Long-Term Outcome of Laparoscopic Duhamel Procedure for Extended Hirschsprung's Disease

Introduction

Hirschsprung's disease (HD) is the most common cause of neonatal intestinal obstruction with a relatively stable incidence of 1/5000 birth. Extended forms of HD (aganglionosis located after the splenic flexure till small bowel) represent around 20% of the cases.

Due to the small number of cases managed in each center and the variety of surgical techniques, ¹ there are few reports of homogenous surgical management especially in laparoscopic techniques. The Duhamel procedure is widely accepted for treatment of extended forms of HD, ² but results and long-term outcome are lacking for the laparoscopic approach.

The single prognosis factor identified in extended HD is the length of ileal involvement ³ and a cutoff at 50 cm proximal to ileocaecal valve is considered in terms of postoperative morbi-mortality. ²

HD-associated enterocolitis is a common occurrence both pre and postoperatively, and stands for the most frequent complication after pull-through in extended HD. ⁴ Its physiopathology is still incompletely understood and its treatment and prevention remain elusive.

The aim of our study was to report our experience in laparoscopic Duhamel procedure in extended forms of HD, focusing on the frequent occurrence of late enterocolitis and its management.

Material and Methods

We performed a monocentric retrospective study from 1991 to 2013 of all patients operated on for HD. We focused on long forms of HD (length of aganglionosis extending to transverse colon and above), operated on by laparoscopic Duhamel procedure.

After approval by the Ethical Board of Robert Debré Hospital, data collection from charts and office notes included sex, age and weight at surgery, length of aganglionosis, comorbidities, type and surgical steps, short postoperative outcome, and mean term outcome in terms of continence and weight-for-age reference curves. We also specifically studied the occurrence of late enterocolitis, results of endoscopy when performed, and the specific management of this complication.

Surgical technique of laparoscopic Duhamel procedure was described elsewhere ⁵ and was performed by only two experienced laparoscopic surgeons during the study period. The gastroenterological team performed the endoscopy under general anesthesia in all patients with late enterocolitis symptoms: recurrent abdominal pain and distension, diarrhea, anemia, and septic and/or inflammatory syndrome on blood tests.

Statistical analysis included a rank test of Wilcoxon and an Fisher exact test with a P < .05 considered statistically significant.

Results

From 1991 to 2013, 171 HD patients were operated on in our referral center for HD. Thirty of them (17.5%) had an extended form of aganglionosis operated on by laparoscopic Duhamel procedure. Patients' characteristics are displayed on Table 1.

Short-term outcome was marked by 13% of postoperative complications requiring redo-surgery: occlusion (n = 2), wall abscess (n = 1), and anastomotic leak (n = 1). Two patients required laparoscopic rectal spur section respectively 3 months and 2.5 years postoperatively and 3 patients underwent Botox injection (two sessions) respectively 2, 2, and 3 years postoperatively, resulting in an improvement of recurrent abdominal distension and stool evacuation, even if transiently.

Postoperative mortality was null with a median follow-up (FU) of 5 years (range: 1–19 years).

More than 5 years of FU (midterm FU) was available for 50% of the patients, with 53% of satisfying bowel control (voluntary bowel movements, no constipation and no soiling, no need for laxatives or antimotility medication). Forty-six percent of these patients had a weight-for-age reference curve below 1 SD (standard deviation). Patients with midterm FU had an average of four stools per day, 13% of soiling, 6% of constipation, and 26% of recurrent abdominal distention. Results are displayed on Table 2.

Late enterocolitis occurred in 22% (n = 7) of all the cases: 6 cases of HD with ileum involvement, 1 case extended to the transverse colon, and 2 syndromic forms (1 Mowat Wilson and 1 Waardenbourg).

The mean length of aganglionic small bowel was of 15 cm (min–max: 1–25) in patients without late enterocolitis (n = 8, Group 1), against 30 cm (min–max: 2.5–60 cm) in the group with late enterocolitis (n = 6, Group 2), which was not statistically significant (Fig. 1). Late enterocolitis was more likely to occur in the group of total aganglionosis with ileum involvement compared to the group of colic forms of HD, with a significant statistical difference, according to the exact test of Fisher: P = .039.

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FIG. 1.

Occurrence of late enterocolitis according to the length of ileum involvement. Group 1, no enterocolitis; Group 2, enterocolitis; Wilcoxon test, nonsignificant in this series.

Endoscopy was performed in every case and showed longitudinal peri anastomosis Crohn-like ulcerations in 100% of the cases. Median delay between surgical procedure and ulceration diagnosis was 3 years (range: 1–8 years). Four of them have had botox injection and three rectal spur sections before assessment of late enterocolitis. Lesions were difficult to control despite corticosteroids (n = 5), immunosuppressors (n = 2) and/or anti-TNFa biotherapies (n = 1). Three patients required redo-ileostomy for recurrent abdominal distension, weight loss, and failure to thrive. Pathology showed severe chronic inflammation and eosinophilic infiltration without specific features of Crohn's disease (CD).

The video published in Videoscopy Journal shows the different steps and tricks for laparoscopic Duhamel procedure and an example of endoscopy with Crohn-like ulcerations.

Discussion

In our experience, laparoscopic Duhamel procedure was a safe and effective method for the management of extended forms of HD, according to a low postoperative morbidity. However, frequent occurrence of late enterocolitis associated to Crohn-like ulcerations impairs the late outcome of these children in terms of growth and bowel function.

Since 2000 in our referral center, surgical management of HD has been consisting of Swenson trans anal pull through for left and recto sigmoid forms of HD and in laparoscopic Duhamel procedure for extended forms of HD, meaning aganglionosis located above the splenic flexure till small bowel, with colo or ileorectal anastomosis. In our series, during the time period, 17% of HD patients presented an extended form, which is consistent with the literature. Our team published in 2001 the surgical technique of laparoscopic Duhamel procedure in 5 cases of extended forms of HD. ⁶ A comprehensive review of surgical procedures for total colonic HD in 2009 ¹ enlightened the high variability of employed techniques, but a failure to bring out a superior operative method according to morbidity, enterocolitis and functional outcome in such little series. To date, most surgeons accept Duhamel procedure as the best option for extended forms of HD considering long-term function.^2,7 With the spread of laparoscopy these last years, we confirmed that this technique was safe and effective in a series of 30 patients. Indeed, our findings of 13% rate of postoperative complications were quite low, in addition to the absence of mortality. Regarding the 2 patients, which presented a rectal spur, diagnosed on both constipation and rectal examination, they were successfully treated under laparoscopy. However, there are some tricks to try to avoid this complication: during the laparoscopic Duhamel procedure, the proximal end of the rectum must be resected by endo-GIA through a 12 mm trocar placed in the inguinal right area, a few millimeters above the last staples of the rectal pouch. Focusing on midterm outcome, our results were concordant with a systematic review and meta-analysis published in 2012 showing 42% of enterocolitis, 60% of bowel control, 33% of soiling, and 7% of definitive ileostomy in total colonic HD. ⁴

Despite advances in the treatment of HD, enterocolitis remains a relatively common complication with significant morbidity and mortality. Its incidence varies markedly according to published series (17% to 50%) because of different diagnostic criteria. ⁸ Pastor et al. developed in 2009 a standardized definition and validated a scoring system using a Delphi analysis to gain consensus from a panel of experts. ⁹ This very detailed score is however difficult to use in a retrospective analysis. Many risks factors for enterocolitis have been identified: long segment disease (as confirmed in our series), previous episodes of enterocolitis, familial forms of HD, or Down syndrome.^8–10 Various hypothesis have been postulated to explain its pathogenesis: partial mechanical obstruction by the remaining aganglionic segment or spastic internal sphincter, qualitative changes in the intestinal mucin, increased prostaglandin E1 activity, marked deficiency in the transfer of secretory IgA across gastrointestinal mucosa, impaired motility associated to protein sensitization, sucrose isomaltase deficiency, defective white cell functions, retained neuronal dysplasia within ganglionic colon, and retrovirus or Clostridium difficile infection.^11–16 Identifying underlying causes must comprise rectal exam looking for anastomotic stricture, review of pathology specimen looking for retained aganglionic segment, and contrast enema looking for stricture, rectal pouch, or twist of pull through segment. ¹⁰

After elimination of these mechanical causes, 7 patients in our series have been diagnosed with chronic enterocolitis and have undergone an endoscopy, as proposed by Pini Prato et al. in 2008 in their algorithm for management of persistent chronic diarrhea after pull through for HD. ¹⁷ They therefore identified 1 patient with sucrose isomaltase deficiency and 2 with inflammatory bowel disease. Rare cases of an association between HD and CD were described in literature with a familial history of CD and granuloma on pathology in half of the cases.^18–20 In our series, even if none of the 7 patients had neither familial history of CD nor granuloma on pathology, 1 out of our 4 patients with an extended HD and assessed for NOD2 mutations was carrying a variant known to be associated with CD. ²¹ This point was challenged by the results of Lacher et al. in 2010, who did not find any correlation between HD-associated enterocolitis and NOD2 variant in a series of 52 patients with various forms of HD. ²² Moreover, classic treatment of CD did not show evident improvement of symptoms related to these chronic ulcerations in our series. Link between extended HD associated enterocolitis and CD required to be further investigated, possibly focusing on dysbiosis induced by ileorectal anastomosis.^23,24

Conclusion

Laparoscopic Duhamel procedure is a safe and effective surgical technique in the management of extended forms of HD, according to a low postoperative morbidity. However, the high occurrence of late enterocolitis with Crohn-like ulcerations visualized in endoscopy impairs the late outcome of these children in terms of growth and bowel function. Link between HD and CD still requires to be investigated.