The Duhamel Procedure for Hirschsprung's Disease
By Paul T. Stockmann and Arvin I. Philippart
Detroit, Michigan
A HALF CENTURY has passed since Swenson de-
scribed the first successful definitive operation for
Hirschsprung's disease.' In the ensuing 15 years, the
DuhameF and Soave3 procedures were devised as alterna-
tives. All three procedures remain viable options, particu-
larly for "traditional" Hirschsprung 's disease with a
rectosigmoid transition zone. All three are performed by
their devotees with similar results in terms of bowel
function . Morbidity and mortality results in large series
of each procedure are similar. All three procedures have
been modified by subsequent generations of pediatric
surgeons for specific reasons or applications. It is our
belief that no one of the three procedures is "best" for all
circumstances. At least the Swenson or Soave and the
MartinlDuhamel should be in the armamentarium of the
pediatric surgeon. Furthermore, some insight into the
pathophysiology of Hirschsprung's disease and its vari-
ants as well as the history of the evolution of the three
procedures is valuable in considering not only the choice
of operation but also staged or primary reconstruction.
PATHOPHYSIOLOGY
The clinical observations by Ehrenpreis 4 and the
pathophysiological ones by Swenson' utilizing manom-
etry, further confirmed by the radiological observations of
Swenson and Neuhauser,s were seminal in identifying the
colon distal to the transition zone as the cause of
obstruction, and led Swenson to introduce resection of
the colon distal to the transition zone.
Essentially simultaneously, Zuelzer and Wilson6 and
Whitehouse and Kernohan? independently identified the
absence of ganglion cells distal to the transition zone as
the etiology or histopathological correlate that became
the sine qua non for the clinical diagnosis of Hir-
schsprung's disease. However, less than perfect func-
tional outcomes in large series of operated patients
progressively led to the realization that the pathophysiol-
ogy of Hirschsprung's disease is more than the simple
presence or absence of ganglion cells. Recent years and
further study of the histology of the autonomic nervous
system, first by Meier-Ruge8 and more recently by Puri,9
have identified abnormalities of the autonomic nervous
system superimposed upon, or separate from, the simple
presence or absence of ganglion cells. These additional
observations are currently grouped in the general cat-
egory of intestinal neuronal dysplasia (IND). This cohort
of observations, as well as the earlier recognition of the
presence of a hypertonic internal anal sphincter, is
important in understanding why clinical outcomes of any
Seminars in Pediatric Surgery, Vol 7, No 2 (May). 1998: pp 89-95
operation were imperfect in earlier decades and remain so
today. Incomplete understanding of the pathophysiology
as described, as well as technical complications, led to
modifications of each of the current three "standard"
operations for Hirschsprung's disease. A superficial knowl-
edge of the history of the development of the "standard"
operations and their modifications should provide insight
into current choices of definitive operation for situations
other than rectosigmoid transition zones if not also for the
common presentations.
OPERATIVE PROCEDURES
The current goal of all procedures for Hirschsprung's
disease is to place normally functioning ganglionated
intestine within 1 cm of the anal verge. The Swenson
procedure was not only the first to accomplish this but
also the standard against which all other procedures must
be measured (Fig 1). A near-total excision of the agangli-
onic colon and rectum was performed with a low oblique
colorectal anastomosis. Careful dissection of the rectal
wall was deemed necessary to preserve the nervi eri-
gentes. The complications included recurrent enterocoli-
tis and anastomotic leaks, with stricture and fecal inconti-
nence in some.
Duhamel's retrorectal pull-through procedure was de-
scribed in 1956 and avoided the extensive pelvic dissec-
tion anterior to the rectum. 2 The native rectum was left in
situ, and an end-to-side colorectal anastomosis was
created. The adjacent walls of the native rectum and
pulled-through colon were crushed within Kocher clamps
placed through the anus. The clamps later sloughed,
resulting in a side-to-side anastomosis. This component
was simplified by mechanical stapling devices. Martin
modified Duhamel's technique to obliterate the blind end
created by the aganglionic rectal pOUCh.1O The recent
development of smaller laparoscopic stapling devices has
facilitated the application of this procedure in the neona-
tal period.
The Soave operation 3 was reported in English in 1964
and is an application of the technique described by
Ravitch and Sabiston in 1948." These procedures are
based on a mucosal proctectomy and low endorectal
From the Departments of Pediatric General and Thoracic Surgery,
ChiLdren S HospitaL of Michigan, Detroit, MI.
Address reprint requests to Arvin I. Philippart, MD, Children S
Hospital of Michigan, Pediatric GeneraL and Thoracic Surgery, 3901
Beaubien BLvd, Detroit, M148201 .
Copyright © 1998 by WE. Saunders Company
1055-8586/98/0702-0003$08.00/0
89
90
State Swenson
® pull-through procedure is universally superior based on
Du hamel/Mar tin
Duhamel
®
- Our technique for the Duhamel pull-through procedure
Soave/1'>otey
© resume a more normal size before the definitive pull-
Fig 1. Graphic representation in lateral view of the three major
surgical procedures for Hirschsprung's disease. Evolution of each
from left to right. Unshaded native rectum is aganglionic. Shaded
pulled-through bowel contains ganglion cells. (A) State's procedure
was a prototypic anterior resection of dilated rectosigmoid. Lengthy
aganglionic segment remained. With the Swenson procedure, an
oblique anastomosis resulted in ganglion cells within 1 cm of the
verge posteriorly. (B) In the original Duhamel operation, the over-
sewn native rectum enlarged as a blind loop, which resulted in a
fecaloma that caused partial obstruction. With Martin's modification,
the blind loop is obviated by complete division of the septum and
anastomosis of the anterior walls of the native rectum and pulled-
through colon. Bowel that contains ganglion cells reaches within 1
cm of the anal verge posteriorly. (e) In the original Soave procedure,
full-thickness colon that contained ganglion cells was advanced
through the demucosalized native rectal sleeve. Excess colon ex-
tended from the anus for several weeks before transection and
delayed anastomosis. With the Boley modification, a primary anasto-
mosis is performed 1 cm above the verge. Ganglion cells are present
circumferentially at that level. (Reprinted with permission. 17 )
anastomosis. Soave's technique included prolapse of
excess pulled-through colon through the anus. A sponta-
neous anastomosis occurred 2 to 3 weeks later. Boley's
"modification" included a primary sutured anorectal
anastomosis. 12
All three procedures with their modifications have
been successful in experienced hands, but the disease-
specific complications of Hirschsprung's disease (ie,
stricture, leak, enterocolitis, and incomplete fecal conti-
STOCKMANN AND PHILIPPART
nence) have occurred after successful pull-throughs of all
types. One or more modifications have been developed
by other surgeons in an attempt to avoid some of these
complications. At this point, it seems that no one
outcome.
In this report we describe our approach for application
of the Duhamel procedure for children with Hir-
schsprung's disease. This is based on our institutional
experience in the surgical management of more than 300
children with Hirschsprung's disease. 13
Operative Technique
has been described previously. 14 The majority of patients
have undergone a two-stage reconstruction with an initial
colostomy at or proximal to the transition zone as the first
stage. This helps to prevent significant enterocolitis and
to allow growth and development in neonates and infants.
A diverting colostomy in patients presenting with marked
proximal intestinal dilatation allows the intestine to
through is performed. Intestinal length is salvaged by
avoiding an extensive resection of the dilated but normal
intestine. For infants, the second-stage procedure is
performed at 6 months of age, after demonstration of
normal growth. In the older child, the second-stage
procedure is postponed until colonic size is appropriate
for pull-through. This generally requires at least 4 to 6
months of decompression.
Patients are admitted to the hospital the day before the
definitive pull-through procedure, and undergo mechani-
cal and antibiotic bowel preparation. A rectal examina-
tion is performed at the time of admission to ensure that
the rectum is empty.
The patient is placed on the operating table in the
supine position. The skin is prepped from the nipples to
the knees, including the buttocks and lower back. Intrave-
nous access is established in an upper extremity. The
patient is positioned so that the buttock overlies the break
in the table, allowing the perineal portion of the operation
to be performed with the surgeon in the sitting position.
The lower extremities are wrapped individually in sterile
stockinettes, and a Foley catheter is placed after sterile
drapes are in place. Intravenous antibiotics are adminis-
tered before the skin incision.
The operation is initiated with a laparotomy. For
patients with a left-lower-quadrant colostomy, an oblique
incision is made surrounding the colostomy and extend-
ing toward the pubis. A vertical midline incision is used
for patients with proximal transition zones to allow
complete visualization of the colon and its mesentery. A
low transverse incision is recommended for patients
undergoing a primary pull-through. After the colostomy
DUHAMEL PROCEDURE 91

is completely dissected free from the abdominal wall

fascia, the colon is divided. The proximal ganglionated
intestine designated for the pull-through is then mobi-
lized. For rectosigmoid disease, this usually requires
ligation of the inferior mesenteric artery near its origin,
with preservation of the remaining arcades and the
marginal artery. Adequate length to reach the perineum is
established before the pelvic portion of the operation. For
longer segment colonic disease, it is often necessary to
ligate the middle colic artery. In this case, the colon is
rotated in a counterclockwise direction to keep the
mesentery straight and avoid compression of the terminal
ileum by the pulled-through colon. The distal aganglionic
colon is mobilized by dividing the superior hemorrhoidal
vessels. As the dissection continues toward the pelvis, a
plane is developed directly adjacent to the posterior rectal
wall with the rectosigmoid on traction. This is a relatively
avascular plane. This dissection is developed toward the
perineum using blunt dissection with the index finger. It
is critical to stay in the plane between the rectum and the
striated muscle complex near the anal verge. At this level,
the coccyx can be palpated with the dissecting finger. The
adequacy of the dissection can be confirmed by visualiz-
ing the dentate line through the anus with the dissecting Fig 2. Author's technique. (A) Dissection of the presacral space
finger in the retrorectal space. At this point, a small folded separates the native rectum from the striated muscle complex
sponge is placed within the tips of a curved ringed clamp, posteriorly (lateral view). A tightly wound sponge and forceps are
inserted in the space created. (8) Anterior view of the distal anorec-
which is then placed down into the retrorectal space tum from the perineum after a "smile" incision over a sponge in the
(Fig 2). The previously mobilized proximal intestine posterior wall of the anorectum. The apex of the smile incision is 0.5
cm from the anal verge. Traction sutures are placed in the midline on
designated for the pull-through is reinspected for ad-
the superior and inferior lips as well as the lateral extremes before
equate perfusion before the perineal portion of the complete removal of the sponge and subsequent advancement of
operation is begun. proximal ganglionic bowel. (C) Completed end-to-side one-layer silk
anastomosis with two sutures on either side of the midline septum
The patient is repositioned by elevating the legs and
left in place for traction during insertion of a stapling device along the
suspending them to the ether screen with gauze rolls. The dotted line. (D) Completed Duhamel anastomosis as modified by
end of the operating table is lowered so the surgeon can Martin. (Reprinted with permission.17)
assume a sitting position if desired. The anorectum is
exposed with retractors, and the dentate line is visualized. and the pulled-through colon. Traction is placed on the
The sponge in the retrorectal space is identified by sutures on the anterior aspect of the anastomosis while
inspection and palpation. Using electrocautery, a semicir- the stapler is set and employed. While the stapler is still in
cular incision is made in the posterior rectal wall parallel place, the tips are palpated and the native rectum is
to and 0.5 cm above the dentate line, extending for divided at this point. The stapler is removed, and the
approximately one third of the circumference of the integrity of the anastomosis is established by inspection
rectum. Sutures are placed in each quadrant to provide and palpation. The pulled-through colon is opened trans-
traction and maintain orientation. The sponge clamp in versely just above the transected native rectum. The most
the retrorectal space is removed, and a similar curved ring proximal portion of the anastomosis is completed using
clamp is placed through the posterior rectal incision. The interrupted 3-0 silk sutures. The abdomen is closed in the
mobilized proximal colon is gently and carefully pulled usual fashion. No drains are employed.
down through the retrorectal space and out the posterior Postoperatively, nasogastric drainage is maintained
rectal incision, onto the perineum. Care is taken to insure until bowel function returns. The Foley catheter is usually
that the mesentery is straight and oriented posteriorly. removed I to 2 days postoperatively. Intravenous antibi-
The previously placed traction sutures are used to create otics are continued for 72 hours. Generally patients are
the four comers of the end-to-side colorectal anastomo- discharged 4 to 6 days after surgery. On the day of
sis. This anastomosis is performed using a single layer of discharge, the operating surgeon performs a gentle rectal
3-0 silk sutures. After the anastomosis is completed, a examination to ensure separation of the adjacent staple
linear mechanical stapler is placed into the native rectum lines.
92
RESULTS
Our institutional experience includes more than 300
children treated at the Children's Hospital of Michigan
from 1960 to the present and represents the composite
experience of several surgeons. These results have been
reported previously.13
The distribution of disease involvement included 58%
with rectosigmoid disease, 26% with long-segment agan-
glionosis, and 12% with total colonic aganglionosis.
During the 1980s, 20% of patients treated for Hir-
schsprung's disease had total colon involvement. The
majority of patients were male (78%); however, this was
less for those with total colon involvement (68%). Eight
percent of patients had been born prematurely. The mean
age at diagnosis was 10 months; but in the last decade it
has decreased to 2.5 months. For patients with total
colonic Hirschsprung's disease, the diagnosis was made
within the first month of life.
The most common presenting symptoms include ab-
dominal distension (80%), constipation (80%), and vom-
iting (58%). Overt signs of malnutrition were unusual.
Vomiting was the predominant symptom (73%) in pa-
tients who presented with total colonic Hirschsprung's
disease. Barium enema was diagnostic in 80% of cases
with rectosigmoid and long-segment disease but in only
55% of those with total colon involvement. The diagnosis
was confirmed by rectal biopsy; although presently the
suction rectal biopsy is used more frequently, historically,
85% underwent full-thickness rectal biopsies. Enterocoli-
tis has occurred before the colostomy, after the colos-
tomy, and after definitive pull-through procedures. The
incidence of enterocolitis was highest after pull-through
procedures and in patients with total colonic Hir-
schsprung's disease, consistent with experience of others.
The mean age at which children undergo definitive
pull-through has decreased and is presently 15 months,
which includes those with a prolonged delay in diagnosis.
The majority of patients have undergone a two-staged
approach with initial colostomy followed by definitive
pull-through. The Duhamel operation is used for children
with all levels of disease and represents approximately
25% of all pull-throughs performed at our institution. The
Duhamel pull-through is preferred for total colonic
Hirschsprung's disease.
Favorable outcome has been achieved for the majority
of children undergoing definitive operations, and our
results have been consistent with those of other series.
Postoperative complications, including enterocolitis, oc-
curred in 12% of patients after the pull-through. The
complication rates varied according to the type of proce-
dure performed. The Soave endorectal pull-through had a
complication rate of 17%, including 0.38 episodes of
enterocolitis per patient during long-term follow-up.
Complications occurred in 13% of patients after a
STOCKMANN AND PHILIPPART
Duhamel procedure, including 0.95 episodes of enteroco-
litis per patient. This includes patients with all levels of
disease involvement. For patients with total colonic
Hirschsprung's disease, the incidence of enterocolitis was
lower after the Duhamel operation (0.75 episodes per
patient) than the Soave endorectal pull-through (1.0
episodes per patient). In our experience, postoperative
mortality has been negligible.
DISCUSSION
Based on this experience, we have made the following
conclusions. (1) The Soave endorectal procedure is the
more popular operation. (2) The Duhamel pull-through
has fewer overall complications but a greater risk of
enterocolitis postoperatively. (3) The Soave procedure
has the lowest rate of enterocolitis with rectosigmoid and
long-segment disease, but a greater overall complication
rate. (4) The Duhamel pull-through and the Soave
operation are equally effective for patients with rectosig-
moid and long-segment disease. (5) For total colonic
aganglionosis, the Duhamel procedure is preferred be-
cause of its lower complication rate, lower risk of
enterocolitis, and more satisfactory functional result
regarding stool frequency. (6) Enterocolitis continues to
be a significant problem even after a colostomy or
pull-through. The risk is highest with total colonic
disease but is no different for rectosigmoid and long-
segment Hirschsprung's disease. The clinical definition
of enterocolitis after diversion or pull-through remains
problematic. Diarrhea with significant fluid and electro-
lyte loss often is attributable to acquired intercurrent
illness rather than to pseudo-obstruction and can be
exacerbated by more proximal diversion or anastomosis.
In either case, hospital admission is justified and the
condition is considered to be an episode of enterocolitis.
More than 30 years ago, Duhamel reviewed his
experience with 270 pull-through procedures. IS Constipa-
tion or diarrhea occurred in 3.7%, but 100% were
continent. Others have reported a postoperative enteroco-
litis rate of 12% and continence rate of 100% after 15
years. 16 In another reported series of patients who under-
went the Duhamel procedure, there was no postoperative
mortality. Postoperative enterocolitis occurred in 10%,
and more than 90% had normal bowel function within 1
year of the surgery.17 Favorable short-term results have
been reported with the primary Duhamel procedure in
newborns. IS
Primary Versus Staged Approaches
The disadvantages of the staged approach includes
stoma-related complications and the need for an addi-
tional operation and hospitalization. Colostomy complica-
tions (prolapse, stricture) requiring surgical revision are
not common and the incidences range from 2% to
DUHAMEL PROCEDURE
13%.16.19 These problems can be minimized with careful
technique and appropriate selection of stoma type. Loop
and end colostomies generally are equivalent in most
circumstances, but end stomas are recommended for
cases with massive colonic distension or small bowel
transition zones. In these cases, specific measures to
reduce the size of the stoma are necessary to minimize
prolapse because the caliber of the colon gradually
diminishes after diversion.
Primary pull-through procedures are appropriate in
many cases of Hirschsprung's disease. The outcome
should be favorable in well-selected cases (Table 1).
Suction rectal biopsy usually is satisfactory in children
under than 1 year of age; however, this technique may not
provide adequate tissue. In this event, a trans anal open
rectal biopsy should be performed. An experienced
pathologist is essential for accurate interpretation.
It is recommended that a barium enema be performed
before a primary pull-through to establish the transition
zone and the degree of proximal intestinal dilatation.
However, up to 20% of cases will not have a demon-
strable transition zone, most commonly in newborns or
with total colon aganglionosis. A primary pull-through is
most appropriate with rectosigmoid disease and minimal
proximal dilatation. In such instances, a primary proce-
dure should be performed early if malnutrition and
enterocolitis are not present. Significant intestinal dilata-
tion often is associated with a prolonged delay in
diagnosis. A primary pull-through should not be done if
the ganglionated bowel is so large that extensive resec-
tion is required or if a tenuous anastomosis would be
created. Patients who present with enterocolitis or signifi-
cant malnutrition require hospitalization for initial man-
agement. A definitive procedure should be postponed
until these complications are resolved completely. Pa-
tients with severe enterocolitis should be managed with a
staged approach. Primary procedures are not recom-
mended for cases of long-segment or total colonic
aganglionosis or those with unclear frozen section re-
sults. In such cases, a stoma followed by permanent
section confirmation and months of demonstrated clinical
function reduce complications and better predict a good
Table 1. Primary Versus Staged Pull-Throughs:
Appropriate Applications
Parameter Primary Staged
Pathology Confirmed Uncertain
Disease involvement Rectosigmoid Long segment.
total colon
Enterocol itis Absent/resolved Active/severe
Intestinal dilatation Minimal Marked
Nutritional state Normal Depleted
Gestational age (newborn) Full term Premature
Colonic irrigation Effective decom- No decom-
pression pression
93
result from a second-stage pull-through. Extensive colon
resection combined with a definitive pull-through based
on multiple intraoperative frozen section biopsies is not
recommended. Hirschsprung's disease is not common in
premature newborns. A preliminary colostomy is appro-
priate, and a reevaluation, including another rectal bi-
opsy, should occur before the definitive pull-through.
In the last decade, numerous reports have been pub-
lished on the early results of primary pull-through
procedures. 17-22 In these series there is variation in patient
age, surgical procedure, and level of disease involvement.
These are retrospective reviews that compare children
treated during different periods. A summary of the current
literature showed an overall major complication rate
(excluding enterocolitis) of 12% (range, 0% to 31%), a
postoperative enterocolitis rate of 6% (range, 0% to
20%), and a reoperation rate (stoma or redo pull-through)
of 10% (range, 0% to 15%).21 The consensus is that for
many cases of Hirschsprung's disease, primary pull-
through procedures are highly effective, with results
comparable to those of staged reconstructions. Long-term
results (> 10 years) have not been reported.
Patients with a poor outcome after initial pull-through
require careful evaluation. This includes a contrast enema
and a full-thickness rectal biopsy under direct vision. The
histopathology of the specimens submitted at the initial
pull-through should be reviewed. Then an assessment can
be made to determine whether the outcome is attributable
to patient disease (internal sphincter dysfunction) or an
error in either technique (high anastomosis) or disease
recognition (inaccurate pathology). In many cases, out-
come can be improved with other interventions, rather
than consideration of a repeat pull-through. These include
medical management, anal dilatation, and internal sphinc-
terotomy. If these measures fail, and the radiological and
pathological findings show an inadequate initial pull-
through, repeat pull-through should be considered. Al-
though repeat Soave and Swenson pull-throughs can be
accomplished, the Duhamel retrorectal pull-through prob-
ably is the safest and most straightforward approach.
Satisfactory outcomes have been reported for this tech-
nique.23 .24
Primary Neonatal Duhamel Procedure
The first neonatal primary pull-through procedures
were performed using the Soave25 or Swenson26 opera-
tion. The drawback of the modified Duhamel procedure
has been the size of the linear stapling devices, which
were too large for use in newborns. With the development
of the Endo-GIA (United States Surgical Corp, Norwalk,
CT) device for laparoscopic use, a smaller stapler became
available, facilitating the use of a primary Duhamel
procedure in neonates. 1\vo reports have documented
94
excellent early results using this technique. J8,27 There
were no deaths in either series, and the combined
incidence of postoperative enterocolitis was 6%. No
anastomotic strictures or leaks were reported.
The Duhamel procedure has theoretical advantages
when applied as a newborn primary pull-through. Anasto-
motic complications (stricture and leak) are more com-
mon with the Soave and Swenson procedures. 28 Signifi-
cant morbidity and mortality are associated with these
complications in newborns and infants.29 Anastomotic
complications have not occurred in our experience with
newborn correction of Hirschsprung's disease by the
Duhamel technique.
Total Colonic Hirschsprung's Disease
Hirschsprung's disease involving the entire colon
poses an additional challenge. Removing the entire colon
from the fecal stream influences not only defecation
patterns but also fluid and electrolyte metabolism. Al-
though these early problems improve with time, they may
be formidable to manage during the transition phase,
especially in neonates. Modifications of the pull-through
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STOCKMANN AND PHILIPPART
procedures have been developed in an attempt to dimin-
ish this morbidity. Duhamel described leaving the agan-
glionic colon in situ. Martin extended his modification of
the Duhamel procedure, which led to a long side-to-side
anastomosis between the small bowel and the aganglionic
rectum and lower sigmoid colon.3o Recurrent enterocoli-
tis has resulted in neither of these procedures being
widely considered as an alternative.
Kimura modified the Swenson procedure to patch the
aganglionic right colon to the ileum before the pull-
through.3J In a similar fashion, Boley modified the Soave
technique with a right colon patch to the ileum before the
endorectal pull-through.32 Other surgeons have reported
satisfactory long-term outcome with the modified Martini
Duhamel procedure or the Soave endorectal pull-through.
It does appear to be advantageous to leave some of the
colon in the fecal stream to improve water and electrolyte
absorption. We believe that the standard Duhamel proce-
dure best accomplishes the goals of improved reabsorp-
tion and increased capacitance of the rectal reservoir, and
does so with at least one fewer surgical procedure than
the colon patch techniques.
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