Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_927_Библиотеки_им_академика_М_И_Перельмана.pdf
ANAL AND PERIANAL REGION
81
utback procedure is another alternative for patients with per-
A c
ineal stulas and can be performed when one is dealing with very
small (premature) or very sick babies. is procedure consists of
making a deep cut in the posterior wall and the sphincter and suturing rectal wall to the skin. e functional results seem to be as good
as with an anoplasty, although cosmetically the results are less than
optimal. If the surgeon is not familiar with the delicate, meticulous
technique required for an anoplasty, it is preferable to perform a cutback procedure.
Limited P
A limi
stulas. ese patients require only irrigations of the colon distal to
the colostomy preoperatively. e patient is placed in the prone position with the pelvis elevated. A midline, posterior sagittal incision is
used, extending from the vestibule to a point located halfway along
the distance to the coccyx. e sphincter mechanism is divided,
including only the muscle complex and the external sphincter. Uniform traction is applied to the stula, and the lower rectum is meticulously mobilized posteriorly to be placed within the limits of the
sphincter mechanism. Special attention is directed to the separation
of rectum from the vagina because both structures share a common
wall. One must create two walls out of one, which is a very delicate
maneuver and may be the main source of complications when it is
not performed properly.
osterior Sagittal Anorectoplasty with or without
P
osterior Sagittal Anorectoplasty
ted posterior sagittal anorectoplasty is used to repair vestibular
Laparotomy/Laparoscopy
A p
osterior sagittal anorectoplasty is used to repair all other defects
except for cloacae. A Foley catheter is placed in the bladder, and the
patient is placed in the prone position with the pelvis elevated. e
midsagittal incision extends from the middle portion of the sacrum
down through the anal dimple. e entire sphincter mechanism is
divided, and the rectum is then meticulously separated from the urinary tract, preserving the urethra intact, as well as the vas deferens,
seminal vesicles, and prostate. e most delicate part of the operation is the separation of the rectum from the urinary tract because
both structures share a common wall, without a plane of separation,
for about 5 to 20 mm above the stula site. is common wall also
exists between the rectum and the posterior urethra, even in patients
without a stula.
Once the rectum has been separated it is mobilized down to the
perineum and placed within the funnel-like sphincter, and an anoplasty is performed. Sometimes it is necessary to tailor the rectum in
order for it to be placed within the sphincter mechanism.
In cases with a recto-bladder-neck stula (which represents
10% of all cases in male patients), it is necessary to nd and mobilize the rectum via a laparotomy or laparoscopy. e abdominal
approach is used rst, the rectum is identied, and the stula is
ligated. A posterior sagittal incision is performed to create the path
through which the rectum will be pulled down, within the limits of
the sphincter mechanism. Tapering is performed when a bulbous
rectum is identied. An anoplasty represents the last part of the
operation.
osterior Sagittal Anorectovaginourethroplasty with
P
or without Laparotomy
osterior sagittal anorectovaginourethroplasty is used to repair
A p
a cloaca. ese operations are usually long, technically demanding
procedures that must be performed by surgeons with special dedication and experience. Via a posterior sagittal approach, the rectum
is separated from the genitourinary tract; the urethra and vagina
frequently are separated and mobilized down, or both may be mobilized together to create a separate urethra, vagina, and rectum.
About 40% of the time, it is necessary to open the abdomen to
reach a very high rectum, a very high vagina, or both. Patients with
very small or absent vaginas may require a form of vaginal replacement, which is usually performed with colon or small bowel. A signicant number of patients require a ureteral reimplantation. Others
need a bladder augmentation and some form of continent diversion,
which means the creation of a tube (appendix, ureter, fallopian tube,
or tubularized bowel) implanted in the bladder with an antireux
mechanism to avoid leakage of urine and allow the emptying of the
bladder through use of intermittent catheterization.
Management of Functional Sequelae
e most devastating functional sequela seen in patients with anorectal malformations is fecal incontinence, which occurs in at least 30%
of these patients. is complication occurs either because the patients
are born without the potential for bowel control (based on the type
of malformation and the quality of the sacrum), as previously mentioned, or because some form of damage occurs during the repair of
a defect with a good prognosis. A special form of bowel management
program has been designed to keep these patients clean and improve
their quality of life. Most patients have fecal incontinence and a tendency toward constipation. e bowel management implemented in
that type of patient includes the use of large enemas given every day
to keep the colon empty. e fact that these patients tend to have
constipation happens to be serendipitous because it keeps the patient
completely continent in between enemas.
In some patients the rectosigmoid or some other portion of their
colon was resected at the time of the main repair. is procedure was
used in patients who underwent endorectal techniques, or else a portion of the colon was sometimes resected for various other reasons.
e nal consequence is a tendency to experience diarrhea. In these
types of cases, the bowel management program includes not only
the use of daily enemas but also the administration of a constipating
diet and medication to slow down the colon motility. is last group
of patients is more dicult to manage. Overall, in our experience,
95% of patients can be kept continent through use of a form of bowel
management.
Once the patient is continent with the implementation of a daily
enema through the rectum, and as the patient grows and wants to
become more independent, an operation called a continent appendicostomy is oered. e appendix is implanted at the patient’s umbilicus. e cecum is usually plicated around the appendix to create
a one-way valve mechanism. For patients who lost their appendix
in the past, a continent neoappendicostomy is created by making a
neoappendix with a tubularized ap of cecum plus a plication of the
cecum around it to provide continence.
Constipation is the most common sequela experienced by patients
who were born with an imperforate anus and underwent a repair in
which the original rectum and colon of the patient were preserved.
Paradoxically, the lower the defect, the worse the constipation expe
ienced by the patient. is symptom must be treated energetically
r
because untreated constipation creates more constipation. e degree
of constipation also correlates with the degree of megarectum that
the patient experienced originally. e megarectum is more severe
in patients subjected to a transverse colostomy and is the worst in
patients with loop colostomies that allow the spillage of stool from
proximal to distal stoma. Severe constipation and chronic fecal
impaction may subsequently provoke overow pseudoincontinence,
which is another reason to treat the constipation to ensure that the
rectum empties every day.
Urinary incontinence in male patients with imperforate anus
occurs only in those born with an absent sacrum or in those who sustained nerve damage during the repair of their otherwise benign anorectal malformation. In female patients, on the other hand, urinary
-
82
AnorectAl congenitAl DisorDers
continence occurs in 20% of girls who have a cloaca with a com-
in
mon channel shorter than 3 cm and in 70% of girls who have a cloaca
and a common channel longer than 3 cm. Most of these patients can
be kept dry through the use of intermittent catheterization.
u
S
B
ischo A, Levitt M, Bauer C, et al. Treatment of fecal incontinence
Bischo A, Levitt MA, Peña A. Bowel management for the treatment of pedi-
Bischo A, Peña A, Levitt MA. Laparoscopic-assisted PSARP—the advantages
Bischo A, Tovilla M. A practical approach to the management of pediatric
Chatoorgoon K, Peña A, Lawal T, etal. Neoappendicostomy in the manage-
Levitt MA, Bischo A, Breech L, Peña A. Rectovestibular stula—rarely recog-
S t
g g e
with a comprehensive bowel management program. J Pediatr Surg.
2009;44:1278–1284.
atric fecal incontinence. Pediatr Surg Int. 2009;25:1027–1042.
of combining both techniques for the treatment of anorectal malformations with recto-bladderneck or high prostatic stulas. J Pediatr Surg.
2013;48:367–371.
fecal incontinence. Semin Pediatr Surg. 2010;19:154–159.
ment of pediatric fecal incontinence. J Pediatr Surg. 2011;46:1243–1249.
nized associated gynecologic anomalies. J Pediatr Surg. 2009;44:1261–1267.
e d
R
e
a d i n g
S
Levitt MA, Bischo A, Peña A. Pitfalls and challenges of cloaca repair; how
to reduce the need for reoperations. J Pediatr Surg. 2011;46:1250–1255.
Levitt MA, Soffer SZ, Peña A. Continent appendicostomy in the bowel
management of fecal incontinent children. J Pediatr Surg. 1997;32:
1630–1633.
Peña A. Anorectal malformations. Semin Pediatr Surg. 1995;4:35–47.
Peña A. Posterior sagittal anorectal plasty; results in the management of 332
cases of anorectal malformations. Pediatr Surg Int. 1988;3:4–104.
Peña A. Surgical management of persistent cloaca: results in 54 patients treat-
ed with a posterior sagittal approach. J Pediatr Surg. 1989;24:590–598.
Peña A. Total urogenital mobilization—an easier way to repair cloacas.
J Pediatr Surg. 1997;2:263–268.
Peña A, Migotto-Krieger M, Levitt MA. Colostomy in anorectal malforma-
tions: a procedure with serious but preventable complications. J Pediatr
Surg. 2006;41:748–756.
Rangel SJ, Lawal TA, Bischo A, etal. e appendix as a conduit for ante-
grade continence enemas in patients with anorectal malformations: les-
sons learned from 163 cases treated over 18 years. J Pediatr Surg. 2011;46:
1236–1242.
Rich M, Brock W, Peña P. Spectrum of genitourinary malformations in pa-
tients with imperforate anus. Pediatr Surg Int. 1988;3:110–113.
Torres P, Levitt MA, Tovilla JM, etal. Anorectal malformations and Down’s
syndrome. J Pediatr Surg. 1998;33:1–5.
H
D
INTR
ODUCTION AND HISTORY
irschsprung disease represents a common cause of intestinal
H
obstruction in newborns. ese patients do not have an actual
mechanical obstruction but rather a serious disturbance in normal
colonic peristalsis because of a lack of ganglion cells. e disease was
named aer Dr. Harald Hirschsprung, who presented what is considered a classic description of this condition at the Pediatric Congress in Berlin in 1886. His description, however, referred only to a
congenital dilation of the colon, without a real understanding of the
histologic features and pathogenesis.
e recognition that aganglionosis in the distal part of the colon
is the main cause of symptoms in these patients led to the surgical
treatment used presently. Swenson and Bill described the rst such
approach, consisting of resection of the aganglionic segment and
pull through of a normal piece of colon to be connected to the anal
canal. Variations of this basic principle were subsequently introduced
into the literature, including the Duhamel procedure, the Rehbein
approach in 1959, the Soave operation, the Boley modication,
and the De La Torre transanal resection of aganglionic bowel. More
recently, laparoscopic techniques have been added to the treatment
approach.
ETIOLOGY, PATHOPHYSIOLOGY, AND INCIDENCE
e absence of ganglion cells in the submucosal (Meissner)
Th
plexus and in the intermuscular (Auerbach) plexus in the distal
intestine, as well as a marked increase in nerve fibers that extend
into the submucosa, are the hallmarks of this condition. Patients
also have an increase in acetylcholinesterase activity. The lack of
ganglion cells affects the most distal part of the rectosigmoid in
about 80% of cases—the so-called “typical cases” of Hirschsprung
disease. The extent of the aganglionosis varies; it may reach anywhere from the descending colon to the transverse colon, where
it is called “long-segment Hirschsprung disease,” and it may even
affect the entire colon (“total colonic aganglionosis”). Reports
have been made of patients with universal aganglionosis (total
intestinal aganglionosis), a condition that thus far has been
incompatible with life.
e fecal stasis that occurs in the proximal normal bowel does
not produce a fecal impaction alone, as in cases of idiopathic constipation. ese patients have other poorly understood functional
abnormalities that may explain other more serious symptoms. e
fecal stasis leads to bacterial overgrowth, which produces explosive
diarrhea, abdominal distention, fever, and a very serious toxic condition. An inammatory inltrate of the intestinal mucosa occurs,
the mucosa becomes ulcerated, and bacteria then traverse the
intestinal epithelium. Abnormal bacteria proliferate, particularly
Andr
ea Bischoff and Alberto Peña
lostridium dicile. is condition is called “enterocolitis” and may
C
occur from the rst hours aer the baby is born before he or she
undergoes surgery or even aer completion of a successful operation. Unknown factors contribute to this serious and mysterious
condition. e babies become extremely sick, with development of
an endotoxemia that is lethal if it is not treated aggressively and
eectively.
e incidence of Hirschsprung disease has been reported to be 1
in 4000 to 1 in 7000. e disease has a denite male preponderance
except for the long-segment type of Hirschsprung disease, in which
the ratio seems to be 1:1 male to female. Ospring of male and female
patients with long-segment Hirschsprung disease have an approximately 20% chance of being aected.
CLINIC
Traditionally, Hirschsprung disease was suspected in children who
had constipation. However, during the past several decades the index
of suspicion for this condition has increased, leading to earlier diagnosis. In the United States today, it is rather unusual to nd an undiagnosed school-aged patient with this condition.
e most common manifestation of Hirschsprung disease during
the newborn period is the lack of passage of meconium during the
rst 24 hours, which is highly suspicious for Hirschsprung disease.
Subsequently, the abdomen becomes distended and the baby vomits.
Symptoms progress as in any other case of colonic obstruction. e
baby looks toxic, lethargic, dehydrated, and septic. Rectal stimulation with a thermometer or a nger provokes characteristic explosive bowel movements, followed by passage of large amounts of fetid
gas, giving the baby temporary relief. However, a few hours later the
symptoms recur.
A small percentage of patients may survive without medical help.
In time, clinical signs of severe constipation, abdominal distension,
and failure to thrive develop.
Untreated patients with Hirschsprung disease who survive and
reach school age have severe megacolon. These patients must be
differentiated from patients with idiopathic constipation. Patients
with Hirschsprung disease have a large amount of stool located
in the very dilated proximal colon. However, the distal, aganglionic segment is usually empty. Patients with severe idiopathic
constipation have a megarectum and fecal impaction located all
the way down to the area of the anal canal. A huge fecal impaction can be felt upon examination with a finger, whereas patients
with Hirschsprung disease are described as having an empty rectum. Patients with Hirschsprung disease characteristically do not
soil their underwear. Only a very small percentage of all patients
with Hirschsprung disease reach this age. Most undiagnosed
patients die in the early stages of the condition because of a lack
of treatment.
AL MANIFESTATIONS
83
84
HirscHsprung Disease
DIAGNOSIS
A
high index of suspicion is the most important element for the early
diagnosis and treatment of Hirschsprung disease. A plain abdominal
radiograph shows very dilated loops of bowel. Unfortunately, during the newborn period, it is extremely dicult to dierentiate small
bowel from large bowel on a plain abdominal lm. Aected patients
have a dilated colon proximal to the aganglionic portion.
Babies with Hirschsprung disease do not have a severe degree of
megacolon. However, as time goes by, the colon proximal to the aganglionic segment becomes more and more dilated, showing a dramatic
contrast with the nondilated distal bowel and the transition zone. e
clinician should not expect to nd a conspicuous megacolon with a
distal narrow segment in the newborn.
A contrast enema is extremely important for the diagnosis of
this condition. Contrast enemas do not demonstrate a transition
zone in all cases, and their value varies from institution to institution, depending to a large extent on the experience of the observer.
A positive contrast study shows a nondilated distal large bowel with
a dilated proximal segment. Sometimes the diagnosis is obvious, but
sometimes the transition between dilated and nondilated sections
is not well demarcated. In patients with total colonic aganglionosis,
no colonic dilatation occurs; the dilatation mainly aects the small
bowel.
e contrast study in these babies must be performed by an experienced pediatric radiologist. A catheter is introduced through the
anus not more than 4 cm. Passing the catheter more than 4 cm will
result in the injection of the contrast material directly into the dilated
portion of the colon, bypassing the nondilated portion and the transition zone and therefore precluding a diagnosis. Failure to pass the
contrast material in the 24 hours aer the study is considered to be
highly suggestive of Hirschsprung disease.
Occasionally, an unattended baby may experience a bowel perforation. e perforation is usually in the cecum, a presentation which
in itself is suggestive of Hirschsprung disease. When a bowel perforation occurs, emergency surgery is necessary.
Anorectal Manometry
I
nation of a balloon in the rectum of a healthy person produces
a drop in pressure in the anal canal. is drop in pressure is called
the “rectoanal inhibitory reex” and is present in all healthy persons. Anorectal manometry in children with symptoms suggestive of
Hirschsprung disease is performed with the goal of eliciting such a
reex. A lack of relaxation of the anal canal is considered diagnostic
of Hirschsprung disease. However, a signicant degree of controversy
exists about the value of this diagnostic test, and most pediatric centers and surgeons do not use it.
Rectal Biopsy
ectal biopsy is the denitive diagnostic test in children with
A r
Hirschsprung disease; however, it requires interpretation by an experienced pediatric pathologist. Most surgeons and pediatric centers
use a suction biopsy for the diagnosis of this condition. A suction
biopsy can be performed at the bedside and is considered highly
diagnostic. One of the limitations of this type of procedure is that
the specimen may not include submucosa and therefore is not sufcient for an accurate diagnosis. Another limitation is performing
the biopsy too distal in the rectum, where absence of ganglion cells is
supposedly normal. Although the diagnosis of aganglionosis can be
made with a suction rectal biopsy, the length of the abnormality cannot be determined with this biopsy.
A full-thickness rectal biopsy provides a much better specimen;
however, induction of anesthesia is required to perform this biopsy.
Full-thickness rectal biopsies are still performed by pathologists who
re not familiar with using a suction biopsy to diagnose Hirschsprung
a
disease and by surgeons who do not have access to the suction biopsy
device. An increase in the activity of acetylcholinesterase is also considered diagnostic, but not all pathologists rely on this analysis.
TMENT
TREA
Resuscitation
A n
ewborn baby who has abdominal distension, vomiting, dehydration, and explosive fetid bowel movements and who looks toxic and
lethargic needs emergency management. Administration of intravenous uids and antibiotics and—most importantly—rectal irrigations must be started promptly.
We do not know much about the pathophysiology of the enterocolitis that may lead to death in these children. However, stasis seems
to be the most important predisposing factor, and therefore decompressing the colon leads to dramatic improvement; this maneuver can
be performed on a temporary basis by passing a rectal tube. Sometimes the characteristics of the meconium and the fecal matter may
interfere with the drainage of gas and liquid from the colon through
the tube. erefore, the tube should be irrigated with small amounts
of saline solution (10 to 15 mL) and moved back and forth. is technique produces a spectacular decompression of the colon with a dramatic improvement of the patient’s symptoms. Sometimes the babies
are so sick that one cannot consider performing a contrast enema
because of the risk of perforation. e rectal irrigations and administration of antibiotics and intravenous uids allow improvement so
that other diagnostic and therapeutic procedures can be performed.
When the baby recovers from the acute enterocolitis, a rectal biopsy
is performed, establishing the denitive diagnosis.
Surger
T
in three stages. e rst stage consisted of a diverting colostomy,
which decompressed the colon and allowed the baby to recover and
go home. Most surgeons create a colostomy in the right transverse
colon (or an ileostomy was created in cases of total colonic aganglionosis). e second stage was resection of the aganglionic segment
and pull through of the normal bowel to be anastomosed to the anal
canal with the technique of choice. e third stage was closure of the
colostomy aer the baby recovered completely from the main pullthrough procedure.
tion, a colostomy was created immediately proximal to the transition
zone, called a “leveled colostomy.” In the second and denitive stage,
surgeons performed the pull through, pulling down what used to be
the colostomy into the anal canal and leaving the patient without a
protective colostomy.
through during the rst few days, weeks, or months of life as a primary procedure without a protective colostomy. is approach
avoids two operations (a colostomy and colostomy closure) and is
feasible without adding extra morbidity. However, a colostomy is still
an extremely valuable option for some babies with Hirschsprung disease. For instance, a very sick baby who has other aggravating factors and/or enterocolitis still could be a candidate for this type of
preliminary procedure. In general, most surgeons take these babies
to the operating room for a pull-through procedure with a plan for
a colostomy if necessary. When the technical circumstances of a
pull-through procedure are not satisfactory and the surgeon has any
questions about the viability of the pulled-through bowel in terms of
blood supply or any other type of technical diculty, he or she can
always protect the pull through with a proximal colostomy. Also, in
y
raditionally, patients with Hirschsprung disease were treated
A two-stage repair was subsequently devised. In the rst opera-
e most recent approach involves performing the main pull
ANAL AND PERIANAL REGION
85
h
ospitals, cities, or countries with inadequate conditions and infra-
structure, a surgeon can save the baby’s life with a colostomy.
To perform the primary neonatal pull through, one should expect
to have a newborn baby in excellent clinical condition with a colon
that has been completely decompressed. Several days are usually
required to prepare such a baby for surgery, using parenteral nutrition and colonic irrigations.
Main Repair
enson Procedure
Sw
e Swenson procedure was the rst to be designed specically
to treat Hirschsprung disease and was used for many years by Dr.
Swenson himself. A few surgeons still perform the original Swenson operation. For this type of pull-through operation, a total body
preparation is performed so the surgeon can approach the abdomen
or the perineum several times either by turning the patient from
the supine into the prone position or simply by liing the legs up to
approach the perineum and then down to approach the abdomen.
Transabdominally, the aganglionic portion of the colon is resected,
including the most dilated portion of the bowel (Fig. 18-1, A). In a
case of typical Hirschsprung disease, only the splenic exure must
be mobilized. On the other hand, when dealing with long-segment
type disease, it may be necessary to mobilize the right colon to obtain
enough length for the pull-through procedure. e aganglionic portion of the colon below the peritoneal oor is dissected in a very precise manner, staying as close as possible to the rectal wall down to the
level of the levator ani muscle. e dissection of the rectum includes
the ligation of the middle hemorrhoidal vessels and the use of cautery
in the perirectal vasculature (Fig. 18-1). Special care is taken to preserve the anal canal above the dentate line. e aganglionic segment
of the colon is resected and normal colon is pulled through (Fig. 18-1,
B and C) and anastomosed by a conventional, transanal, hand-sewn
technique. e basic principles of this procedure are still observed in
most modern operations. Dr. Swenson developed an extraordinary
experience with this operation with good outcomes. However, other
surgeons reported damage of important pelvic nerves, and thus other
procedures were designed, such as those created by Duhamel and
Soave.
Duhamel Procedure
Duhamel designed a procedure with the specic purpose of avoiding extensive pelvic dissection by preserving the pelvic portion of the
aganglionic rectum, which entails dividing the bowel at the peritoneal reection as distally as possible (Fig. 18-2, A). e aganglionic
rectal stump is then closed. e normoganglionic bowel is pulled
down right in front of the sacrum in a safe space behind the rectum
that is created by blunt dissection (Fig. 18-2, B). Lateral dissection
is therefore avoided, which prevents nerve damage. e posterior
rectal wall is incised above the dentate line, entering the previously
dissected retro rectal space. e normoganglionic bowel is pulled
through the rectal incision in the posterior wall of the rectum, and a
gastrointestinal anastomosis (GIA) stapler is used to create an anastomosis between the aganglionic rectum and the normoganglionic
pulled-down bowel (Fig. 18-2, C–F). e anastomosis between the
pulled-through colon and the aganglionic rectum is created to be as
wide as possible, and the rectal stump must be as small as possible to
avoid fecal accumulation (Fig. 18-2, G).
is procedure is very appealing because it is easy to perform and
technically reproducible. e main problem with this operation is
that the aganglionic rectum continues to be aected by the dysmotility disorder of the primary Hirschsprung condition. Stool tends to
accumulate in the rectal stump, and patients oen return for followup with a hypertrophic aganglionic rectum and a large fecal impaction. Oen they require a repeat operation to remove the aganglionic
portion and create an end-to-end anastomosis as in the Swenson
operation.
Soave Procedure
Franco Soave, an Italian surgeon, created an ingenious and appealing
operation with the specic purpose of avoiding the consequences of
nerve damage caused by an imprecise Swenson dissection. e aganglionic rectosigmoid is resected via an endorectal dissection, theoretically minimizing the risk of pelvic injury (Fig. 18-3, A and B).
e normoganglionic colon is passed through the rectal muscular cu that is le aer the endorectal dissection (Fig. 18-3, C and
D). ere is no aganglionic segment of rectum le, such as with the
Duhamel procedure. is procedure is less reproducible than the
Duhamel procedure and requires meticulous technique in the dissection of the mucosa from the muscular cu. e endorectal dissection
is performed immediately around the mucosal layer and inside the
smooth muscle layer of the bowel (Fig. 18-3, C). is dissection is
usually initiated 1 or 2 cm above the peritoneal reection. It is carried
down to a point about 1 cm above the dentate line to preserve the anal
canal with its sphincters and sensation. e normoganglionic colon
is anastomosed to the anal canal about 1 cm above the dentate line
(
Fig
. 18-3, D). Soave originally performed this operation without a
colostomy, leaving a portion of the pulled-through colon protruding
AB C
FIGURE 18-1 Sw
pletion of the operation.
enson surgical technique.
A, Resection of the aganglionic and dilated bo
wel. B, Pull through of normal ganglionic bowel. C, Com-
86
HirscHsprung Disease
D
B
A
C
E
FIGURE 18-2
C, Pull through of normal ganglionic bowel. D, Incision of the posterior rectal wall, above the dentate line. E, Pull through of normal aganglionic
colon through the window in the posterior rectal wall. F, Creating a wide anastomosis between normal ganglionic and aganglionic segments.
G, Completion of the operation.
w
ell beyond the anal skin margin. About a week later, this portion of
the bowel was resected and an anastomosis was created between the
normoganglionic bowel and the anal canal. is operation was modied by Boley, who proposed a primary anastomosis, thus avoiding
the second procedure.
La
paroscopic Procedures
L
aparoscopic techniques have been used in the management of
Hirschsprung disease. e purpose of using laparoscopic techniques
is to substitute a minimally invasive technique for a formal laparotomy, resulting in less postoperative pain and leading to a smoother
recovery and earlier discharge. Laparoscopy has been shown to be
useful in the management of these patients. Surgeons can still use
their preferred technique (e.g., Swenson, Duhamel, Soave), but the
procedure is less invasive when laparoscopy is used instead of a laparotomy. We believe that the use of laparoscopy will not have any negative impact on the results, and we favor use of this technology when
possible.
T
ransanal Approach
M
ore recently, De La Torre created a simple but ingenious and
original way to treat Hirschsprung disease. He demonstrated rst
The Duhamel technique. A, Resection of the dilated por
F
tion and part of the aganglionic segment. B, Presacral, retrorectal dissection.
in animals and then in human beings that the rectosigmoid can be
resected in a purely transanal fashion. e resection is performed
and the aganglionic segment is pulled down simultaneously through
the anus. Interestingly, in the most common type of Hirschsprung
disease (the so-called “classic type”), the entire aganglionic segment
can be resected transanally and the normoganglionic colon can be
anastomosed to the anal canal, also transanally avoiding any type of
laparotomy or laparoscopy. Fortunately, the so-called classic form
of Hirschsprung disease represents approximately 80% of cases,
which gives extra value to this ingenious technique. e transanal
approach has been adopted rapidly, and larger series of cases have
been reported.
When the transanal approach is used, the patient is placed in the
lithotomy or prone position with the pelvis elevated. A Lone Star
retractor is used to expose the anal canal (Fig. 18-4, A). e great
advantage of this retractor is that it can open the anus in a symmetric,
circumferential manner and expose the anal canal very clearly. Multiple 6-0 silk sutures are placed in the rectal mucosa above the anal
canal to exert uniform traction, which is very important to enable an
ecient and neat dissection of the rectum to be performed, above the
dentate line (Fig. 18-4, B). e original De La Torre technique actually is an endorectal technique performed from below. At our institution, we have been using a full-thickness dissection, which could be
called a Swenson procedure performed transanally. Both techniques
are equally useful and can be performed, depending on the experience of the operator. While applying uniform traction to the multiple
G
ANAL AND PERIANAL REGION
87
A
C
6-0 s
titches, a circumferential dissection is performed either submucosally or full thickness, dividing and cauterizing the extrinsic blood
supply of the rectum. In this manner we can gain length in the dissection, and surprisingly, one can reach the peritoneal reection very
rapidly (Fig. 18-4, C). As the dissection progresses, one can obtain
full-thickness biopsy specimens.
We advise obtaining full-thickness biopsy specimens every 5
cm during this dissection. e dissection must continue until the
normoganglionic bowel is reached. If at that point the surgeon perceives that the colon is already normoganglionic but very dilated, it
is mandatory to continue the dissection until a normoganglionic and
nondilated portion of the colon is reached. One can usually comfortably and safely dissect and resect the rectosigmoid up to the junction
of the sigmoid with the descending colon. e procedure becomes
rather risky and uncomfortable when trying to go higher than that,
even when it is feasible to do so. At that point, one can continue laparoscopically or with a laparotomy.
It is our practice to perform an end-to-end anastomosis with two
layers of interrupted 6-0 long-term absorbable sutures between the
normoganglionic, nondistended portion of the colon and the anal
canal (Fig. 18-4, D). e patient will receive parenteral nutrition, and
oral feedings are initiated 5 days aer the operation.
COMPLIC
W
e believe that operative complications in patients with Hirschsprung
disease can be divided into two categories; preventable and nonpreventable. Preventable complications include fecal incontinence,
ATIONS
B
D
FIGURE 18-3
of dilated colon plus the intra
segment. B, Endorectal intrapelvic dissection.
C, Resection of the m
down to the dentate line. D, Pull through of
normal ganglionic bowel through the muscle cuff
and anastomosis 1 cm above the dentate line.
dehiscence, retraction, stula formation, and constipation. ese
complications can be minimized through use of meticulous surgical
technique.
F
ecal Incontinence
F
ecal incontinence is still a relatively frequent problem in patients
who undergo surgery for Hirschsprung disease. We believe that
fecal incontinence occurs mainly when the surgeon violates basic
principles, regardless of the type of technique used. Basically, fecal
incontinence means that the anal canal is damaged. is crucial area,
extending from 1 cm above the dentate line down to the skin, must
be preserved, regardless of the surgical technique used in the treatment of Hirschsprung disease. e anal canal contains the most sensitive area of the gastrointestinal tract and the sphincter mechanism.
Patients with sphincter damage benet from a bowel management
program.
The Soa
ve technique.
ucosal aganglionic segment
A, Resection
peritoneal aganglionic
Dehiscence and Retraction
nastomotic dehiscence and retraction of the colon usually occur
A
as a consequence of a decient technique in the management of the
blood supply of the normoganglionic bowel. It is very important to
observe the blood supply of the colon very carefully before making
a decision to ligate a branch of the inferior mesenteric, middle colic,
or ileocolic vessels. e segment to be pulled down must have good
blood supply through the collateral arcades of the colonic vessels. In
88
HirscHsprung Disease
A B
FIGURE 18-4
orre) technique. A, A Lone Star
T
retractor is used to expose the
rectal mucosa and pectinate line.
B, Multiple silk stitches after
repositioning the Lone star hooks
deeper than the pectinate line,
taking rectal mucosa above the
pectinate line. C, Full-thickness
dissection of the rectum after the
peritoneal reflection and beyond.
D, Anastomosis of normal ganglionic
bowel to the rectum 1 cm above
the pectinate line.
addi
tion, the pulled-through colon must lie in its position without
T
ransanal (De La
CD
any tension.
Constipation
C
onstipation occurs mainly in patients in whom normoganglionic
but dilated colon has been pulled down. We have learned through the
years that a very dilated piece of colon is almost as bad as an aganglionic segment. Evidence indicates that abnormally dilated portions of any hollow viscus lose their peristalsis capability. erefore,
regardless of the technique used by the surgeon, it is imperative to
pull down a nondilated normoganglionic part of the colon. However,
there may be as yet undened factors responsible for the hypomotility observed in normoganglionic colon because many patients still
experience postoperative constipation in spite of pulling down a nondilated portion of the colon.
Nonpr
En
unpredictable complication seen in patients with Hirschsprung disease. Some surgeons claim that they do not see enterocolitis in their
patients. Perhaps they do not look for this specic complication,
only thinking of enterocolitis when they see an extremely sick child.
eventable Complications
terocolitis is the most feared, mysterious, nonpreventable, and
W
e try to detect these cases very early. We start by obtaining radiographs very early postoperatively. One can see dierent degrees
of dilatation of the colon, as well as irregularities in the bowel
mucosa that indicate that the patient is at risk for the development
of enterocolitis. We treat these patients with rectal irrigations and
administration of metronidazole by mouth and sometimes by rectum. In this manner we try to avoid a full picture of enterocolitis
and prevent patients from experiencing this complication. We are
unaware of any specic ways to prevent enterocolitis. Some surgeons claim that myectomies and anal dilatations give good results
in the management of enterocolitis. Other surgeons use botulinum
toxin, supposedly injected into the internal sphincter. Results have
been good, but only for short periods.
RESUL
M
ortality as a result of Hirschsprung disease has been reported to
TS
be between 0 and 3.3%. Advocates of the Swenson procedure report
that 13% of their patients have temporary soiling and 16% to 27%
have postoperative enterocolitis. Advocates of the Duhamel procedure report a 10% complication rate, whereas advocates of the Soave
procedure report a 12% incidence of strictures.
We believe that enterocolitis has been underestimated and underreported. e incidence of this complication in dierent analyses varies from 10% to 30%.
ANAL AND PERIANAL REGION
89
esults of the transanal approach of Hirschsprung disease are still
R
considered preliminary because of the lack of long-term follow-up.
Early follow-up indicates that the results are similar to those obtained
with previously used techniques. e transanal approach has obvious
advantages compared with the other operations because it is the least
invasive, including laparoscopy. e main concern with the transanal
approach, of course, is the possibility of interfering with bowel control because of the stretch of the sphincter mechanism that is required
to expose the anal canal.
T
OTAL COLONIC AGANGLIONOSIS
T
otal colonic aganglionosis is a particular variant of Hirschsprung
disease that continues to represent a serious technical challenge. Different treatments have been designed to deal with this condition, but
all of them are less than ideal in terms of quality of life. In 1968, Lester Martin described his technique for the treatment of total colonic
aganglionosis. Patients with total colonic aganglionosis require resection of the entire colon and pull-through of the normoganglionic
small bowel to be connected to the anal canal. As expected, these
patients experience multiple bowel movements during the day and
night, severe diaper rash, and a tendency to be dehydrated. To avoid
these symptoms, Martin thought that it could be useful to preserve a
signicant portion of the aganglionic colon to take advantage of its
water absorption capacity and also to create a type of reservoir that
would allow the patient to hold the stool and decrease the number
of bowel movements per day. Dr. Martin proposed preserving the
rectosigmoid and sometimes even longer portions of the colon. e
normal ganglionic terminal ileum is then pulled down through the
presacral space (as in the Duhamel technique) and anastomosed to
the rectum. A long side to side anastomosis is created between the
terminal ileum and the rectosigmoid. However, patients undergoing
this procedure frequently have abdominal distention and bouts of
enterocolitis. We now know that the small bowel functions well with
rapid transit. Stasis in the small bowel produces an inammatory
process that results not only in lack of absorption of water but also
a secretory type of diarrhea and enterocolitis. Many of these patients
have to undergo a repeat operation to resect the pouch or patch of
aganglionic bowel. Others have tried to modify Martin’s original procedure. Kimura, for instance, created a right colon patch basically following the same principles. Martin or Kimura procedures are usually
performed with a protecting ileostomy.
Our routine practice for treating patients with total colonic aganglionosis includes an ileostomy with or without a total colectomy with
ileoanal anastomosis at the time of presentation. We close the ileostomy only aer the patient meets two criteria: he/she is toilet trained
for urine and he/she accepts rectal irrigations. e rationale behind
this rather conservative approach is based on the fact that babies normally do not hold their stool at young ages, and the constant passage
of liquid stool from an ileum-anal pull-through procedure produces
a severe diaper rash that is not easily manageable. Once the patient
is toilet trained for urine, even with frequent episodes of liquid stool,
the patient is able to reach the toilet. Acceptance of rectal irrigation is
necessary because the longer the aganglionic segment, the higher the
chances of enterocolitis.
Boley S. An endorectal pull-through operation with primary anastomosis for
Hirschsprung’s disease. Surg Gynecol Obstet. 1968;127:353.
Chatoorgoon K, Peña A, Lawal TA, etal. e problematic Duhamel pouch in
Hirschsprung’s disease: manifestations and treatment. Eur J Pediatr Surg.
2011;21:366–369.
Cilley RE, etal. Denitive treatment of Hirschsprung’s disease in the newborn
with a one-stage procedure. Surgery. 1994;115:551.
De La Torre L, Ortega J. Transanal endorectal pullthrough for Hirschsprung’s
disease. J Pediatr Surg. 1998;33:1283.
Duhamel B. A new operation for the treatment of Hirschsprung’s disease.
Arch Dis Child. 1960;35:38.
Elhalaby EA, et al. Enterocolitis associated with Hirschsprung’s disease: a
clinical-radiological characterization based on 168 patients. J Pediatr Surg.
1995;30:76.
Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pullthrough for
Hirschsprung’s disease in infants and children. J Pediatr Surg. 1995;30:1017.
Hirschsprung H. Stuhltragheit neugeborner in folge von dilatation and hyper-
trophie des colons. Jaharb Kinderch. 1887;27:1.
Kimura K, Mishijima E, Muraji T. A new surgical approach to extensive agan-
glionosis. J Pediatr Surg. 1981;16:840.
Kleinhaus S, etal. Hirschsprung’s disease—a survey of the members of the
surgical section of the American Academy of Pediatrics. J Pediatr Surg.
1979;14:588.
Lake BD, Malone MT, Risdon RA. e use of acetylcholinesterase (AchE) in
the diagnosis of Hirschsprung’s disease and intestinal neuronal dysplasia.
Pediatr Pathol. 1989;9:351.
Lake BD, Puri P, Nixon HH, et al. Hirschsprung’s disease: an appraisal of
histochemically demonstrated acetylcholinesterase activity in suction
rectal biopsy specimens as an aid to diagnosis. Arch Pathol Lab Med.
1978;102:244.
Langer JC, Durrant AC, De La Torre L, etal. One-stage transanal Soave pull-
through for Hirschsprung’s disease. A multi-center experience with 141
children. Ann Surg. 2003;238:569.
Langer JC, Minkes RK, Mazziott MV, etal. Transanal one stage Soave proce-
dure for infants with Hirschsprung’s disease. J Pediatr Surg. 1999;34:148.
Levitt M, Martin C, Olesevich M, et al. Hirschsprung’s disease and fecal
incontinence: diagnostic and management strategies. J Pediatr Surg.
2009;44:271–277.
Martin LW. Surgical management of Hirschsprung’s disease involving the
small intestine. Arch Surg. 1968;97:183.
Rescorla FJ, etal. Hirschsprung’s disease. Evaluation of mortality and long-
term function in 260 cases. Arch Surg. 1992;127:934.
Schoeld DE, Devine W, Unis EJ. Acetylcholinesterase-stained suction rectal
biopsies in the diagnosis of Hirschsprung’s disease. J Pediatr Gastroenterol
Nutr. 1990;11:221.
So HB, Schwartz DL, Becher JM, etal. Endorectal pullthrough without pre-
liminary colostomy in patients with Hirschsprung’s disease. J Pediatr Surg.
1980;15:470.
Soave F. Hirschsprung’s disease: a new surgical technique. Arch Dis Child.
1964;39:116.
Swenson O, Bill AH. Resection of rectum and rectosigmoid with preservation
of the sphincter for benign spastic lesions producing megacolon. Surgery.
1948;24:212.
Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an
analysis of 501 patients. J Pediatr Surg. 1973;8:587.
Vane D, Grosfeld J. Hirschsprung’s disease, experience with the Duhamel op-
eration in 195 cases. Pediatr Surg Int. 1986;1:95.
S u g g e S t e d
B
ischo A, Levitt M, Bauer C, etal. Treatment of fecal incontinence with
a comprehensive bowel management program. J Pediatr Surg. 2009;44:
1278–1284.
Bischo A, Levitt MA, Peña A. Bowel management for the treatment of pedi-
atric fecal incontinence. Pediatr Surg Int. 2009;25:1027–1042.
Bischo A, Levitt MA, Peña A. Total colonic aganglionosis: a surgical chal-
lenge. How to avoid complications? Pediatr Surg Int. 2011;27:1047–1052.
Bischo A, Tovilla M. A practical approach to the management of pediatric
fecal incontinence. Sem Pediatr Surg. 2010;19:154–159.
R
e a d i n g
This page intentionally left blank