Print this page Email this page
Users Online: 87
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
ORIGINAL ARTICLE
Year : 2020  |  Volume : 10  |  Issue : 1  |  Page : 17-21

The outcome of single-stage management of Hirschsprung's disease at a tertiary care center


Department of Pediatric Surgery, Indira Gandhi Institute of Medical Science, Patna, Bihar, India

Date of Submission30-May-2020
Date of Acceptance01-Oct-2020
Date of Web Publication06-May-2021

Correspondence Address:
Dr. Zaheer Hasan
ADD- D6/2 Quarter, Indira Gandhi Institute of Medical Science, Raja Bazar, Patna - 800 014, Bihar
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_27_20

Rights and Permissions
  Abstract 


Background: Hirschsprung's disease (HD) was initially treated as a multistage procedure. Subsequently, it has been recognized that surgery can be done as a single-stage at an early age as this approach is safe, cost-effective, and avoids the morbidity associated with stomas. We study the effectiveness and safety of single-stage management of HD using Langer's or Georgeson's techniques, to analyze the outcome of surgery, and its comparison with other similar studies.
Patients and Method: The study was retrospectively carried out in our institution from October 2016 to September 2019. They were operated in a single-stage either by Langer's procedure or by laparoscopy-assisted Georgeson's procedure. Inclusion criteria were clinically diagnosed cases of HD with an obvious transition zone. Patients without a funnel (on barium enema films) were excluded. Results were analyzed using IBM SPSS statistics 20.00 software.
Result: The present series included 28 patients. The mean age of the patients was 16.24 months. Mean intra operative blood loss for Langer's series was 30 ml and Georgeson's series was 20 ml. The mean operative time for Georgeson's procedure was 150 min and for Langer's, it was 90 min. Postoperative enterocolitis 3 (10.7%), persistent constipation 2 (7.5%), and perianal excoriation 9 (32.14%) were some of the important complications noted.
Conclusion: Single-stage surgery for HD is safe and avoids the need for multiple operations including stoma and its related complications.

Keywords: Hirschsprung's disease, postoperative enterocolitis, transanal Hirschsprung's management


How to cite this article:
Thakur VK, Hasan Z, Chaubey D, Yadav R, Prasad R, Karim A. The outcome of single-stage management of Hirschsprung's disease at a tertiary care center. Arch Int Surg 2020;10:17-21

How to cite this URL:
Thakur VK, Hasan Z, Chaubey D, Yadav R, Prasad R, Karim A. The outcome of single-stage management of Hirschsprung's disease at a tertiary care center. Arch Int Surg [serial online] 2020 [cited 2023 Feb 8];10:17-21. Available from: https://www.archintsurg.org/text.asp?2020/10/1/17/315395




  Introduction Top


Hirschsprung's disease is a developmental disorder of the enteric nervous system. It was initially treated as a multistage procedure. Subsequently, it has been recognized that surgery can be done as a single-stage at an early age. Georgeson's et al. reported a technique of laparoscopic rectal dissection in single-stage endorectal pull-through.[1],[2],[3],[4] Transanal pull through an extension of Georgeson's procedure without laparoscopic intra-abdominal dissection reported by Langer's in 1999.[5] In this technique, the tedious abdominal rectal mucosectomy performed in the Soave procedure was avoided. The objective of this study was to review the outcome of single-stage surgery in the management of Hirschsprung's disease using Langer's or Georgeson's technique, to analyze the outcome of surgery in our institute and its comparison with other studies.


  Patients and Method Top


This retrospective study was carried out in our teaching institute from October 2016 to September 2019. The diagnosis was suspected on clinical examination and radiological confirmation with contrast enema. Although we did not perform any preoperative biopsy, intraoperative confirmation of disease was done by of frozen section biopsy. The present series included 28 patients. The inclusion criteria for single stage surgery were a clinically diagnosed case of HD with an obvious transition zone on barium enema films. Patients with absence of funneling (barium enema finding), history of recurrent enterocolitis, major congenital anomalies, and other cause of constipation were excluded from the study.

The method of treatment was Transanal Soave procedure (Langer's procedure), or Laparoscopic assisted pull through (Georgeson's procedure

The operative procedure was evaluated under operative time, blood loss, length of resected bowel, duration of hospital stay, complications, and follow-up.

The sample size of 30 (15 in each group) was calculated assuming alfa error of 0.05, beta error 0.20, and mean (SD) of 12 ± 3.5 and 18 ± 5.5. Chi-square test was used for the inter-group comparison categorical data (for postoperative complication). Significance was assumed at the level of P < 0.05 using IBM SPSS statistics 20.00 software.

Langer's procedure

In this procedure colon and rectum were excised through transanal route. In lithotomy position, patient was catheterized and a nasogastric tube was placed. Anal and rectal mucosa was exposed with traction sutures. Rectal mucosa was circumferentially incised using pointed cautery approximately 0.5 cm above the dentate line which corresponds to a lower margin of mucosal rugosity. Rectal submucosal dissection was carried 2 cm proximally which it can be extended 5 cm further up. Thereafter, dissection becomes full thickness. The rectum and colon were mobilized circumferentially till transition and the dilated part comes into view. The intestine was mobilized till the normal caliber of the colon is reached and then resected. To avoid any residual aganglionic segment of the colon, the most proximal segment of the resected specimen was subjected to a frozen section examination to look for the presence of ganglion cells. Following confirmation of the absence of ganglion cells by frozen sections, further resection of colonic segment was done. The rectal muscular cuff was cut on its posterior wall to avoid constriction of the pull-through segment. The colo-anal anastomosis was performed with vicryl 4-0 and vicryl 3-0 used for older children.

Georgeson's procedure

This is a laproscopic procedure where mobilization of spastic segment and colon of normal caliber is prepared for pullthrough. In the Trendelenburg position, pneumoperitoneum was created with pressure 6 − 8 mm of Hg rectal dissection was initiated at the peritoneal reflection, the dissection was continued circumferentially down to the recto-prostatic region using harmonic cautery. In female dissection was carried up to the upper part of the vagina. After adequate mobilization of the rectum trans-anal procedure was completed in a lithotomy position similar to Langer's procedure.

In the postoperative period, feeding was allowed on the 3rd post-operative day when the abdomen was soft, nasogastric aspirate was nil and the patient was passing stool frequently. The patients were discharged on 5 − 8 postoperative days. The anus was calibrated with a dilator after 3 weeks of surgery, then weekly for the next 3 months.


  Results Top


Age at presentation is shown in [Table 1]. The mean age was 16.24 months. Majority of the patients were from the age group 1-6 months (50%). There were 20 males (71.4%) out of 28 patients. Male to female ratio was 2.5:1. Characteristics of the patients are shown in [Table 2]. In our study, 16 patients underwent Georgeson's procedure and 12 patients underwent Langer's procedures respectively depending upon the level of aganglionosis. Georgeson's and Langer's procedures were mostly performed in the age group of 1-6 months. [Table 3] The operative time varied between 45 min and 150 min depending upon the type of procedure performed (mean operative time for Georgeson's procedure was 150 min and for Langer's it was 90 min.). In Georgeson's and Langer's procedures, the mean length of the resected specimen was 30 and 25 cm respectively that included the spastic, transition, and the part of dilated gut. Mean intra operative blood loss for Langer's series was 30 ml and for Georgeson's series, it was 20 ml. One patient required blood transfusion. The mean duration of hospital stay in Georgeson's procedure was 8 days while in Langer's procedure it was 7 days.
Table 1: Age at presentation

Click here to view
Table 2: Characteristics of the patients

Click here to view
Table 3: Age-wise operative procedure done

Click here to view


Postoperative complications [Table 4] include postoperative enterocolitis 3 (10.7%) (Georgeson's series it was 12.5% and for Langer's series it was 8.34%), persistent constipation 2 (7.1%), and perianal excoriation 9 (32.14%) were some of the important complications noted which were not found to be statistically significant. Continence assessment was done in eight patients 12/28 (seven of Georgeson's series and five of Langer's series) using Kelly's clinical scoring method. Eleven of them had (6/6) scores and one had (5/6). The frequency of stool in following the procedures is shown in [Table 5]. There was no mortality in the present series.
Table 4: Post -operative complications

Click here to view
Table 5: Frequency of stool in our procedure

Click here to view



  Discussion Top


Although many operations have been described for definitive management of Hirschsprung's disease, no single operation achieves a perfect functional outcome. The most commonly performed operations include the Swenson, Duhamel, and Soave procedures.[6] These operations could be done either in a one-stage or in the multistaged procedure. Each of these operations has its own merits and demerits. One stage management of HD is meant for reducing hospital stay, and morbidity associated with staged pull-through procedures especially avoiding stoma.[1] Application of trans-anal endorectal pull-through in the treatment of Hirschsprung's disease is advantageous as 75% of patients have a transition zone at the level of the recto-sigmoid region which is an important criterion in selecting patients for trans-anal endorectal pull-through.[7] According to them if a transitional zone is present, the positive predictive value for the patient having HD is 100%. In this technique, the tedious abdominal rectal mucosectomy performed in the abdominal Soave procedure was avoided.[8] The trans-anal approach has the advantage of a significantly shorter hospital stay when compared to the trans-abdominal approach. It excludes the time spent in laparotomy and therefore abolishing the risk of postoperative intestinal adhesions. Besides, it offers better cosmesis in the surgical correction of HD. The endorectal dissection preserved anorectal sphincters as well as local blood supply and innervation so that fecal and urinary continence pathways were untouched.[9] The transanal approach offers a smaller operative field, a potential hazard with this technique could be uncontrolled bleeding when the colon is being mobilized especially in older children.[10]

The first series of one-stage neonatal pull-through was described by So et al.[11] who reported 20 patients with HD, all diagnosed and treated within the 1st month of life. In Langer's procedure, the mean operative time was 90 minutes (80-120 minutes) which was comparatively less than other series, as shown in [Table 5].[12],[13],[14],[15] The mean operative time for Georgeson's procedure was 150 min (120 − 210 min) similar result was reported by Jona in 2005.[16] In our series for Langer's procedure mean length of the resected bowel was 25 cm. Studies shown by different authors showed the mean length of bowel resection varied from 9.5 to 30 cm.[13],[15],[17],[18] The reason for resecting a longer segment of bowel in our series that extended beyond the dilated segment was to avoid bowel dysfunction owing to associated hypo or dysganglionosis. Thakkar et al. reported variability of transition zone in HD and advised resection of bowel in excess of spastic segment.[19] Various authors[12],[15] reported blood loss (≤25 ml) in their series of Langer' procedure. We report marginally higher blood loss (30 ml) as the majority of our patients were operated beyond the neonatal period The parents complained of more stool frequency in the immediate postoperative period in the patients where we excised a comparatively longer segment of colon. Contrast enema was the mainstay in the delineation of the transition zone. Various authors reported sensitivity of 89%-94% in detecting transition zone on contrast enema examination in HD.[17],[20],[21] Gangopadhyay et al. mentioned pull-through procedure (laparoscopic or transanal) are ideal for short segment disease, though long segment or unclear transition zone should not be considered absolute contraindication because if the transition zone can't be reached from below, one can switch over to laparotomy. Now with the wide availability of frozen section biopsy, doubts of residual aganglionic segment is almost gone. The mean duration of the hospital stay in our series was comparable to other series. All the patients were advised for a monthly visit for the first 6 months then 3 monthly follow-up for 3 years. Overall, the incidence of enterocolitis in our study was 10.71% (3/28) which was significantly lower than other studies.[13],[14] Constipation was observed in 7.14% (2/28) in the present series and it was less than in other reported series.[22] Less frequency of these complications were might be due to shorter mucosal dissection before full-thickness dissection.[23] In one of our cases, operated by Langer's procedure developed stricture as the patient was lost to follow up in the postoperative period, it subsequently resolved by Hegar's dilatation for six months.

The average frequency of stool in Langer's procedure was 3.8/day and it was 4.2/day in Georgeson's procedure at three weeks of the postoperative period which decreased drastically to 1.6/day (Langer's) and 1.8/day (Georgeson's) at one year of follow-up [Table 5]. We conducted a continence study in our twelve patients which were found appropriate in follow-up (age >3 years). Seven (Georgeson's) and five (Langer's) patients were subjected to continence assessment using Kelly's score; no one was found to be incontinent in our study. The functional results were preliminary, and a continous assessment of fecal continence and bowel function are needed.


  Conclusion Top


Single-stage surgery for Hirschsprung's disease is safe and can be done in young children with ease. It avoids the need for multiple operations including diversion colostomy and its related complication. One stage trans-anal pull-through takes less operative time, less blood loss, and limited bowel resection with earlier recovery if compared with other conventional surgery mentioned for Hirschsprung's disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Somme S, Langer's JC. Primary vs. staged pull-through for the treatment of Hirschsprung disease. Semin Pediatr Surg 2004;13:249-55.  Back to cited text no. 1
    
2.
Skarsgard ED, Superina RA, Shadling B, Weseon DE. Initial experience with a one-stage endorectal pull-through procedure with Hirschsprung's disease. Pediat Surg Int 1996;11:480-2.  Back to cited text no. 2
    
3.
Cilley RE, Statter MB, Hirschl RB. Definitive treatment of Hirschsprung's disease in a newborn with a one-stage procedure. Surgery 1994;115:551-6.  Back to cited text no. 3
    
4.
Georgeson's KE, Cohen RD, Hebra A, Jona JZ, Powell DM, Rothenberg SS, et al. Primary laparoscopic-assisted endorectal colon pull-through for Hirschsprung's disease: A new gold standard. Ann Surg 1999;229:678-83.  Back to cited text no. 4
    
5.
Langer's's JC, Minkes RK, Mazziotti MV, Skinner MA, Winthrop AL. Transanal one stage Soave procedure for infants with Hirschsprung's disease. J Pediatr Surg 1999;34:148-52.  Back to cited text no. 5
    
6.
Huddart SN. Hirschsprung's disease: Present UK practice. Ann R Coll Surg Engl 1998;1:46-8.  Back to cited text no. 6
    
7.
Kroll-Wheeler L, Wilson AM. Educational case: Hirschsprung disease. Acad Pathol 2019;6:2374289519893088.  Back to cited text no. 7
    
8.
Hackam David J, Superina RA, Pearl RH. Single-stage repair of Hirschsprung's disease: A comparison of 109 patients over 5 years. J Pediatr Surg 1997;2:1028-32.  Back to cited text no. 8
    
9.
Chen Y, Nah SA, Laksmi NK, Ong CCP, Chua JHY, Jacobsen A, et al. Transanal endorectal pull-through versus transabdominal approach for Hirschsprung's disease: A systematic review and meta-analysis. J Pediatr Surg 2013;48:642-51.  Back to cited text no. 9
    
10.
Torre LDL, Salgado JAO. Transanal endorectal pull-through for Hirschsprung's disease. J Pediatr Surg 1998;33:1283-6.  Back to cited text no. 10
    
11.
So HB, Schwartz DL, Becker JM, Daum F, Schneider KM. Endorectal “pull through” without preliminary colostomy in neonates with Hirschsprung's disease. J Pediatr Surg 1980;15:470-1.  Back to cited text no. 11
    
12.
Pratap A, Shakya VC, Biswas BK, Sinha A, Tiwari A, Agrawal CS, et al. Single-stage transanal endorectal pull-through for Hirschsprung's disease: A perspective from a developing country. J Pediatr Surg 2007:42;532-5.  Back to cited text no. 12
    
13.
Hadidi A. Trans- anal endoanal pull-through for Hirschsprung disease. A comparison with the open technique. Eur J of Ped Surg 2003;13:176-80.  Back to cited text no. 13
    
14.
Srivastava P, Upadhyaya V, Gangopadhyay AN, Hasan Z, Upadhyaya A, Kumar V, et al. Comparison of two techniques for single-stage treatment of Hirschsprung disease in neonates. Int J Surg 2007;17:1-5.  Back to cited text no. 14
    
15.
Teeraratkul S. Transanal one-stage endorectal pull-through for Hirschsprung's disease in infants and children. J Pediatr Surg 2003;38:184-7.  Back to cited text no. 15
    
16.
Jona JZ. Laparoscopic pull-through for Hirschsprung's disease in infants. J Indian Assoc Pediatr Surg 2005;10:28-30.  Back to cited text no. 16
  [Full text]  
17.
Pratap A, Gupta DK, Tiwari A, Sinha AK, Bhatta N, Singh SN, et al. Application of a plain abdominal radiograph transition zone in Hirschsprung's disease. BMC Pediatr 2007;7:5.  Back to cited text no. 17
    
18.
Langer JC, Durrant AC, de la Torre L, Teitelbaum DH, Minkes RK, Caty MG, et al. One-stage transanal Soave pullthrough for Hirschsprung disease: A multicenter experience with 141 children. Ann Surg 2003;238:569-83; discussion 583-5.  Back to cited text no. 18
    
19.
Thakkar HS, Blackburn S, Curry J, De Coppi P, Giuliani S, Sebire N, et al. Variability of the transition zone length in Hirschsprung disease. J Pediatr Surg 2020;55:63-6.  Back to cited text no. 19
    
20.
Peyvasteh M, Askarpour S, Ostadian N, Moghimi MR, Javaherizadeh H. Diagnostic accuracy of barium enema findings in Hirschsprung's disease. Arq Bras Cir Dig 2016;29:155-8.  Back to cited text no. 20
    
21.
Alehossein M, Roohi A, Pourgholami M, Mollaeian M, Salamati P. Diagnostic accuracy of a radiologic scoring system for evaluation of suspicious Hirschsprung's disease in children. Iran J Radiol 2015;12:12451.  Back to cited text no. 21
    
22.
Zimmer J, Tomuschat C, Puri P. Long-term results of transanal pull-through for Hirschsprung's disease: A meta-analysis. Pediatr Surg Int 2016;32:743-9.  Back to cited text no. 22
    
23.
Toree LDL, Langer's JC. Transanal endorectal pull-through for Hirschsprung's disease: Technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Seminars Ped Surg 2010;19:96-106.  Back to cited text no. 23
    



 
 
    Tables

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



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Results
Discussion
Conclusion
Patients and Method
References
Article Tables

 Article Access Statistics
    Viewed1191    
    Printed54    
    Emailed0    
    PDF Downloaded125    
    Comments [Add]    

Recommend this journal