plus ice cream. Let’s say
that then he/she says chocolate; we give him/her
chocolate, and he/she has “accidents” (meaning
passing stool in the underwear); now we know
that chocolate is a real “offender” and should go
to the list of forbidden types of food, most likely
on a permanent basis. Following a similar routine,
for the following weeks and months, the parent

328
20 Bowel Management for the Treatment of Fecal Incontinence
will be able to tailor a specifi c diet that is the
best for his/her daughter or son that will be most
tolerable.
We do something similar with the administration of the loperamide. 14 For instance, we may
know that with a full dosage of loperamide 14
and the diet, the child remains completely clean.
Yet we want to fi nd out the minimum amount
of loperamide 14 capable of keeping the patient
completely clean. If the medication had being
administered three times per day, the fi rst step
would be to decrease the administration to twice
per day for one entire week and see the patient’s
reaction. If the patient is still clean, that means
that he or she only needs the administration of
loperamide 14 twice a day. Subsequently, we
decrease to once a day. We keep decreasing the
dosage to determine the minimal required amount
to keep the patient clean. Some patients may stay
completely clean without administration of loperamide 14 . Some others need a minimal amount.
We like to say that fecal incontinence is not a
psychological problem but rather a physiologic
one. Many of our patients have already seen psychiatrists and psychologists before coming to our
clinic. Certainly, fecal incontinence produces
secondary psychological problems. Most of the
times, however, those problems disappear when
the patient is clean in the underwear.
Many patients have also been on biofeedback,
which we believe is useless, particularly if the
patient was born with what we call a “bad malformation” and does not have anatomic elements
for bowel control.
During the last visit to our clinic (the last day
of the week), we explain to the parents that a successful bowel management is something adjustable. At that point, we know that that particular
enema with or without diet and/or loperamide
15
works for the patient at that particular time.
However, we explain that the same enema may
not work next week. We have patients that have
been on the same regime for 10 years with no
changes, and they remain clean. Other times, parents will call us after a month because, even when
14
See footnote 1.
15
See footnote 1.
they are giving the same enema that we prescribed,
the patient is passing stool in the underwear during the day, in between enemas. The advice we
give to these parents is very specifi c: take a plain
x-ray abdominal fi lm, and send it to us by e-mail.
We take a look at the image, and most of the times
we can say what is happening. If we see a lot of
stool in the colon (Fig. 20.7 ), it means that either
the enema that used to work no longer works or
the parents are not giving the enema correctly. We
then go over the technique of administration of an
enema with the parents to be sure that the enema
is really being given and not leaking. If the enema
has been given correctly, we conclude that the
enema that used to clean the colon now does not
work for unknown reasons. We can then increase
the volume and/or the concentration of the ingredients. On the other hand, if the x-ray fi lm that the
parents sent us shows a completely clean colon
(Fig. 20.12 ) and the patient is having “accidents,”
that means that for some reason the patient is having hypermotility of the colon. It is either because
he/she suffers from diarrhea, he/she has a virus,
he/she is eating something that upsets his/her
colon, or the phosphate enema is producing a
spastic type of colon. The recommendation is
then to discontinue the phosphate if they have
been using it or to reduce the amount of glycerin
if they are using it. If there is evidence of a viral
diarrhea episode in the family, we simply tell
them to stop the enema until the patient goes 24 h
without a bowel movement and then restart the
bowel management again.
The parents should know that during an episode of diarrhea (viral or bacterial), the bowel
management must be suspended. The patient
must remain at home and should not go to school
because there is no way to avoid the leakage of
stool.
Once we are successful with the bowel
management, we discuss with the parents the
possibility of performing a Malone procedure
(continent appendicostomy or continent neoappendicostomy) or any other kind of ACE
type of operation. In Chap.
21 , we will discuss
our technique and the type of operation that
we perform for the administration of antegrade
enemas.

20.9 Bowel Management for the Treatment of Severe Diaper Rash
329
We like to perform that operation when the
patient is capable of understanding its advantages.
Some parents want us to do a Malone procedure
too early (on a 3-year-old child). We believe that
is not appropriate, because perhaps they want the
operation to make things easier for them, but not
necessarily for the child. We like the participation
of the children. The patients that benefi t the most
from antegrade enemas are patients who are usually older than 8 years of age and want to become
more independent. They want to give the enemas
themselves because they feel embarrassed when
the mother or the father is giving the enema. They
want to be able to go on camping overnights and
be more independent in general. Teenagers are
the population who benefi ts the most from this
kind of operation.
20.9 Bowel Management
for the Treatment of Severe
Diaper Rash
It has been our experience that patients who had
an anorectal malformation already repaired, followed by a colostomy closure, suffer from severe
diaper rash as a consequence of the constant
passing of stool. Sometimes this occurs in other
colorectal conditions as well. Fortunately, this
is not a permanent condition. As time goes by,
the number of bowel movements decreases and
mothers are able to manage the severity of the
diaper rash. During the acute stage, the passing
of stool is so frequent that it makes it impossible
to change the diapers frequently enough. Also,
the use of different types of creams to avoid the
contact of stool with the skin fails when patients
pass stool constantly.
Under these circumstances, we give the parents a long explanation, so they can understand
that the goal of the treatment is to avoid the contact of stool with the skin. The different types of
ointments and creams that we prescribe only represent a potential barrier between the skin and the
stool. Our team of nurses tries different strategies
to avoid contact by stool with the skin, including
when possible keeping the baby with no diapers
and near a fan to keep the skin dry, to be able to
see when the baby passes stool and be able to
clean them with soap and water without rubbing
the skin.
In spite of all of this, the diaper rash sometimes is very diffi cult to treat. This led us to the
new idea of implementing a sort of bowel management, consisting in the administration of a
small glycerin enema, repeated twice per day.
Even when the patient recently had the colostomy closed, and therefore has a suture line in
the colon, we have found that the administration of a 15-mL glycerin type of enema, twice
per day, does not represent a risk for the suture
line of the colostomy. Yet, that small enema produces a large bowel movement that will allow
the baby to remain several hours without passing
stool, which is very important for the treatment of
the diaper rash. After a few days or weeks, most
patients with anorectal malformation with an
intact colon eventually will develop constipation,
which means a lack of bowel movements, which
will help in the management of the diaper rash.
Yet, frequently, constipation is manifested by the
frequent passing of small amounts of soft stool
in the diaper, which exacerbates the diaper rash.
Arbitrarily, we decided that once the patient
is over 1 month post-colostomy closure, the
patient may be a candidate for the implementation of a full bowel management, even when he/
she is only a baby (before 3 years of age). The
bowel management in these cases is not implemented for the treatment of fecal incontinence,
but for the treatment of diaper rash. We follow
the specifi c protocol of bowel management as
described in this chapter, but because most of
these patients are babies, we start usually with
an enema of 150 mL of saline solution or larger
volumes depending very much on the age of the
patient. The bowel management has a spectacular effect in curing the diaper rash, and the parents like it very much. Even when a normal baby
has no bowel control and uses diapers, there is
a big difference between him and a baby who
was born with a poor prognosis type of anorectal
malformation. These babies have a tendency to
pass stool constantly, which is a refl ection of a
motility disorder of the rectosigmoid. The constant presence of stool in the diaper makes the

330
20 Bowel Management for the Treatment of Fecal Incontinence
rash extremely diffi cult to manage. This is seriously exacerbated in cases with urinary incontinence. The bowel management implemented in
babies under these circumstances has been very
successful.
20.10 Bowel Management Through a Stoma
Many patients have come to our center with a
colostomy created at another institution, a colostomy that was considered a permanent one.
Usually this is because the patients were born
with a poor prognosis type of defect (rectobladder neck fi stula, absent sacrum, poor sacrum,
tethered cord, myelomeningocele), which understandably makes the surgeons believe that the
patient will never have bowel control. Based on
that assumption, the surgeons open an end colostomy and explain to the parents that this is going
to be for life. Many of these parents look for us
and come for consultation with the hope and
expectation that we offer an operation that will
allow them to get rid of the stoma. Some of these
patients belong to the group of cloacal exstrophies or covered exstrophies. As is well known,
patients with cloacal exstrophy or covered exstrophy frequently have a very abnormal colon. It is a
spectrum that goes from a normal length colon to
an almost absent colon. In between, we see
patients with a very short and bizarre-looking
colon, with a very abnormal blood supply, and
we are not sure whether or not that amount of
colon is enough to absorb enough water, so as to
be able to form solid stool, in order to respond to
a bowel management program consisting of a
daily enema. Most of the patients with cloacal
exstrophy or covered exstrophy have a poor
innervation of the pelvic organs, due to a very
defective sacrum, defective spine, or meningocele. We call covered exstrophy to a specifi c
defect consisting in separated pubic bones (see
Chap.
17 ), absent bladder neck, and abnormal
colon; yet, these patients do not have omphalocele or bladder exstrophy; in other words, the
lower abdominal wall is intact. Inside their abdomen, however, they have a very similar anatomy
to the one seen in classic cloacal exstrophies,
meaning very abnormal colonic blood supply and
different degrees of short colon. For this group of
patients, most surgeons will open a colostomy
and assume that it is going to be a permanent one.
We look at the quality of the stool that comes
through their stoma; if they have solid stool, there
is a strong possibility that they will respond to
the bowel management (enema) administered
through the stoma. If they respond to the bowel
management through the stoma (meaning that we
are capable of keeping the patient completely
free of stool coming out through the stoma for
24 h), it means that we must consider the possibility of a pull-through of the stoma down to the
perineum. The parents understand that the same
enema that was demonstrated to be effi cient
given through the stoma must be given through
the rectum or through an antegrade continence
mechanism after the pull-through, in order for the
patient to remain clean. Sometimes, the patients
have extremely short colons, and we are not sure
whether or not they have enough water absorption capacity to respond to the bowel management. In that particular group of patients, we are
afraid of offering the family a pull-through of the
stoma, which will be equivalent to a perineal,
incontinent stoma that will make their life miserable. The bowel management through the stoma
20.13 ) consists in following the same sys-
(Fig.
tematic protocol of administration of enemas
through the stoma, monitoring with abdominal
fi lms the amount of stool in the colonic pouch
and following the principles already described.
Often we must add a constipating diet, loperamide, and pectin to help bulk the stool. We have
tried bowel management through the stoma in 13
patients. In four of them, we were unsuccessful
due to the fact that the patients had a very short
colon and therefore incapacity to form solid
stool. Our management was successful in nine
patients; seven of them had a pull-through procedure even when we knew that the patient had a
bad prognosis for bowel control. These patients
had an ACE procedure at the same time of the
pull-through. The patients expressed their satisfaction, and they seemed to believe that it is much
better, in terms of quality of life, to have no stoma

References
331
Fig. 20.13 Bowel management through a stoma
and receive bowel management than to have a
permanent stoma for life. Two patients were hesitant about the pull-through; yet, they decided to
continue giving themselves the bowel management through the stoma, because that, itself, represents an enormous advantage in their quality of
life, since they have now a completely empty
stoma bag for 24 h, which allows them to be
much more active, play sports, without risking a
leakage of stool from the stoma bag, which represents a potentially embarrassing experience.
These two indications (bowel management for
severe diaper rash and bowel management
through the stoma) have never been reported by
others as far as we know. We are very happy with
the use of bowel management for these kinds of
problems.
Biofeedback
We are aware of the enthusiasm of some authors
that related with the use of this modality of treatment [ 30 – 32 ]. Our experience with this form of
treatment was never published. The patients who
received this kind of management for several
months were able to improve their manometric
parameters. However, such improvement did not
translate into a real, clinical, signifi cant, positive
change for the patient.
Medication
The use of medication such as loperamide [ 33 ],
cholinergic drugs [ 34 ], or cholestyramine [ 35 ]
has been used. We believe that medication alone,
used in patients suffering from fecal incontinence
due to anorectal malformation, has very few possibilities of being successful. However, as we
mentioned previously, medications can be used
as an adjunct to our bowel management program.
Patients suffering from borderline fecal incontinence may benefi t from the use of drugs to regulate the colonic motility. In cases of overfl ow
pseudoincontinence, the laxatives used as indicated can make the difference between incontinence and bowel control.
Electrical Stimulation
This kind of treatment, posterior tibial or sacral
[ 35 – 37 ], is receiving a lot of attention, mainly
between adult colorectal surgeons. We have no
experience with this type of treatment. However,
since there is no rational explanation of the mechanism of action, we remain skeptical about it.
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10.1055/s-0030-1253406

Operations for the Administration of Antegrade Enemas
2 1
21.1 Introduction
As discussed in our previous chapter, the bowel
management program, consisting mainly in the
administration of enemas, plus sometimes treatment with specifi c medication and a specifi c diet,
greatly changes the quality of life of many children. Giving an enema to a small child is a relatively easy task. It is easy for the parents to
position the child on their lap for the enema, and
usually the child tolerates this procedure very
well. As times goes by, as the patients grow, particularly in those patients that require an enema
for long periods of time or on a permanent basis,
it becomes logistically more diffi cult, simply
because of the size of the patient. If one tries to
imagine how to give an enema in a teenager, taking advantage of gravity, it is easy to understand
that is diffi cult. Sometimes the patients’ squat on
the tub to give themselves the enema, and the
whole procedure becomes very messy. We have
been trying to use different methods to teach
teenagers to administer their own enemas while
sitting on the toilet, which is not easy, because
the enema is then given against gravity. We teach
them to pass a Foley catheter into the rectum, to
infl ate the balloon inside the rectum, and while
they are applying traction on the catheter, to
administer the enema. This is easy to say but not
easy to do. As a consequence, most teenagers
need some help from another person to receive
the enema, which is an embarrassing event.
Imagine a 13-year-old girl receiving enemas
every day with the assistance of her father or the
other way around, a 14-year-old boy assisted by
his mother to have an enema.
Malone, Ransley, and Kiely had a great idea
[ 1 ] of creating a mechanism for the administra-
tion of an enema in an antegrade fashion. They
thought that an enema could be given through an
orifi ce created in the abdominal wall, connected
to the appendix, which allowed the passage of a
small catheter directly into the colon to give the
enema while the patient is sitting on the toilet
[ 2 – 7 ]. This was a very creative concept that
gained popularity quickly. The original idea of
Malone, Ransley, and Kiely included a technique
consisting of dividing the base of the appendix,
rotating the appendix 180°, and reimplanting it in
the wall of the cecum, in a submucosal fashion, to
avoid leakage of stool through the appendix. In
retrospect, this represents a complex maneuver
that we now believe is unnecessary. The basic
idea of Malone and Kiely is still extremely valuable, but now we know that we do not have to go
through all the surgical maneuvers they described.
They also advocated the opening of the orifi ce in
the right lower quadrant, which is something that
we do not do at the present time; we prefer the
umbilicus [
Fairly soon, after Malone and Kiely popularized their idea, many others [ 11 – 16 ] came up
with different ways to achieve the same goal.
Other variants included a “noninvasive” or “minimally invasive” procedure to introduce a Silastic
button into the cecum. That technique became
8 – 10 ].
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_21, © Springer International Publishing Switzerland 2015
333

334
21 Operations for the Administration of Antegrade Enemas
very attractive because it is minimally invasive, it
does not require a big operation, and it is technically simple. It can be done under fl uoroscopy,
with endoscopy, or with laparoscopy. However,
we have been exposed to several patients who
had a button cecostomy in the past; they came to
our clinic very unhappy about the multiple longterm complications and sequelae of that procedure
that included malfunction of the button, leakage
of stool around the device, prolapse of the colonic
mucosa, and granulation tissue around the button. These problems have been seen by others
[ 17 , 18 ]. Many patients also dislike the visible
foreign body on the abdomen and are self-conscious about it, avoiding swimming and other
activities. Those patients required a revision,
removal of the button, and creation of a continent
appendicostomy. It is because of that experience
that we do not favor the insertion of button
cecostomies.
Several authors [ 19 – 24 ] had the idea of per-
forming these types of procedures laparoscopically. This, of course, has advantages but also
some limitations as will be seen in this chapter.
21.2 Our Preferred Technique
a
b
Fig. 21.1 External aspect of the abdomen of a child who
underwent a continent appendicostomy operation (Malone
procedure). ( a ) Without a feeding tube. ( b ) With a feeding
tube
We believe that the essential purpose of this type
of procedure is to improve the quality of life of
the patient. Therefore, our goal is to perform an
operation that will allow the patient to have an
active life, play sports, swim, and not be embarrassed of an orifi ce that rather looks like a colostomy or to have to hide a plastic device protruding
from the abdomen. We believe that these patients
benefi t the most by the creation of a little hidden,
umbilical orifi ce that should be as inconspicuous
as possible (Fig.
21.1 ). We create that little orifi ce
in the deepest portion of the umbilicus, making it
invisible. Yet, making a tiny orifi ce and avoiding
stricture requires a technically challenging and
meticulous surgical technique.
We think that we should use the appendix as
much as possible. Yet, we are limited by the fact
that about 30 % of the patients in whom we plan
to do this operation have no appendix because it
had been removed in the past for other reasons.
The old surgical tradition of doing an appendectomy as part of a laparotomy luckily seems to be
disappearing as surgeons become more cognizant
of the potential use of the appendix for the care of
the incontinent child. In addition, nowadays, urologists are using the appendix to create a conduit
that goes from the abdominal wall to the bladder
for intermittent catheterization which is known
as a Mitrofanoff procedure (see Chap. 23 , Sect.
23.13 ). That is another reason why the appendix
is not always available for us for this operation.
Because of that, we had to learn to make appendices out of the colonic or small bowel tissue.
We call that a “continent neo-appendicostomy”
[ 10 ]. We make the appendix out of a fl ap of the
colonic wall that is tubularized around a feeding tube to make a new appendix. We have been
doing this, mainly from the ascending colon, but
we have cases in which, because of anatomic

21.2 Our Preferred Technique
335
circumstances, we were obligated to make the
neo- appendix from the transverse or the sigmoid
colon. We have done this, in cases with multiple peritoneal adhesions or “frozen abdomens.”
In such cases, we have no choice but to use that
part of the colon that we have access to. Ideally,
we prefer to create the new appendix from the
ascending colon near the cecum but away from
the ileocecal valve.
We have done approximately 200 of these
operations (appendicostomies and neo- appendicostomies). On two occasions, we opened the
abdomen and for a couple of hours tried to divide
the multiple peritoneal adhesions between the
loops of bowel and decided to abort the procedure, because we had the feeling that we would
do more harm than benefi t to the patient.
A patient that has his or her native appendix does not need colonic preparation to do this
operation. The operation in a patient with minimal peritoneal adhesions, a thin abdominal wall,
a normal appendix, and a mobile cecum becomes
minimally invasive, although non-laparoscopic,
because it only requires a small incision (about
4 cm long) running from the umbilicus down.
The whole procedure can be done through that
incision in about 1 hour; the patient can be fed the
same day and discharged the following day from
the hospital. On the other hand, when the patient
has the appendix located in a rather bizarre position (behind the duodenum or behind the liver)
or has multiple peritoneal adhesions, in addition,
suffers from obesity, and has no appendix, the
procedure becomes technically very demanding,
taking an unpredictable number of hours. In similar cases, provided the peritoneal adhesions are
not too dense, the laparoscopy may be helpful to
avoid a laparotomy in mobilizing the cecum.
It is very important, prior to the planning of
these operations, to know whether or not the
patient has an appendix. Some radiologists are
very optimistic and believe that an ultrasound is a
reliable study to determine whether or not the
patient has an appendix, but we all know that
such a study is not 100 % accurate in visualizing
a normal appendix. A contrast enema that shows
an appendix gives us 100 % evidence that the
patient has one, but if the contrast enema does not
show an appendix, it does not mean necessarily
that the patient does not have an appendix. A CT
scan seems to be a better study.
The importance of learning about the presence
or absence of an appendix prior to the operation
resides in the fact that creation of a neo-appendix
requires full bowel preparation with administration
of GoLYTELY. It is not advisable to make a new
appendix with a colon full of stool which would
increase the chances of infection. We are very proud
of the fact that we have not had any cases of infection. In case of doubt, therefore, we go ahead with a
total bowel preparation the day before surgery with
GoLYTELY as described in the corresponding
chapter in this book (see Chap. 7 ).
Continent appendicostomy and neo- appendicostomy, in our experience, have two main late
sequelae or complications: one is stricture (or
acquired atresia) of the little stoma that we create
between the tip of the appendix or neo- appendix
and the skin of the umbilicus. The other is the
problem of leakage of stool.
In our series, the frequency of stricture or
acquired atresia was 18 %. In trying to avoid this
problem, we have been practicing a technique
that takes time to learn and to master, but we
believe will eventually decrease the frequency
of stricture. This technique consists of creating a triangle of skin from the umbilicus that
will be accommodated in a spatulated appendix
(Fig. 21.3 ) [ 9 ].
Concerning the problem of leakage of stool
through the appendix, it is very common to hear
that some authors believe that the appendix is
naturally continent. This we found to be true, half
of the time. The leakage of stool through the
Malone orifi ce occurred in 2.9 % of our cases
who underwent a plication of the cecum around
the appendix, and in contrast, it occurred in 29 %
of those cases without plication. In addition, during the performance of many of our continent
appendicostomy procedures, prior to making a
decision about plication or no plication of the
cecum around the appendix, we tested the natural
continence of the appendix by fi nger clamping
the ascending colon and the terminal ileum and
injecting saline solution through a feeding tube
introduced in the cecum through the tip of the
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