Long-term results of transanal pull-through for Hirschsprung’s disease: a meta-analysis

Introduction

Several operative techniques have been developed for the treatment of Hirschsprung’s disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD.

Methods

A systematic literature search for relevant articles was performed in four databases using the following terms “Hirschsprung/Hirschsprung’s disease”, “aganglionosis”, “transanal”, “pullthrough/pull-through”, “longterm/long-term” “results”, “follow-up” and “outcome”. A meta-analysis was conducted for relevant articles for one-stage transanal pull-through for HD with a minimal follow-up of median 36 months regarding constipation, incontinence/soiling, enterocolitis and secondary operations. Odds ratio (OR) with 95 % confidence intervals (CI) were calculated.

Results

Six studies with 316 patients matched the set criteria and were included in this analysis. Overall 45 (14.2 %) patients had disturbances of bowel function (OR 0.05, 95 % CI 0.03–0.07, p < 0.00001). Of these, 24 (53.3 %) patients experienced constipation, 8 (17.8 %) incontinence/soiling and 13 (28.9 %) enterocolitis. 10 (3.2 %) patients developed complications requiring secondary surgery. Most patients had a daily defecation frequency of 1–3 bowel movements 3 years postoperatively, resembling the stooling patterns of healthy controls.

Conclusion

Nearly 15 % of all patients operated with TAPT for HD continue to experience persistent bowel symptoms with constipation as the main problem. Further studies on the long-term outcome of children operated with this technique for HD are necessary to evaluate stooling patterns, urinary and sexual function as well as general quality of life during adolescence and adulthood.

     

Usefulness of endoscopic marking for determining the location of transanal endorectal pull-through in the treatment of Hirschsprung’s disease

In the treatment of Hirschsprung’s disease, transanal endorectal pull-through (TEPT) is being performed without laparoscopic assistance or laparotomy for short-segment aganglionosis. Preoperative ascertainment of the extent of aganglionosis is required, as this affects the planning for TEPT. The present study investigated the usefulness of preoperative endoscopic marking as an intraoperative marker of the location of pull-through. Since 1998, we have performed TEPT using a prolapsing technique for the treatment of Hirschsprung’s disease. Subjects comprised 17 patients with short-segment aganglionosis and 2 patients with long-segment aganglionosis in whom endoscopic marking was performed preoperatively. Median age at time of surgery was 2.7 months. The relationship between pathologic findings at the marked area and location of actual pull-through was investigated. For endoscopic marking, each patient was sedated using intravenous ketamine hydrochloride. The junction between normal bowel with peristalsis and aganglionic bowel without peristalsis (“shorebreak” finding) was marked by either tattooing or clipping. Normal ganglion cells were seen in the marked area of 14 patients, and pull-through was performed at the marked area in each of these patients. In three patients, ganglion cells existed in the marked area, but the number of ganglion cells was considered insufficient. Additional frozen sections were thus prepared to ascertain the area with normal ganglion cells, showing that normal ganglion cells were seen 1, 3 or 5 cm proximal to the marked area. In one patient, no ganglion cells were seen in the marked area, but were present 5 cm proximal to the marked area. In the remaining one patient, normal ganglion cells were seen 7 cm distal to the marked area. Pathologic findings revealed ganglion cells at the shorebreak finding in 17 of the 19 patients (89.5%), suggesting that this junction basically matches the distribution of ganglion cells. Endoscopic marking of the junction is very useful for determining the tip of pull-through.     

Transanal mucosectomy for endorectal pull-through in Hirschsprung’s disease: comparison of abdominal,extraanal and transanal approaches

BACKGROUND/PURPOSE: The authors compared the clinical outcome between laparoscopically assisted endorectal pull-through (EPT) with an extraanal approach (EA) and conventional abdominal approach (AB) in Hirschsprung's disease (HD), and found that the former approach was less invasive and can provide a better clinical outcome. Recently, they introduced transanal approach (TA) without laparoscopical assistance and compared the clinical outcomes of these three approaches. METHODS: In the period between 1990 and 1997, 20 cases of HD underwent EPT with AB (Group A), between 1998 and 2001, 21 cases underwent EPT with EA (Group E), and between 2004 and 2005, eight cases underwent EPT with TA (Group T). There was no difference in age and weight distribution among three groups. Clinical outcome was assessed 3 years after surgery. RESULTS: The operation time in Groups A, E and T was comparable (4.9 +/- 0.8, 5.2 +/- 0.8 and 4.3 +/- 0.8 h), whereas blood loss (98 +/- 52, 36 +/- 30 and 36 +/- 30 ml) and the incidence of postoperative complications requiring surgical intervention (25, 0 and 0%) was significantly lower in Groups E and T compared with Group A. The incidence of postoperative enteritis (25, 29 and 13%) was lower in Group T compared with Groups A and E. In Group E, two cases had persistent constipation, which required anorectal myotomy. Voluntary defecation (>once/2 days) was comparable among the groups (70, 87 and 88%). Soiling (small amount of involuntary passage of stool) was significantly less frequent in Groups E and T (45, 14 and 0%). CONCLUSION: EPT with perineal approaches is less invasive and can provide a better clinical outcome than EPT with AB in terms of postoperative soiling. Compared with EA and TA, EA tended to develop stagnant enteritis or residual constipation.     

Long-term outcome of children after single-stage transanal endorectal pull-through for Hirschsprung’s disease

Background  

Single-stage transanal endorectal pull-through (TEPT) is a new technique for surgery of Hirschsprung’s disease (HD). TEPT can be assisted by laparoscopy (laparoscopic assisted transanal pull-through, LATP) or with non-additional procedure (total transanal endorectal pull-through, TTEP). This study was undertaken to evaluate the long-term outcome of these approaches in children with HD.     

Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung’s disease

Purpose  

The transanal one-stage pull-through procedure (TERPT) has gained worldwide popularity over open and laparoscopic-assisted one-stage techniques in children with Hirschsprung’s disease (HD). It offers the advantages of avoiding laparotomy, laparoscopy, scars, abdominal contamination, and adhesions. However, enterocolitis associated with Hirschsprung’s disease (HAEC) still remains to be a potentially life-threatening complication after pull-through operation. The reported incidence of HAEC ranges from 4.6 to 54%. This meta-analysis was designed to evaluate postoperative incidence of HAEC following TERPT procedure.     

What is the most common complication after one-stage transanal pull-through in infants with Hirschsprung’s disease?

Background  

Hirschsprung’s disease (HD) is a relatively common congenital disease that could be suspected by clinical symptoms, abdominal plain X-ray, and finally diagnosed by rectal biopsy. In 80% cases, rectosigmoid junction is involved. Recently, one-stage transanal pull-through (TAPT) procedure has been popular and may have several complications.     

Management of Hirschsprung’s disease: a comparison of Soave’s and Duhamel’s pull-through methods

The aim of this study was to compare the safety and benefits of Soaves and Duhamels pull-through procedures for the management of Hirschsprungs disease (HD). The patients consisted of 33 boys (85%) and six girls (15%), a ratio of 5.5:1. Their ages ranged from 1 day to 8 years. Rectal biopsy was performed to confirm the diagnosis of HD. Twenty-five patients (64%) underwent Soaves pull-through, and 13 patients (33%) underwent Duhamels pull-through. Twenty children (80%) out of the 25 undergoing Soaves pull-through recovered uneventfully, compared with 11 out of the 13 (84%) undergoing Duhamels pull-through. The complications following Soaves procedure included strictures in two patients (8%), enterocolitis in another two (8%), and anastomotic leakage in one (4%). Additional operations were required in two patients (8%). The complications following Duhamels procedure included stricture in one patient (7.6%) and enterocolitis in another (7.6%). An additional operation was required in one patient (7.6%). The rate of constipation was 16% after the Soaves pull-through compared with 15% after the Duhamels pull-through. There was no significant difference between the two procedures in postoperative surgical morbidity or in long-term risk of enterocolitis. In the light of present findings, both procedures appear comparable in terms of efficiency and associated complications.     

Primary pull-through for Hirschsprung’s disease

The first report of a successful primary pull-through for Hirschsprung’s disease using the endorectal pull-through (ERPT) was by So et al. (J. Pediatr. Surg. 15 (1980) 470; J. Pediatr. Surg. 33 (1998) 673). Subsequently, because of the simplified nature of this approach and the potential for cost savings, several groups have reported excellent results with this procedure. In addition to the ERPT, both the Duhamel and Swenson procedures have been performed in a one-stage fashion. More recently, primary laparoscopic approaches for each of these techniques have been utilized. The purpose of this review is to discuss the technique of primary pull-through, the peri-operative management and a summary of clinical results.     

Clinical experience with a new modified transanal endorectal pull-through for Hirschsprung’s disease

Single-stage transanal endorectal pull-through (TEPT) procedure is now widely used for rectosigmoid Hirschsprung’s disease (HD). However, this procedure is associated with some common complications like stricture, enterocolitis, constipation, anastomotic dehiscence and perianal excoriation. The aim of this study was to evaluate the clinical outcomes of a modified TEPT operation in the management of rectosigmoid HD. 45 patients with rectosigmoid HD admitted between August, 2004 and July, 2008 were included in this study. Pre-operative barium enema was done in all of them. Patients in whom the transition zone was well delineated were included in the study. Frozen section biopsy was done to confirm the presence of ganglion cells in the pull-through bowel. All odd number patients were included in group A, where conventional TEPT procedure was performed. All even number patients and the last five patients of the series were in group B. In this group, a modified transanal endorectal procedure was done. The initial part of submucosal dissection and mobilization of aganglionic rectum was the same as in the conventional procedure. The anorectal mucosa was anastomosed end-to-side to the seromuscular coat of the ganglionic bowel. Mobilized bowel was divided at about 2 cm distal to the anastomosis. Six hitching stitches were applied from the free edge of the bowel to the perianal skin so that the anastomosis remains outside the anal verge. The distal redundant bowel was excised on eighth post-operative day. Pre-operative bowel preparation was done in all patients in group A. The bowel preparation was not mandatory in group B patients. Post-operative results of both these groups were compared and analyzed using the Mann–Whitney U test. Mean operative time in group A was 86 min (range 75–95 min) versus 90 min (range 70–100 min) in group B. Average length of the bowel resected was 20 cm (range 10–26 cm) in group A and 22 cm (12–40 cm) in group B. Post-operative feeding was started after 48 h in group A and after 6 h in group B. Average hospital stay was about 8 days in both the groups. Median follow-up was 36 months in group A (range 14–54 months) and 32 months (range 12–52 months) in group B. Anastomotic stricture or stenosis was noted in two patients in group A and none in group B (p < 0.01). Partial anastomotic dehiscence was noted in two patients in group A and none in group B (p < 0.01). Average bowel movement in group A was 6–8 per day at 1 month and 2–6 per day at 6 months. In group B, it was 8–10 at 1 month and 2–8 at 6 months. Cuff narrowing causing constipation was noted in two patients in group A and none in group B (p < 0.01). Enterocolitis following pull-through was noted in two patients in group A and three patients in group B. Four patients in group A and one patient in group B had post-operative constipation. All of them responded to bowel management program: only one of them (group A) required enema; soiling was noted in one patient each in both the groups. Modified TEPT procedure is associated with excellent clinical outcome with fewer complications, and permits early post-operative feeding. Operating time, hospital stay and post-operative bowel movement were comparable with the conventional technique.     

Comparison of the postoperative bowel function between transanal endorectal pull-through and transabdominal pull-through for Hirschsprung’s disease: a study of the feces excretion function using an RI-defecogram

Purpose  

Herein, we compared the bowel function after a transabdominal and a transanal procedure for Hirschsprung’s disease (HD) using the clinical score and a quantitative evaluation of the feces excretion function based on the findings of an RI-defecogram.     

Laparoscopic-assisted pull-through operation for Hirschsprung’s disease: a systematic review and meta-analysis

Purpose

In the last two decades, laparoscopic-assisted pull-through (LAPT) has gained much popularity in the treatment of Hirschsprung’s disease. The aim of this meta-analysis was to determine the long-term outcome of patients treated laparoscopically.

Methods

A systematic literature-based search for relevant cohorts was performed using the terms “Hirschsprung’s disease and Laparoscopy”, “Laparoscopic-assisted pull-through outcome”, “Laparoscopic-assisted Soave pull-through” “Laparoscopic-assisted Swenson pull-through” and Laparoscopic-assisted Duhamel pull-through. The relevant cohorts of laparoscopic operated HD were systematically searched for outcome regarding continence, constipation, secondary surgery related to the laparoscopic approach and enterocolitis. Pooled incidence rates and odds ratios (ORs) with 95 % confidence intervals (CI) were calculated using standardized statistical methodology.

Results

Sixteen studies met defined inclusion criteria, reporting a total of 820 patients. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. The median cohort size consisted of 28 patients (range 15–218). In the long-term follow-up, 97 patients (11.14 %) experienced constipation (OR 0.06, 95 % CI 0.05–0.08, p < 0.00001), 53 (6.46 %) incontinence/soiling (OR 0.01 95 % CI 0.01–0.01, p < 0.00001), 75 (9.14 %) recurrent enterocolitis (OR 0.02 95 % CI 0.01–0.02, p < 0.00001) and 69 (8.4 %) developed complications requiring secondary surgery (OR 0.01 95 % CI 0.01–0.02, p < 0.00001). Overall events in long-term follow-up occurred in 225 (27.5 %) patients (OR 0.24 95 % CI 0.20–0.30, p < 0.00001).

Conclusions

This meta-analysis shows that nearly one-third of the patients continue to have long-term bowel problems, such as constipation, soiling and recurrent enterocolitis following LAPT. Many patients treated by LAPT require secondary surgery. Large randomized studies with long-term follow-up are necessary to determine the difference in outcome between LAPT and completely transanal pull-through operation.

     

Diagnosis and outcome of Hirschsprung’s disease: does age really matter?

Although Hirschsprungs disease (HD) typically presents in the newborn period, it is often diagnosed in older children, in whom the presentation and management remain poorly defined. We hypothesized that older patients with HD have a milder variant of the disease with an improved prognosis compared with those diagnosed earlier. Children with HD (1995–2001) were divided into Group I (diagnosis before 30 days) and Group II (after 30 days). Patients with total colonic disease were excluded. There were 66 patients; 47 in Group I and 19 in group II. Mean age at diagnosis was 7.1±1.3 days (range 1–30 days) versus 27±10 months (1.3 months–19 years). Older children differed mainly in the symptoms at presentation and the length of the involved segment of aganglionosis. Surgical strategies were applied equally in both groups. Complications, including postoperative enterocolitis, occurred equally, but the length of stay and costs were lower in Group II. The delayed diagnosis of HD does not worsen outcomes of older children with HD. This finding implies that these children have a milder form of the disease, perhaps because of adaptation to the aganglionic state.     

Is high amplitude propagated contraction present after transanal endorectal pull-through for Hirschsprung’s disease?

The transanal endorectal pull-through (TAEPT) procedure is now widely performed for Hirschsprung’s disease (HD), however, the colorectal function after TAEPT has not yet been adequately assessed. We evaluated the postoperative clinical outcome and colorectal function based on lower colonic manometry after TAEPT. Twenty-one cases of HD underwent TAEPT from 1998 to 2005. We examined the clinical outcome based on the requirement of enemas/suppositories, the number of defecations per day, the severity of perianal erosion, and the incidence of enterocolitis requiring hospital stay. Using a three-channel Dentsleeve catheter and UPS-2020 measuring device, we performed postoperative colonic manometry at three locations: (1) the anal canal, (2) 5 cm proximal to the anal canal, and (3) 10 cm proximal to the anal canal, during defecation, and then we measured the appearance of high-amplitude contraction (HAC) (duration ≥10 s, amplitude ≥100 cmH₂O). Four of 21 cases were treated with enemas/suppositories no longer than 1 year. The number of defecations decreased gradually from 4 to 5 per day in the early postoperative period, reaching 2 to 3 per day about 1 year postoperatively. Perianal erosion was noted in 3 of 21 cases but it disappeared within three postoperative years. Two cases had enterocolitis. Lower colon manometry was performed in eight cases. Manometry during defecation was successfully monitored in six cases. HAC occurred in five of six cases (83.3%). The clinical outcomes after TAEPT were satisfactory in almost all cases. These good outcomes were possibly due to the occurrence of HAC during defecation, though HAC did not fill the definition of high amplitude propagated contractions (HAPCs). The occurrence of HAC after TAEPT might be caused by keeping any damage to the mesenteric ascending cholinergic nerve to a minimum during surgery. Further long-term observations are still required to make an adequate assessment of such cases.     

Is there a hidden mortality after one-stage transanal endorectal pull-through for patients with Hirschsprung’s disease?

One-stage transanal pull-through (TAP) has become a standard definitive procedure for the treatment of Hirschsprung’s disease (HD). Short-term results of this operation seem to be excellent, but long-term outcome is still obscure. We evaluated the morbidity and mortality of our patients with one-stage TAP, and we reviewed the literature. We performed a TAP without a colostomy in 21 patients with HD. The primary outcome measures are age, sex, complications during surgery, enterocolitis (EC) attacks after surgery, postoperative stooling problems and mortality. All patients were called over telephone, and their clinical and functional outcomes were obtained. Case series of TAP in the literature were also reviewed in terms of postoperative problems. Twenty-one patients with full thickness rectal biopsy-proven HD underwent one-stage TAP. Average follow-up was 28 months. One early postoperative EC and three more late attacks of EC were observed. All survived patients had normal bowel habits. Three patients had perianal excoriations, three patients soiling, seven cases required anal dilatations and four patients experienced a diarrhea after surgery. We have been informed that four patients died after discharge from hospital. Two of them were a sudden death (one patient had metabolic problems, the other might have had an EC attack). The cause of death of one patient with an associated Down syndrome was a severe pneumonia, and one other case died of a septic shock of unknown etiology. None of these patients had a diarrhea or abdominal distention, which could have been an evidence of an EC attack prior to their deaths. We observed similar fatal cases, when reviewed the published series in the literature. There might be a hidden mortality within the long-term period after TAP for HD. Therefore, we recommend a close follow-up for all patients with any associated health problem and those from low socioeconomic regions after one-stage pull-through.     

Complete innervation profile of whole bowel resected at pull-through for Hirschsprung’s disease. Unexpected findings

We used Acetylcholinesterase (AchE) staining and neural cell adhesion molecule (NCAM) immunoreactivity to examine such resected lengths to determine the complete innervation profile of resected bowel in Hirschsprung’s disease (HD). Resected specimens of colon obtained at pull-through surgery from 15 patients with HD [short type (S-type: n=5), recto-sigmoid type (RS-type: n=5), long type (L-type: n=5)] were sectioned at 1.5 cm intervals and stained conventionally with AchE histochemistry and NCAM immunohistochemistry. The number of positive nerve fibers (PNFs) in the lamina propria and smooth muscle layers was assessed on a scale of 0 to 3 where 0 meant no PNF and 3 meant many PNFs. The three types of HD had different AchE and NCAM innervation profiles, especially the L-type. There were also different AchE and NCAM innervation patterns seen within the same aganglionic or transitional segments of bowel depending on the site of sampling. The mean proportion of transitional segment in resected specimens from RS-type HD was significantly larger than that of S-type HD (P<0.001) although the proportion of aganglionic segments from S- and RS-type HD were almost the same. Our results suggest that the etiology of L-type HD may be different from the etiology of S- and RS-type HD because of different innervation profiles. Because segments of the excised bowel would appear to have different innervation patterns depending on whether the specimen is sampled proximally or distally, it is important to record the exact site of sampling to allow valid comparisons between types of HD to be made.     

Transanal one-stage endorectal pull-through for Hirschsprung’s disease: a comparison with the staged procedures

Transanal one-stage endorectal pull-through (TOSEPT) has been presented as the most recent progression in treatment of Hirschsprung's disease (HD), which may be able to replace various previous techniques. This prospective study was designed to compare the efficacy and probable complications of the commonly used open surgery with those of TOSEPT technique in management of HD. Forty-two children (35 boys and 7 girls) between 3 days and 12 years of age underwent surgical treatment for HD-all after being diagnosed by barium enema over an 18-month period. In a manner of systematic random selection, 21 patients were operated by TOSEPT and the other 21 by Swenson procedures. All patients were followed-up at least for 12 months after hospital discharge. The necessary data containing age, sex, length of the resected aganglionic segment, intraoperative details, duration of hospital stay and postoperative functional results or complications were collected during hospitalization and follow-up period. There was no significant difference in age at the first operation, sex distribution and length of resected bowel between the two groups. Rate of postoperative complications were significantly higher in Swenson procedure group (P < 0.01). Narcotics were needed in all patients of Swenson procedure group but only in two patients operated by TOSEPT also required laparotomy (P < 0.001). The hospitalization period of TOSEPT group was also less than that of Swenson group (P < 0.001). The total cost of treatment by Swenson procedure is considerably higher than by TOSEPT. TOSEPT can significantly diminish postoperative pain, surgical complications, hospital stay and cost burden caused by Hirschsprung's disease. Besides, this procedure is cosmetically preferable because no visible scar remains.     

Experience with primary laparoscopy-assisted endorectal pull-through for Hirschsprung’s disease

Abstract From October 1996 to July 2001, 61 patients received primary laparoscopy-assisted endorectal pull-through (LAERPT) for biopsy-proven Hirschsprungs disease (HD) at Mackay Memorial Hospital. The patients ages at operation ranged from 7 days to 8 years. The patients were followed up for an average of 3.8 years (range 1–5.5 years). Major complications occurred in five (8.1%) patients, of whom four had surgical complications (two instances of anastomotic leakage, one colon perforation, and one delayed formation of colovesical fistula) and one had postoperative Salmonella infection-induced colonic stricture. The five patients required a diverting colostomy and a redo-pull-through procedure. Postoperative enterocolitis developed in 13 (21%) infants. All of them had enterocolitis before the diagnosis of HD was established. The majority of the episodes of postoperative enterocolitis were mild and resolved spontaneously, but four neonates were hospitalized with significant systemic manifestations, and two of them needed a second operation to solve the problem. After primary LAERPT, stooling frequency in young infants declined rapidly from 10.5±3.2 to 4.4±1.6 bowel movements per day in the first 3 months and more slowly thereafter. Most of these infants had regular bowel movements one to two times per day 1 year after operation. Continence evaluation of the 43 patients over 3 years of age was graded as normal in 24 (56%) patients and good in 19 (44%) patients. Of the 13 patients between 1 and 3 years of age, 11 (85%) had regular normal stools, and two (15%) had occasional soiling. From the current study, the authors concluded that primary LAERPT is a safe and effective method of managing HD with excellent continence results. Considering the complications of surgery and postoperative enterocolitis, patients with long segment aganglionosis, severe enterocolitis, or prominently dilated colon are not good candidates for primary LAERPT.     

Hirschsprung’s disease in twins: a systematic review and meta-analysis

Aim of the study

Hirschsprung’s disease (HSCR) is known to occur in families. The reported overall incidence of familial cases is 7.6%, with a higher incidence of 15–21% in total colonic aganglionosis and 50% in the rare total intestinal aganglionosis. HSCR is extremely rare in twins. The aim of this study was to systematically analyse the patterns of HSCR in twins published in the literature.

Methods

Electronic databases Pubmed and Medline were screened for relevant articles using the keywords “Hirschsprung’s disease”, “aganglionosis”, “twins”, “monozygotic”, and “dizygotic”. Examining reference lists identified further relevant papers.

Main results

Twelve studies with a total of 18 twin pairs were included in this analysis. 67% twins were discordant. HSCR was found in 24 out of 36 twin subjects (67%), of which 83% affected were male. Rectosigmoid type was reported in 71% of patients, long-segment disease in 21, and 8% presented with a total aganglionosis. Three twin pairs had at least one family member affected with HSCR.

Conclusion

HSCR was found in two-thirds of twin subjects with a male predominance. Rectosigmoid aganglionosis was the most common variant. Disease discordance was identified, where environmental insults were postulated to be predisposing factors to disease expression. Future studies investigating the disease-associated mutations in the already identified HSCR genes should provide insights into the genetic basis of HSCR in twins.