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Year : 2015  |  Volume : 28  |  Issue : 1  |  Page : 38-42

Clinical and radiological assessment of transanal endorectal pull-through in children with Hirschsprung's disease

1 Department of General Surgery, Faculty of Medicine, Menoufia University, Menoufia, Egypt
2 Department of Pediatric Surgery, Ministry of Health, Egypt

Date of Web Publication29-Apr-2015

Correspondence Address:
Ahmed Mohamed Nabil
Diarb Najm, Sharkia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-2098.155936

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The aim of this study was to evaluate the late outcome of transanal endorectal pull-through technique (TEPT) clinically and radiologically in patients with Hirschsprung's disease (HD).
HD is a relatively common neonatal developmental disorder of the enteric nervous system. It leads to absent peristalsis in the affected bowel, which results in functional intestinal obstruction. Many surgical operations have been used to treat it; here we discuss TEPT, especially the late results of it.
Patients and methods
A retrospective study was conducted on 40 patients in whom TEPT was performed from 2010 and who were followed up for 2 years.
The outcome of TEPT is satisfactory for both surgeons and patients, especially with respect to bowel habits; however, as it is a new surgical technique, we are awaiting further study results to support its more widespread use in the treatment of HD.
The clinical outcome is satisfactory. Postoperatively, a gradual recovery could be noted in the stooling patterns over time. The younger the patient operated on, the faster the recovery of stooling function.

Keywords: Clinical evaluation, neonatal developmental disorder, radiological evaluation

How to cite this article:
Loulah MA, EL-Gammal AS, Nabil AM. Clinical and radiological assessment of transanal endorectal pull-through in children with Hirschsprung's disease. Menoufia Med J 2015;28:38-42

How to cite this URL:
Loulah MA, EL-Gammal AS, Nabil AM. Clinical and radiological assessment of transanal endorectal pull-through in children with Hirschsprung's disease. Menoufia Med J [serial online] 2015 [cited 2020 Feb 24];28:38-42. Available from: http://www.mmj.eg.net/text.asp?2015/28/1/38/155936

  Introduction Top

Harold Hirschsprung, a Danish pediatrician, is credited with the first definitive description of the disease that bears his name. Hirschsprung's disease (HD) is a relatively common pediatric surgical problem (1 : 2000-5000) [1].

Since Swenson performed his first successful pull-through operation in 1964, many procedures have been developed aiming to excise the aganglionic segment and to perform pull-through coloanal anastomosis with minimal complications [2]. Despite numerous surgical techniques and their modifications, the surgical treatment of HD remains controversial in terms of choosing the optimal technique to avoid both early and late postoperative complications (incontinence, soiling, staining, strictures, and enterocolitis), with the incidence of complications being higher in multicentric studies [3].

Classic techniques were introduced decades ago by Swenson, Duhamel, and Soave with subsequent numerous modifications of these three basic procedures. Laparoscopic surgery has been introduced lately in the treatment of HD, but, although less invasive, it is also based on classic surgical techniques [4]. Transanal mucosectomy was practiced for many years as a part of conventional and laparoscopically assisted pull-through procedures for HD. The turning point in the surgical approach emerged in 1998 when De la Torre-Mondragon and Ortega-Salgado published the latest development - an entirely transanal endorectal pull-through technique (TEPT) [5].

The TEPT represents a major revolution in treating HD. This procedure can be performed as a primary pull-through or staged with a prior colostomy to achieve optimal results [3].

Complications can be classified as either early (weeks to months) or late (months to years). Early postoperative complications include anastomotic leak and cuff abscess, bowel obstruction, perineal excoriation, stoma complications, and wound infection. Late complications include bowel obstruction, constipation, enterocolitis, incontinence, and stricture. There is some overlap between the early and late complications [6].

This study reviews the results of treatment of HD using the TEPT.

  Patients and methods Top

This is a retrospective descriptive study that included 40 patients with HD who were treated in 2010 or earlier and were followed up for at least 2 years after TEPT. This study also included patients with HD who were operated on using TEPT in the last 5 years. The diagnosis was confirmed by rectal biopsy and site of transitional zone in the rectosigmoid colon, diagnosed with contrast enema.

All patients were subjected to full physical examination, necessary laboratory investigation, and imaging studies (gastrographin enema and plain abdominal radiograph), and the patients' parents were counseled.

The follow-up criteria were age at presentation, age at surgery, number of defecations per day, rectal sensation, soiling, continence, number of attacks of enterocolitis, abdominal distension, and perianal skin excoriation.

Statistical analysis

Data were checked, entered, and analyzed using SPSS (version 2.0; SPSS Inc., Chicago, Illinois, USA), and were represented as numbers and percentage for categorical variables. The c2 -test or Fisher's exact test was used when appropriate. A P value less than 0.05 was considered statistically significant.

  Results Top

The results are shown in [Table 1],[Table 2],[Table 3],[Table 4],[Table 5] and [Table 6].
Table 1: Percentage of abdominal distension

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Table 2: Recurrent attacks of enterocolitis (6– 12 months after surgery)

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Table 3: Frequency of defecation

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Table 4 Continence

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Table 5: Soiling

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Table 6: Rectal sensation

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Fourteen (35%) patients had normal defecation. Fourteen (35%) patients had a frequency of defecation of 3-5 days. Eight (20%) patients had a frequency of defecation of 6-7 days. Four (10%) patients had a frequency of defecation of 8 or more days. All of these are positive results and agree with those of other studies [Table 3].

Thirty-two patients had true fecal incontinence and had a nondilated colon on contrast enema and a tendency to have diarrhea and did not respond to medical treatment. Four patients had partial fetal incontinence and four patients had incontinence. All of these results confirm the efficacy of transanal endorectal pull-through technique and promise us with better results in the future [Table 4].

Postoperative soiling accidents were observed occasionally in four (10%) patients and often in 18 (45%) patients. Soiling was not observed in 18 (45%) patients. Soiling is not a subtype of incontinence according to our study, although some consider it as a subtype as it does not include any affection in anal sphincter muscles and on further follow-up it improves with enema in comparison with incontinence [Table 5] and [Figure 1],[Figure 2],[Figure 3],[Figure 4] and [Figure 5].
Figure 1: Cross-sectional anatomy of the anal canal.

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Figure 2: H&E-stained rectal biopsy.

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Figure 3: Steps of the operation.

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Figure 4: Inoperative anal verge and outcome 1 month after operation.

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Figure 5: Postoperative follow-up gastrographin enema showing colon dilation.

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  Discussion Top

HD is one of the most common congenital malformations, occurring in 1 : 2000-5000 live births. One-stage TEPT has been increasingly used for HD [7].

The treatment of HD is primarily surgical. Surgical therapy for HD implies the removal of aganglionic bowel and bringing normally innervated intestine to the anus. Although all of the three most commonly performed basic techniques for the correction of HD (Swenson, Soave, and Duhamel) accomplish this goal, the modified Soave operation has an important advantage of avoiding injury to the pelvic nerves responsible for urinary incontinence and sexual dysfunction [4].

The use of the transanal approach (TEPT) is a relatively new concept. The technique became popular since the paper of Mondragon and Salgado [8] in Mexico, who used the technique on five patients with HD; the whole operation was carried out transanally without intra-abdominal intervention.

The TEPT has gained support from a vast majority of pediatric surgeons as it is a minimally invasive procedure. It has little postoperative complications, with short hospital stay. It is cost effective and a relatively easy procedure. The risk of contamination and adhesion formation is eliminated. The procedure does not damage the pelvic structures and has the most optimal cosmetic result [5].

In this series, 32 (80%) patients had true fecal continence and a nondilated colon on contrast enema and a tendency to have diarrhea. Four (10%) patients had partial fecal incontinence and four (10%) patients had incontinence.

As regards abdominal distension, 14 patients showed no distension, 24 patients showed mild distension, and two patients showed moderate to severe abdominal distension. A history of previous attack of enterocolitis was positive in all patients.

Hadidi [9] reported a 4.4% enterocolitis rate utilizing the transanal approach in comparison with a 12% incidence in a preceding series of open pull-through procedures. This is a very low incidence of post-pull-through enterocolitis, which differs from most reports reported in the literature. The rate of enterocolitis varies widely in published reports, ranging from a higher rate of 54% by Van Leeuwen et al. [10] to a lower rate of 4.55% in the study by Zhang et al. [11] .

Ekema et al. [4] in their study on 15 children reported that seven (46.6%) patients had constipation, six (40%) patients had neonatal intestinal obstruction, and one (6.6%) patient had enterocolitis.

Postoperative soiling accidents were observed occasionally in four (10%) patients and often in 18 (45%) patients. Soiling was not observed in 18 (45%) patients. Soiling was observed at night in 16 (40%) patients and during both day and night in six (15%) patients.

Enterocolitis continued to be a troublesome problem that disturbed some patients and most doctors; in this series all patients had a history of enterocolitis that varied from three attacks per year in 55% of patients to more than six attacks per year in 10% of patients. This high incidence may be due to long cuff, defective follow-up, and anal dilatation, or due to endemic gastroenteritis in Egypt.

In the series reported by Teitelbaum et al. [6], 48% of patients presented with enterocolitis preoperatively and 39% had episodes of enterocolitis after pull-through operation. There was no correlation between pre-pull-through and post-pull-through enterocolitis in their patients or with the type of pull-through operation. In most patients, episodes of enterocolitis occurred in the first few years after pull-through operation.

Most episodes of enterocolitis occurred within the first 2 years after the pull-through procedure. Engum and Grosfeld noted that infants who experienced enterocolitis before operative intervention had an increased risk of occurrence for this complication following the pull-through procedure.

Zhang et al. [11] studied 76 children who underwent staged Swenson operation for HD; they showed that constipation occurred in 6.7% of patients, incontinence in 6.7%, and soiling in 11% of patients. The rate of postoperative enterocolitis was 37.8%.

Elhalaby et al. [2] reported transient soiling and increase in bowel movements in a significant number of their patients who underwent TEPT, which was due to overstretching.

Zhang et al. [11] studied 58 patients who underwent TEPT for HD. The results were as follows: soiling in nine (15.5%) patients and constipation in five (8.6%); no incontinence was seen in any patient.

El-Sawaf et al. [12] studied 41 patients who underwent TEPT for HD; there was a 53.7% overall incidence of post-pull-through enterocolitis. This higher rate of enterocolitis may be because of the low threshold in diagnosing early cases of enterocolitis. Levitt et al. [9] reported that postoperative enterocolitis was more frequent in patients who had enterocolitis before the pull-through procedure.

We agree with all the results cited here, even those that did not match ours [13], as the follow-up time of the patients differed among studies. Moreover, the net results are not only surgeon-dependent or hospital-dependent but also based on the socioeconomic level of country and patients. Above all, the criteria of judgment differed among studies (e.g. soiling in our study is different from continence and has its own classification; some define it as a subtype of continence).

  Conclusion Top

TEPT is a good surgical technique and can be performed by any good surgeon. Frozen section biopsy is mandatory to obtain good results and overcome inadequate surgical margin complications. Patient follow-up should comprise a cooperative team of surgeons, pediatrics, and nutritionists. The educational level of parents could improve results as they can identify early signs of complications. Rectal enema is the golden standard for treating enterocolitis (the serious postoperative complication) and the technique must be taught to the mother.

  Acknowledgements Top

Conflicts of interest

There are no conflicts of interest.

  References Top

De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Semin Pediatr Surg 2010; 19 :96-106.  Back to cited text no. 1
Elhalaby EA, Teitelbaum DH, Coran AG, Heidelberger KP. Enterocolitis associated with Hirschsprung′s disease: a clinical histopathological correlative study. J Pediatr Surg 1995; 30 :1023-1026; discussion 1026-1027.  Back to cited text no. 2
El-Sawaf MI, Drongowski RA, Chamberlain JN, Coran AG, Teitelbaum DH Are the long-term results of the transanal pull-through equal to those of the transabdominal pull-through? A comparison of the 2 approaches for Hirschsprung disease. J Pediatr Surg 2007; 42 :41-47; discussion 47.  Back to cited text no. 3
Jamieson DH, Dundas SE, Belushi SA, Cooper M, Blair GK Does the transition zone reliably delineate aganglionic bowel in Hirschsprung′s disease? Pediatr Radiol 2004; 34 :811-815.  Back to cited text no. 4
Langer JC, Minkes RK, Mazziotti MV, Skinner MA, Winthrop AL. Transanal one-stage Soave procedure for infants with Hirschsprung′s disease. J Pediatr Surg 1999; 34 :148-151; discussion 152.  Back to cited text no. 5
Georgeson KE. Hirschsprung′s disease. In: Holcomb GW, Murphy JP. editors. Aschcraft′s pediatric surgery. Saunders: Elsevier; 2010. 456-467.  Back to cited text no. 6
Pratap A, Shakya VC, Biswas BK, Sinha A, Tiwari A, Agrawal CS, Adhikary S. Single-stage transanal endorectal pull-through for Hirschsprung′s disease: perspective from a developing country. J Pediatr Surg 2007; 42 :532-535.  Back to cited text no. 7
Mills JL, Konkin DE, Milner R, Penner JG, Langer M, Webber EM. Long-term bowel function and quality of life in children with Hirschsprung′s disease. J Pediatr Surg 2008; 43 :899-905.  Back to cited text no. 8
Liem NT, Hau BD, Son HT. Modified Soave procedure through the posterior sagittal approach for Hirschsprung′s disease. J Pediatr Surg 2005; 40(3):547-550.  Back to cited text no. 9
Holschneider AM, Steinwegs I. Functional diagnosis. In: Holschneider AM, Puri P, editors. Hirschsprung′s disease and allied disorders. 3rd ed. Berlin, Heidelberg: Springer-Verlag; 2008. 153-184.  Back to cited text no. 10
McAlhany A, Popovich D. Hirschsprung disease. Newborn Infant Nursing Rev 2007; 7 :151-154.  Back to cited text no. 11
Murphy F, Menezes M, Puri P. Enterocolitis complicating Hirschsprung′s disease. In: Holschneider AM, Puri P, editors. Hirschsprung′s disease and allied disorders. 3rd ed. Berlin, Heidelberg: Springer-Verlag; 2008. 133-143.  Back to cited text no. 12
Little DC, Snyder CL. Early and late complications following operative repair of Hirschsprung′s disease. In: Holschneider AM, Puri P, editors. Hirschsprung´s disease and allied disorders, 3rd ed. Berlin, Heidelberg: Springer-Verlag; 2008. 375-385.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]


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