Correspondence to: Amin Saleh, MD, Department of Surgery, Faculty of Medicine, Zagazig University. E-mal: aminsaleh65@yahoo.co.uk

Annals of Pediatric Surgery Vol 5, No 1, January 2009, PP 27-30

Original Article

Hirschsprung's Disease: Early and Late Outcome after Correction by Transanal Pull-through

Amin M. Saleh, Hassan A., Wesam A, Amr A. Department of Surgery, Faculty of medicine, Zagazig University

Background/purpose: Transanal pull-through for patients with Hirschsprung's disease is gaining popularity in many pediatric surgical centers. The aim of this study was to analyze the early and latepostoperative rsults.

Patients and methods: All children with Hirschsprung's disease, who were surgically treated by transanal pull- through at Zagazig University Hospital between 2002 and 2006 were included in this study. Preoperative variables were enterocolitis, bowel obstruction, age at surgery, and number of operations performed. Outcome measures included intraoperative or postoperative complications, postoperative enterocolitis, bowel functional results.

Results: Forty children with Hirschsprung's disease had been treated by trans-anal pull- through. Their ages ranged from 6 months to 2 years.The minimum follow up period was 18 months. Twenty five patients (62.5%) experienced at least one or more of the following complications : sphincter spasm (n=2, 5%); cuff abscess (n=2, 5%) , enterocolitis (n=8, 20%) , perineal excoriation (n=10, 25%), increased stool frequency (n= 14, 35%), anal stenosis (n=3, 7.5%), constipation (n=10, 25%), Fecal incontinence (n=3, 7.5%). Fecal continence improved with time; four patients required redo pull- through

Conclusions: Both early and late outcomes of patients with Hirschsprung's disease treated by transanal pull- through are acceptable. However, long term follow up are needed to detect and to treat potential complication or bowel dysfuction.

Key words: Hirschsprung's disease, trananal pull through, complications, enterocolitis

INTRODUCTIONhe surgical correction of Hirschsprung's disease (HD) is usually performed early in life. Several

operative procedures have proven effective. As the population of surgically treated patients for HD has grown older, the long term complications have also been investigated.1 Both constipation and fecal incontinence have been recognized as chronic problems in a significant proportion of these patients.1

The transanal endorectal pull- through (TEPT) approach represents a major revolution in treating Hirschsprung's disease.2 This procedure can be performed as primary pull- through as early as the first week of life. 3

The purpose of this study was to analyze the early and late outcome after trans-anal endorectal pull- through for patients with Hirschsprung's disease, and to tivestigate the relationship between increasing age and both bowel function and quality of life.

PATIENTS AND METHODS The current study included all children with Hirschsprung's disease trated by transanal pull- through at Zagazig University Hospital in the period from 2002 to 2006, and follow up was performed till 2008.

T

Saleh A. et al

Annals of Pediatric Surgery 28

All patients had rectal biopsy verified Hirschsprung's disease. The aganglionic segments were confined to rectum and or sigmoid colon in all cases treated entierly through transanal approach. Transanal pull- through was performed by the technique described by De la Torre. 2

Data chart was designed to collect the following information: (1) preoperative risk factors, (2) problems encountered before surgery, (3) operative details including the level of starting the submucosal resection, length of seromuscular cuff, length of excised segment, estimated blood loss and blood transfusion, conversion to laparotomy and its cause, and operative time, (4) early postoperative complications particularly entercolitis, cuff abscess, and anastomtic leak, (5) late postoperative complications such as increased stool frequency, anastomotic stricture, and enterocolitis. Clinical outcome, assessed by interviews regarding stool patterns including continence, frequency, and need for laxatives or other medications.

Early anal dilatation started on the 7th postoperative day in all children to relieves sphincter spasm, and to avoid development of enterocolitis, and to keep the coloanal anastomosis wide and patent. Dilatation continued once daily up to 3 months.

Constipation scoring system by Agachan et al 4 was validated using physiologic measurement of bowel evacuation. Higher scores correspond to more severe constipation.

Fecal continence was assessed according to the established scoring system proposed by Templeton and Ditesheim5,6, Higher scores correspond to better

fecal continence, and continence can be graded as good (4-5 points), fair (2-3.5 points), and poor (0-1.5 points)

RESULTS Forty patients underwent transanal pull- through procedure over a period of 4 years. Their characteristics are shown in table 1.

Thirty patients (75%) underwent a primary transanal pull-through and 10 (25%) underwent staged pull- through. The minimum follow up period was 18 months and maximum was 30 months after surgery. Postoperative complications occurred in 25 patients (62.5%), Twenty five (62.5%) patients experienced at least one or more of the following complications : sphincter spasm (n=2, 5%), cuff abscess (n=2, 5%), enterocolitis (n=8, 20%), perineal excoriation 10 (n=10, 25%), anal stenosis (n=3, 7.5%), constipation (n=10, 25%), fecal incontinence (n=3, 7.5%). Fecal continence improved with time (Table 2).

Sixteen patients showed increased stool frequency, 10 of them developed severe perineal excoriations, their ages were younger at time of pull- through. Postoperative enterocolitis ocuured more frequently in this group. Preoperative enterocolitis occurred in 20 patients (50%), 8 of them developed postoperative enterocolitis. Another two died after developing fulminant enterocolitis preoperatively and were not included in this series. Constipation developed in 10 patients (25%); those patients were younger age at operation. In those patients, a shorter segment of the colon were removed during transanal pull- through.

Table I. Operative findings and managment of 40 patients:

Total N.

>3years 1-3y 6-12m 1-6 m Patients data

13 1 2 3 7 Preoperative enterocolitis managed conservativly

7 - 1 2 4 Preoperative enterocolitis required colostomy

4

30

6

2

3

-

-

2

1

1

13

2

1

12

3

Transition zone: Rectum

Rectosigmoid

Mid sigmiod 31

9

4

1

3

2

9

2

15

4

Length of cuff < 5cm

> 5cm

45 35 25 20 Length of excised colon (cm)

Saleh A. et al

29 Vol 5, No 1, January 2009

Table II Postoperative complications:

No& % of total

> 3 years

1-3 years

6-12 months

1-6 months

Complications

2( 5%) - - - 2 Sphincter spasm 2(5%) 1 1 - - Cuff abscess 8(20%) 1 1 2 4 Enterocolitis 10(25%) 1 - 1 8 Perineal excoriations 16(40%) 1 1 3 11 Increased stool frequency. 3(7.5%) - - 1 2 Anal stenosis 10(25%) - 1 3 6 Constipation 3(7.5%) 1 1 - 1 Fecal incontinence

DISCUSSION

Since the description of the transanal pull- through procedure for Hirschsprung's disease by De la Torre Mondragon and Ortega Salgado 2 , the approach has become widely used by pediatric surgeons.3 The length of aganglionic segment has a great impact on the feasability of entirley transanal pullthrough approach3. If the aganglionic segments extend beyond the sigmoid colon, an assisted laproscopy mobilization or abdominal mobilization of the colon becomes neessary.

Some authors reported that there is a correlation between the length of excised segments of bowel and the increased frequency of bowel movment and perianal escoriation and the occurrence of fecal incontinence.7,8 We noted that 20-25 cm of the colon were excised in 30 patients, 8 patients of them developed increased stool frequency, and 4 of them developed perineal excoriation. In 10 patients 35-45cm of the colon were excised, 8 patients of them showed increased stool frequency and 6 developed perineal excoriation.

Ten patients (25%) experienced constipation during follow up periods, three of them showed stenosis at the suture line and responded to frequent dilatations, four equired redo transanal pull- through to excise more length of the colon, the other 3 patients responded to saline enemas and stimulants for intestinal motility.

Enterocolitis has been considered one of the main problems in patients with HD both before and after definitive treatment. The frequency of post operative entercolitis in the current series is still less than reported following other surgical approaches.9.10 The relative low incidence of enterocolitis after TEPT in the current series may be related in part to the short seromuscular cuff, the low coloanal anastomosis, and our policy of routine postoperative anal dilatation particularity in neonates and infants.. We agre with finding in EPSA

study3 hat showed 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.

Several authors11,12 reported that preoperative enterocolitis significantly increase the incidence of postoperative enterocolitis. This has been attributed to predisposing immunologic factors shared by patients of Hirschsprung's disease, who seem to be prone to develop enterocolitis both pre and postoperatively. Furthermore, severe perineal excoriations are presumably correlated to the chronic diarrhea experienced in cases of entercolitis.13, 14 In this study, 20 patients developed preoperative enterocolitis, 8 of them developed postoperative enterocolitis, 16 developed postoperative diarrhea and 10 of them developed perineal excoriations.

Sphincter spasm was noted in 2 patients (5%), those patients showed delayed passage of stool and abdominal distention for more than 24 hours after pull- through. Akshay et al 9 reported 6.1% incidence of sphincter spasm after transanal pull- through in patients with Hirschsprung's disease.

Postoperative fecal continence improved with time, only 3 (7.5%) patients developed incontinence, it was not affected by the age at surgery, the level of lesion or the length of the excised colon.

CONCLUSION

Transanal pull- through is both feasible and safe for the management of patients with Hirschsprung's disease It is associated with acceptable morbidity and mortality. Preoperative enterocolitis increases the incidence of

Saleh A. et al

Annals of Pediatric Surgery 30

postoperative diarrhea, enterocolits, and perineal excoriation. Both the transitional zone and a part of the dilated colon proximal to it should be excised to decrease the incidence of postoperative constipation.

REFERENCES 1. Menezes M., Corbally M., Puri P. Long term results of bowel function after treatment for hirschsprung disease :a 29 year review. Pediatr Surg Int. 22:987-990, 2006

2. De la Torre-Mondragon L, Ortega-Salago JA. Transanal endorectal pull-through for Hirschsprung disease. J Pediatr Surg. 33:1283-1286, 1998

3. Elhalaby E, Hashish A, Elbarbary MM, et al. Transanal One-Stage Endorectal Pull-Through for Hirschsprung’s Disease: A Multicenter Study. J Pediatr Surg, 39:345-351, 2004

4. Agachan F, Chen T, Pfeifer J, et al. A constipation scoring system to simplify evaluation and management of constipated patients. Dis Colon Rectum. 39:681-685, 1996

5. Temleton JM, Ditesheim JA. High imperforate anus quantitative results of long term fecal continence. J Pediatr Surg. 20:645-652, 1985

6. Yanchar NL, Soucy P..Long term outcome after Hirschsprung disease: patients' perspectives. J Pediatr Surg. 34:1152-1160, 1999

7. Ludman Spitz L, Tsuji H, et al. Hirschsprung disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis. Arch Dis Child. 86:348-351, 2002

8. Alessio P.P, Valerio G, Camilla G, et al. Hirschsprung disease: do risk factors of poor surgical outcome exist? J Pediatr Surg. 43:612-619, 2008

9. Akshay P, Devendra KG, Vikal CS, et al. Analysis of problems, complications, avoidance and management with transanal pull- through for Hirschsprung disease.J Peditr Surg. 42:1869-1876, 2007

10. Elhalaby EA, Coran AG, Blane CE, et al. Enterocolitis associated with Hirschsprung’s disease: A clinical-radiological characterization based on 168 patients. J Pediatr Surg 30:76-83, 1995

11. Gao Y, Li G, Zhang X, et al. Primary transanal rectosigmodectomy for Hirschsprung’s disease: Preliminary results in the initial 33 cases. J Pediatr Surg 36:1816-1819, 2001

12. Fouquet V, De Lagausie P, Faure C, et al. Do prognostic factors exist for total colonic aganglionosis with ileal involvement? J Pediatr Surg. 37:71-75, 2002

13. Elhalaby EA, Teitelbaum DH, Coran AG, et al. Enterocolitis associated with Hirschsprung’s disease: A clinical histopathological correlative study. J Pediatr Surg 30:1023-1027, 1995

14. Murphy F, Puri P. New insight into the pathogenesis of Hirschsprung associated enterocolitis . Pedatr Surg Int. 21:773-779, 2005