Long-Term Results of One-Stage Laparoscopic-Assisted Endorectal Pull-Through for Rectosigmoid Hirschsprung's Disease in Patients Aged Above 5 Years

Abstract

Purpose:

Despite the fact that the laparoscopic-assisted endorectal pull-through (LAEPT) for Hirschsprung's disease (HD) was introduced over 20 years ago, published outcomes in older children and adolescent are lacking. To address this, we studied the long-term results of LATEP for rectosigmoid HD in patients above 5 years of age.

Materials and Methods:

A retrospective review was conducted on all nonsyndromic patients above 5 years of age and who underwent one-stage LAEPT for rectosigmoid HD between January 2002 and December 2017. Late Hirschsprung-associated enterocolitis (HAEC) was defined as HAEC occurring 1 year after the pull-through operation. Postoperative bowel function was assessed using the Krickenbeck classification.

Results:

Forty-one patients (37 males and 4 females) were included in this study with a median follow-up of 9.0 years. The median age at surgery was 55 days. Two patients had anastomotic leakage. No patient had late HAEC, rectal prolapse, anastomotic stricture, or intestinal obstruction. According to the Krickenbeck classification, all patients had voluntary bowel movements without constipation. Overall, 65.8% of patients had no soiling. However, on subgroup analysis, only 45.4% of patients younger than 11 years of age had no soiling compared with 89.5% in patients at or older than 11 years of age (P = .003).

Conclusions:

Our results showed that LAEPT for rectosigmoid HD was a safe procedure. Nearly 90% of patients had normal bowel function by puberty. Further studies are needed to address the problem of soiling in patients younger than 11 years of age.

Introduction

Hirschsprung's disease (HD) is characterized by absent ganglion cells in the distal bowel. Over 85% of patients have diseased bowel limited to the rectum and the sigmoid colon. The definitive treatment is a pull-through operation to remove the diseased bowel. There has been the evolution of surgical techniques from the traditional 3-stage open, to 1-stage either laparoscopic-assisted or totally transanal pull-through (TTAP) for about 25 years.^1–3 However, since Georgeson's first report on the use of primary laparoscopic-assisted endorectal pull-through (LAEPT) in 1995,⁴ there are only scanty reports on long-term outcomes in older children and adolescents.^5–7 In addition, it is very difficult to compare the outcomes of the various surgical techniques due to the different extent of disease and continence scoring systems reported in these studies.^8–13 It is, therefore, the aim of the study to review the long-term outcomes of LAEPT in nonsyndromic patients above 5 years of age with rectosigmoid HD using the Krickenbeck classification system.

Materials and Methods

A retrospective review was conducted on all nonsyndromic patients who were above 5 years of age and who had one-stage LAEPT performed for rectosigmoid HD between January 2002 and December 2017. In our institute, the diagnosis of HD was made by suction rectal biopsy. Preoperative barium enema to delineate the level of aganglionosis was not performed. Patients with primary colostomy, Down's syndrome, or HD extended beyond the descending colon–sigmoid junction were excluded. All patients had regular anal dilatation for a few months after surgery. In the early part of the study, these patients were kept in the hospital for two weeks when the anal wound was ready for anal dilatation. In the recent 4 years, they were discharged when the anal wound was stable and would come back at around two weeks postoperatively for anal dilatation. All patients were regularly followed-up in the Pediatric Surgery Gastrointestinal Clinic. Bowel function assessment using the Krickenbeck classification system was performed when appropriate.

Surgical technique

At laparoscopy, seromuscular colonic biopsies were taken at the dilated distal colon proximal to the macroscopic transitional zone for frozen section. The rectum was then mobilized above the peritoneal reflection. The superior rectal vessels were divided to facilitate the mobilization of pull-through colon to the anus. After adequate mobilization, as guided by the frozen section, the operation is then shifted to transanal dissection. The Lone Star retractor (Cooper Surgical) was used at the latter part of the study to facilitate the anal dissection. Depending on the age of the patient, mucosectomy was started at 3–5 mm above the dentate line. The transanal submucosal dissection was continued until the dissected rectum could be prolapsed out of the anal wound. The seromuscular layer was incised leaving a 3–5 cm muscular cuff that was split in the midline longitudinally. The pull-through colon was then delivered through the anal wound. After confirming good alignment of the mesentry and bowel by laparoscopy, anocolic anastomosis was performed. Rectal tubes were not used.

The demographics of patients, operative time, perioperative complications, and the length of hospital stay were recorded. The long-term bowel function was assessed at clinical follow-ups using the Krickenbeck classification.¹⁴ Normal bowel function was defined as spontaneous bowel movements without soiling and constipation. The bowel function was compared between 2 groups of patients at a cutoff age of 11 years, which was the median age of onset of puberty in our locality.¹⁵ In group A, the age of patients was <11 years. In group B, the age was ≥11 years. Hirschsprung-associated enterocolitis (HAEC) was diagnosed on the basis of fever, abdominal distension, and feeding intolerance. Late HAEC was defined as HAEC occurring at least 1 year after the pull-through operation.

Statistical methods

Statistical analysis was accomplished using the SPSS program for Windows 22.0 (SPSS, Chicago, IL). The Mann–Whitney U test was used to compare the continuous data. Fisher's exact test was used to compare the categorical data. P < .05 was considered statistically significant. The study is approved by the local Clinical Research Ethics Committee.

Results

Forty one children (37 males and 4 females) above 5 years of age were included in this study. The demographics are summarized in Table 1. LAEPT was successfully performed in all patients. Three patients had early postoperative surgical complications. One had a pelvic abscess requiring surgical drainage. Two had anastomotic leakage requiring fecal diversion with an ileostomy. No patient had transitional zone pull-through. At a median follow-up of 9.0 years, 7 patients had postoperative HAEC, all occurring within the first 6 months after surgery. All of these settled with rectal washout and antibiotics. There was no long-term complication, including late HAEC, intestinal obstruction, anastomotic stricture, or rectal prolapse.

Table 1.

Patient's Demographics and Long-Term Bowel Function

	N = 41
Age (years) (median, range)	10.3 (5.8–18.1)
Age at surgery (days) (median, range)	55 (14–1965)
Sex (M:F)	37:4
Body weight at surgery (kg) (median, range)	3.26 (1.1–19.1)
Operative time (minutes) (median, range)	240 (180–500)
Length of hospital stay (days) (median, range)	15 (5–214)
Postoperative HAEC	7 (17%)
Follow-up time (years) (median, range)	9.0 (3.0–18.0)
Bowel function (Krickenbeck classification)
Voluntary bowel movement	41 (100%)
Soiling
No soiling	27(65.8%)
Grade 1	8(19.5%)
Grade 2	6 (14.6%)
Grade 3	0 (0%)
Constipation
No constipation	41 (100%)
Grade 1	0 (0%)
Grade 2	0 (0%)
Grade 3	0 (0%)

All occurred within 6 months after surgery.

HAEC, Hirschsprung-associated enterocolitis.

In subgroup analysis, no statistical difference was observed regarding the age and body weight at surgery and the operative time. However, the length of stay was significantly longer in group B (P = .001) (Table 2).

Table 2.

Comparison of Demographics Between the 2 Groups

	Group A (n = 22)	Group B (n = 19)	P
Age (years) (median, range)	7.3 (5.8–10.4)	12.4 (11.2–18.1)	<.0001^*
Age at surgery (days) (median, range)	71.5 (14–1829)	34 (14–1965)	.43
Sex (M:F)	20:2	17:2	.64
Body weight at surgery (kg) (median, range)	3.18 (1.82–12.6)	3.38 (1.09–19.1)	.374
Operative time (minutes) (median, range)	265 (190–500)	230 (180–403)	.15
Length of hospital stay (days) (median, range)	8 (5–214)	20 (6–57)	.001^*
Postoperative HAEC	5 (23%)	2 (11%)	.27
Follow-up time (years) (median, range)	7.4 (3–10.4)	11.9 (7.3–18)	<.0001^*

All occurred within 6 months after surgery.

HAEC, Hirschsprung-associated enterocolitis.

P < .05 was considered to be statistically significant.

The detailed result of bowel function assessment was stated in Table 3. All patients had voluntary bowel movement. No patient suffered from constipation. Overall, 65.8% patients had normal bowel function without constipation and soiling. When comparing the results of the two groups, 89.5% of patients in group B had normal bowel function, whereas only 45.5% in group A had normal bowel function. For the two 15-year-old patients in group B having soiling, one had attention-deficit/hyperactivity disorder (ADHD) and the other had very poor motivation resulting in grade 2 soiling. Regarding the 3 patients with early postoperative complications, 2 out of 3 had normal bowel function. One in group A had grade 2 soiling.

Table 3.

Comparison of Long-Term Bowel Function Between the 2 Groups

Bowel function (Krickenbeck classification)	Group A (n = 22)	Group B (n = 19)	P
Bowel function (Krickenbeck classification)	n (%)	n (%)	P
Voluntary bowel movement	22 (100)	19 (100)	—
Soiling
No soiling	10 (45.5)	17 (89.5)	.003^*
Grade 1	8 (36.4)	1 (5.3)	.019^*
Grade 2	4 (18.2)	1 (5.3)	.22
Grade 3	0 (0)	0 (0)	—
Constipation
No constipation	22 (100)	19 (100)	—
Grade 1	0 (0)	0 (0)	—
Grade 2	0 (0)	0 (0)	—
Grade 3	0 (0)	0 (0)	—

P < .05 was considered to be statistically significant.

Discussion

The study focused on the long-term outcome on nonsyndromic patients with rectosigmoid HD. Our series had demonstrated one-stage LATEP was technically feasible and safe even in small infants. Compared with the traditional 3-stage open pull-through, our patients avoided the fashioning and closure of a stoma.¹⁶ The other advantages of LAEPT include accurate intraoperative delineation of the transition zone to avoid transition zone pull-through, proper alignment of pull-through bowel, and avoidance of excessive tension on the mesentry of pull-through bowel. Also, at a median follow-up of 9.0 years, no patient developed adhesive intestinal obstruction.¹⁷

The length of stay was statistically longer in group B (P = .001) (Table 2). The difference was likely related to the change in our clinical practice. In the early part of the study, we kept our patients in the hospital until the wound was ready for anal dilatation. With increase in experience, patients were discharged earlier and returned at 2 weeks after surgery for anal dilatation. Thus, the median hospital stay in group A was significantly shorter.

In our series, HAEC occurred in 17.1% of patients after pull-through operation and all happened within the first 6 months after surgery. All these responded to rectal washout and antibiotics. It was likely that early postoperative HAEC was due to transient internal sphincter spasm instead of anastomotic stricture or muscular cuff obstruction. The structural causes, if not corrected, will lead to recurrent HAEC even long time after surgery.

TTAP first reported in 1998, has become one of the popular methods in the management of rectosigmoid HD.¹⁸ In this method, it is mandatory to perform preoperative barium enema to assess the suitability of TTAP.^19–21 However, it is not uncommon for the contrast study to have underestimated the extent of HD resulting in additional laparoscopy or even laparotomy to complete the bowel mobilization.⁵ In addition, without the laparoscopic guidance in the TTAP, there is a potentially higher chance of twisting the mesentry and the torsion of the pull-through bowel, although such complications were reported in both methods in the literature.^16–21 In the series, we did not encounter such complications as we always tried to ascertain the good alignment of the bowel using laparoscopic guidance before transanal bowel anastomosis. Surgeons favoring the use of TTAP may also argue that the additional laparoscopic part in LAEPT increases the operative time when compared with TTAP.^20,21 However, the slight extra time is well spent as the bowel can be mobilized safely under laparoscopic guidance and the extent of trans-anal dissection can also be minimized. We have found in the series that the main limiting factor was the waiting time for frozen section. We usually finished the laparoscopic mobilization and started the transanal dissection before the result of frozen section was ready.

The ultimate goal of pull-through operation in HD is to achieve normal bowel function without soiling and constipation. Different incontinence scoring methods used in previous studies precluded comparison between different surgical techniques.^8–13 Thus, a standardized scoring system is required to have a meaningful comparison among studies. The Krickenbeck classification was by far the most objective system and was used in this study to assess the bowel function.²² This might set the platform for future studies.

There have been reports on bowel function after LAEPT and TTAP, but most of these have relatively short duration of follow-up of less than 5 years.^8,17,23 The median follow-up time of 9 years in the current series is indeed the longest in the English literature to date. Up to this moment, there were only reports on long-term bowel function in adolescents and adults with HD after open Duhamel or Swenson operations.⁷ In these studies, constipation was reported in up to 30% of patients.⁷ This post pull-through constipation may be attributed by poor motility of retained dilated colon, muscular cuff obstruction, anastomotic stricture or retained aganglionosis.²⁴ On the contrary, we did not observe any postoperative constipation in this series. We might have minimized the risk of cuff obstruction by keeping the muscular cuff short and divided.²⁵ In addition adequate laparoscopic mobilization of the colon and mesentry would minimize the risk of tension in the anocolic anastomosis and hence the risk of anastomotic stricture.²¹ All of these contribute to the good outcome observed in the series.

One of the aims of the pull-through operation is to maintain fecal continence after surgery. Unfortunately a certain proportion of patients did suffer from fecal incontinence after the pull-through operation. Incontinence may be secondary to intraoperative damage of the anal sphincter, removal of the transitional epithelium, or the lack of a rectal reservoir.²⁶ Overstretching of the anal sphincter in transanal dissection was attributed as one of the risk factors of incontinence in endorectal pull-through.^27,28 The extent of transanal dissection, hence the chance of overstretching, is indeed minimized in LAEPT as compared with TTAP.²⁸

Bowel function after pull-through operations seems to improve with age. Menezes et al. had reported only 30.3% of patients had normal bowel function at 5 years of age, but the majority had acquired normal bowel function by 15 years of age.²⁹ Similar findings were demonstrated in the current series. The study showed that only less than half of patients younger than 11 had normal bowel function while nearly 90% at or older than 11 years could achieve normal bowel function. There is so far no accountable reason for the improvement of continence at puberty. Increase in self-awareness,^7,29 increase in muscle bulk, and strength of sphincter muscle at puberty may be factors for improvement. All of these may contribute to overcome the factors for fecal incontinence, including deficiency of rectal reservoir and shortened colonic length after pull-through operations. We also observed similar situation in the current study (Table 3). On the other hand, the situation is not expected to improve at puberty if the soiling is due to the damage of transitional epithelium, anal stenosis, or damage in the anal sphincter.²⁹ As mentioned earlier, overstretching of the anal sphincter accounts for the higher incidence of soiling after the transanal dissection. We have employed the laparoscopic assistance to minimize the extent of transanal dissection and more recently the Lone Star retractor to facilitate the transanal dissection. These may help to decrease the chance of overstretching of the anal sphincter and this may in turn lead to a better functional outcome. Further studies are definitely needed to confirm this.

Apart from age, there are other nonsurgical factors that may affect fecal continence. Patient factors such as personal ability and motivation, parental support, and bowel management program also play an important role in achieving continence.²⁹ This may explain the poorer outcome in syndromic patients.³⁰ We, therefore, excluded these in the current study so as to have a clearer understanding on the effect of surgery on the outcome. At the time of review, two patients in group B still had soiling. The patient with ADHD had actually made improvement at puberty. The other boy had poor motivation to keep clean. He had the sensation but just soiled his pant instead. We were still trying to inspire him and hope he may improve in future.

The strength of this study is that this is the first report in literature focusing on the long-term outcome after LAEPT in adolescent patients. We assessed bowel function by the Krickenbeck classification, which sets a better platform for comparison in future studies. The functional outcome was structurally assessed at clinical follow-ups instead of by questionnaire. This minimized the risk of bias, poor response rate, and “survey fatigue.”²² The limitation of this study included retrospective data collection, relatively small number of patients,¹⁷ and multiple surgeons. It is expected to have minor modifications of the surgical techniques over the whole study period of 15 years when multiple surgeons were involved.

In conclusion, this study demonstrated that LAEPT was a safe procedure without any long-term surgical complication, such as intestinal obstruction or late HAEC. All nonsyndromic patients had spontaneous bowel movement without constipation. Normal bowel function could be achieved in nearly 90% of patients older than 11 years. Further studies are needed to address the problem of soiling in prepubertal patients.

Footnotes

Disclosure Statement

No competing financial interests exist.

Funding Information

No funding was received for this article.

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