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428
24 Hirschsprung’s Disease
Fig. 24.26 Patulous anus
Fig. 24.27 Abdominal fi lm showing retention of stool
post-Hirschsprung’s operation
to the opposite (narrow, non-dilated) tendency to
diarrhea, hypermotility type (Fig. 24.28 ). As we
explained in the chapter about bowel management, those groups require a completely different
regimen of bowel management in order to be successful incontinence [ 131 , 132 ].
Fig. 24.28 Contrast enema – spastic, hyperactive colon
If the patient has an intact anal canal, we
believe that the patient has potential for bowel
control. Some patients (49/103) even with intact
anal canals suffer from fecal incontinence, and
we do not know exactly why. However, the contrast enema in those cases will tell us whether
the patient suffers from severe constipation and
megacolon (hypomotility) or non-dilated colon
and tendency to diarrhea (hypermotility). The
management of each group is different. We give
all these patients the opportunity to see how
much bowel control they have, helping them to
improve their specifi c motility problem. In other
words, if the patient has constipation and megacolon, we give them laxatives. If the patient has
tendency to diarrhea and a non-dilated colon,
then we administer a constipating diet, pectin,
and Imodium.
3
In both groups, we radiologically
monitor the effects of our treatment. The patients
are managed on an ambulatory basis over a period
of 1 week (see Chap. 20 ). They are expected to
3
Imodium – active ingredients: loperamide HCl 2 mg and
simethicone 125 mg (in each caplet); slows the rate at
which the stomach and intestines move. It also increases
the density of stools and reduces the amount of fl uid in the
stool.
24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
Table 24.1 Medical management in patients with fecal incontinence after Hirschsprung’s operation
Patients with constipation Patients with tendency to diarrhea
Clean with
enemas
33.8 % 5.8 % 8.8 % 8.8 % 33.8 % 8.8 %
Remain incontinent in
spite of the use of
laxatives
Continent with the
use of laxatives
Continent with the
use of
constipating diet
and bulking
agents
Clean with the
use of enemas
and constipating
diet
Remain
incontinent in
spite of enemas
and constipating
diet
429
Fig. 24.29 Contrast enema in a patient with retained
aganglionic segment
come to the clinic every day. Abdominal x-ray
fi lms are taken daily to monitor the amount of
stool in the colon, and with that information, we
determine if the amount of laxative that we are
giving is adequate. By doing that, many patients
who suffer from constipation and “incontinence”
now behave like continent patients which means
that perhaps they were actually suffering from
overfl ow pseudoincontinence.
In cases of patients with a non-dilated colon
with tendency to diarrhea, a decreased borderline
sensation and perhaps a partially damaged
sphincter cannot cope with a hyperactive bowel
that has constant peristalsis trying to pass stool.
Therefore, the use of specifi c fi ber (pectin) that
makes the stool more bulky plus a constipating
diet and Imodium* and regular meals trying to
decrease the number of bowel movements may
make the patients behave like fecally continent.
Table 24.1 shows our results with the manage-
ment of patients with fecal incontinence after
Hirschsprung’s operations. The contrast enema,
in addition, occasionally may show changes
consistent with retention of an aganglionic piece
of colon (Fig. 24.29 ); in such cases, during the
examination under anesthesia, a rectal biopsy is
done. If the results of the biopsy confi rm the
absence of ganglion cells, a secondary resection
and pull-through is performed.
24.13.2 Non-preventable Complications
We think that enterocolitis is a non-preventable
and non-predictable complication. For us, this
complication represents a mystery. Fortunately,
we know some of the predisposing factors that
contribute to generate this problem of enterocolitis, but we still do not know its intrinsic
pathophysiology.
There is something characteristic about
Hirschsprung’s disease patients; in general, they
do not tolerate fecal stasis. A normal human
being who retains stool develops a clinical picture consistent with what we call constipation,
but they generally do not develop toxemia and
proliferation of bad bacteria. In Hirschsprung’s
disease, the patients usually behave differently,
as previously mentioned. They tend to suffer
from enterocolitis. So far, our management of
this problem that has been described here includes
only the use of frequent irrigations and metronidazole. If that is not enough, we offer the patients
subsequent resections of bowel, even knowing
that the resection will include normoganglionic
bowel. It is expected that those resections may
430
24 Hirschsprung’s Disease
decrease the chances of the patient to suffer from
enterocolitis. Again, the contrast enema may
show changes consistent with the possibility that
the patient still has a piece of aganglionic
segment.
24.13.3 Partially Preventable Complications
In this category, we include the problem of constipation. We believe that if we leave an extremely
dilated piece of bowel, even when it is normoganglionic, most likely the patients will suffer from
postoperative constipation. The old, demonstrated principle that a hollow viscus (ureter,
colon, small bowel, and esophagus) that is
allowed to suffer from severe viscus dilatation
will lose its normal peristalsis is applicable here.
A colon that is extremely dilated, by defi nition,
will suffer from hypomotility, and therefore, it is
expected that the patient will have severe constipation. Therefore, we emphasize the need to
resect not only the aganglionic segment but also
the dilated normoganglionic portion of the colon.
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Idiopathic Constipation and Other Motility Disorders
2 5
25.1 Defi nition and Terminology
The term idiopathic constipation refers to the
incapacity or diffi culty to pass stool regularly and
effi ciently. In addition, we believe that it also
means incapacity to empty the colon.
We use the term “idiopathic” because we do
not know the etiology of this condition. We are
aware of many proposed explanations to understand the pathophysiology of this condition, but
we fi rmly believe that none of those explanations
have solid scientifi c basis. In agreement with
Benjamin Disraeli, we believe that “to be conscious that you are ignorant is a great step to
knowledge” [ 1 ].
We intentionally avoided other terms frequently used in the literature, basically because
many of those names implied an accepted etiology. Some of those names include: “puborectalis
spasm syndrome,” “descending perineum syndrome,” “chronic obstipation,” and “spastic pelvic fl oor syndrome.” In addition and most
importantly the treatments that we have to offer
to patients with constipation are not different,
regardless of the category or type of constipation
that the patient suffers from.
We also refer to idiopathic constipation as the
central subject of this chapter because, by far, the
greatest number of patients coming to our clinic
Electronic supplementary material Supplementary
material is available in the online version of this
chapter at
10.1007/978-3-319-14989-9_25 .
with a colonic motility disorder, requesting a surgical consultation, belongs to this particular
group.
25.2 Incidence, Social Impact, and Relevance
Idiopathic constipation is by far the most common defecation disorder and colonic motility
disorder seen in children. It represents a common
cause for surgical consultation [ 2 – 4 ]. It affects
millions of Americans, as well as patients of
other countries, but perhaps most important is
the fact that it is an incapacitating condition
when it is not treated properly. In fact, it produces a form of fecal incontinence known as
encopresis or overfl ow pseudoincontinence that
makes the patient socially rejected and
discriminated.
25.3 Etiology
We are aware of multiple publications proposing
different possible causes for this condition.
However, most of those explanations have no scientifi c basis, and therefore we do not embrace
them. We prefer to take a healthy and potentially
more productive attitude by declaring our ignorance about the origin of this condition.
Some authors believe that diet is very impor-
tant as an etiologic factor of constipation [ 5 , 6 ].
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_25, © Springer International Publishing Switzerland 2015
435
436
25 Idiopathic Constipation and Other Motility Disorders
There is no question that different types of food
have either a laxative or a constipating effect on
our bodies. In addition, we recognize the existence of personal idiosyncrasies that explain why
one type of food may act as a laxative for one
individual and have a constipating effect for
another one. Although we recognize that diet is
important to regulate colonic motility, we believe
that the therapeutic value of diet is negligible in
the most serious forms of constipation. We must
keep in mind that this chapter belongs to a book
of surgical treatments of colorectal problems in
children. The type of constipation that is manageable by diet belongs to the pediatric clinics.
Those patients do not come to our clinic because
they are treated either by a pediatrician or gastroenterologist. The patients that come for surgical
consultation are patients that have already
received all kinds of unsuccessful medical managements previously.
There are also many articles [ 7 – 12 ] that try to
explain the problem of idiopathic constipation on
psychological basis. The psychodynamic mechanisms proposed are interesting and sometimes
picturesque; including, strict demanding parents.
Strict demanding parents who impose rigid rules
on a child during the toilet training process, children that supposedly retain the stool to manipulate the parents to achieve their own purposes. All
these mechanisms may have an element of truth,
but we do not believe that they can explain the
severe forms of constipation in patients with fecal
pseudoincontinence, giant megacolon, sometimes megabladder, serious nutritional and developmental disturbances, and sometimes death. We
think that it is certainly not easy to retain stool
voluntarily in an otherwise autonomous normal
rectosigmoid with normal peristalsis.
It is true that most patients suffering from
idiopathic constipation also have a psychological
disorder, but we do not think it is a primary one.
Any human being suffering from severe constipation and soiling, ostracized and discriminated
understandably, must have a very signifi cant secondary psychological problem.
Surgeons, on the other hand, have proposed
different potential mechanisms to explain this
problem. For instance, a rather simplistic expla-
nation is that there is a lack of relaxation of the
“internal sphincter” also known as achalasia of
the internal sphincter [ 13 – 16 ]. This is, obviously,
a very attractive and popular idea. In other words,
a simplistic logic dictates that incontinence
means “lack of sphincter”; therefore, constipation most likely means “too much sphincter.”
However, as discussed in the chapter of ultrashort
Hirschsprung’s, we do not believe that the “achalasia of the internal sphincter” is an entity that
can explain the symptoms of these patients.
Many patients suffering from idiopathic constipation are subjected to rectal manometry.
Many of them have no relaxation refl ex. The lack
of relaxation has been described as a diagnostic
of Hirschsprung’s disease, and therefore the next
step is usually to take a rectal biopsy. If the rectum has no ganglion cells, the diagnosis of
Hirschsprung’s disease is confi rmed. On the
other hand, if the rectal biopsy shows ganglion
cells, the patient then receives the diagnosis of
“achalasia of the internal sphincter.”
25.3.1 Ultrashort Segment
Hirschsprung’s Disease
Ultrashort Hirschsprung’s disease has been
defi ned as a condition in which a small length of
the distal bowel has no ganglion cells. The specifi c length of this aganglionic zone has not been
defi ned. Under normal circumstances, human
beings have an area of aganglionosis above the
pectinate line [
aganglionic zone has not been well established at
different ages from premature life to the adult
size. Consequently, it is very diffi cult to know if
a biopsy that shows absent ganglion cells was
taken from this normal aganglionic area of the
rectum. At what point an aganglionic zone is considered normal and at what point is considered
typical Hirschsprung’s? Both are unanswered
questions.
Many authors believe that this is a common
cause of constipation [ 39 – 42 ]. We believe that
“ultrashort segment Hirschsprung’s disease” and
the so-called internal sphincter achalasia are
highly debatable conditions. From the clinical
43 – 45 ]. The length of this normal
25.3 Etiology
437
and radiologic point of view, patients who have
been labeled with these conditions cannot be differentiated from those suffering from idiopathic
constipation clinically or from the radiologic
point of view. In other words, the three groups of
patients suffer from severe constipation, they
have a tendency to soil the underwear when the
constipation is severe, they have a very dilated
rectum, and most importantly, the three groups
respond to the use of laxatives. Those authors
who support the existence of these two conditions (ultrashort segment Hirschsprung’s and
internal sphincter acatalasia) [ 13 – 16 , 33 – 40 ]
claim that they can make the diagnosis based on
manometric studies [ 17 – 21 ] and confi rmed by
sophisticated histochemical techniques not
always available to the clinician.
We have serious questions about the existence
of these conditions for several reasons:
25.3.2 Rectal Manometry
During a regular rectal manometry study, a balloon is placed inside the lumen of the rectum, and
the pressure of the anal canal is recorded. Under
normal circumstances, the infl ation of the balloon
in the rectum elicits a response from the patient
consisting in a drop of pressure in the anal canal,
which the manometrists interpret as “relaxation
of the internal sphincter.” The fi rst question that
comes to our mind is how do they know that the
internal sphincter is the structure that is relaxing?
If we think about the real anatomy of normal
individuals (see Chap.
called internal sphincter has been defi ned as a
thickening of the circular layer of smooth muscle
of the most distal portion of the bowel. However,
surrounding the entire rectum, there is an obvious
and powerful striated skeletal muscle structure or
voluntary sphincter mechanism. During very
early manometric studies, some investigators
used paralyzing agents in animals and even in
human volunteers in order to be able to discriminate the relaxation of the striated muscle, from
relaxation of the smooth muscle (internal sphincter), and they concluded that it was the smooth
muscle that was relaxing. In addition, they even
2 ), we fi nd that the so-
reached the conclusion that 85 % of the bowel
control depended on the “internal anal sphincter!” Yet, during all the clinical manometric evaluations of patients, muscle relaxants are not used,
and therefore, we do not know if the drop of pressure in the anal canal is due to a relaxation of the
striated muscle complex or to the smooth
muscle.
Another very serious question that comes to
our mind when discussing rectal manometry is
the fact that most patients evaluated manometrically suffer from constipation, and, by defi nition,
most of them have a very dilated rectum. In order
for the patient to have a relaxation refl ex, it is
necessary to stretch the rectal wall with the
infl ated balloon. That means that we are supposed to use different volume balloons for different patients depending on the size of the rectum.
We are unaware of this kind of methodology.
Most manometries use the same size balloon for
all patients, and certainly we have never seen 1 or
2 L balloons, yet we have seen many patients
with giant rectums. As a consequence, it is conceivable that the infl ation of a relatively small
balloon that does not stretch the rectum would
produce a negative refl ex. In other words, the
pressure in the anal canal would not drop because
actually the rectum was never distended due to
the fact that they used a standard size balloon in a
megarectum. When we asked these questions to
different proponents of manometry, we do not
have a reasonable answer.
25.3.3 Doubts and Questions About
the Anatomy of the Internal
Sphincter
The “internal sphincter” has been defi ned as a
thickening of the circular layer of the smooth
muscle of the bowel in its most distal portion, the
anorectum. Yet, real photographs documenting
the presence of this structure are extremely
unusual to see (see Chap. 2 ). A description of the
specifi c thickness and upper and lower limits of
this elusive structure at different ages is inexistent. There are plenty of diagrams but very few
photographs, and those photographs do not show