Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Comparing SOAVE Technique in the Management of Early and Late Diagnosed Cases of Hirschsprung Disease: A Retrospective Single Center Experience

Ubaidullah Khan, Murad Kitar, Imed Krichen, Kais Maazoun, Naglaa M Kamal, Rasha A, Mostafa YL Khalif

Pediatric Surgery, Department of Surgery, Alhada Armed Forces Hospital, Taif, Saudi Arabia

 

Correspondence:

Ubaidullah Khan

Pediatric Surgery, Department of Surgery

Alhada Armed Forces Hospital, Taif, Saudi Arabia

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Phone: 00966547005971

 

Abstract

Background and objectives: Soave transanal procedure for Hirschsprung disease (HD) is most commonly performed at early age with good outcome. In our center we adopted this technique in many patients in different age groups without assisted laparoscopic abdominal approach. We herein aim to describe different technical aspects, outcome and success rate of Soave procedure among our patients.

Methods: We retrospectively reviewed our series of HD patients who underwent the Soave transanal approach, for postoperative stricture, anastomotic leak, enterocolitis, and subsequent bowel functions. Patients were divided into two groups: group 1: the early neonatal group (0-1 month) and group 2: the late presentation group ( 3-14years).

Results: Among the 17 patients with HD operated in our center, 13 underwent Soave transanal resection, 1 was lost from follow up, 2 had a levelling colostomy prior to referral and 1 had ileostomy. The average length of resection was 20±10.5 cm. The mean follow-up period was 12.2 months (range 5–22 months). Seven patients were 3-14 years old. At follow-up none had fecal incontinence or constipation requiring laxatives. All patients had voluntary bowel movements.

Conclusions: Our results support the fact that a single stage Soave approach without transabdominal dissection is an excellent technique for patients presenting with late HD with an excellent outcome. A multicenter prospective study with larger number of patient is essential to validate our results.

Keywords: Hirschsprung disease, Soave, transanal


 

Introduction

Hirschsprung’s disease (HD) is the commonest congenital gut motility disorder and is characterized by a lack of ganglion cells (aganglionosis) in a variable length of the distal gut. Affected infants usually present shortly after birth with signs of distal intestinal obstruction that are invariably fatal if left untreated. It has been more than 60 years since the original description of the aetiology of HD, elucidated by Dr. Ovar Swenson [1]. Since then, numerous techniques have been described for removal of the aganglionic distal colon. Removal of the aganglionic bowel, pulling through ganglionic bowel, and preserving the anal canal and sphincter mechanism, remain the principles to surgical repair regardless of the technique.

The operative management of HD has evolved dramatically, from full-thickness rectosigmoid dissection (Swenson) [2], endorectal dissection (Soave) [3], retrorectal pouch procedure (Duhamel) [4,5], low anterior resection (Rehbein) [6] and more recently a primary repair [7,8] that can be done transanally [9,10] and using the laparoscopictechnique [11]. Despite all this, still there is debate about which technique provides the best short and long-term results.

Soave is most commonly done nowadays with good results in the young age group. The transanal Soave procedure is a natural evolution of the laparoscopic operation. Transanal resection of the rectum was shown to be possible in animal models and in the initial series of children with HD who were published by de la Torre and Ortega-Salgado [34] and Langer et al. [35] in the late 1990s. The transanal approach has the principal benefit of avoiding the need for intraabdominal mobilization of the rectum through either laparotomy or laparoscopy [27].

We herein aim to describe our experience with Soave technique in our cohort of HD and compare its outcomes in the early and late presenters groups.

Patients and methods

We retrospectively reviewed electronic records of pediatric patients, age 0 to 14 years, fulfilling the inclusion criteria of confirmed diagnosis of HD who underwent surgical repair in the period between January 2016 and July 2017 in the Pediatric Surgery department of Alhada Armed Forces Hospital, Taif, Saudi Arabia. The study was approved by the research and ethical committee of the hospital.

Exclusion criteria were patients with confirmed diagnosis of HD but not yet operated, and rectal biopsy histopathological result other than HD. Patients were divided into two groups according to the age at presentation and transanal resection: group 1 - before one month of age and group 2 - later than one month of age.

All patients’ files were reviewed for the following information: demographic data (age, gender), medical history (age at presentation, presenting symptoms, other medical problems), surgical history (for any operation done other than the primary repair of HD), confirmation of HD (contrast imaging of each patient was assessed for the level of transition zone, rectal biopsy with histopathological assessment), preoperative data (preoperative bowel preparation and intravenous antibiotics use), operative data(efficiency of bowel preparation, age at the time of surgical correction, surgical technique used for repair, performance of intra-operative full thickness frozen section biopsy, length of each resected specimen), hospital course and post-operative follow-up (information on early and late post-operative complications was evaluated as well as long-term outcomes especially fecal incontinence and constipation). Rates of stricture formation, pelvic abscess, and enterocolitis were noted as well.

Statistical analysis

Data were coded and statistical analysis was done using Statistical Package for Social Sciences software program (SPSS) Version 20. Quantitative data were expressed as means ± standard deviation. Although qualitative data were expressed as percentages. A Chi-square and Fisher exact tests were applied for bivariate data analysis to test the statistical significance of associations, the level of significance was P ≤ 0.05.

Results

Eighteen patients were diagnosed withHD but one female patient was excluded as she was above 14 years old and referred to adult surgeon services. The remaining 17 patients fulfilled all the study inclusion criteria and were enrolled in the study with 9 patients in group 1 and 8 patients in group 2.

Age at the time of operation ranged from 10 days to 14 years with mean age of 14±12 days in group 1 and 7.9 ± 5.8 years in group 2. There were 13 male and 4 female patients with a ratio of 3.2:1. Each group included two female patients.

Three patients didn’t go to direct primary repair of HD due to bowel perforation at few days of birth from which 2 of them underwent colostomy and one underwent ileostomy.

Preoperative evaluation with contrast enema was done in all patients which revealed a transition zone in the rectosigmoid in 12 patients (70.5%), left colon in 4 (23.5%) and transverse colon in 1 (5.8%) as shown in Fig. 1. HD was confirmed by preoperative rectal biopsy with histopathologic evidence of aganglionosis. All patients underwent mechanical bowel preparation along with rectal irrigation prior to their definitive operation aiming for best operative and post-operative outcomes. Six patients in group 2 had afecalom on presentation and three of them necessitated irrigation under anaesthesia to wash well.

Figure 1. Contrast study showing dilated proximal colon (blue arrow) and distal portion (black arrow) with clear transition zone

11.2 1 1

Preoperative intravenous antibiotics with third generation cephalosporin and metronidazole were given to all patients with provisions made for allergies.

One surgical technique was used in the studied cohort with transanal rectal dissection, short sleeve, Soave approach with careful preservation of the dentate line. The three patients with fecal diversion prior to definitive repair underwent transanal resection in the neonatal period. The transverse colostomy in two of them was left untouched for later reversal as a third stage; the ileostomy in the third patient required a stoma reversal in a next stage to pull-through procedure.

In the remaining 14 patients who had a well defined sigmoid or lower left colon transition zone on contrast enema (Fig. 2), a purely Soave transanal resection was performed without preoperative diversion. The transanal dissection was performed using a lithotomy position. Sutures were placed initially just inside the anal canal at the level of the mucocutaneous junction for retraction, in this way the distal 1.5 cm of anal canal was preserved. Interrupted sutures were placed 1.0 cm proximal to the dentate line in a circumferential fashion to provide uniform traction. Monopolar electrocautery was used to perform submucusal dissection leaving 3 cm cuff and then a full-thickness dissection along the traction line of sutures. The dissection was carried into the peritoneal cavity and the colon was pulled-through to the appropriate level. The anastomosis was created in two layers, 1.0 cm proximal to the dentate line. In all patients, full thickness frozen section intraoperative biopsies were checked in the operating theatre to assure that the pull-through segment had ganglionic cells and the muscularis and submucosa had normal sized nerve trunks.

Figure 2. Well excised specimen to demonstrate with contrast study (arrows)

11.2 1 2

Laparoscopy or laparotomy were not used to assist the dissection in patients with disease that extended even above the mid-sigmoid, except for the 3 patients who had previous levelling stomas. The average length of resected specimen was 22.6 ± 10.9 cm in both groups with no statistically significant difference (Fig. 2). The time consumed during the operation was slightly in group 2 but didn’t reach a statistical significance. Operative and post-operative complications were compared in both groups with no statistically significant difference. In both groups, none of the patients required perioperative blood transfusions, none had urinary retention, wound infection, enterocolitis, anastomotic leak or strictures, or intraabdominal abscesses.

All patients were followed up in the pediatricsurgey outpatient clinic at 2 weeks, 2 months and 6 months. At two weeks, rectal examination using Hagar dilators was done to assess the need for rectal dilations. Two patients from group 2 needed anal dilation in the first two clinic visits and improved after dilation. One patient in group 2 had constipation requiring intermittent laxative usage. All patients had multidisciplinary care follow-up plane with pediatric surgery, pediatric gastroenterology and clinical nutrition departments.

Group 2 patients were assessed for fecal and bowel functions on follow up where voluntary bowel movements and spontaneous voiding with good control was reported in 100% of patients (Table 1).

Table 1: Showing results of bowel function after Soave in both groups

 

Bowel Function

Total

Normal

NA

Age

less than a month

Count

9

0

9

% within age

100.0%

0.0%

100.0%

less than 5 years

Count

1

0

1

% within age

100.0%

0.0%

100.0%

less than 10 years

Count

6

0

6

% within age

100.0%

0.0%

100.0%

more than 10 years

Count

0

1

1

% within age

0.0%

100.0%

100.0%

Total

Count

16

1

17

% within age

94.1%

5.9%

100.0%

Discussion

HD is one of the most common congenital gut motility disorders facing pediatric surgeons with a mainstay of treatment involving removing the aganglionic bowel segment and pulling through the ganglionic bowel while preserving the anal sphincter. Many surgical techniques had evolved with many published HD series treated with either the laparoscopic or transanal pull-through techniques from almost every continent with excellent outcomes [16,17,18,33,28].

Few studies compared the laparoscopic to the transanal approach [19]. Miyano and his colleagues [27] adopted the laparoscopic approach, while Pratap and his group [36] highlighted the high feasibility of the transanal pull-through as it can be done by any pediatric surgeon, including those without laparoscopic skills, and by pediatric surgeons in parts of the world where access to appropriately laparoscopic equipment is limited.

In our center we adopted the Soave transanal technique in all our patients irrespective of the age at presentation and age at surgical intervention. We divided patients into two groups: neonatal and postneonatal, Soave procedure results were almost similar in both groups. None experienced anastomotic leaks, strictures or intraabdominal abscesses, this compares favourably with literature results which quotes leak rates of 5–7%, stricture rates of 5–24%, and abscess formation in 2–6% [20-25] which might be explained by the fact that we performed the Soave procedure without opening the abdomen or creating a stoma as compared to diversion before pull through done by others. The complications described by them beyond the neonatal age were attributable to the stoma done before Soave not the Soave itself [31]. In those series surgeons preferred preliminary defunctioning stoma in older children for resolution of the dilatation and thickening, so that a subsequent transanal approach can be safely used, however in our series like Onishi and his group, we used good colonic irrigations to decompress the dilated colon. We didn’t encounter any difficulty during transanal dissection to pull down this dilated segment of the rectum or colon (Fig. 2) and a single stage Soave procedure was performed without any appreciable complications in follow up as compared to the neonatal group (Table 1).

Full thickness frozen biopsies sections were performed intraoperative in all patients in our cohort. Based on the report of Muller et al. 2012 [22], we adopted our protocol where we don’t only depend on confirmation of presence of ganglion cells in intraoperative biopsies studies prior to performing an anastomosis, but also to have pathologic assurance of normal appearing nerves without hypertrophied nerve trunks in a circumferential biopsy sample which includes muscularis and submucosa. These protocols explain the slightly prolonged operative time in group 2 which was attributed to the wait time for the results of the intraoperative biopsies. This means that practically speaking the time of the technique itself is almost equal in both groups.

Only one patient in group 2 had constipation with intermittent laxative use and none had bowel dysfunction in both groups as excessive pull of the anal canal and external sphincter were avoided. In concordance with Levitt and his group [26], we completely removed the aganglionic bowel apart from the preserved 1 cm above the dentate line, without leaving behind a long cuff as we only leave around 3 cm cuff.

Although we have a study limit because of a small cohort population, we can conclude that the Soave approach is a safe, reproducible and elegant technique if properly planed irrespective of the patient’s age. A larger multicenter prospective study is warranted to solidify our conclusion.

 

 

References:

1. Swenson O, Rheinlander HF, Diamond I. Hirschsprung’s disease: a new concept of the etiology. N Engl J Med 1949;241:551-6.

2. Swenson O, Bill AH. Resection of rectum and rectosigmoid with preservation of sphincter for benign spastic lesions producing megacolon: an experimental study. Surgery 1948;24:212.

3. Soave F. A new operation for the treatment of Hirschsprung’s disease. Surgery 1964;56:1007-14.

4. Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35:38-9.

5. Duhamel B. Une nouvelle opération pour le mégacolon congénital: l’abaissement rétrorectal et trans-anal du colon, et son application possible au traitement de quelques autres malformations. Presse Med 1956;64:2249-50.

6. Rehbein F, Von Zimmermann H. Results with abdominal resection in Hirschsprung’s disease. Arch Dis Child 1960;35:29-37.

7. So HB, Schwartz DL, Becker JM, et al. Endorectal “pull through” without preliminary colostomy in neonates with Hirschsprung’s disease. J Pediatr Surg 1980;15:470-1.

8. Teitelbaum DH, Coran AG. Primary pull-through for Hirschsprung’s disease. Semin Neonatol 2003;8:233-41.

9.De la Torre-Mondrago’n L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 1998;33:1283-6.

10. Langer JC, Minkes RK, Mazziotti MV, et al. Transanal one-stage Soave procedure for infants with Hirschsprung’s disease. J Pediatr Surg 1999;34:148-52.

11. Georgeson KE, Fuenfer MM, Hardin WD. Primary laparoscopic pull through for Hirschsprung’s disease in infants and children. J Pediatr Surg 1995;30:1017-22.

12. Levitt MA, Peña A. Imperforate anus and cloacal malformations. In: Holcomb GW, Murphy JP, editors. Ashcraft’s pediatric surgery. 5th ed. Philadelphia (Pa): Saunders Elsevier; 2010. p. 468-90.

13. Lawal T, Chatoorgoon K, Collins M, et al. Redo pull-through for obstructive symptoms due to residual aganglionosis and transition zone in Hirschsprung’s disease. Pediatr Surg 2011;46:342-7.

14. Swenson O. Follow-up on 200 patients treated for Hirschsprung’s disease during a ten-year period. Ann Surg 1957;146:706-14.

15. Langer JC, Durrant AC, de la Torre-Mondragón L, et al. One-stage transanal Soave pullthrough for Hirschsprung disease: A multicenter experience with 141 children. Ann Surg 2003;238:569-76.

16. Pratap A, Shakya VC, Biswas BK, et al. Single-stage transanal endorectal pull-through for Hirschsprung’s disease: Perspective from a developing country. J Pediatr Surg 2007; 42:532-35.

17. Sookpotarom P, Vejchapipat P. Primary transanal Swenson pull through operation for Hirschsprung’s disease. Pediatr Surg Int 2009; 25:767-73.

18. Vu PA, Thien HH, Hiep PN. Transanal one-stage endorectal pull through for Hirschsprung disease: Experiences with 51 newborn patients. Pediatr Surg Int 2010; 26:589-92.

19. Singh R, Cameron BH, Walton JM, et al. Postoperative Hirschsprung’s enterocolitis after minimally invasive Swenson’s procedure. J Pediatr Surg 2007; 42:885-9.

20. Fortuna RS, Weber TR, Tracy TF, et al. Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 1996;131: 520-24.

21. Somme S, Langer JC. Primary versus staged pull-through for the treatment of Hirschsprung disease. Semin Pediatr Surg 2004;13: 249-55.

22. Muller CO, Mignot C, Belarbi N, et al. Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung’s disease? Pediatr Surg Int 2012;28:597-601.

23. Nasr A, Langer JC. Evolution of the technique in the transanal pullthrough for Hirschsprung’s disease: Effect on outcome. J Pediatr Surg 2007;42:36-9.

24. Langer J, Caty M, de la Torre-Mondragon L, et al. IPEG colorectal panel. J Laparoendosc Adv Surg Tech A 2007;17:77-100.

25. Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung’s disease: A systematic review and meta analysis. Pediatr Surg Int 2011; 27:1053-7.

26.Marc A. Levitt, Miller C. Hamrick. Transanal, full-thickness, Swenson-like approach for Hirschsprung disease. Journal of Pediatric Surgery 2013; 48, 2289–2295.

27.Miyano G, Takeda M, Koga H, Okawada M Hirschsprung's disease in the laparoscopic transanal pull-through era: implications of age at surgery and technical aspects. Pediatr Surg Int. 2017: 5.

28.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(8):743-9.

29.Lu C, Hou G, Liu C, Geng Q, Xu X et al. Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and non neonates: A multicenter study. J Pediatr Surg. 2017 Jul;52(7):1102-1107.

30.Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel Function and Quality of Life After Transanal Endorectal Pull-through for Hirschsprung Disease: Controlled Outcomes up to Adulthood. Ann Surg. 2017 ;265(3):622-629.

31.Onishi S, Nakame K, Yamada K. Long-term outcome of bowel function for 110 consecutive cases of Hirschsprung's disease: Comparison of the abdominal approach with transanal approach more than 30 years in a single institution - is the transanal approach truly beneficial for bowel function? J Ped Surg 2016;51(12):2010-2014

32.Patrycja Sosnowska, Michał Błaszczyński. A 15-Year Experience with the One-Stage Surgery for Treatment of Hirschsprung’s Disease in Newborns, Infants, and Young Children. Indian J Surg 2015; 77(Suppl 3): S1109–S1114.

33.Amine Ksia, Houssem Yengui, Manel Ben Saad. Soave transanal one-stage endorectal pull-through in the treatment of Hirschsprung's disease of the child above two-year old: A report of 20 cases. Afr J Paediatr Surg 2013; 10 (4): 362—366.

34. de La Torre-Mondragon L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung’s disease. J Pediatr Surg 1998;33: 1283-6.

35. Langer JC, Minkes RK, Mazziotti MV, et al. Transanal one-stage Soave procedure for infants with Hirschsprung disease. J Pediatr Surg 34:148-52.

36. Pratap A, Shakya VC, Biswas BK, et al. Single-stage transanal endorectal pull-through for Hirschsprung’s disease: perspective from a developing country. J Pediatr Surg 2007; 42:532-5.

 

Bowel Function

Total

Normal

NA

less than a month

Count

9

0

9

 

% within age

100.0%

0.0%

100.0%

 

less than 5 years

Count

1

0

1

 

% within age

100.0%

0.0%

100.0%

 

less than 10 years

Count

6

0

6

 

% within age

100.0%

0.0%

100.0%

 

more than 10 years

Count

0

1

1

 

% within age

0.0%

100.0%

100.0%

 

Total

Count

16

1

17

% within age

94.1%

5.9%

100.0%