Long-term bowel function and quality of life in children with Hirschsprung's disease

doi:10.1016/j.jpedsurg.2007.12.038

Journal of Pediatric Surgery

Volume 43, Issue 5, May 2008, Pages 899-905

https://doi.org/10.1016/j.jpedsurg.2007.12.038 Get rights and content

Abstract

Background/Purpose

Little is known about the quality of life (QOL) of children with Hirschsprung's disease (HD) as they grow older. The purpose of this study was to measure the QOL and bowel function of these children as they mature.

Methods

All children who were surgically treated for HD at British Columbia Children's Hospital, Vancouver, British Columbia, Canada between 1986 and 2003 were invited to participate. Each family was sent 3 previously validated questionnaires exploring current QOL and bowel function.

Results

Fifty-one families participated (49%), with children between the ages of 3 and 21 years. Fecal continence improved significantly with age (P = .04) and was the strongest predictor of QOL scores of all variables in our study. There was no statistically significant difference in QOL scores between children with HD and healthy children, although a clinically relevant impairment in QOL may be present, especially in psychosocial scores.

Conclusions

Fecal continence is an important predictor of overall QOL in children surgically treated for HD. Although continence tends to improve with age, a number of older children still have ongoing continence problems, and they seem to be a group at risk for impaired QOL. Our study indicates that interventions for children with incontinence may offer gains in QOL as well as bowel function.

Section snippets

Sample

British Columbia Children's Hospital (BCCH), Vancouver, British Columbia, Canada, is the tertiary pediatric surgery center in the province of British Columbia and the Yukon Territory. A database of all children surgically treated for HD at BCCH was used to identify eligible families for this study. The current study included all children born between October 1986 and October 2003. Children younger than 3 years were excluded because it is difficult to assess fecal continence in this age group.

Sample

One hundred forty-four children underwent surgical repair of HD between October 1986 and October 2003. Four of these children have since passed away with the following causes of death: withdrawal of care because of total bowel aganglionosis at 1 month of age, septic shock at 10 months of age, and unknown cause and age in the other 2 children. Current contact information was available for 119 families, but 14 of these packages were returned to sender and presumed lost to follow-up. In total, 105

Discussion

It is well established that children who undergo surgical correction for HD are at significant risk of developing long-term bowel dysfunction. The incidence of bowel dysfunction ranges widely from study to study. Constipation has been reported from approximately 15% [8], [15] to a high of 60% [16]. Fecal incontinence has ranged from a low of less than 10% [7] to a high of between 75% and 80% [1], [16]. Our study population had a lower rate of constipation (8%) but an incontinence rate in the

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Cited by (65)

Health-related quality of life in children with Hirschsprung disease and children with functional constipation: Parent-child variability
2022, Journal of Pediatric Surgery
Health-related quality of life (HRQOL) is an important outcome among children with Hirschsprung Disease (HD), but there are challenges in interpreting findings in previous studies owing to the choice of a comparator group and informant. We compared parent-proxy versus child self-report HRQOL in children with HD to children with functional constipation (FC) and examined predictors of HRQOL.
Data of 126 children (5–18 years, 60.3% male, HD: n = 52, FC: n = 74) were acquired from the Pediatric Colorectal and Pelvic Learning Consortium. Demographics, clinical variables, HRQOL (Pediatric Quality of Life Inventory parent-proxy; child self-report) and functional outcomes (Baylor Continence Scale, Cleveland Clinic Constipation Scoring System) were collected.
Parent and child HRQOL was similar for both cohorts, with higher scores on physical functioning and lower scores on emotional and school functioning. For children with HD, demographics and clinical variables did not predict HRQOL in multivariable regression models. For children with FC, greater severity of constipation predicted lower HRQOL (parent-proxy: B = -2.14, p < 0.001; child: B = -1.75, p = 0.001). Parent-child agreement on HRQOL scores was poor to moderate in the HD group (intraclass correlations (ICC)=0.38–0.74), but moderate to excellent in the FC group (ICC=0.63–0.84). Furthermore, parents of children with FC and ≤10 years overestimated children's HRQOL (proportional OR 4.59 (1.63, 13.85); p = 0.004).
Clinical symptoms and demographic factors did not predict HRQOL among children with HD, highlighting the need to examine other biopsychosocial factors to understand long term HRQOL. Low parent-child HRQOL agreement in children with HD demonstrates the importance of obtaining parent and child perspectives.
III.
Prognosis study.
Patient-reported outcome measures in pediatric surgery - A systematic review
2022, Journal of Pediatric Surgery
With improved long-term survival rates, measuring the quality of surgical care has gradually shifted from clinical morbidity and mortality to patient-reported outcome measures (PROMs). Since the use of PROMs in pediatric surgery is still limited, we undertook a study to identify current PROMs, assess their characteristics, and identify gaps and areas for improvement.
A search was conducted in eight databases from their inception until May 2021 to identify PROMs that have been used in pediatric surgical patients. PRISMA standards were followed, and screening was completed by two independent reviewers. The quality of the included studies was appraised using the AXIS and the Mixed Methods Appraisal Tool.
Of 8282 studies screened, 101 articles met the inclusion criteria. Most of the studies (99%) were cross-sectional. We identified 85 different PROMs among the studies, 53 being disease-specific and the rest generic. The PedsQL™ was the most frequently used tool (42 studies). Almost half of the instruments (41 studies) were not validated, and 28% were developed ad hoc for each specific study. Significantly, all PROMs encountered were standardized (consisting of pre-determined domains), with no individualized tools currently in use. The overall quality of the included studies was good.
PROMs are increasingly used in pediatric surgery. Disease-specific PROMs predominate the field, yet validated and especially individualized PROMs are notably absent. Future efforts are needed to develop robust tools that reflect individual patient and family needs, preferences, and values, with the aim of furthering family-centered pediatric surgical care.
Functional outcome, quality of life, and ‘failures’ following pull-through surgery for hirschsprung's disease: A review of practice at a single-center
2020, Journal of Pediatric Surgery
Citation Excerpt :
At the time of this study none of these patients have had, or are being considered for, reversal of the long-term stoma. Over the last few decades there have been several studies on long-term outcomes for HD [3,10] and there is increasing recognition of the poor long-term outcomes for bowel function, especially with regards to the persistence of soiling [10,21–28]. However, these studies have several deficiencies rendering comparison of results difficult if not impossible.
The purpose of this study was to assess bowel function and quality of life (QoL) in patients with Hirschsprung's disease (HD) and identify patients who have ‘failed’ treatment.
A review of a single-center HD cohort treated with pull-through surgery from 2004 to 2017 was completed. Bowel function of patients five years and above and QoL of all patients were assessed using validated questionnaires. Patients who ‘failed’ treatment were defined as above five years with one or more of: a) long-term stoma, b) needing an antegrade continence enema or transanal irrigation, c) severe soiling, or d) severe constipation. Statistical analysis was performed with P < 0.05 deemed significant. Data are given as mean [range].
Seventy-one patients presented with HD within the study period. Mean follow-up was 5.4 years [0.7–13.3]. Of 38 eligible patients, bowel function was assessed in 24 patients (nine had a stoma, five lost to follow-up). The mean incontinence score was 17 [0–28)], and the mean constipation score was 17 [5–25]. Incontinence and constipation scores were worse than healthy controls (P < 0.001 and P = 0.001, respectively) and did not improve with age. Fifty-six patients had QoL assessed with no difference between our cohort (81 [25–100]) and healthy controls (81 [unknown]); (P = 0.85). Thirty-three patients were assessed for ‘failure’ (bowel function score n = 24; stoma n = 9). Thirty of 33 (91%) children older than five years can be considered to have ‘failed’ treatment.
Patients have worse bowel function than healthy children, which does not improve with age. QoL is comparable to healthy controls. A significant proportion of patients have poor outcomes and have ‘failed’ treatment.
Level III.
The long-term quality of life outcomes in adolescents with Hirschsprung disease
2018, Journal of Pediatric Surgery
Citation Excerpt :
Previous literature focused upon QoL outcomes in HD patients has had significant limitations, primarily owing to the variable methods used to measure QoL, and the use of heterogeneous patient populations. The results from the current study are consistent with some previous studies that have utilized PedsQL as a general measure of QoL in HD patients [4,6]. Other authors have reported lower QoL outcomes associated with the dysfunction in these patients, but they used either nonvalidated measures of QoL created by the authors, or questionnaires relating directly to bowel function which did not assess global QoL [12].
Postoperative outcomes for Hirschsprung disease (HD) remain variable, with many patients affected by constipation and/or fecal incontinence. The long-term impact upon quality of life (QoL) for HD patients is unclear. We measured long-term QoL outcomes in adolescents with HD using validated questionnaires.
Patients with HD, managed at a large tertiary pediatric institution between 1997 and 2004, were identified. Patients and/or their proxy completed validated questionnaires. Results were compared with published healthy population controls. Two questionnaires assessed QoL: Pediatric Quality of Life Inventory (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FICQOL). Three measures assessed functional outcomes: Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Symptom Survey.
Interviews were completed for 58 (70% response rate) patients [M:F, 49:9; median age, 14.5 years (11.1–18.7)]. No significant differences were found in general QoL scores between patients and healthy controls (84.84 versus 81.49, p = 0.28). Disease-specific questionnaires revealed reduced QoL in patients and families, with 17% of parents reporting the bowel dysfunction stopped their child from socializing and 47% of parents experiencing some degree of anxiety/depression regarding their child's bowel condition. Fecal incontinence (r = − 0.59, p < 0.01), constipation (r = − 0.36, p = 0.01), and dysfunctional elimination (r = − 0.59, p < 0.01) all negatively correlated with QoL scores.
In this study, generic QoL in the adolescent HD population was comparable to healthy populations. However, children with HD have ongoing bowel dysfunction which negatively impacts upon their QoL.
Prognosis study:– level II (prospective cohort study).
Anorectal malformation & Hirschsprung's disease: A cross-sectional comparison of quality of life and bowel function to healthy controls
2018, Journal of Pediatric Surgery
Patients with anorectal malformation (ARM) and Hirschsprung’s disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires.
A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation. Age-matched and gender-matched controls were enrolled from our patients with minor outpatient complaints. All participants completed the following PedsQL™ scales (maximum score 100): 4.0 Generic Core Scales, 3.0 General Well-Being (GWB) Scale and 2.0 Family Impact (FI) Module. All were also scored on bowel function (BFS), with a maximum score 20. Appropriate statistical analysis was performed, with significance level < 0.05.
There were 193 participants: 87 controls, 62 ARM, 44 HD. When comparing Core, GWB and FI scores, there were no significant differences between groups although controls had best scores indicating best QOL and general wellbeing, with least impact of the child’s health on the family. BFS was significantly different with controls having best and ARM worst scores. There were no significant differences in scores between parent and child indicating intradyad consistency. There was significant positive correlation between BFS and Core (p < 0.0001), and between BFS and GWB scores (p < 0.005); and significant negative correlation between BFS and FI scores (p < 0.0001).
Bowel function impacts quality of life. Those with ARM and HD can achieve good quality of life comparable to controls, based on patient and caregiver self-reported outcomes.
Prospective comparative study
Level II.
Quality of life outcomes in children with Hirschsprung disease
2017, Journal of Pediatric Surgery
Citation Excerpt :
Studies, utilizing validated questionnaires for moderately sized cohorts have reported no significant difference in the QoL outcomes of male compared to female children with HD [27,28]. The studies that have found noticeable differences between female and male children with HD are severely underpowered due to their very small cohorts of female children [10]. Whilst our cohort was adequately sized, we were limited in our ability to investigate the effects of disease severity and chromosomal anomalies on QoL.
Morbidity following repair of Hirschsprung disease (HD) is common. However, quality of life (QoL) results focused on HD children are contradictory. We aimed to measure QoL outcomes in HD children using validated questionnaires.
Patients with HD, managed at a large tertiary pediatric institution between 2004 and 2013, were identified. Parents completed validated questionnaires. Results were compared with published healthy population controls. QoL outcomes were measured using Pediatric Quality of Life (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FIC QOL). Functional outcomes were assessed using Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Syndrome Survey.
Parents of 60 HD patients [M:F 49:11; median age 6.4 years (2.3–10.9)] were interviewed (59% participation). The majority (47/60, 78%) had rectosigmoid disease. There was significant reduction in psychosocial (social and emotional) QoL compared with healthy children (p = 0.03). Psychosocial functioning was affected by increasing age (r = − 2.72, p < 0.001), fecal incontinence (r = − 0.475, p = 0.007), constipation (r = − 1.58, p = 0.006), and dysfunctional elimination (r = − 2.94, p = 0.004). Fecal incontinence also reduced physical functioning QoL (r = − 0.306, p = 0.007). Children with HD had significantly higher levels of fecal incontinence (p < 0.01).
We have demonstrated that HD children have significant reductions in psychosocial QoL and functional outcomes.
Prognosis Study – Level II (Prospective cohort study).

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^☆: Presented at the 39th Annual Meeting of the Canadian Association of Pediatric Surgeons, August 23-26, 2007, St John's Newfoundland, Canada.

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CAPS original papersLong-term bowel function and quality of life in children with Hirschsprung's disease☆

Abstract

Background/Purpose

Methods

Results

Conclusions

Section snippets

Sample

Sample

Discussion

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Ambul Pediat

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Adolesc Health

Critical analysis of the operative treatment of Hirschsprung's disease

Arch Surg

CAPS original papers
Long-term bowel function and quality of life in children with Hirschsprung's disease☆