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X
- •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

98
6 Imaging
cystograms, and urodynamic studies, as well as a
frequent nephrologic monitoring. Patients with
neurologic defi cits including abnormal sacrum,
tethered cord, or complex cloacas are, by defi nition, urologic patients that require very close
follow-up monitoring of the urinary function.
Many of the complex cloacas eventually will
require urinary reconstructions including bladder
augmentations and Mitrofanoff.
For patients that have been operated on at other
institutions and come to us, for instance, for bowel
management due to fecal and/or urinary incontinence, we evaluate the entire colorectal anatomy,
pelvic anatomy, and urinary tract. We specifi cally
order a magnetic resonance imaging (MRI) study
with a specifi c technique that includes the placing
of a big Foley catheter in the rectum (Fig.
6.30a
and b ). This will allow us to see the location of the
rectum as related to the sphincter mechanism. In
addition, it is an excellent study to make a diagnosis of a posterior urethral diverticulum which is a
complication that we have seen in the past in
patients that were operated abdominoperineally,
having a rectourethral bulbar fi stula; the surgeons
found it diffi cult to reach the lower end of the
bowel, amputated the bowel, and left a piece of
rectum attached to the urethra (Figs. 6.30c and d ).
That piece of rectum becomes a diverticulum that
produces mucus, forms stones, and produces
pseudourinary incontinence, and in one specifi c
case, the patient developed an adenocarcinoma.
With the advent of laparoscopic procedures, we
are seeing again this problem of posterior urethral
diverticulum, when the surgeons try to approach
laparoscopically a rectourethral bulbar fi stula,
fi nding diffi cult to reach the lower end of the rectum Animation 5 (Posterior urethral Diverticulum
in a patient with a recto-urethral bulbar fi stula
approached laparoscopically). The voiding cystourethrogram may or may not show the posterior
urethral diverticulum and therefore is not the ideal
method of diagnosis. The diagnosis of this entity
is confi rmed cystoscopically, and the repair is
described in Chap.
22 .
When the babies come to us for the fi rst time
with anorectal malformations and did not have
spinal ultrasound during the fi rst 3 months of life,
the only way to know the location of the conus
terminalis is by an MRI study that is the most
accurate way to determine whether or not the
patient has tethered cord (Fig. 6.9c ).
Imaging plays a very important role in the
diagnosis, follow-up, and management of patients
already operated (see Chap. 19 ) .
References
1. Peña A (1987) Anatomical considerations relevant to
fecal continence. Semin Surg Oncol 3(3):141–145
2. Peña A (1996) Anorectal malformations. Semin
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3. Niedzielski J, Midel A (1998) Sacroiliac ratio in children:
natural evolution and clinical implications. Surg Childh
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4. Berdon WE, Baker DH, Santulli TV, Amoury R
(1968) The radiologic evaluation of imperforate anus.
An approach correlated with current surgical concepts. Radiology 90(3):466–471
5. Wangensteen OH, Rice CO (1930) Imperforate anus:
a method of determining the surgical approach. Ann
Surg 92(1):77–81
6. Berdon WE, Baker DH (1967) The inherent errors in
measurements of inverted fi lms in patients with
imperforate anus. Ann Radiol (Paris) 10(3):235–240
7. Narasimharao KL, Nair PM, Mitra SK, Pathak IC
(1984) Hypoxia during invertography. Indian Pediatr
21(12):971–973
8. Narasimharao KL, Prasad GR, Katariya S, Yadav K,
Mitra SK, Pathak IC (1983) Prone cross-table lateral
view: an alternative to the invertogram in imperforate
anus. AJR Am J Roentgenol 140(2):227–229
9. Willital GH (1971) Advances in the diagnosis of anal
and rectal atresia by ultrasonic-echo examination.
J Pediatr Surg 6(4):454–457
10. Schuster SR, Teele RL (1979) An analysis of ultra-
sound scanning as a guide in determination of “high” or
“low” imperforate anus. J Pediatr Surg 14(6):798–800
11. Oppenheimer DA, Carroll BA, Shochat SJ (1982)
Sonography of imperforate anus. Radiology 148(1):
127–128
12. Baunin C, Blancher A (1986) Radiologic examination
of anorectal malformations. Chir Pediatr 27(5):
239–245
13. Donaldson JS, Black CT, Reynolds M, Sherman JO,
Shkolnik A (1989) Ultrasound of the distal pouch in
infants with imperforate anus. J Pediatr Surg 24(5):
465–468
14. Tashev P, Chatalbashev N, Kazakov K (1991)
Application of ultrasonography in the evaluation of
imperforate anus. Folia Med (Plovdiv) 33(3):36–40
15. Wagner ML, Harberg FJ, Kumar AP, Singleton EB
(1973) The evaluation of imperforate anus utilizing
percutaneous injection of water-soluble iodide contrast material. Pediatr Radiol 1(1):34–40
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The value of transperineal injection for the diagnosis
of imperforate anus. Ann Surg 190(5):668–670
17. Kurlander GJ (1967) Roentgenology of imperforate
anus. Am J Roentgenol Radium Ther Nucl Med 100(1):
190–201
18. Kohda E, Fujioka M, Ikawa H, Yokoyama J (1985)
Congenital anorectal anomaly: CT evaluation.
Radiology 157(2):349–352
19. Ikawa H, Yokoyama J, Sanbonmatsu T, Hagane K,
Endo M, Katsumata K, Kohda E (1985) The use of
computerized tomography to evaluate anorectal
anomalies. J Pediatr Surg 20(6):640–644
20. Krasna H, Nosher JL, Amorosa J, Rosenfeld D (1988)
Localization of the blind rectal pouch in imperforate
anus with the CT scanner. Pediatr Surg Int 3:
114–119
21. Martuciello G, Taccone A, Fondelli P, Moran Penco
JM, Dodero P (1990) Tomografía Axial
Computerizada en las malformaciones anorectales:
¿Una indicación pre y postoperatoria? Cir Pediatr
4(3):173–178
22. Taccone A, Martucciello G, Dodero P, Delliacqua A,
Marzoli A, Salomone G, Jasonni V (1992) New concepts in preoperative imaging of anorectal malformation. New concepts in imaging of ARM. Pediatr
Radiol 22(3):196–199
23. McHugh K (1997) The role of radiology in children
with anorectal anomalies; with particular emphasis on
MRI. Eur J Radiol 26(2):194–199
24. Cremin BJ, Cywes S, Louw JH (1972) A rational
radiological approach to the surgical correction of
anorectal anomalies. Surgery 71(6):801–806
25. Lernau OZ, Jancu J, Nissan S (1978) Demonstration
of rectourinary fi stulas by pressure gastrografi n
enema. J Pediatr Surg 13(6):497–498
26. Gross GW, Wolfson PJ, Pena A (1991) Augmentedpressure colostogram in imperforate anus with fi stula.
Pediatr Radiol 21(8):560–562
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10.1016/j.

Bowel Preparation in Pediatric Colorectal Surgery
7
Colorectal operations are considered “contaminated procedures” and represent, by defi nition,
cases with a higher risk of infection. Therefore,
patients must be subjected to some forms of bowel
preparation prior to the operation. In addition, the
use of prophylactic antibiotics must be considered.
Through the last 30 years, we have been following the evolution of the concepts related with
the different modalities of bowel preparation, as
well as the use of different antibiotics by mouth,
intravenously, and pre- and postoperatively.
Most of the literature is related with adults.
There are an enormous number of publications
related to adult conditions. We selected only a
few papers representative of the tendency to
avoid mechanical bowel preparation [ 1 – 4 ] and
antibiotics. Some surgeons found no evidence to
support the use of mechanical bowel preparations. Even more, some publications present data
indicating that the bowel preparations seem to
result in a higher incidence of infections and
dehiscence! The incidence of wound infections
in the adult literature varies from 4 to 19 % with
minor variations in favor of those patients who
did not receive preoperative colonic preparation.
The question for us, pediatric surgeons, is if
we should change our routines, based on the
information obtained from adult data. Our personal answer is no. The incidence of wound
infections reported in adult patients with and
without bowel preparations is still much higher
than in our cases [
tive colostomies without a single case of infec-
5 ]. If we closed 649 consecu-
tion, following preoperative management, we
fi nd no convincing reason to change. We understand that perhaps, we could have the same good
results without bowel preparation; however, it is
not easy to change a routine that resulted in zero
infections. We also suspect that a meticulous surgical technique is the key to avoid complications,
rather than the bowel preparation or the antibiotics. Interestingly, during the last 30 years, we
have changed the type of antibiotics used preand postoperatively according to the recommendations of the epidemiology departments of the
institutions in which we worked, yet the results
were the same: no infections.
According to Breckler et al. [
whelming majority of pediatric surgeons in the
United States still use mechanical bowel preparation and preoperative intravenous antibiotics.
Leys et al. [
anastomotic leaks and infections in pediatric
patients who underwent a mechanical bowel preparation. Breckler et al. reported 14 % of wound
infections in colostomy closures and no differences between those who received antibiotics and
those patients who did not [ 8 ]. Serrurier et al.,
also in pediatric cases [ 9 ], found signifi cantly
higher number of wound infections (14 %) in a
series of 272 colostomy closures, of patients who
received mechanical bowel preparation, whereas
only 5 % of those without preparation suffered
from this complication. Similar results were
reported by others [ 10 , 11 ]. Pennington et al. [ 12 ]
in a retrospective analysis of the series of 42
7 ] found slightly higher number of
6 ], the over-
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_7, © Springer International Publishing Switzerland 2015
101

102
7 Bowel Preparation in Pediatric Colorectal Surgery
American Children’s Hospitals, (5,473 patients)
not surprising, found that only 22.9 % of all
patients received an “evidence-based preparation.” However, the “evidence-based guidelines”
that they allude to are related with adult patients.
The question comes again: Should we change our
routines based on the adult literature? The question is even more diffi cult to answer when our
results are excellent. In summary, we are not convinced that following adult “evidence- based” routines will benefi t our patients.
We are very proud of our excellent record of
zero wound infections in colostomy closures. We
close all wounds primarily and leave no drains.
The patients received irrigations of the proximal
stoma, with saline solution and intravenous antibiotics during anesthesia and 48 h postoperatively.
On the other hand, we had cases of infections or
minor dehiscent anoplasties in cases subjected to
primary pull-throughs without a colostomy. Those
patients received GoLYTELY ® (total bowel preparation). The dehiscences that we have seen
occurred usually between the 5 and 8 days postoperatively while the patients are still fasting. We
take those patients to the operating room, resutured the dehiscent areas, and prolong the period
of fasting for 2–3 more days. A complete dehiscence will receive a colostomy, which is a very
unusual event. A question to be answered is if a
dehiscence, occurring in a case with bowel preparation, has less serious consequences than in cases
without bowel preparation. We believe it does.
The type of colonic preparation that we recommend varies, depending on the type of surgical
procedure that the patient is going to receive, as
well as the specifi c circumstances of the patient.
Thus, the bowel preparation required for a
newborn baby that is going to be subjected to a
primary, major, colorectal procedure without a
colostomy is different than the one required for
an older patient.
There is plenty of evidence related with the
safety of using GoLYTELY
1
GoLYTELY® PEG 3350 236 g, sodium sulfate 22.74 g,
sodium bicarbonate 6.74 g, sodium chloride 5.86 g, and
potassium chloride 2.97 g (4,000 mL) [regular and pineapple fl avor].
® 1
(polyethylene gly-
col electrolyte solution) to clean the entire gastrointestinal tract in pediatric patients [ 13 – 18 ].
7.1 Major Procedures
Major procedures include primary or secondary
pull-throughs for anorectal malformations, for
Hirschsprung’s disease, or for idiopathic constipation, performed without a protective colostomy.
Anoplasties and resection of rectal prolapse are
also included in this category. These patients must
be admitted, most of the time, 24 h before the
operation. Severely constipated or fecally
impacted patients, sometimes, are admitted 2 days
before surgery to be subjected to our protocol of
disimpaction before the cleaning of the gastrointestinal tract (See Chap. 25 , Sect. 25.7.1 ).
The morning of admission the patient is only
allowed to take clear fl uids by mouth. Usually
around noon time, the administration of
GoLYTELY ® 2 is started, at the rate of 25 mL/
kg/h until clear. Most of the time, this is administered through a nasogastric tube. Occasionally,
some adolescents volunteer to drink GoLYTELY ®
at the right speed, in order to try to avoid a nasogastric tube. Many of them try, yet, usually the
attempt is unsuccessful because they do not drink
fast enough or they have nausea, and therefore,
they need the nasogastric tube. It usually takes
about 4 h for the colon to be completely cleaned.
Some patients suffer from nausea and vomiting
during the administration of GoLYTELY
that happens, the patient benefi ts from the administration of an antiemetic medication, but we continue the administration of the GoLYTELY ® , at a
slightly slower rate, because we have seen that
most of the GoLYTELY ® still goes through the
pylorus and through the bowel. During the
administration of GoLYTELY ® , the patient usually suffers from a certain degree of abdominal
distention and cramps. Soon enough, they start
passing stool through the rectum. Later on, this
becomes liquid stool that becomes more and
more clear, until it is free of stool. Patients with
Hirschsprung’s disease frequently require rectal
2
See footnote 1.
®
. When

7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period
103
irrigation to help relieve this distention during the
bowel prep. After 4 h of the administration of
GoLYTELY ® , the nurse or the resident must see
what is coming out through the rectum of the
patient. A yellow or greenish color represents
bile that is excreted in the bowel and is considered acceptable. On the other hand, the presence
of particles of fecal matter is considered unacceptable, and therefore, the administration of
GoLYTELY ® should continue. Subsequently, the
resident or the nurse should check what is coming out of the rectum every hour as the prep continues to run, until the goal of a clean colon is
achieved. At that point, the nasogastric tube can
be pulled out, and the patient is allowed to drink
clear fl uids. (Clear fl uids by mouth are not
allowed during the administration of the
GoLYTELY ® because we were told by the company that produces GoLYTELY ® that the glucose
contents allow for infl ow of fl uid into the colon
lumen, leading to dehydration.)
Most patients pass clear liquid through the
rectum after 4 h, but others require a longer
period of time. Occasionally, we see patients that
continue having GoLYTELY ® through the night,
and then come to the operating room, only for us
to fi nd out that they are still not clean.
It has been our experience that many patients
who received GoLYTELY ® the day before an
operation come to the operating room suffering
from some degree of dehydration and a mild
degree of metabolic acidosis. This seems to be
more signifi cant in babies. Therefore, it is our
routine to administer intravenous fl uids during
the entire process to patients younger than 2 years
of age. In patients older than 2 years of age, intravenous fl uids are desirable, but not vital.
In patients with a signifi cant degree of megacolon and chronic fecal impaction, it is very
important to go through the process of fecal
disimpaction prior to the administration of
GoLYTELY
®
. The administration of
GoLYTELY ® through a nasogastric tube in
patients with fecal impaction and severe megarectosigmoid sometimes makes them feel very
uncomfortable. Their abdomen becomes very
distended, they complain of severe cramps, and
they feel very sick. The protocol of fecal disim-
paction consists of the administration of three
enemas per day and daily radiologic monitoring,
to be sure that the colon is free of fecal impaction
(see chapter on Idiopathic constipation). When
this is achieved, the patient can be admitted to
follow the protocol of administration of
GoLYTELY ® .
Once the patient is in the operating room,
prior to the operation, we routinely pass a large
Foley catheter through the rectum, to evacuate
the remaining bowel prep fl uid that is in the
colon.
Otherwise, this fl uid becomes an inconvenience
during the surgical procedure. Also, we pass that
tube, in order to do a last irrigation before we start
the operation. Surprisingly, sometimes, even when
it was reported that the patient was clean, we fi nd
stool in the colon. Occasionally, we have to cancel
a case because we were not successful in cleaning
the colon even in the operating room. We are very
proud of our postoperative results.
7.2 Primary Procedures
for the Treatment
of Anorectal Malformation
During the Newborn Period
There are some malformations that we repair primarily without a colostomy during the newborn
period. It has been our experience that these
patients behave better from the point of view of
the possibility of infection, dehiscence, and
retraction, as compared to older patients. We
speculate that, perhaps, due to the fact that the
meconium has not been colonized during the fi rst
few days of life, the chances of a wound infection
may be less.
Full-term, stable newborns, who are otherwise healthy, without major associated defects,
born at our institution or nearby and having what
we call a “benign anorectal malformation,”
receive a primary procedure during the newborn
period without a full bowel preparation. These
cases include rectovestibular fi stulas, recto-perineal fi stulas in males and females, anorectal malformation without fi stula in both male or female
patients, or rectourethral bulbar fi stulas. In the

104
7 Bowel Preparation in Pediatric Colorectal Surgery
last two types of malformations, we expect to
see the distal rectal end, full of gas, located
below the coccyx, on the cross table lateral fi lm
(See Chap. 25 , Sect. 25.7.1 ), meaning that we
will be able to reach the rectum comfortably
through a posterior sagittal incision without taking unnecessary risks of injuring the urinary
tract. These patients are operated on at our institution usually within the fi rst 48 h of life without
bowel preparation.
7.3 Primary Pull-Through in Newborn Patients with Hirschsprung’s Disease
When a baby is born at our institution, or is
brought during the fi rst days of life, with the
diagnosis of Hirschsprung’s disease, we fi rst confi rm the diagnosis, and then, we introduce a nasogastric tube for gastric decompression and start a
program of rectal irrigations, performed as often
as necessary to decompress the colon. It is very
important for doctors and nurses to understand
the difference between a rectal irrigation and an
enema. A patient with Hirschsprung’s disease has
a congenital, intrinsic incapacity to empty the
colon due to a severe motility disorder. Therefore,
in general, enemas are to be avoided, as they may
simply be retained in the colon and can worsen
the distention. Irrigation, on the other hand, consists of passing a large-lumen tube, through the
rectum and irrigating with small amounts
(10 mL) of saline solution at a time. The saline
solution is injected with a catheter-tip syringe
through the tube just to clear its lumen, to allow
decompression of the colonic contents.
Characteristically, the patient passes gas and liquid stool in an explosive manner through the
lumen of the tube. A dramatic clinical improvement can be observed in these babies after every
irrigation. Within a couple of days, the abdomen
is usually fl at, and what we obtain with the irrigations is only bile. At that point, and provided the
diagnosis has been histologically confi rmed, the
patient is ready to undergo a primary pullthrough. We do not administer GoLYTELY
these kinds of patients. Most of these operations
®
in
are performed without a protective colostomy;
yet, we request consent for a colostomy in case it
is necessary.
7.4 Patients with Hirschsprung’s Disease with Enterocolitis After the Neonatal Period
A baby with enterocolitis is an extremely delicate,
sick patient. Therefore, he or she must be managed
in an expeditious way. The management includes
recto-colonic irrigations, adequate aggressive
hydration, and administration of metronidazole
(intravenously at the beginning, eventually by
mouth and later through rectal irrigations).
Occasionally we add broad-spectrum antibiotics.
The irrigations are performed as often as necessary to decompress the colon and alleviate the
abdominal distention. It is important to keep in
mind what is happening in the colon. Stasis
occurs, leading to bacterial overgrowth, secretory
diarrhea, and dehydration. It is this cycle that
must be broken by the irrigations. During the
acute stages of enterocolitis, the irrigations will
produce a characteristic type of stool that is particularly fetid and frequently with abnormal bacteria, such as Clostridium diffi cile . We keep these
patients fasting, receiving parenteral nutrition
and with the irrigations per rectum until they
become asymptomatic. Fairly soon, the patient
has a fl at abdomen, no vomiting, and no diarrhea.
In addition, the fetid stools disappear and are
replaced by clear fl uid with bile. At this point, we
consider that the patient is free of enterocolitis
and therefore ready to be operated on. If we feed
these patients, they may develop enterocolitis
again. Therefore, we prefer to do the operation at
this point. These patients require longer periods
of rectal irrigations and fasting than neonates
before they are ready for an operation. In fact,
some of these patients receive a diverting colostomy when we feel that the patient is not improving with the medical treatment described.
Sometimes it takes 1 or 2 weeks for the patient to
be ready for the operation to be sure that there is
no evidence of enterocolitis. We do not administer GoLYTELY
®
in these kinds of patients.

References
105
7.5 Patients with Hirschsprung’s Disease Beyond the Neonatal Period, Without Enterocolitis
Occasionally, we see patients at school age, or
even adolescence, who come to us suffering
from Hirschsprung’s disease. They usually have
a very severe megacolon and yet no history of
episodes of enterocolitis. We believe that this
type of patient suffers from a “benign type” of
Hirschsprung’s disease. For reasons that we do
not know, these patients never had an episode
of enterocolitis and behave in a more benign
way than the rest of the patients with
Hirschsprung’s disease. The colon preparation
in these patients is the same as the one described
for major pull- throughs. In other words, if the
patient is fecally impacted, we fi rst disimpact
them, and then we bring them to the hospital to
administer GoLYTELY ® , 1 or 2 days before the
operation.
7.6 Colostomy Closures
The preparation of the bowel in these patients is
much more simple. The patient has a normal
breakfast the day prior to surgery and comes to
the hospital thereafter. The rest of the day, the
patient is maintained on clear fl uids by mouth
until 6 h before surgery. During that day, the
nurses irrigate the proximal stoma with saline
solution. There is no need to irrigate the distal
stoma, which is, by defi nition, clean and free of
stool. The patient receives irrigations through a
no. 10 Foley catheter with injections of 10–20 mL
of saline solution, allowing the saline to come
back out through the stoma. This maneuver is
repeated as many times as necessary, until the
saline solution recovered is clear. We do not use
GoLYTELY
When the patient has two stomas that are
located too close one to the other, and both stomas are included under the same stoma bag, it is
our routine to irrigate both stomas the day before
surgery. The same routine is applied for patients
who have a loop colostomy.
®
for this preparation.
We have done over 700 colostomy closures, and
we are very proud of the fact that none of our
patients suffered from a wound infection [ 5 ]. None
of the patients had any kind of Penrose drainage
left in the wound or in the abdomen, and all wounds
were closed primarily including a subcuticular
stitch. Only two patients had postoperative complications: one of the patients had an anastomosis
dehiscence, and the other patient had a colonic perforation proximal to the anastomosis. We do not
have an explanation for that complication.
7.7 Patients with a Colostomy
Who Will Have a Repair
of an Anorectal
Malformation
When the colostomy is totally diverting (completely separated stomas, with the bag covering
only the proximal stoma), and the surgeon is sure
that the malformation can be repaired from below
(perineally or posterior sagittally), it is not necessary to irrigate the proximal stoma. It is only necessary to irrigate the distal one.
Sometimes, even when the rectum is considered reachable from below, the length of the
colon available between the distal stoma and the
most distal end of the rectum is so short that it is
fair to assume that this distance will interfere
with the pull-through, making it necessary to
mobilize the proximal stoma. In such cases, it is
necessary to clean the entire gastrointestinal tract
with GoLYTELY
®
as previously described. In
addition, in certain cases that may require a vaginal replacement, a clean colon allows the surgeon
to consider the bowel from the proximal stoma as
an option for the neovagina.
References
1. Stellato TA, Danziger LH, Gordon N, Hau T, Hull
CC, Zollinger RM Jr, Shuck JM (1990) Antibiotics in
elective colon surgery. A randomized trial of oral, systemic, and oral/systemic antibiotics for prophylaxis.
Am Surg 56(4):251–254
2. van Geldere D, Fa-Si-Oen P, Noach LA, Rietra PJ,
Peterse JL, Boom RP (2002) Complications after

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7 Bowel Preparation in Pediatric Colorectal Surgery
colorectal surgery without mechanical bowel preparation. J Am Coll Surg 194(1):40–47
3. Wille-Jørgensen P, Guenaga KF, Matos D, Castro AA
(2005) Pre-operative mechanical bowel cleansing or
not? An updated meta-analysis. Colorectal Dis
7(4):304–310
4. Dahabreh IJ, Steele DW, Shah N, Trikalinos TA
(2014) Oral mechanical bowel preparation for
colorectal surgery [Internet], Report No.
14-EHC018-EF. Rockville: Agency for Healthcare
Research and Quality (US)
5. Bischoff A, Levitt MA, Lawal TA, Peña A (2010)
Colostomy closure: how to avoid complications.
Pediatr Surg Int 26(11):1087–1092. doi:
s00383-010-2690-6
6. Breckler FD, Fuchs JR, Rescorla FJ (2007) Survey of
pediatric surgeons on current practices of bowel preparation for elective colorectal surgery in children. Am
J Surg 193(3):315–318
7. Leys CM, Austin MT, Pietsch JB, Lovvorn HN 3rd,
Pietsch JB (2005) Elective intestinal operations in
infants and children without mechanical bowel preparation: a pilot study. J Pediatr Surg 40(6):978–981
8. Breckler FD, Rescorla FJ, Billmire DF (2010) Wound
infection after colostomy closure for imperforate anus
in children: utility of preoperative oral antibiotics. J
Pediatr Surg 45(7):1509–1513. doi:
jpedsurg.2009.10.054
9. Serrurier K, Liu J, Breckler F, Khozeimeh N, Billmire
D, Gingalewski C, Gollin G (2012) A multicenter
evaluation of the role of mechanical bowel preparation in pediatric colostomy takedown. J Pediatr Surg
47(1):190–193. doi:
10. Víctor D, Burek C, Corbetta JP, Sentagne A, Sager C,
Weller S, Paz E, Bortagaray JI, Lopez JC (2012)
Augmentation cystoplasty in children without preop-
10.1016/j.jpedsurg.2011.10.044
10.1007/
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erative mechanical bowel preparation. J Pediatr Urol
8(2):201–204. doi:
11. Leal AJ, Tannuri AC, Tannuri U (2013) Mechanical
bowel preparation for esophagocoloplasty in children:
is it really necessary? Dis Esophagus 26(5):475–478.
10.1111/j.1442-2050.2012.01378.x
doi:
12. Pennington EC, Feng C, St Peter SD, Islam S, Goldin
AB, Abdullah F, Rangel SJ (2014) Use of mechanical
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evidence-based? J Pediatr Surg 49(6):1030–1035.
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doi:
13. Tuggle DW, Hoelzer DJ, Tunell WP, Smith EI (1987)
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17. Engum SA, Carter ME, Murphy D, Breckler FM,
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10.4103/0189-6725.120889
doi:
10.1016/j.jpurol.2011.01.015

Recto-perineal Fistula
8
8.1 Defi nition, Frequency,
and Prognosis
Perineal fi stula is an anal malformation in which
the anal opening is located anterior to the center
of the sphincter. In females, the anal opening is
located somewhere between the location of the
normal sphincter and the female genitalia in the
area known as the perineum or perineal body.
The anterior mislocation of the orifi ce could be
minimal (a few millimeters) (Fig. 8.1 ) or severe,
becoming borderline with a malformation called
vestibular fi stula (Fig. 8.2 ). When the anal orifi ce
is located at the junction of both labia majora, the
malformation sometimes receives the French
name “fourchette malformation” (Fig. 8.3 ), which
is considered a defect intermediate between the
vestibular and perineal area. Yet, most anorectal
malformations in females can be clearly differentiated between perineal and vestibular. This is,
perhaps, the anorectal defect subjected to more
controversies, both in semantics and treatments.
In female patients, some surgeons use different
terms to refer to this condition, including “ectopic
anus” and “anterior displacement of the anus”
[
1 – 13 ]. We prefer the term perineal fi stula for the
following reasons:
• The anal opening is most frequently strictured
or stenotic.
• There is no anal canal.
• The orifi ce is not surrounded 360° by a sphinc-
ter mechanism.
These facts make us believe that this is not a
real anus. For similar reasons, we believe that
in order to call a malformation “anterior anus,”
it would be necessary for the patient to have a
non- stenotic orifi ce, with normal anal canal,
and surrounded 360° by a sphincter mechanism
(as electrically demonstrated). If we accept
this, as the defi nition of an anterior anus, then
we must say that we have never seen that specifi c type of defect. The fact that we have not
seen such defect does not mean that it does not
exist, but it certainly means that it must be
Fig. 8.1 Picture of a perineal fi stula with minimal
anterior mislocation of the opening
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_8, © Springer International Publishing Switzerland 2015
107

108
a b
Fig. 8.2 Perineal fi stula with a signifi cant anterior mislocation of the anal opening. ( a ) Picture. ( b ) Diagram
8 Recto-perineal Fistula
Fig. 8.3 “Fourchette” type of fi stula
extremely rare. In addition, we believe that
in the event of seeing a case suffering from
such condition, we would be very hesitant to
operate.
Perineal fi stula is one of the most common
anorectal defects. Our series includes 90 males
and 84 females, occupying the second place in
frequency in males after the rectourethral fi stulas
(bulbar and prostatic) together and the third place
in females after vestibular fi stula and cloacas.
However, we have reasons to believe that the
perineal fi stula is perhaps one of the two most
frequent defects in females. Most likely, the
majority of perineal fi stula cases are not referred
to us, probably because they are considered easy
to repair. Ours is a referral center, and we receive
mainly complex and (or) complicated patients
from other institutions. That may be the reason to
explain the elevated number of cloacas that we
have, as well as the rather low number of cases
with perineal fi stula. We conclude that the relative
frequency of presentation of these malformations
in our series is not representative of the frequency
in the general population.
Perineal fi stulas are the most benign of all
anorectal defects in terms of functional prognosis
for bowel control. In fact, in our experience,
100 % of the patients operated by us have bowel
control provided the sacrum is normal. The
majority of patients have a good sacrum, but
there are exceptions, as we will be discussing
below.
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