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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

References
295
urinary tract. If the patient has short colon, obviously the urologist cannot use the colon or even
small bowel for the augmentation of the bladder,
but rather must think in all the possibilities such
as gastric augmentation. If the patient has a
normal- sized colon, then the urologist can be
more liberal to use a piece of bowel that he wants
for the bladder augmentation.
Concerning the surgical management of the
colorectal component of the malformation, as
well as the genitalia reconstruction, the surgeon
must follow the same principles recommended in
the management of cloacas and cloacal exstrophies (see Chap. 16 ).
The functional prognosis of these patients
seems to be similar or better than in cases of cloacal exstrophy. Therefore, we put together the
results of covered cloacal exstrophy and cloaca
exstrophy in Tables 17.1 and 17.2 of this chapter.
Our experience includes 28 cases and was
reported in 2013 [ 67 ] .
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General Principles for the Postoperative Management of Patients with Anorectal Malformations
1 8
18.1 General Care
As discussed in each one of the specifi c anorectal
malformations, patients operated with a protective colostomy that underwent a posterior sagittal
repair, without opening the abdomen, are usually
fed the same day of the operation. The pain that
these patients suffer from is minimal. We try to
stay away from strong pain medications, such as
morphine. The patients feel comfortable once
they feel the presence of the parents. We keep
them in the hospital for 48 h in order to administer intravenous antibiotics. We do not have cases
of infections after a posterior sagittal anorectoplasty, performed with a protective colostomy.
When the patient is discharged home, we ask
them to come back to the clinic 2 weeks after the
operation, to start the process of anal dilatations.
Occasionally, patients suffer from vomiting after
the operation; we believe that it is related with the
anesthesia, since we did not perform any abdominal surgery.
When we perform a posterior sagittal anorectoplasty without a colostomy as described in the
chapter on bowel preparation, the patients receive
GoLYTELY®
gastrointestinal tract. They also receive a PICC
line or central venous catheter in order for them to
receive parenteral nutrition. We tell the family that
the patient will remain with nothing by mouth for
1
(Polyethylene glycol/electrolytes) Braintree Laboratories,
Braintree, MA., USA
1
; in other words, we clean the entire
a period of 7–10 days after the operation; we
explain that he or she will not be hungry because
we will be providing parenteral nutrition. After
7 days from the procedure, we look at the perineum
of the patient. If everything looks well and it seems
to be healing nicely, we let them eat. On the other
hand, if we have any doubts or we see dehiscences
of 1, 2, 3, or 4 sutures, either in the anoplasty, in
the perineum, or in the posterior sagittal incision,
we continue the period of fasting for 2 or 3 more
days, take the patient to the operating room, and
resuture the dehiscent areas. Very rarely, we have
patients that suffer from a complete dehiscence of
the anoplasty without a colostomy. In those cases,
we perform a colostomy and wait until the wound
is healed, not less than 3 months, in order to
reoperate.
Patients who underwent a posterior sagittal
anorectoplasty in addition to a laparotomy usually start having po feeds 2–4 days after the operation. That period of time depends very much on
the degree of ileus that the patient suffers from,
which is related with the manipulation of the
bowel during the laparotomy. Sometimes, we
operate on patients who had previous multiple
operations, and we spend long periods of time in
the operating room taking down all the peritoneal
adhesions. The longest operation that we performed so far, nonstop, has been 18 h in a patient
with a cloacal exstrophy in which we were able to
repair the rectal component, the vagina, and the
urinary tract. Those patients obviously would
remain with nothing by mouth for longer periods
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_18, © Springer International Publishing Switzerland 2015
299

300
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
of time because they have a prolonged ileus. We
followed the general guidelines that most surgeons follow-up after complicated laparotomies.
Babies subjected to long operations (longer
than 6 h) always go to the intensive care unit. We
have seen a tendency for them to suffer from
respiratory arrest postoperatively.
Occasionally, we operate on patients that
came to us with a loop colostomy. As we previously discussed in the chapter of colostomies,
some loop colostomies are totally diverting, and
we see that the entire stool goes into the stoma
bag. However, when those loop colostomies
retract a little bit, they pass stool into the distal
bowel. When that happens, after the operation, to
avoid contamination, there are several maneuvers
that may prevent fecal contamination. One can be
the introduction of a Foley catheter in the distal
colonic limb, with the balloon infl ated for several
days, to avoid the passing of stool. Another one is
to place a purse string suture in the distal stoma
with a heavy, long-term, absorbable suture to prevent the spillage of stool into the distal limb.
18.2 Local Care
We do not restrain the movements of our patients.
We do not tie them to the bed. We do not keep the
legs together. We let them walk around and move
in any way they want. However, we encourage
the mother to use a double diaper, to serve as a
cushion for the bottom. Also, we prevent the
patients from jumping and sitting in a rough way
after the operation. Because the posterior
approach is basically painless, the patients start
jumping around, and sometimes they injure
themselves. We keep the double diaper for
1 month. We like to use antibiotic ointment on the
posterior sagittal incision, the anoplasty, and the
area of the genitalia, in cases of female malformations. However, we use this ointment only for
5 days, because we have seen that after that, the
patient has a tendency to suffer from fungus
infection in the perineum.
We do not believe that the urine contributes to
producing dehiscences. In female babies, with a
rectovestibular fi stula or rectovaginal fi stula, we
do not leave a Foley catheter. In cloaca patients,
the type of urinary diversion that we use postoperatively depends on the type of cloaca that the
patient has (see Chap. 16 ). We leave a Foley cath-
eter for 2 weeks in a case of a posterior sagittal
anorectovaginourethroplasty and total urogenital
mobilization with a short common channel.
When the patient has a common channel longer
than 3 cm and we enter into the abdomen, we prefer to leave a suprapubic tube particularly if we
believe that the patient will be able to urinate normally, before 3 months after the operation. When
the patient has severe kidney damage, hydronephrosis, megaureters, and complex malformations and we believe that it is going to take longer
than 3 months for them to pass urine normally,
we prefer to leave a vesicostomy.
In male patients with rectourinary fi stulas, we
leave a Foley catheter for 1 week. After a week,
the patient comes to the clinic, our nurses pull the
catheter out early in the morning and ask the parents to take the baby to walk around and drink a
lot of fl uids, to be sure that the patient is passing
urine normally before sending him home.
Occasionally, the patient shows signs consistent
with burning of the urethra during the voiding
episodes; we encourage them to take more fl uids
and eventually the symptom disappears. It is
extremely unusual to see patients in urinary
retention after a technically correct posterior sagittal operation. However, if the patient has a poor
sacrum, tethered cord, and a very high malformation, we can anticipate that, occasionally, they
may have urinary retention. Under those circumstances, we leave the Foley catheter for a few
more days and remove it again. If we already
know that the patient has neurogenic bladder,
then sometimes we leave a suprapubic tube, in
order for our colleague urologists to evaluate the
bladder postoperatively with a urodynamic study.
18.3 Anal Dilatations
Prior to the repair of anorectal malformations, the
parents of our children receive a piece of paper
describing our protocol of anal dilatations
(Fig. 18.1 ).

18.3 Anal Dilatations
301
Fig. 18.1 Anal dilatation.
( a ) Protocol. ( b ) Size of
dilators according to age
a
• 14 days post-operative the surgeon will determine the initial size of the
Hegar dilator. He will demonstrate how to pass the dilator.
• The parents must perform the dilatation twice a day, passing the dilator
and leaving it inside for 30 seconds.
• Dilatations must be performed before meals.
• The baby must be restrained, keeping the knees against the chest.
• The size of the dilator must be increased every week, until reaching the
desired size, according to the patient’s age.
• Once final size has been reached, dilatations must continue twice a day
until the dilator passes easily. At that point, the parents must start tapering
the frequency of dilatations, guided by the following sequence:
– Once a day for one month
– Every other day for one month
– Every third day for one month
– Two times a week for one month
– Once a week for one month
Anal Dilatation Protocol
– Once a month for three months
Size of Dilators According to Age
b
Age
0 – 4 months
4 – 8 months
8 – 12 months
1 – 3 years
3 – 12 years
> 12 years
(Hegar numbers represent millimeters in diameter)
We encourage the parents of our patients to
read the protocol and memorize it, before coming
to the clinic 2 weeks after our procedure.
We have been in national and international
meetings, in which some surgeons question the
need to perform anal dilatations in patients with
anorectal malformations. In fact, some surgeons
have expressed a negative impression about the
use of dilatations, because of the potential psy-
Hegar #
#12
#13
#14
#15
#16
#17
chological trauma produced in the children, due
to the pain that we provoke with these
dilatations.
As an answer for criticisms related with our
protocol of anal dilatations, we always explain
that patients subjected to pull-throughs following old, rather rude, grotesque surgical techniques end up having an anoplasty that actually
looks like a colostomy (Fig. 18.2 ). We agree

302
Fig. 18.2 Grotesque, prolapsed anoplasty. Does not
require dilatations
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
Fig. 18.3 Postoperative appearance of a technically cor-
rect anoplasty, in a patient with good sphincter mechanism. The anus is closed. The patient needs anal
dilatation
that in the type of repair, the patients do not
need dilatations. However, in patients that are
subjected to technically correct surgical techniques to repair anorectal malformations, particularly if the patient has good sphincter
mechanism and the rectum is placed within the
limits of the sphincter, at the end of the procedure, as can be seen in the pictures that we
show after the surgical repair of each malformation, the anus looks closed (Fig. 18.3 ). This
is consecutive to the effect of the sphincter.
This is seen in good anorectal malformations
subjected to good repairs. If the patient is left
like that with a colostomy, for several months,
the anus is going to heal that way and is going
to suffer from a stricture. We believe that what
we do are not really dilatations, but we rather
want the anus of the patient to heal with the
rectum open, in the way we left it at the end of
the procedure. When we do the anoplasty in the
way that can be seen in diagrams and pictures
in the chapters of this book, one can see that at
the end of the procedure, the anus has the size
of a normal anus for the age of the patient.
However, when we remove the retention
sutures that are holding the anus open, the anus
closes by the effect of the sphincter mechanism
that surrounds it. If we leave it that way, it is
going to heal that way. That is the reason why
we believe that it is necessary to follow our
protocol of anal dilatations. We emphasized
the importance of starting the dilatations
2 weeks after surgery, not earlier, not later.
Earlier than that, we would be concerned about
damaging the sutures; later than that, we would
be concerned about fi nding already fi brosis
that would make the dilatation process most
diffi cult.
Diffi cult dilatations that produce a lot of pain
and bleeding occur when the rectum that was
pulled down suffered from ischemia, and in spite
of the efforts of the doctors, those cases usually
end up having a severe anal stricture.
We defi nitely oppose the routine followed by
some surgeons, consisting of trying to avoid pain

18.4 Avoiding Constipation
303
to the patient and booking the anal dilatations
under anesthesia once a week. The doctors do
that to calm the anxiety of the parents. Under
anesthesia, the patient does not complain, and as
a consequence, the doctors tend to over-dilate the
anus, producing injuries to the suture line. In the
following days, the anus is not dilated; the injuries tend to heal, producing fi brosis. This is followed by another traumatic dilatation every
week, with more fi brosis. In those cases, we see
patients coming to our clinic with severe fi brosis
that is impossible to dilate. Because of that, we
are very strict about our protocol. In the clinic
2 weeks after surgery, we show the parents how
to dilate the anus and we passed a dilator that the
baby does not even feel. It is usually a no. 6 or no.
8 Hegar. We do that in order for the mother to
gain confi dence performing dilatations. We show
the father how to hold the baby with the knees
against the chest, to really immobilize him/her in
order to avoid showing the mother a “moving target,” which would make the mother more anxious. With the baby completely immobilized, the
mother passes the dilator well lubricated, leaves
the dilator in place for 30 s, and repeats the
maneuver twice in the morning and twice at
night. We encourage the mother to do the dilatation before meals, to avoid the possibility of
vomiting and aspiration. We specifi cally tell the
parents to increase the size of the dilator by one
size per week (1 mm per week) and continue
increasing the size until they reach the size that is
adequate for the age of the patient. Based on our
previous experience, we believe that a normal
anal size for a full-term newborn is a no. 12
Hegar dilator, for a 4-month-old baby a no. 13,
for an 8-month-old baby a no. 14, and for 1-yearold baby a no. 15 Hegar. Older than that, usually
we recommend to go up to no. 16 Hegar dilator
(Fig.
18.1b ).
Once the parents reach the adequate size for
the age of the patient, we encourage them to continue doing the dilatations twice a day until the
dilator goes easily and painlessly. At that point,
the parents are supposed to start tapering the frequency of dilatations. In other words, they move
from the routine of doing it twice a day to once a
day, for a month, and then every other day for a
month, twice a week for a month, once every
2 weeks for a month, and then stop the dilatations.
We are very emphatic when we explain to the parents about the importance of dilatations, and we
explain that not following the routine of dilatations may provoke anal stenosis that would be
impossible to dilate, and the patient may require
an operation. When the anoplasty has been done
correctly, when the blood supply of the distal rectum is good, and when the technique has been
meticulous, the dilatations are not really painful.
Most of our patients subjected to primary
repairs of anorectal malformations were only a
few months old, and it is our experience that
those patients really do not have pain during the
anal dilatations.
When we reoperate patients that are older than
2 years old, dilatations become a problem,
because the patient comes to us already traumatized from previous procedures and painful dilatations. Under those circumstances, particularly
when we know that the patients have a bad prognosis type of malformation and we are dealing
with a reoperation, we actually tried to make the
anus a little larger than normal in diameter, being
aware that during the healing process, it will
decrease a little bit in diameter but still will be
adequate to pass stool. This facilitates the dilatations and avoids any pain. Older patients are very
diffi cult to restrain in order to do dilatations, and
it is a more traumatic experience.
18.4 Avoiding Constipation
Constipation is by far the most common sequela
seen in patients born with anorectal malformations and subjected to surgical procedures that
include the preservation of the rectum. The resection of the rectum in patients with anorectal malformations followed by a pull-through of colon
certainly may avoid the problem of constipation,
but for sure it will produce fecal incontinence.
We keep insisting that the rectum represents the
natural reservoir of the human being and therefore must be preserved as much as possible.
Patients with anorectal malformations suffer
from a hypomotility disorder of the rectosigmoid,

304
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
which translates in constipation. The constipation is more severe in patients with lower defects
(recto-perineal fi stula, rectovestibular fi stula, and
imperforate anus with no fi stula); these patients
suffer from more constipation than patients with
recto-bladder neck fi stulas. We do not know the
reason for that. It is important to remember and to
be more proactive and aggressive in preventing
and managing the constipation of those patients
that are at higher risk. When the patients have
been with a colostomy, for long periods of time
particularly if it is a transverse colostomy, without repair of the malformation, they come to us
with a severe megarectum and sometimes fecally
impacted, they suffer from more severe constipation, and it is important to anticipate that to avoid
aggravation of the problem.
Avoiding constipation must start when we
open a colostomy; we insist on the importance of
cleaning the distal colon at the time of the opening of the colostomy and to keep the colon collapsed from day one. In addition, we try to avoid
transverse colostomies and try to repair the malformations as early as possible to avoid dilatation
of the distal end.
We, as well as the parents, must be ready to
deal with the problem of constipation after the
colostomy is closed (in patients with colostomies)
or after the main repair, when the repair is done
without a colostomy. We have long conversations
with the parents of our patients and try to make
them paranoid against constipation. We tell them
that constipation produces fecal incontinence. We
know that it is actually overfl ow pseudoincontinence, but we try to make the parents aware of the
importance of taking care of the constipation. In
the chapter of bowel management, we described a
group of patients that had a good repair, of a good
prognosis type of malformation, but did not
receive care for constipation; as a consequence,
they suffer from severe fecal impaction, megarectum, and fecal overfl ow pseudoincontinence. We
try to avoid that in our patients.
The parents take home a little glycerin enema
to be used only if necessary. The golden rule is to
be sure that the patient passes stool every day. In
addition, the parents must develop the sense that
the patient is really emptying the colon. Many
persons do not know that passing stool does not
necessarily mean that the patient is not constipated. Sometimes patients can pass many bowel
movements, but they do not empty, and therefore,
they suffer from severe constipation. If the patient
goes one entire day without a bowel movement,
before the baby goes to sleep, the parents must
give the small glycerin enema and must increase
the amount of laxatives or will start the administration of laxatives if they have not given laxatives yet. We prefer to use senna derivative type
of laxatives from day one, because it has a better
and more controlled effect. Pediatricians in general prefer to use a different type of laxatives,
such as lactulose. The problem with those laxatives is that they have a tendency to produce liquid stools, which is one of the enemies of patients
with anorectal malformations. We do not know
the amount of laxatives that every patient needs;
we know that every patient needs a different
amount, and we determine the amount by trial
and error with each patient. As a routine, we
order an abdominal x-ray fi lm 1 week after the
colostomy closure or after the pull-through; subsequently, we order fi lms to be taken after
2 weeks and 1, 3, and 6 months. Even when the
parents tell us that the patient is doing very well
and they think that he is not constipated, by following this routine, we detect many times severe
constipation that the parents were unaware of and
we can give laxatives to avoid that problem. Once
we start with laxatives, we explain to the parents
that that must be given on a permanent basis.
We explain to the parents that from the time
the colostomy is closed and until the age of bowel
control (2 1/2 to 3 years), their work and responsibility consists of:
(a) Avoiding constipation
(b) Trying to achieve regularity in bowel
movements
Patients with anorectal malformations suffer
from a colonic (mainly rectosigmoid) hypomotility disorder, and if we leave them alone, they tend
to pass a small amount of stool through the day,
not really emptying. That is why they need help to
empty the rectosigmoid completely. In addition,
many of them do not have the excellent regularity
of the motility of the rectosigmoid of normal

18.5 Toilet Training
305
human beings, who usually empty the rectosigmoid all at once, every day. After 1 year of age,
we encourage the parents to give the patients only
three meals per day and no snacks in between. By
doing that, we try to produce a gastrocolic refl ex
only three times a day and not seven times, like
many children that have snacks in between. It is
much easier to toilet train a child that has two or
three bowel movements in a day at a predictable
time. The opposite, a patient that suffers from episodes of diarrhea followed by episodes of constipation is very diffi cult to toilet train.
We explain to the parents that the big enemies
of our patients are two:
A. Diarrhea
B. Constipation
We try to keep the patients right in between.
We want them to empty the rectosigmoid every
day and have soft but formed stool. We do all that
trial and error, and we educate the parents to
achieve our goal.
18.5 Toilet Training
It is very important to determine the future functional prognosis, as early as possible in patients
with anorectal malformations. When the patients
belong to the “poor prognosis” group (poor
sacrum, tethered cord, bladder neck fi stula), we
do not encourage them to try to toilet train their
children. We try not to create false expectations.
The toilet training in our patients with a good
or reasonably good functional prognosis usually
starts by the age of 2. If we achieve our goal of
having our patients free of constipation with the
use or without the use of laxatives and also if we
achieve our goal of producing one to three bowel
movements, at regular times, during the day at
predictable times, then it is relatively easy to toilet train the patients.
In cases with poor functional prognosis, we
encourage the family to start our bowel management program by the age of 3. We arbitrarily
selected the age of 3 because many children, at
least in the United States, are in diapers even
between 2 and 3 years of age. In addition, many
parents send the children to school by the age of
3, and we believe that the child must go to school
either toilet trained, like all other children, or
completely clean in the underwear, because of
the bowel management that we provide. We are
very much against the idea of sending a child to
school knowing that everybody is using normal
underwear except our patient. We believe that
that is not good from the psychological point of
view.
For the patients that have malformations with
a functional prognosis that is considered in the
middle of the spectrum, in other words, patients
that have about 50 or 60 % chance to have voluntary bowel movements by the age of 3, such
as cases with rectoprostatic fi stula, if the patient
is not toilet trained by the time that the parents
want to send him to school, we offer our bowel
management program and explain to the parents
that it will not be necessarily for life. Every
year, the patient should come to our clinic, and
we will try to stop the enemas (bowel management) and see how much bowel control the
patient has, when subjected to a regime that
includes giving three meals per day, laxatives,
and fi ber with the purpose of making the stool
bulky. If the patient does not gain bowel control,
we encourage the parents to go back to the
bowel management with enemas, for another
year, and come back later, because every year
we have more chances to toilet train the patient.
Every year, the patient will be more cooperative
and more interested in being clean with the
management plan.
Concerning the urinary control, we can very
much predict what kind of urinary function the
patient will have, based on the original anatomy
and the associated defects of the sacrum and
tethered cord. A normal baby, at that age of
1 year old, most likely is not toilet trained for
urine; however, it is very easy to observe that the
baby passes urine in episodes, about every 3 or
4 h, with a good stream and then remains completely dry in between voiding episodes. That
patient has good functional prognosis, and we do
not need a urodynamic study to know that he will
have urinary control. On the other hand, if we
see a 1-year-old baby dribbling urine constantly,
obviously the patient will have problems with
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