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Hirschsprung Disease Managed With One-Stage Transa
Hirschsprung Disease Managed With One-Stage Transa
Abstract
Background: Over the past few decades, surgery for Hirschsprung’s disease (HD) has evolved into a minimally inva-
sive, single-stage procedure with excellent outcomes. Intraoperative frozen section biopsy is critical for this procedure
to avoid the potential risk of leaving a retained aganglionic segment. However, this facility is not available in most
low-income countries. Therefore, a two-stage procedure with an initial colostomy is still practiced in the developing
world. We aimed to evaluate the outcome of single-stage transanal pullthrough performed in a facility without frozen
section biopsy.
Methods: A retrospective review of all patients who underwent transanal pullthrough in two teaching hospitals over
a 6-year period (2015–2020).
Results: Forty-seven children underwent transanal endorectal pullthrough (TERPT). Age at surgery ranged from
2 months to 6 years and mean weight was 8.7 kg. Barium enema did not show transition zone in 6 patients (12.8%)
while others demonstrated short segment HD. Intraoperatively, the transition zone was visualized in 40 patients (85%).
TERPT alone was performed in 35 (74.5%), TERPT with laparotomy to visualize transition zone in 9 (26.7%) and TERPT
with transabdominal mobilization was required in 3 (6.4%). Definitive histopathologic examination revealed agangli-
onic segment pullthrough in 4 (8.5%) and transitional zone pullthrough in another 4 (8.5%). However, with long term
follow up all eight children remained asymptomatic and no intervention was required.
Conclusions: Transanal pullthrough offers reduced number of surgeries and faster recovery. We have also observed
a good functional outcome despite a discrepancy with pathology results. Overall, our data suggests it is a safe and
viable option for the treatment of short segment HD in facilities where frozen section is not available.
Keywords: Transanal endorectal pullthrough, Frozen section, Hirschsprung’s disease, Ethiopia
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have shown it to be safe, even in newborns, and it has 4. Respond to rectal irrigation until the age of 3 months
become the standard of practice in many centers [8, 9]. or weight of 5 kg (surgery delayed in order to avoid
This procedure became popular because of the simpli- anesthesia-related complications.)
fied nature and potential for cost savings [10]. It is also
a minimally invasive technique that offers faster recov- The soave pullthrough technique was used in all cases.
ery with fewer complications [11, 12]. All these qualities Extent of resection was determined by barium enema
are extremely attractive for low income countries where and intraoperative visualization of transition zone by
there are few pediatric surgery centers with limited the surgeon. Dilated bowel above transition zone was
resources [13]. resected as much as possible until normal caliber bowel
However, the procedure has one drawback. It is was reached. Biopsy (for permanent section) was taken
dependent on frozen section for identification of the nor- from proximal pulled down segment.
mally innervated bowel [6]. Frozen section is described
as a critical part of the procedure because barium enema Results
may be inaccurate in 10% of the cases [14, 15]. Without Over a period of 6 years, 47 children underwent transa-
frozen section confirmation, there is a potential risk of nal soave pullthrough. There were 39 boys (83%) and 8
leaving aganglionic bowel behind in these patients [15]. girls (17%). Half of the children were from Addis Ababa
Frozen section facility is not available in most LMIC (51.1%) while others came from different regions in the
such as ours, thus mandating colostomy for all children country. Mean follow up time was 11.5 months.
with this condition. Nonetheless, we recently started Thirty-eight children (80.9%) had symptoms since
performing single stage pullthrough in selected patients birth. Presenting complaints were chronic constipation
despite the lack of this facility. We aim to report our ini- in 39 children (83%), acute obstruction in 5 (10.6%) and 3
tial experience and to determine whether this is a possi- children (6.4%) had pseudo-incontinence. Only one child
ble alternative for other resource-limited countries. had malnutrition and anemia which was corrected before
the surgery.
Methodology Age at surgery ranged from 2 months to 6 years
We conducted a retrospective hospital-based study at (median 6 months) and mean weight was 8.7 Kg. Barium
Tikur Anbessa Hospital and Menelik Hospital. These are enema did not show transition zone in 6 patients (12.8%)
the largest centers affiliated with Addis Ababa University, while others demonstrated short segment HD (Fig. 1).
Ethiopia. They have a high burden of pediatric surgical Intraoperatively, transition zone was visualized in 40
cases, with more than 2000 procedures being performed patients (85%). Transition zone was in rectum in 2 (4.3%),
yearly. rectosigmoid in 26 (55.3%) and sigmoid in 12 (25.5%).
We included all children less than 15 years of age who TERPT alone was performed in 35 patients (74.5%)
underwent one stage transanal pullthrough over the (Fig. 2). In 9 patients (26.7%) a small left lower quadrant
past 6 years (January 2015–December 2020). Data was incision was made to visualize the transition zone before
obtained from medical records using a structured ques- starting TERPT. Laparotomy for transabdominal mobili-
tionnaire. Variables included demography, symptoms, zation was required in 3 patients (5.4%).
operative details, post-op complications and histopathol- The mean operating time was 118.9 minutes. There
ogy result. Telephone interviews were also conducted were no intraoperative anesthetic complications. Intra-
for follow-up data on July 2021. Data was then entered, operative transfusion was required in 2 children (4.3%).
coded and analyzed using SPSS version 23. Ethical Post-operative hospital stays ranged from 2 days to 1
approval was obtained from the institutional review month (median 4 days). Resumption of oral feeding
board of the college of health sciences, Addis Ababa ranged from 1 to 6 days (mean 2 days).
University. Definitive histopathologic examination (permanent
The single-stage transanal pullthrough procedure was section) revealed aganglionic segment pullthrough in
offered to selected patients with HD in our institution. 4 (8.5%) and transitional zone pullthrough in another 4
The considerations for selecting these patients were (8.5%). All eight children remained asymptomatic and no
intervention was undertaken. The children also did not
1. Hirschsprung disease confirmed with full thickness require laxatives or enemas. In 2 of the children (4.2%)
rectal biopsy the bowel status is unknown because biopsy sample was
2. Short segment disease without significant dilata- lost. (Fig. 3)
tion of the descending colon (diagnosed on barium Post op complications occurred in 25 children (53.2%).
enema) The most common complication was perineal exco-
3. No severe enterocolitis or severe malnutrition riation which occurred in 13 children (27.7%). Other
Discussion
It is proven that TERPT has several advantages over the
two stage pullthrough procedure as it offers reduced we found less operating time (118 min), early resumption
number of surgeries, faster recovery and overall reduced of oral feeding (2 days) and early hospital discharge (5
cost [10, 12]. Our data also substantiates this finding as days) comparable to other literatures on TERPT [16, 17].
Table 1 Postoperative complications to ensure pullthrough of ganglionated bowel [7, 16]. This
Post-op complications N (%)
was the practice in our setting as well.
Retained aganglionosis occurred in 3 of the 7 studies
Perianal excoriation 13 (27.7%) who performed single stage transanal pullthrough with-
Wound infection 2 (4.3%) out frozen section. The study by Shrestha [3] reported
Enterocolitis 5 (10.6%) 1 out of 20 cases which is an acceptable rate. They per-
- Death 1 formed redo pullthrough because of the biopsy result.
Obstructive symptoms 4 (8.5%) Significant rate of retained aganglionosis occurred in the
- Twisted pullthrough 1 study by Sookpotarom (14.8%) [1] followed by our cur-
- Acquired aganglionosis 1 rent study (8.5%). In both studies however, the patients
- Suspected internal sphincter achalasia 2 did not have symptoms and did not undergo interven-
Constipation 3 (6.3%) tion. We deferred redo-surgery because we chose to
Anastomotic leak 1 (2.1%) make decisions based on clinical symptoms rather than
relying on pathology results.
Although uncommon, late diagnosed Hirschsprung
can present with soiling preoperatively [24]. We had two
These factors are especially important low income set- such patients who persisted to have some soiling after
tings as there are limited resources. Moreover, this pro- the procedure. Otherwise, good continence was reported
cedure avoids a colostomy which has a high psychosocial from a majority of the patients which is expected [25].
burden in the developing world [18, 19]. Other complications encountered in our study include
Intraoperative frozen section is considered a standard wound infection, enterocolitis, anastomotic leak, twisted
part of the procedure [6, 15] and we faced ethical con- pullthrough, which have comparable rates to other stud-
cern in performing one stage TERPT without this facil- ies [15, 26, 27]. These complications are also not specific
ity. However, studies on the pathology of HD have shown to one stage TERPT and can occur in the two stage pull-
that even intraoperative frozen section can have a dis- through procedure [28].
crepancy with permanent section in 3–15% [20–22]. We
also considered frozen section may be less reliable in
developing nations as it is dependent on availability of Conclusion
experienced pathologist [20]. Our experience, as well as literature reviewed, has dem-
Additionally, our practice was supported by other onstrated the feasibility of performing transanal pull-
studies that reported contrast enema and/or intraopera- through in the absence of frozen section facility. It had
tive naked eye visualization are sufficient for the identi- acceptable short term and long-term outcomes with none
fication of transition zone [1, 3, 7, 10, 16, 23] (Table 2). of the complications being attributed to remnant agangli-
Some of these authors also recommend resecting nor- onic segment. This procedure should serve as an alterna-
mal appearing bowel more proximal to transition zone tive in the treatment of HD in children from LMIC.
Table 2 Comparison of studies on single stage pullthorugh performed without frozen section
Study Country No of patients Type of PT Length of Aganglionic PT Aganglionic
resection (mean) PT requiring
redo
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