hisrschsprung disease

1. REVIEW ARTICLE
Hirschsprung’s Disease: A Review
Orvar Swenson, MD
B
ecause it is now more than 50 years since both
the discovery of the cause and a treatment for
Hirschsprung’s disease, it seems appropriate
to assess outcomes for those unfortunate children
who have this previously lethal disease. In 1948, the
discovery of the cause of this disease was based on a
series of clinical observations indicating that there
was a defective segment of distal colon producing a
partial bowel obstruction.1 Peristaltic tracings of the
dilated proximal colon recorded progressive contrac-
tions; however, this peristaltic wave did not enter the
more distal narrow segment. This was suggestive
evidence of a physiologic defect in that distal seg-
ment. Therefore, removing this segment would
prove the validity of these observations. An opera-
tive technique was devised and tested in the labora-
tory, and when it proved feasible, a patient’s distal
narrow segment was removed and his intestinal con-
tinuity was restored with a pull-down and reanasto-
mosis. Soon thereafter, the patient’s gastrointestinal
system began to function normally. We concluded
that this operation provided experimental evidence
that proved our assumptions were correct.2
DIAGNOSIS
It is possible to suspect the presence of Hirsch-
sprung’s disease in the newborn by clinical criteria if
the infant does not pass meconium in the first 48
hours or by the presence of vomiting and abdominal
distention.3 In the older child, clinical criteria for
diagnosis includes a history of severe constipation
from birth, failure to thrive, abdominal distension
and often an empty rectal ampulla on rectal exami-
nation. The disease occurs about once in 5000 live
births.4 The incidence of this disease being transmit-
ted to offspring is about 3%. In specific instances in
females where the length of the aganglionic lesion is
extensive, the percentage increases.4
As part of our investigation into the cause of this
disease, a radiographic enema using a small amount
of barium outlined an irregular and narrowed small
colon beginning in the anal canal and extending
proximally before it fanned out and connected with
the more proximal dilated colon.5 Roentgenograms
of this portion of the colon in this disease had never
been previously published and became the definitive
test to establish the diagnosis of congenital megaco-
lon. Delayed clearance of barium from the colon after
the enema was additional evidence suggesting
Hirschsprung’s disease. It soon became apparent
that this test was less sensitive for detecting short
lesions, total colon aganglionosis, and the disease in
the newborn. Therefore, studies were performed us-
ing the surgically removed distal segment. Strips of
tissue were cut from the full length of the specimen
extending down to the anal canal, and, for the first
time, the whole segment was found to be devoid of
Auerbach’s plexus.1 In 1957, this led us to recom-
mend rectal biopsy as the most reliable test to estab-
lish the diagnosis even for those groups with a con-
flicting barium enema radiograph.6 This procedure
required a general anesthetic and was likely to leave
scarring, complicating the definitive operation. Mu-
cosal biopsy and the application of histochemical
staining eliminated these problems and such speci-
mens were often obtained at the bedside by a suction
cup or forceps.7 In a review of 1340 mucosal biopsies,
there were 3 cases of bleeding requiring transfusion
and 3 clinical perforations, 1 resulting in death.8 One
caution in performing rectal biopsy for the diagnosis
of Hirschsprung’s disease is the recognition that
there is normally a 2-cm band in the distal rectum at
the level of the internal anal sphincter devoid of
ganglion cells.9
OPERATIVE TREATMENT
The original operation (the Swenson procedure)
consisted of freeing the defective distal colon from
within the pelvis by careful sharp extrarectal dissec-
tion down to 2 cm above the anal canal and perform-
ing an end-to-end anastomosis. Applying this tech-
nique, the defective aganglionic tissue is completely
removed and the proximal ganglionated colon and
anal canal are left in a normal anatomic position.2
In 1960, Duhamel10 published a modification of
the original operation. He resected a portion of the
distal defective segment as in the original operation
and made a side-to-side anastomosis between the
proximal normal colon and defective rectum. In
1963, Soave11 published another modification. It con-
sisted of making a circumferential cut through the
muscular coat of the colon at the pelvic peritoneal
reflection. Working in an intramural plane, the mu-
cosa is separated from the muscular coat down to the
anal canal. The freed mucosa is excised and the prox-
imal normal colon is pulled through this retained
viable spastic aganglionic muscular sleeve. A tele-
From Charleston, South Carolina.
Received for publication May 11, 2001; accepted Jan 14, 2002.
Reprint requests to (O.S.) 1 Gadsen Way, Apartment 322, Charleston, SC
29412-3574.
PEDIATRICS (ISSN 0031 4005). Copyright © 2002 by the American Acad-
emy of Pediatrics.
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2. scoping type of anastomosis is made that adds an
extra layer of colon wall at the anastomosis. Marks12
reported cutting the sleeve posteriorly, a practice
now widely used. Boley13 further modified the
method by making an extrapelvic anastomosis be-
tween the anal canal mucosa and the mucosa of the
normal colon. Rehbein and von Zimmermann14 used
an anterior approach to partially remove the defec-
tive aganglionic tissue leaving a considerable seg-
ment of aganglionic tissue in place. Some surgeons
using this technique perform sphincterotomy as a
routine part of the operation.15
During the past decade, there has been a trend to
operate on infants in the first weeks of life without a
preliminary colostomy. Carccassonne was a leader in
introducing this change, and he reported results on
98 infants treated in this manner in whom all the
defective tissue was removed (M. Carcassonne, per-
sonal communication). All the patients were under 3
months of age and there were no deaths. One anas-
tomotic leak closed promptly when a colostomy was
instituted (M. Carcassonne, personal communica-
tion). This strategy may prove to be an important
change because it merges the period of incontinence
with the normal period of training.
Contemporary laparoscopic surgery may also fit
well with resecting patients with Hirschsprung’s dis-
ease, especially recognizing that the excised tissue
can be removed through the anal canal. Wulkan and
Georgeson16 have reported an experience with this
technique; however, they leave the defective agan-
glionic muscular sleeve in place and follow with a
prolonged program of daily rectal dilatations. Cur-
ran and Raffensperger,17 along with Arany et al,18
have demonstrated that it is easy to remove all the
defective aganglionic tissue with laparoscopic tech-
nique with no need for daily rectal dilatations post-
operatively. Still another new technique achieves the
entire resection, leveling of ganglion cells, and defin-
itive pull-through via the anal canal without an ab-
dominal incision.19 Small series have been reported
with reasonable complication rates.20
About 4% to 5% of patients with congenital mega-
colon have no ganglion cells in the entire colon.21
These smaller reported series have mortality rates
after surgical treatment ranging from 13% to 23.5%.22
Martin23 suggested a technique that saves a portion
of the defective colon, uniting it with the terminal
ileum with a side-to-side anastomosis, thus provid-
ing additional surface for fluid absorption. Criticisms
of this modified Duhamel technique have been re-
ported because of poor emptying of the retained
aganglionic colon.24,25 Better results in treating this
entity have been reported by Sherman et al.26 Nine-
teen patients were treated with excision of the entire
aganglionic colon with end-to-end anastomosis of
the terminal ileum to the anal canal with no deaths.
These patients were started on oral feedings shortly
after operation; however, they went through a diffi-
cult postoperative period with frequent stools which
at times produced perineal excoriation.27 With per-
severance they all gained stool control. The first pa-
tient treated in this manner graduated from college,
married, had several children and was doing well
until 40 years after his operation when he became
anemic with blood in his stool.28 At sigmoidoscopy
the ileal mucosa was reddened. An ileostomy was
performed and 6 months later he was no longer
anemic, and his stool was negative for blood. A
second patient went through the same experience.
Some surgeons have modified this procedure by con-
structing a pouch to serve as a stool reservoir.29 A
rare case of a colon transplant has been reported with
a short follow-up.30
OPERATIVE MORTALITY
The overall mortality rate of Hirschsprung’s dis-
ease in 880 cases treated with the original Swenson
operation was 2.5%; however, in the last 20 years the
mortality has fallen to 1.25%.26 Soave31 in his own
series of 271 patients reported a 4.5% mortality rate.
In a recent paper reporting 260 patients treated with
the Duhamel modification, the mortality rate was
6.2%.32 Carcassonne et al,33 Weizman et al,34 Shand-
hogue and Bianchi,35 Waldron and O’Donnell36 and
Madonna et al37 in their papers describing their ex-
perience with the original operation reported no
deaths.
OPERATIVE COMPLICATIONS
It is difficult to make a precise comparison of
complications between the original Swenson opera-
tion and the numerous modifications. For instance,
Sherman et al26 reported a careful statistical analysis
of 880 cases from seven children’s centers. A tissue
diagnosis was obtained on all cases and all were
treated with the original operation. There were no
problems with urinary or sexual function. For com-
parison, there is a recent report of 2400 cases from
multiple centers treated with the Duhamel modifica-
tion and subjected to a 30-year follow-up.38 In a
considerable proportion of the cases, there was no
tissue diagnosis. The data were collected by ques-
tionnaire and treatment consisted of 8 different mod-
ifications of the Duhamel procedure. No data were
reported on sexual function.
One of the most serious complications of a Hirsch-
sprung’s disease pull-through is a leak at the anas-
tomosis. In the Sherman series, 26 there was a 5.6%
leak rate. Soave31 states that in his personal series
there were no leaks. However, in the collection of 365
patients treated with his technique, the leak rate was
6.1%.39 In the survey on Hirschsprung’s disease of
the members of the Surgical Section of the American
Academy of Pediatrics, the leak rate after the Soave
Boley modification was 6.9%.40 Harrison et al41 re-
ported the leak rate of patients treated by the Du-
hamel and Soave modifications to be 7%.
Other additional complications characterize the
modified procedures and they do not occur after the
original Swenson procedure. The Duhamel modifi-
cation is reported to have a 10% rectal pouch prob-
lem consisting of impactions and at times bleeding.42
Grosfeld et al43 reported an 8% rate and Baillie et al44
reported a 25% rate of pouch problems. Martin and
Torres45 claim to have eliminated this problem with
their series of modifications. In their review, Bourde-
lat et al38 list 9 modifications of the Duhamel tech-
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3. nique. There also are reports of abscess formation
between the retained aganglionic muscular sleeve,
retraction of the pulled through normal colon, and
mucosal prolapse after the Soave modification, com-
plications that can occur years after the operation.46
Fortuna et al47 has reported secondary reoperation
rates in patients treated by the Duhamel to be 29%
and after the Soave to be 26%. The postoperative
occurrence of enterocolitis was 19% to 20%. Sherman
et al26 reported that in 880 Swenson procedure cases
the secondary reoperation rate was 6%. This
stemmed from an anastomotic leak rate of 5.6%,
which required diverting colostomies, and, in rare
cases, the need to surgically excise strictures. In a
recent report on 97 patients treated with the original
operation, the secondary operation rate was 6%.35
Throughout the 40-year study of the original opera-
tion, the postoperative rate of enterocolitis was
11%.26
There are several reports that some patients had
such poor results from their pull-through that com-
plete reoperation was required.48–50 A small number
of these were for stricture or retained aganglionic
tissue, the remainder were for recurrent enterocolitis,
constipation, or soiling. The entire reoperative group
had either a primary Duhamel or Soave modifica-
tion. In a large series of Duhamel procedures, 4.9% of
patients had such poor results that reoperations were
required.38 Furthermore, Tariq et al51 reported such
unrelenting postoperative constipation in 3.7% of
their patients after treatment with the Soave modifi-
cation that reoperations were required. Sherman et
al,26 Carcassonne et al,33 Weizman et al,34 Shand-
hogue and Bianchi,35 Waldron and O’Donnell36 and
Madonna et al37 all using the original Swenson op-
eration have not encountered problems that required
reoperation.
It is generally believed that all postoperative
Hirschsprung’s disease patients, regardless of the
original operative procedure, improved with time.
This is challenged by Postuma and Corkery,52 who
observed that this was true for a period of time, but
that those treated by the modifications subsequently
had an increase in troubles. Mishalany et al53 and
Heij et al54 have expressed the same concern.
OUTCOMES OF OPERATIVE TREATMENT FOR
HIRSCHSPRUNG’S DISEASE
There have been concerns about how end results
are evaluated. The commonly used method is to list
results as excellent, good, fair, and poor. This
method involves judgment and in collected series,
the evaluation is made by the operating surgeon to
whom parents are reluctant to report problems. In an
attempt to enhance objectivity, the results can be
reported by the normalcy of bowel function. Duth-
rie55 has estimated the normal pattern of bowel func-
tion is one movement or more each 3 days. Drossman
et al56 studied 800 normal adults with no gastroin-
testinal complaints and determined a normal range
was at least 1 movement every three days and no
more than 3 movements per day. Sherman et al26
avoided these criticisms because he was the Swenson
procedure surgeon in only a small number of the
total patients he interviewed about bowel function
and continence. Using the normal pattern of bowel
function to determine outcomes, he found that 96%
of the patients fell into the normal pattern and were
continent. In an attempt to determine the quality of
life the patients experienced, he asked each patient if
they considered their bowel habits normal. Affirma-
tive answers were received from 94%. Rescorla in his
report on 260 patients treated with the Duhamel
modification adhered to the same standards. Only
103 patients were available for long-term follow-up
and the outcomes in 67% were very satisfactory, 27%
required occasional enemas or stool softeners and 8%
had constipation or soiling.32 Heij et al54 reported the
results on 75 patients treated with the Duhamel mod-
ification. Ten of the 49 patients ⬎4 years old were
continent without constipation, 22 had soiling
and/or constipation, and 17 were incontinent.54 In
his review of collected cases treated with his modi-
fication, Soave31 reported 83% as good and 17% as
unsatisfactory. Quinn et al57 investigated the long-
term incidence of constipation after operation. The
incidence was 54% after the Duhamel, 43% after the
Soave, and 4% after the original Swenson operation.
DISCUSSION
The last 50 years have seen the successful applica-
tion of operative therapy to the management of
Hirschsprung’s disease, but controversy continues
over the issue of what is the best operation. The
major deficit after the Duhamel modification is con-
stipation and poor emptying. Martin and Altemeier58
insist that the rectum should be left in place so that it
can perform its normal function. It is difficult to
comprehend how an aganglionic rectum can func-
tion normally. It is true that with a series of modifi-
cations, the troublesome pouch problem may have
been eliminated. However, half of the rectum and
some of the rectosigmoid wall are aganglionic tissue
with limited peristaltic contraction. Additional evi-
dence that when half the circumference of intestine is
made up of aganglionic tissue there is functional
disruption and stasis is suggested by the results of
the Martin procedure used in patients with total
colon agangliosis.24,25
Another concern expressed about operative proce-
dures for Hirschsprung’s disease is data on sexual
function. In the review of 2400 patients treated with
the Duhamel modification, no data on this subject are
included.38 In their report on the Duhamel operation,
Rescorla et al32 do not mention sexual function. Pol-
ley et al59 stated that the advantage of the Soave
modification over the Swenson operation was that
the rectal intramural dissection ensured that no dam-
age would be done to the pelvic neural structures
that might result in urinary and fecal incontinence
and possibly damage sexual function. The report of
Sherman et al26 demonstrated that these hypothetical
fears are groundless and that all the defective tissue
can be removed during extrarectal dissection with-
out these postulated damages. In contrast, Sherman
et al26 state that no defect in urinary or sexual func-
tion occurred in his review of a large number of adult
postoperative patients treated with the Swenson op-
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4. eration. In 1 report of patients treated with the Swen-
son operation, 80 patients were married and collec-
tively there were 146 children.60
The Soave modification is complicated by recur-
rent enterocolitis and constipation. Holschneider et
al61 found in his large review that the Soave modifi-
cation had the highest incidence of postoperative
enterocolitis followed by the Duhamel. The least in-
cidence occurred after the Swenson operation.60,61
Kimura et al62 found persistent rectal achalasia in
postoperative patients treated with the Soave modi-
fication and subjected them to posterior rectal myec-
tomy. They believe that the cause of this was the
telescoping type of anastomosis associated with this
Soave technique, which incorporates an extra layer
into the anal canal. Abbas Banani and Forootan63
reported a series of 37 patients who underwent a
Soave modification and 6 had signs and symptoms
similar to their preoperative state. Conservative ther-
apy did not improve their condition. On examina-
tion, they had high anal canal pressures. After ano-
rectal myectomy, there was a reduction in anal canal
pressure and improvement in their symptoms. Blair
et al64 reported on 10 postoperative patients who
were not relieved of their symptoms. They had been
treated with both the Soave modification and the
Duhamel modification. All were treated with poste-
rior internal sphincterotomies. It is important to note
that Wulkan and Georgeson’s patients treated with a
telescoping anastomosis all required daily rectal di-
latations for prolonged periods, and Soave dilated all
his patients postoperatively.16,31 Postuma and Cork-
ery52 believed that the long aganglionic spastic mus-
cular sleeve that the normal colon is pulled through
limited rectal capacity and contributed to the persis-
tent diarrhea he observed. Rescorla et al32 state that
the variability in long-term results might be related
to the varying amounts of aganglionic tissue left in
place. Touloukian, in discussing these papers, won-
ders if consideration should be given to going back
to the concept of removing all the defective tissue.
The concept that removing the aganglionic rectum
obliterates a reflex that causes relaxation of the
sphincter as Varma and Stephens65 reported is seri-
ously questioned. A series of reports has concluded
that the sensation of rectal fullness is mediated from
sensory endings in the levator and puborectalis sling
rather than the rectum.66–68 Adults treated by the
original operation with removal of the aganglionic
rectum insist that they can detect rectal fullness.
The Duhamel and Soave modifications are less
attractive than the original Swenson operation be-
cause of the greater numbers of complications and
the poorer long-term outcomes. Surgeons who use
these modifications are meticulous, resorting to fro-
zen sections to determine that at the oral end all of
the aganglionic colon is completely removed; yet at
the pelvic end they are content to leave varying
amounts of aganglionic tissue in place. Sherman et
al26 have reported outcomes that can be achieved for
Hirschsprung’s disease patients when treated with
the Swenson operation in a children’s center staffed
with experienced pediatric surgeons backed by pe-
diatric pathologists, radiologists, and pediatricians.
It is important to realize that the surgical procedure
is only the first step in curing these patients; and
there is a variable period for the patient and parent to
be guided to overcome enterocolitis and to gain fecal
control. To ensure optimal operative outcomes, ease
of operative performance of the operative technique
is secondary. Rather, the selection of the course of
treatment should be based on proven long-term re-
sults and outcomes.
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A MOTHER’S QUERY
“I’m currently in my ninth week of pregnancy, and will get a cerclage around
week 13. We do NOT want another micropreemie—hell, we don’t want another
preemie at all, micro or otherwise. I don’t think our family could handle it, and I
don’t want another child to undergo what N did. Short of moving to a third-
world country where there ARE no NICUs, what can we do to ensure that our
wishes are abided by?”
Anonymous
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6. DOI: 10.1542/peds.109.5.914
2002;109;914
Pediatrics
Orvar Swenson
Hirschsprung's Disease: A Review
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