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Orvar Swenson, MD
B
ecause it is now more than 50 years since both been previously published and became the definitive
the discovery of the cause and a treatment for test to establish the diagnosis of congenital megaco-
Hirschsprung’s disease, it seems appropriate lon. Delayed clearance of barium from the colon after
to assess outcomes for those unfortunate children the enema was additional evidence suggesting
who have this previously lethal disease. In 1948, the Hirschsprung’s disease. It soon became apparent
discovery of the cause of this disease was based on a that this test was less sensitive for detecting short
series of clinical observations indicating that there lesions, total colon aganglionosis, and the disease in
was a defective segment of distal colon producing a the newborn. Therefore, studies were performed us-
partial bowel obstruction.1 Peristaltic tracings of the ing the surgically removed distal segment. Strips of
dilated proximal colon recorded progressive contrac- tissue were cut from the full length of the specimen
tions; however, this peristaltic wave did not enter the extending down to the anal canal, and, for the first
more distal narrow segment. This was suggestive time, the whole segment was found to be devoid of
evidence of a physiologic defect in that distal seg- Auerbach’s plexus.1 In 1957, this led us to recom-
ment. Therefore, removing this segment would mend rectal biopsy as the most reliable test to estab-
prove the validity of these observations. An opera- lish the diagnosis even for those groups with a con-
tive technique was devised and tested in the labora- flicting barium enema radiograph.6 This procedure
tory, and when it proved feasible, a patient’s distal required a general anesthetic and was likely to leave
narrow segment was removed and his intestinal con- scarring, complicating the definitive operation. Mu-
tinuity was restored with a pull-down and reanasto- cosal biopsy and the application of histochemical
mosis. Soon thereafter, the patient’s gastrointestinal staining eliminated these problems and such speci-
system began to function normally. We concluded mens were often obtained at the bedside by a suction
that this operation provided experimental evidence cup or forceps.7 In a review of 1340 mucosal biopsies,
that proved our assumptions were correct.2 there were 3 cases of bleeding requiring transfusion
and 3 clinical perforations, 1 resulting in death.8 One
DIAGNOSIS caution in performing rectal biopsy for the diagnosis
It is possible to suspect the presence of Hirsch- of Hirschsprung’s disease is the recognition that
sprung’s disease in the newborn by clinical criteria if there is normally a 2-cm band in the distal rectum at
the infant does not pass meconium in the first 48 the level of the internal anal sphincter devoid of
hours or by the presence of vomiting and abdominal ganglion cells.9
distention.3 In the older child, clinical criteria for
diagnosis includes a history of severe constipation OPERATIVE TREATMENT
from birth, failure to thrive, abdominal distension The original operation (the Swenson procedure)
and often an empty rectal ampulla on rectal exami- consisted of freeing the defective distal colon from
nation. The disease occurs about once in 5000 live within the pelvis by careful sharp extrarectal dissec-
births.4 The incidence of this disease being transmit- tion down to 2 cm above the anal canal and perform-
ted to offspring is about 3%. In specific instances in ing an end-to-end anastomosis. Applying this tech-
females where the length of the aganglionic lesion is nique, the defective aganglionic tissue is completely
extensive, the percentage increases.4 removed and the proximal ganglionated colon and
As part of our investigation into the cause of this anal canal are left in a normal anatomic position.2
disease, a radiographic enema using a small amount In 1960, Duhamel10 published a modification of
of barium outlined an irregular and narrowed small the original operation. He resected a portion of the
colon beginning in the anal canal and extending distal defective segment as in the original operation
proximally before it fanned out and connected with and made a side-to-side anastomosis between the
the more proximal dilated colon.5 Roentgenograms proximal normal colon and defective rectum. In
of this portion of the colon in this disease had never 1963, Soave11 published another modification. It con-
sisted of making a circumferential cut through the
muscular coat of the colon at the pelvic peritoneal
From Charleston, South Carolina. reflection. Working in an intramural plane, the mu-
Received for publication May 11, 2001; accepted Jan 14, 2002. cosa is separated from the muscular coat down to the
Reprint requests to (O.S.) 1 Gadsen Way, Apartment 322, Charleston, SC
29412-3574.
anal canal. The freed mucosa is excised and the prox-
PEDIATRICS (ISSN 0031 4005). Copyright © 2002 by the American Acad- imal normal colon is pulled through this retained
emy of Pediatrics. viable spastic aganglionic muscular sleeve. A tele-
REVIEW
Downloaded from www.aappublications.org/news at Indonesia:AAP Sponsored on July 28, 2021 ARTICLE 915
nique. There also are reports of abscess formation total patients he interviewed about bowel function
between the retained aganglionic muscular sleeve, and continence. Using the normal pattern of bowel
retraction of the pulled through normal colon, and function to determine outcomes, he found that 96%
mucosal prolapse after the Soave modification, com- of the patients fell into the normal pattern and were
plications that can occur years after the operation.46 continent. In an attempt to determine the quality of
Fortuna et al47 has reported secondary reoperation life the patients experienced, he asked each patient if
rates in patients treated by the Duhamel to be 29% they considered their bowel habits normal. Affirma-
and after the Soave to be 26%. The postoperative tive answers were received from 94%. Rescorla in his
occurrence of enterocolitis was 19% to 20%. Sherman report on 260 patients treated with the Duhamel
et al26 reported that in 880 Swenson procedure cases modification adhered to the same standards. Only
the secondary reoperation rate was 6%. This 103 patients were available for long-term follow-up
stemmed from an anastomotic leak rate of 5.6%, and the outcomes in 67% were very satisfactory, 27%
which required diverting colostomies, and, in rare required occasional enemas or stool softeners and 8%
cases, the need to surgically excise strictures. In a had constipation or soiling.32 Heij et al54 reported the
recent report on 97 patients treated with the original results on 75 patients treated with the Duhamel mod-
operation, the secondary operation rate was 6%.35 ification. Ten of the 49 patients ⬎4 years old were
Throughout the 40-year study of the original opera- continent without constipation, 22 had soiling
tion, the postoperative rate of enterocolitis was and/or constipation, and 17 were incontinent.54 In
11%.26 his review of collected cases treated with his modi-
There are several reports that some patients had fication, Soave31 reported 83% as good and 17% as
such poor results from their pull-through that com- unsatisfactory. Quinn et al57 investigated the long-
plete reoperation was required.48 –50 A small number term incidence of constipation after operation. The
of these were for stricture or retained aganglionic incidence was 54% after the Duhamel, 43% after the
tissue, the remainder were for recurrent enterocolitis, Soave, and 4% after the original Swenson operation.
constipation, or soiling. The entire reoperative group
had either a primary Duhamel or Soave modifica- DISCUSSION
tion. In a large series of Duhamel procedures, 4.9% of The last 50 years have seen the successful applica-
patients had such poor results that reoperations were tion of operative therapy to the management of
required.38 Furthermore, Tariq et al51 reported such Hirschsprung’s disease, but controversy continues
unrelenting postoperative constipation in 3.7% of over the issue of what is the best operation. The
their patients after treatment with the Soave modifi- major deficit after the Duhamel modification is con-
cation that reoperations were required. Sherman et stipation and poor emptying. Martin and Altemeier58
al,26 Carcassonne et al,33 Weizman et al,34 Shand- insist that the rectum should be left in place so that it
hogue and Bianchi,35 Waldron and O’Donnell36 and can perform its normal function. It is difficult to
Madonna et al37 all using the original Swenson op- comprehend how an aganglionic rectum can func-
eration have not encountered problems that required tion normally. It is true that with a series of modifi-
reoperation. cations, the troublesome pouch problem may have
It is generally believed that all postoperative been eliminated. However, half of the rectum and
Hirschsprung’s disease patients, regardless of the some of the rectosigmoid wall are aganglionic tissue
original operative procedure, improved with time. with limited peristaltic contraction. Additional evi-
This is challenged by Postuma and Corkery,52 who dence that when half the circumference of intestine is
observed that this was true for a period of time, but made up of aganglionic tissue there is functional
that those treated by the modifications subsequently disruption and stasis is suggested by the results of
had an increase in troubles. Mishalany et al53 and the Martin procedure used in patients with total
Heij et al54 have expressed the same concern. colon agangliosis.24,25
Another concern expressed about operative proce-
OUTCOMES OF OPERATIVE TREATMENT FOR dures for Hirschsprung’s disease is data on sexual
HIRSCHSPRUNG’S DISEASE function. In the review of 2400 patients treated with
There have been concerns about how end results the Duhamel modification, no data on this subject are
are evaluated. The commonly used method is to list included.38 In their report on the Duhamel operation,
results as excellent, good, fair, and poor. This Rescorla et al32 do not mention sexual function. Pol-
method involves judgment and in collected series, ley et al59 stated that the advantage of the Soave
the evaluation is made by the operating surgeon to modification over the Swenson operation was that
whom parents are reluctant to report problems. In an the rectal intramural dissection ensured that no dam-
attempt to enhance objectivity, the results can be age would be done to the pelvic neural structures
reported by the normalcy of bowel function. Duth- that might result in urinary and fecal incontinence
rie55 has estimated the normal pattern of bowel func- and possibly damage sexual function. The report of
tion is one movement or more each 3 days. Drossman Sherman et al26 demonstrated that these hypothetical
et al56 studied 800 normal adults with no gastroin- fears are groundless and that all the defective tissue
testinal complaints and determined a normal range can be removed during extrarectal dissection with-
was at least 1 movement every three days and no out these postulated damages. In contrast, Sherman
more than 3 movements per day. Sherman et al26 et al26 state that no defect in urinary or sexual func-
avoided these criticisms because he was the Swenson tion occurred in his review of a large number of adult
procedure surgeon in only a small number of the postoperative patients treated with the Swenson op-
REVIEW
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and personal observations over 50 years. J Pediatr Surg. 1996;31: disease: indications, techniques and results. J Pediatr Surg. 1999;34:
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31. Soave F. Endorectal pull-through 20 years experience. J Pediatr Surg. following the modified Soave operation for Hirschsprung’s disease.
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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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