A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease

doi:10.1016/j.jpedsurg.2017.01.001

Journal of Pediatric Surgery

Volume 52, Issue 9, September 2017, Pages 1458-1464

https://doi.org/10.1016/j.jpedsurg.2017.01.001 Get rights and content

Abstract

Objective

Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome.

Methods

Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records.

Results

73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n = 31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P < 0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1–15, P = 0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1–5.7, P = 0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs.

Conclusion

A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted.

Prognosis Study: Level II.

Section snippets

Patients

All HD patients above four years of age with aganglionosis not extending oral to the sigmoid colon operated with primary endorectal pull-through at four Nordic tertiary pediatric surgical centers, were eligible for inclusion. The only exclusion criterion was severe language problems precluding reliable assessment of bowel function.

Surgical management

All patients had the diagnosis verified preoperatively by a rectal biopsy, and a contrast enema, indicating the location of the transition zone, was performed in all

Patients

A total of 258 patients with rectosigmoid HD were operated with a primary transanal endorectal pull-through at the four hospitals until 2010. The technique was introduced in the departments in Helsinki, Oslo, Stockholm, and Lund in 1987, 1999, 2001, and 2005, respectively. 55/258 patients/parents did not respond, and three were excluded owing to language problems. Consequently, 200 patients (169 males) were enrolled. 75, 68, 30 and 27 patients were recruited from the hospitals in Helsinki,

Discussion

This multicenter study on a large population of HD patients with rectosigmoid aganglionosis operated with transanal endorectal pull-through shows that a significant number of patients continue to have long-term problems, mainly related to fecal incontinence. We have also shown that syndromic patients have less favorable functional outcome. Our results also suggest that a completely transanal procedure may predispose to long-term bowel functional defects. Importantly, for the majority of the

Acknowledgments

The authors would like to thank nurse practitioner Astri Austrheim for conducting the telephone interviews with the patients.

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Cited by (50)

Sexual function and lower urinary tract symptoms after minimally invasive endorectal pull-through in adolescent males with Hirschsprung disease
2023, Journal of Pediatric Surgery
Citation Excerpt :
One male adolescent had a neurologic disease without intellectual impairment, and was the only one with low satisfaction with own bladder function. It is also important to emphasize that the frequency of LUTS in this and most studies we have compared our results with, report findings in non-syndromic HD patients [9,15,34]. In syndromic HD patients, both frequency and severity of LUTS are different from those without a syndrome [35,36].
Effects of colorectal resection on sexual function and the lower urinary tract are inadequately studied in patients with Hirschsprung disease (HD). This study aimed to increase existing knowledge on sexual function and lower urinary tract symptoms (LUTS) in HD male adolescents operated with minimally invasive endorectal pull-through (ERPT).
Non-syndromic male adolescents (12–18 years) operated with ERPT were invited to participate in this single-center cross-sectional study which included a semi-structured interview on sexual function and LUTS, a questionnaire recording LUTS, and a urodynamic study. Uroflowmetry curves were eligible for evaluation if the voided volume was >50% of expected bladder capacity. Ethical approval and informed consent were obtained.
Of 37 eligible male adolescents, 35 (95%) with a median age of 14.9 (12.0 -18.3) years were included. 94% had rectosigmoid aganglionosis, and 97% underwent a minimally invasive ERPT. 34 (97%) visited the outpatient clinic. The ability to get erections and to ejaculate was assessed in 25/35 (71%) adolescents. 25/25 (100%) could get an erection, and of adolescents ≥15 years 14/15 (93%) could ejaculate. 32 (91%) returned the questionnaire and underwent urodynamic studies. 15/32 (47%) reported LUTS, but most had only sporadic LUTS. 31/32 (97%) were highly satisfied with their bladder function. Sporadic urinary incontinence was reported by 4/32 (13%), but none reported social problems due to this. Of the eligible 28/32 (88%) uroflowmetry curves, 15/28 (54%) were normal.
Minimally invasive ERPT seems to preserve sexual function and does not induce lower urinary tract symptoms in adolescent HD males.
III.
Pull through for Hirschsprung disease without planned rectal decompression is safe
2023, Journal of Pediatric Surgery
Definitive surgery for Hirschsprung disease (HD) is typically deferred to allow rectal washouts. In contrast, we have performed pull through on the next available operating list following diagnostic biopsy. Our aims are to compare outcomes of surgery without planned decompression vs. surgery where the timing of pull through was deferred.
A consecutive series undergoing pull-through. Timing of surgery was at surgeon's discretion and classified as either no planned decompression surgery or other. Outcomes were i) complications graded >3b Clavien-Dindo (CD) within 30d, or ii) a need for a revisional pull through or iii) a post-pull-through stoma. We excluded total colonic aganglionosis.
156 children (116 boys) underwent pull-through. 71 had surgery without planned decompression. The indications for timing in the other 85 were: planned after home washouts (n = 28), planned after stoma (n = 29), diagnostic error (n = 19), prematurity (n = 3), late presentation (n = 2) and other (n = 4). The mean age at surgery in the surgery without planned decompression group was 18 days (range 1–49 days) vs. 310 days (range 14–4084 days).
5/72 (7%) undergoing surgery without planned decompression experienced a complication of ≥CD 3b, compared to 7/85 (8%) of delayed surgery (p = 0.8). 3/71 (4%) of the surgery without planned decompression group required a stoma following pull through compared to 11/83 (13%) of the delayed group (p = 0.059). 5/71 (7%) of the surgery without planned decompression group required revisional pull through compared to 13/85 (15%) of the delayed surgery group (p = 0.1).
Surgery without planned rectal decompression for HD leads to no increase in significant complications, requirement for stoma or revision. Early definitive surgery is safe, and the use of pre-operative stoma or rectal washouts is not always necessary.
III
Comparison of clinical outcomes after total transanal and laparoscopic assisted endorectal pull-through in patients with rectosigmoid Hirschsprung disease
2022, Journal of Pediatric Surgery
Citation Excerpt :
Furthermore, TERPT is suggested to have shorter operative time and faster recovery as well as being less expensive than LERPT [3,4]. However, there are concerns that TERPT causes more anal sphincter damage because the exposure of the anal canal is longer and more forceful when the whole operation is performed through the anus [5–7]. The literature comparing bowel function after total transanal and transabdominal ERPT procedures is inconclusive [4,8-11].
Total transanal (TERPT) and laparoscopic endorectal pull-through (LERPT) are the most common procedures to treat rectosigmoid Hirschsprung's disease (HD). Since few studies have compared the two methods, we aimed to assess clinical outcomes after TERPT and LERPT in this cross-sectional study.
All patients with rectosigmoid HD operated with TERPT and LERPT between 2001 and 2018 were eligible. Peri-operative data were registered from patients’ records, and bowel function was assessed according to the Krickenbeck classification.
91/97 (94%) patients were included; 46 operated with TERPT and 45 with LERPT. Bowel function was assessed in 80 patients at median seven (4–17) years. There was no difference in functional outcome between the procedures. Unplanned procedures under general anesthesia were frequent; 28% after TERPT and 49% after LERPT (p = 0.04). 11% of TERPT and 29% of LERPT patients got botulinum toxin injections (p = 0.03). In the TERPT group, patients operated in the neonatal period had poorer outcome (78%) than those operated later (24%) (p = 0.005). No difference in operative time, length of hospital stay, and rate of early and late complications was found between the procedures.
There was no difference in long-term bowel function in patients with rectosigmoid HD operated with TERPT or LERPT. More LERPT patients had an unplanned procedure under general anesthesia, mostly due to obstructive symptoms.
III.
Hirschsprung disease outcomes
2022, Seminars in Pediatric Surgery
Citation Excerpt :
Studies related to Health-related quality of life (HRQOL) in children have been equivocal with some studies reporting poorer HRQOL compared to their healthy peers14,15 and others demonstrating little or no impairment despite worse disease specific functioning.16 Improvement in bowel function and quality of life have been shown to occur with age2,14,17–20 although a recent study which included adults reported good bowel function scores in 52% with a poor score in 12%. This correlated to an impaired Gastrointestinal QOL score in 25% of adults.21
Hirschsprung disease (HD) is a complex surgical and medical problem that appears to have varied health and social outcomes with the age and neurodevelopmental state of patients. In general, long-term outcomes are thought to be good for the majority of patients despite recognized problems with constipation and/or fecal incontinence. However, there are no universally accepted pathways regarding post-operative bowel management programs nor clearly defined follow-up pathways making the current outcome measures difficult to interpret. Further, other factors that may influence outcome including age at the time of procedure and procedure type continue to lack consensus. Improved support of children in resource limited environments and during periods of transition into the adult medical care environment are needed to improve outcome. Recent proliferation of multidisciplinary care teams and consortia may help to better understand outcomes and address current knowledge gaps. Continuing these collaborations will be imperative to continuing improvements in care which may ultimately impact outcome.
Optimal timing for Soave primary pull-through in short-segment Hirschsprung disease: A meta-analysis
2022, Journal of Pediatric Surgery
Citation Excerpt :
Several recent studies have aimed to address the question of whether the timing of primary ERPT surgery for SS-HSCR is associated with functional outcomes. The results have varied, with some studies showing inferior outcomes in younger infants [19–21], others showing no association between outcomes with age at surgery [22,23], and one showing improved outcomes with younger age at ERPT [24]. With the goal of identifying an optimal age for ERPT surgery in infants with SS-HSCR, we performed a systematic review of the current literature and meta-analysis of the relevant studies with the addition of our institutional data to determine whether age at primary ERPT influences clinical outcomes.
The optimal age for endorectal pull-through (ERPT) surgery in infants with short-segment Hirschsprung disease varies, with a trend toward earlier surgery. However, it is unclear if the timing of surgery impacts functional outcomes. We undertook the present study to determine the optimal timing of ERPT in infants with short-segment Hirschsprung disease.
The NCBI PubMed database was searched for English-language manuscripts published between 2000 and 2019 analyzing functional outcomes for patient following the initial Soave ERPT for short-segment Hirschsprung disease. Raw data from these studies was obtained from the corresponding author for each manuscript. We combined data from these papers with our own institutional data and performed a meta-analysis.
A total of 780 infants were included in our meta-analysis. Constipation occurred in 1.0–31.7%, soiling 1.3–26.0%, anastomotic stricture 0.0–14.6%, and anastomotic leak 0.0–3.4%. Regarding age at ERPT, younger infants at the time of initial corrective surgery had higher rates of soiling, stricture, and leak. On sub-group analysis, patients <2.5 months at their initial corrective surgery had higher rates of soiling (25.9% vs. 11.4%, p<0.01), as well as stricture (10.0% vs 1.7%, p<0.01) and leak (5.5% vs 1.3%, p<0.01).
While age at Soave endorectal pull-through for short-segment Hirschsprung disease has decreased over time, functional outcomes associated with this trend have only recently been examined. Our findings suggest that patients <2.5 months old at the time of endorectal pull-through may have worse functional outcomes, emphasizing the need to consider further study of the timing of surgery in this population.
Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee
2021, Journal of Pediatric Surgery
Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD.
Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed.
66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder.
A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

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^☆: Funding source: This work was funded by South-Eastern Norway Regional Health Authority.

^☆☆: Financial disclosure: The authors declare no financial relationships relevant to this article.

^★: Conflicts of interests: The authors declare no conflicts of interest relevant to this paper.

^★★: Preliminary results were presented at the Annual meetings of the European Association of Paediatric Surgeons, June 2015, Ljubljana, Slovenia and June, 2016, Milan, Italy.

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ClinicalA Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease☆,☆☆,★,★★

Abstract

Objective

Methods

Results

Conclusion

Section snippets

Patients

Surgical management

Patients

Discussion

Acknowledgments

J Pediatr Surg

Semin Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Semin Pediatr Surg

Clinical
A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease☆,☆☆,★,★★