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Correspondence to: Hussam S. Hassan, MD.

Lecturer of Pediatric Surgery, Tanta University, Tanta, Egypt, E-mail:


hassanh_30@hotmail.com



Annals of Pediatric Surgery, Vol 5, No 1, January 2009 PP 21-26
Original Article

One-Stage Transanal Endorectal Pull- through Procedure for Hirschsprungs
Disease in Neonates
Hussam S. Hassan
Pediatric surgery unit, Departments of Surgery, Tanta University, Tanta, Egypt

Background/ Purpose: Traditionally, Hirschsprung's disease (HD) was treated in a staged procedure of colostomy, the
definitive procedure then closure of colostomy. Eventually many pediatric surgeons became more interested in the one-stage
approach, and results have been favorable when compared with a staged procedure. This study aims at evaluation of
management of HD using one-stage transanal endorectal pull-through (TEPT) early in the neonatal period.
Materials & Methods: A retrospective review of 42 cases operated in the neonatal period between 2003-2007 was done.
Data collected from records included age, sex, diagnostic procedure, length of aganglionic segment, operative time, blood loss,
length of resected segment, and post operative complications.
Results: Twenty two males and 15 females were included in the study. The mean operative time was 80 min, mean blood
loss was 20 mL, and mean hospital stay was 5 days. Perianal excoriation occurred in 11 cases, enterocolitis in 5 cases, cuff
abscess in 2 cases and anastomotic stricture in 2 cases. Cases completed 3 years follow up showed complete continence in
83.3% of cases, while the remaining cases showed good resting sphincter tone and powerful squeeze pressure on manometry.
Conclusion: One stage TEPT is both feasible and safe in the neonatal period. The mucosectomy, operative time, and
intraoperative blood loss are favourable compared to previously published large series in older children. Likewise, the
postoperative complications and the functional outcome are comparable to cases operated in infancy and childhood period.

Index Word: Hirschsprung's disease, neonatal period, transanal endorectal pullthrough.


INTRODUCTION
irschsprung's Disease (HD) affects one in 5,000
newborns. The diagnosis often is suspected
when a newborn fails to pass meconium in the first 48
hours of life, has abdominal distension and
vomiting.
1

Surgical therapy for HD implies removal of
aganglionic bowel and bringing of normally
innervated intestine to the anus. Traditionally, this
was achieved by creation of a colostomy followed by
one of the pull-through procedures (Swenson,
Duhamel, Soave), then colostomy closure in the same
setting or later (2 or 3 stages).
2
Eventually many
pediatric surgeons became more interested in the one-
stage approach, and results have been favorable when
compared with a staged procedure.
3, 4

One-stage pull-through has been noted to be
particularly beneficial in neonates, due to the fact that
the colon above the aganglionic segment is less
dilated, and episodes of enterocolitis are less frequent,
making dissection much easier.
5

Transanal endorectal pull-through (TEPT) represents
the latest development in the concept of the
minimally invasive surgery for HD; the novel
H
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Annals of Pediatric Surgery 22

description of an entirely TEPT approach by De la
Torre-Mondragon and Ortega-Salgado in 1998
6
was
rapidly followed by similar promising early
experiences from various centers.
7-9

This study aims at evaluation of the feasibility and
safety of TEPT in the neonatal period with respect to
early and late complications compared to the reported
complications in previously published large series of
patients treated later after neonatal period.

PATIENTS AND METHODS
A retrospective review of the files of all patients with
HD operated between June, 2003 and April, 2007 was
done. The inclusion criteria included only patients
with proven tissue diagnosis of HD, who were
operated as one stage TEPT in the neonatal period.
The exclusion criteria included older children or
neonates with either severe enterocolitis or neglected
bowel obstruction not responding to bowel
decompression, or those who were referred to us after
initial colostomy was done elsewhwere. Neonates
with preoperative known long aganglionic segments
(proximal to sigmoid colon) were excluded from this
series.
The diagnosis was made by contrast study and rectal
biopsy. The feasibility of an entirely TEPT at the
neonatal period was assessed by the level of the
transitional zone and the general condition of the
patient. If the radiologic transitional zone was at
rectosigmoid or sigmoid colon, the patient was
considered eligible for one stage TEPT.
All patients had the following data extracted from
records for further analysis: (1) Age and sex; (2)
clinical presentation and investigations; (3) operative
details including position of patient, the level of
starting the submucosal dissection, degree of
difficulties in submucosal dissection and/or colon
mobilization, length of the remaining cuff, iatrogenic
injury of any structure during surgery, length of the
excised specimen, estimated blood loss and blood
transfusion, conversion to laparotomy and its cause,
and operating time; (4) early postoperative course
particularly time at regain of peristalsis, timing of first
passage of stool, and starting of oral feeding; (5)
postoperative complications such as significant
perineal excoriation, anastomotic leak, anorectal
stricture, enterocolitis, and perineal or pelvic
infection; (6) functional outcome as judged by bowel
habits, recurrent abdominal distension, and anorectal
continence status; and (7) need for a secondary
surgical procedure such as myectomy, or redo pull-
through. Postoperative investigations including
contrast enema, anorectal motility, and
electromyogram (EMG) were performed only for
patients with complications or problems with bowel
control.
All cases had undergone one-stage TEPT.
Preoperative bowel preparation of the colon using
warm saline was done until effective decompression
of the bowel was achieved.
Surgical technique:
After the induction of general endotracheal anesthesia
and the placement of intravenous lines, the patient
was given one of the third generation cephalosporin
intravenously. Rectal irrigation was performed with a
dilute solution of betadine. The patient was then
placed supine with the pelvis elevated at the end of
the operating table with the lower limbs attached to
an inverted U-shaped bar. The abdomen and
perineum were prepared in the standard fashion. A
bladder catheter was not routinely inserted. The anal
canal was exposed with Loan-Star anal retractor.
Submucosal injection of epinephrine or saline was not
routinely used. A circumferential row of 4-0 silk stay
sutures was inserted approximately 0.5 to 1 cm above
the dentate line. The rectal mucosa was incised just
distal to the traction sutures and lifted
circumferentially using fine diathermy needle to
develop the submucosal plane. Once the submucosal
plane was established, the dissection was easily
continued proximally using blunt dissection and
cauterization of submucosal infiltrating vessels. The
traction on mucosal tube facilitated proximal
extension of mucosal dissection until the level
proximal to peritoneal reflection (approximately 10 to
15 cm above the dentate line). Four stay sutures were
inserted to control the upper end of muscular cuff,
which was incised circumferentially allowing
exposure of the full-thickness sigmoid colon.
Mobilization of the colon was continued as proximal
to the grossly obvious or histologically confirmed
transition zone as possible by dividing the
rectosigmoid vessels after cauterizing them. Ligatures
were rarely needed in neonates. The long
seromuscular cuff was inverted outside the anus and
shortened to less than 5 cm in length before returning
it to its normal position. After resection of the
aganglionic segment, the normally innervated bowel
was pulled through the muscular cuff and
anastomosed to the remaining mucosa above the
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23 Vol 5, No 1, January 2009

dentate line using 4-0 slowly absorbable suture
material. Feeding was allowed at first or second
postoperative day. The patients were discharged 3-5
days after surgery if no complications occurred. First
rectal digital examination was performed after 10
days. Routine anal dilations were performed in all
neonates once or twice weekly for at least 3 weeks.
Follow-up was arranged once weekly for 3 weeks
followed by once monthly for 3 months then every 3
months thereafter.

RESULTS
The study included 42 neonates; all of them were
referred from paediatricians for evaluation of
neonatal intestinal obstruction or constipation and
distension.. There were 27 males and 15 females.
Their age ranged from 2 weeks to 2 months. The level
of aganglionosis was rectum, n = 11 (26.5%);
rectosigmoid, n = 19 (45%); sigmoid, n = 12 (28.5%);

Operative details:
All cases were operated as planned (TEPT). No case
required conversion to laparotomy. The mean
operating time was 80 minutes 23.5 (range, 75-100
minutes). Submucosal dissection and colon
devascularization was easy, due to the fact that there
is no enough time to have repeated episodes of
enterocolitis, no long standing dilated hypertrophied
colon and thickened mesentery.
The average length of resected bowel was 20.8 12.4
cm (range, 15 to 30 cm). The average estimated
intraoperative blood loss was 20 ml 2.4 mL (range,
20-25mL). No case required blood transfusion.
The average time of the first passage of stools post
operatively was 1 day. Feeding was allowed 3 days
post operatively. The mean hospital stay was 7 days
(5-10 days). There was no mortality in this series.

Early Postoperative morbidity:
Significant transient perianal excoriation occurred in
11 cases (26%), which responded to medical
management including the use of zinc oxide
ointment, keeping the baby dry and frequent change
of diapers, and use of constipating medication to
decrease frequency of bowel motions.
Enterocolitis after the pull-through was noted in 5
cases (11.9%). The clinical grade of enterocolitis was
grade 1in 3 patients; grade 2 in 1 patients; and grade 3
in 1 patient; according to the grading system
established by Elhalabyet al.
13
All patients with grade
1 enterocolitis were treated successfully as
outpatients, whereas those of clinical grade 2 or 3
were hospitalized treated with colonic decompression
and antibiotics. Two patients had cuff abscess; 1
required drainage under anaesthesia, the other one
drained spontaneously after digital rectal
examination.

Late Postoperative morbidity:
Two patients (4.8%) had significant anastomotic
stricture that necessitated dilatation at least once
under general anesthesia. A tight sphincter was
reported in another 4 patients (9.5%), but all of them
responded adequately to anal dilatation. (table 2).

Table 1. Patients' characteristics.
Characteristics No. of patients (%)
Sex Male 27 (64%)
Female 15 (36%)
Transitional zone
Rectum 11 (26.5%)
Rectosigmoid 19 (45%)
segmoid 12 (28.5%)
Operative time (min) 80 23.5
Operative blood loss (mL) 20 2.4
Average Hospital stay (days) 5

Table 2. Post operative complications.
Post operative Complications No. of
patients (%)

Early postoperative (within10days)
Perianal excoriation.
Enterocolitis.
Cuff abscess.


11 (26%)
5 (11.9%)
2 (4.8%)

Late postoperative
Anastomotic stricture.
Tight sphincter.


2 (4.8%)
4 (9.5%)

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Annals of Pediatric Surgery 24



Fig 1. Anal exposure using Loan Star retractor Fig 2. Traction stitches 2 cm above dentate line

Fig 3. Anorectal mucosectomy Fig 4. Circumferential division of the cuff and starting
the full thickness mobilization of the colon


Fig 5. The seromuscular cuff is everted and then
shortened to < 5 cm
Fig 6. Coloanal anastomosis

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25 Vol 5, No 1, January 2009

Follow up:
The median follow up period was 20 months (range 5-
36 months). Five cases had no regular follow up.
The mean stool frequency was 7 (6-9) at first month of
follow up. This gradually improved to average of 5
times at 3 months post operatively, then to average of
3 times at 1 year of follow up period.
Twelve cases completed 3 years follow up). Complete
anorectal continence was confirmed in 10 of those 12
children (83.3%), whereas soiling still occur in 2
patients who continue to show a steady improvement
of their continence status. Anorectal motility and
EMG mapping of the anal sphincter of those 2
patients showed good resting tone and powerful
squeeze pressure.


DISCUSSION
The aim of this study was to evaluate the feasibility of
one-stage TEPT procedure in the neonatal age group,
with special emphasis on the operative time, difficulty
of mucosal dissection and colon mobilization, blood
loss, post operative complications.
The age of patients included in the study ranged from 2
weeks to 2 months. All cases were referred to us by the
neonatology unit; with whom we conducted seminar to
discuss the merits of performing surgery early in
neonatal period once the final diagnosis is made .
When cases were referred to us, a Barium enema was
done. In many cases, the characteristic features of HD
were evident as early as 2 weeks of age. In all cases the
diagnosis was established by rectal biopsy.
The mean operative time in this series (80 min) is
shorter than in other series including older ages.
10, 11

This is because mucosal dissection is much easier in
neonatal age due to the presence of less adherent
mucosa (no repeated attacks of enterocolitis), and the
mesentery and colon are less substantial and more easy
to mobilize.
The average amount of intra operative bleeding was 20
ml 2.4 mL. This amount is less than reported in
literature series for older infants and children.
12
This is
explained by the fact that dissection is easier in
neonates due to the same factors described above.
In all cases, the seromuscular cuff was shortened to
become less than 5 cm to avoid stenosis and recurrence
of obstructive symptoms, which may predisposes to
enterocolitis.
There was no case that had to be converted from TEPT
to laparotomy. This is because cases were selected in
this series when they have a clear transitional zone not
further than the sigmoid colon.
Enterocolitis has been considered one of the main
problems in patients with HD both before and after
definitive treatment.
13,14
The incidence of postpull-
through enterocolitis reported in the literature varies
widely, with some studies reporting rates as high as
32% to 42%.
15,16
Hackman et al
15
studied the risk
factors for postoperative enterocolitis and found that
both the presence of anastomotic leak or stricture and
the development of postoperative intestinal obstruction
secondary to adhesions increased the relative risk and
subsequent enterocolitis by approximately 3-fold. The
relative low incidence of enterocolitis after one stage
TEPT in the current series may be related in part to the
short seromuscular cuff, the low coloanal anastomosis,
and the policy of routine postoperative anal dilatation.
There is a general tendency to reserve anal dilation or
bouginage to cases with existing or potential risk of
stricture formation.
7
We believe that postoperative
routine anorectal bouginage is an effective tool to
prevent the occurrence of anal stricture and to decrease
both the frequency as well as the severity of
enterocolitis particularly in neonates and young infants.
Cuff abscess occurred in 2 (4.8%) patients. This
complication occurs when the mucosal tube is damaged
during dissection, when there is retraction of the
anastomosis, and when there is poor blood supply to
the pull-through colon. These events cause spillage of
intestinal contents to surrounding tissues, which initiate
an inflammatory process that culminates in abscess
formation. This incidence is less than reported in some
series,
17
perhaps due to the extra care taken during
dissection to avoid mucosal injury as possible and
avoiding anastomosis under tension by avoiding
excessive traction on the colon before excision and
anastomosis (to avoid upwards retraction of the colon
pulling on the anastomosis). These precautions also are
responsible for not having any case of anastomotic leak.
Although every effort was done to avoid anastomosis
under tension, and meticulous coloanal anastomosis to
avoid leakage, we had two cases (4.8%) of anastomotic
stricture. The two cases, however, responded to
repeated anal dilatation.
Regarding to the concern that delicate structures such
as the muscular sphincters may be over stretched and
injured during TEPT, 83% of cases who completed 36
months follow up had complete anorectal continence.
Anorectal motility and EMG mapping of the anal
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Annals of Pediatric Surgery 26

sphincter of the remaining 17% of patients showed
good resting tone and powerful squeeze pressure.
These results are the same as in children operated
later in life and those whom operated by other
techniques than TEPT
18



CONCLUSION
We conclude that one stage TEPT is feasible in the
neonatal period. Operative time and blood loss are less
than in published series of older children. Post
operative complications occur at similar rate to cases
operated in infancy and childhood period. Continence
results are similar to cases operated later in life using
techniques other than TEPT.
We recommend early operation, in the neonatal period,
for cases of HD. We also recommend good
communication with the paediatricians to speed up the
diagnostic workup and to convince them about the
potential benefits and limitation of one stage surgery at
neonatal period.


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