Long-term Outcomes of Patients Surgically Treated for Hirschsprung Disease

Nasr, Ahmed; Grandpierre, Viviane; Sullivan, Katrina; Wong, Coralie A; Benchimol, Eric I

doi:10.1093/jcag/gwaa026

Cited by 9 publications

(8 citation statements)

References 20 publications

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“…This could be explained by a loss at follow-up of patients with better outcomes and low statistical power, thus preventing us from confirming this theory, but the increasing trend over time was also shown in other studies. Another Canadian study in 2020 demonstrated a higher incidence of constipation in HD patients than in a control group with new-onset chronic constipation that continued to occur even after 5 years (17). Although they did not differentiate syndromic patients from isolated diseases, it is still interesting to take into consideration that our results demonstrate a greater use of bowel continence medications in the SA-HD group at 5 years.…”

Section: Discussionmentioning

confidence: 99%

Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

Gagnon

Duguay

Prasil

et al. 2023

Journal of Pediatric Gastroenterology &Amp; Nutrition

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Introduction: Given the lack of data to help caregivers in the follow-up of Hirschsprung disease (HD), this study aimed to compare the functional outcomes of isolated Hirschsprung disease (I-HD) to syndrome-associated Hirschsprung disease (SA-HD) at 1, 3, 5, and 10 years. Methods: A retrospective chart review of patients diagnosed with HD between January 1990 and May 2021 at our pediatric center was performed to collect data on patient characteristics, investigations, and treatments. Ninety-five patients were identified, of whom 76 were included in the study. SA-HD is defined as a syndrome known to be associated with HD or cognitive impairment. Results: Patient characteristics were comparable between groups (P > 0.05). There were 52 patients with I-HD and 24 with SA-HD. The patients median age was 9 days at diagnosis and 1.5 month at surgery. SA-HD patients became bowel continent at a significantly older age (mean age 8.43 vs 4.94 years, P = 0.0471) and received more bowel continence medications. At 5 years, SA-HD patients requiring ≥2 medications for bowel continence represented 54.5% versus 11.1% of I-HD patients (P = 0.009). Lastly, SA-HD patients had urinary incontinence at a significantly older age (P = 0.0136, 5 years). Conclusion: Clinicians should be aware that SA-HD patients are more prone to bladder dysfunction and became bowel continent at an older age than I-HD patients. They need more and prolonged bowel management medications, and other important complications need to be addressed in patient care. These results should prompt a longer follow-up period for these patients, especially in SA-HD.

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Section: Discussionmentioning

confidence: 99%

Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

Gagnon

Duguay

Prasil

et al. 2023

Journal of Pediatric Gastroenterology &Amp; Nutrition

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“…Our study provided another report of the functional bowel outcomes between three different pull-through techniques from a different population, i.e. , Indonesia ( vs. a Western country [ 9 ]). Another strength of our study is a prospective design and the length of follow-up of at least three years after definitive surgery.…”

Section: Discussionmentioning

confidence: 99%

“…Several studies have reported the functional outcomes of HSCR patients following pull-through [ 4 – 8 ]. However, the studies that compare long-term functional outcomes from these different surgical approaches are very limited [ 9 ]. Long-term outcomes are defined by the patient’s ability to control defecation, social function, and quality of life [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

Long-term functional outcomes of patients with Hirschsprung disease following pull-through

et al. 2022

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Background Hirschsprung disease (HSCR) is a common congenital disorder presenting with functional obstruction due to aganglionosis of the colon. There are numerous types of pull-through surgery for managing HSCR, such as transabdominal endorectal (Soave), Swenson, Duhamel, transanal endorectal pull-through (TEPT), and laparoscopic (Georgeson) approach. Here, we aimed to describe the long-term outcome of patients with HSCR who underwent transabdominal Soave, Duhamel, and TEPT in our institution. Methods We performed a cross-sectional analysis for patients who underwent Duhamel, Soave, and TEPT at our institution from January 2012 to December 2015. Long-term functional outcome was determined by bowel function score (BFS). The BFS was obtained by interviewing patients who had completed at least three years of follow-up. Results Twenty-five patients were included in this study who underwent transabdominal Soave (n = 8), Duhamel (n = 4), and TEPT (n = 13). There were 24 patients with short aganglionosis type. The median age of HSCR diagnosis was 10 (IQR = 1–39) months, while the median age of pull-through surgery was 17 (IQR = 7–47) months. The median follow-up of BFS level for HSCR patients after pull-through was 72 (IQR, 54–99) months. There were 11 patients with good BFS level and 10 patients with normal BFS level. Additionally, 50% of Duhamel patients had poor BFS level, while 50% of Soave patients had good BFS level, and 54% of TEPT patients had normal BFS level (p = 0.027). As many as 50% of Duhamel patients showed daily soiling and required protective aids, while 38.5% of TEPT had staining less than 1/week and no change of underwear required, and 50% of Soave patients revealed no soiling, respectively (p = 0.030). Furthermore, 75% of Duhamel patients had accidents, while 75% of Soave and 46.2% of TEPT patients had no accidents (p = 0.035). Conclusion Our study shows that the type of definitive surgery might affect the long-term bowel functional outcome; particularly, the TEPT approach might have some advantages over the transabdominal Soave and Duhamel procedures.

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“…6 The functional outcome of the pull-through surgery needs to be assessed post-operatively in a long-term follow-up period. 7 Functional outcome is defined by the child's ability to control defecation, social function and in the long term, by the quality of life. 8…”

Section: Introductionmentioning

confidence: 99%

Assessment of Functional Outcome Following Duhamel Retro-Rectal Pull-Through Surgery for Hirschsprung's Disease - A Follow-up Study

M.,

Maniam,

Dhanasekarapandian

et al. 2023

Journal of Coloproctology

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Context Hirschsprung's disease (HD) is one of the commonest problems requiring surgery in children. More than 95% of children present during new-born period, when they are treated with leveling colostomy and are followed with pull-through surgery a few months later, once the child has gained adequate weight to withstand a major surgery. The commonest pull through surgery done is the Duhamel retro-rectal pull-through (DRPT) repair. Settings and Design This is a retrospective study of children who presented to one unit in our institute, a tertiary care referral hospital for children less than 12 years, with HD and underwent DRPT procedure during the period between July 2017 to June 2020. The children were evaluated after three years of follow-up for fecal incontinence and constipation. The study was conducted in children diagnosed with classical segment recto-sigmoid HD who underwent surgery. The children who were diagnosed with HD other than classical segment, who underwent primary pull through surgery and who underwent other repairs for HD were excluded from the study. Results Thirty-two children underwent DRPT procedure during the study period. Of them, five (15.6%) children were lost on follow-up and one (3.1%) child had expired in the immediate post-operative period. Twenty-six children were included in the study. The bowel function score was calculated. The mean age of definitive surgery was 4.2 years. The follow-up period was a minimum of three years. Only two children had a “good” score of eighteen and above. Nineteen children had a “fair” score of 13–17. Five children had a “poor” score of less than thirteen, and among them, two had a “very poor” score of less than nine. The mean BFS was 13.72. Conclusions Functional outcomes following Duhamel procedure are satisfactory, with 7.7% of children are in the fringe of requiring another surgery for constipation and pseudo-incontinence.

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Long-term Outcomes of Patients Surgically Treated for Hirschsprung Disease

Cited by 9 publications

References 20 publications

Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

Long-term functional outcomes of patients with Hirschsprung disease following pull-through

Assessment of Functional Outcome Following Duhamel Retro-Rectal Pull-Through Surgery for Hirschsprung's Disease - A Follow-up Study

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