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Objectives: Hirschsprung disease (HD) requires surgical resection of affected bowel, but the current evidence is inconclusive regarding the optimal age for resection. The aim of this study was to assess whether age at resection of the aganglionic segment is a determinant for surgical outcomes., Methods: A cross-sectional cohort study was done including all consecutive patients with HD between 1957 and 2015, aged 8 years or older (n = 830), who were treated in 1 of the 6 pediatric surgical centers in the Netherlands. Outcome measures were mortality, postoperative complications, stoma rate and redo surgery rate, retrieved from the medical records. Additionally, constipation and fecal incontinence rate in long term were assessed with the Defecation and Continence Questionnaire (DeFeC and P-DeFeC)., Results: The medical records of 830 patients were reviewed, and 346 of the 619 eligible patients responded to the follow-up questionnaires (56%). There was a small increase in the risk of a permanent stoma [odds ratio (OR) 1.01 (95% confidence interval {CI}: 1.00-1.02); P = 0.019] and a temporary stoma [OR 1.01 (95% CI: 1.00-1.01); P = 0.022] with increasing age at surgery, regardless of the length of the aganglionic segment and operation technique. Both adjusted and unadjusted for operation technique, length of disease, and temporary stoma, age at surgery was not associated with the probability and the severity of constipation and fecal incontinence in long term., Conclusions: In this study, we found no evidence that the age at surgery influences surgical outcomes, thus no optimal timing for surgery for HD could be determined., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Transanal endorectal pull-through (TERPT) is a common surgical procedure in Hirschsprung disease (HD). Aim of this study was to gain insight in the prevalence and severity of postoperative complications within 30-days after TERPT and to identify patient and perioperative characteristics, associated with the development of short-term postoperative complications., Methods: This study retrospectively analyzed data of children with HD and treated with TERPT in our center between 2005 and 2020. Complications emerging within 30-days after surgery were assessed using Clavien-Dindo (CD). Patient and perioperative characteristic as predictor of a complication were tested using (multivariable) logistic regression analysis., Results: Twenty-two of 106 (21%) included patients (17 transanal only; 77 laparoscopic-assisted; 12 laparotomy-assisted) developed 35 complications, including two patients (1.8%) that deceased. We suspect postoperative rectal irrigation leading to perforation as cause of death in both patients. Six patients (6%) had a minor (CD<3) and 16 patients (15%) a major (CD≥3) complication. Anastomotic leakage (n = 4, 11%), abdominal abscess (n = 3, 9%) and anastomotic stricture (n = 3,9%) occurred most frequently. Predictive factors for developing a complication were older age at time of surgery (OR 1.03 1.00-1.01, p = 0.041), laparotomy-assisted surgery (OR 12.65, CI 1.712-93.07, p = 0.013) and long-segment HD (OR 4.09 CI 1.09-15.39, p = 0.037)., Conclusions: We found a CD-graded short-term postoperative complication rate of 21% following TERPT, reporting anastomotic complications most frequently. In patients at risk a diverting stoma should be considered. We suspect postoperative rectal irrigation being the cause of two lethal perforations. Therefore, we recommend to place a rectal transanastomotic tube in all patients receiving TERPT., Level of Evidence: Level III., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Patients with Hirschsprung disease (HD) are at risk of persistent constipation, fecal incontinence or recurrent enterocolitis after surgical treatment, which in turn may impact physical and psychosocial functioning. Generic health-related quality of life (HRQoL) and disease-specific health-related quality of life are relevant outcome measures to assess the impact of HD on the QoL of these patients., Aim: To summarize all available evidence on HRQoL of patients with HD after surgery and the impact of possible moderating factors., Methods: Pubmed, Web of Sciences, PsycInfo and Embase were searched with search terms related to 'Hirschsprung disease', 'Pediatrics' and 'Quality of life'. Mean and standard deviation of generic HRQoL overall and domain scores were extracted from each study, as well as data describing potential factors associated with QoL. Random effect models were used for meta-analytic aggregation of generic HRQoL scores. Meta-regression was used to assess the relationship between patient and clinical characteristics and generic HRQoL. Disease-specific HRQoL outcomes of patients with HD were systematically reviewed., Results: Seventeen articles were included in the systematic review ( n = 1137 patients) and 15 in the quantitative meta-analysis ( n = 1024 patients). Four studies reported disease-specific HRQoL. Patient's age ranged between 0 and 21 years. Meta-analytic aggregation showed a non-significantly impaired generic HRQoL ( d = -0.168 [95%CI: -0.481; 0.145], P = 0.293, I 2 = 94.9) in patients with HD compared to healthy controls. Physical ( d = -0.042 [95%CI: -0.419; 0.335], P = 0.829, I 2 = 95.1), psychosocial ( d = -0.159 [95%CI: -0.458; 0.141], P = 0.299, I 2 = 93.6) and social HRQoL ( d = -0.092 [95%CI: -0.642; 0.457], P = 0.742, I² = 92.3) were also not significantly lower compared to healthy controls. There was no relation between health-related outcomes and the sex of the patients and whether generic HRQoL was measured by parental proxy or self-report. Disease-specific complaints of patients with HD impaired physical HRQoL, but not psychosocial and social HRQoL., Conclusion: In this systematic review and meta-analysis, no evidence was found for impaired generic HRQoL in patients with HD compared to healthy controls, neither for moderating effects of sex, parental proxy or self-report., Competing Interests: Conflict-of-interest statement: All authors have no conflict of interest to disclose., (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.)

Introduction: Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of pre- and postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval., Material and Methods: A retrospective cohort study was performed with all 146 HD patients treated between 2000 and 2017. Data were retrieved from the medical records. HAEC was defined as presence of clinical signs of bowel inflammation, that required treatment with intravenous antibiotics and admittance to the hospital during at least two days. To identify risk factor for HAEC, patients with and without a history of postoperative HAEC were compared. Kaplan-Meier and Cox-regression were used to assess HAEC free intervals before and after surgery., Results: Out of 146 patients, 12 patients had pre-operative HAEC (8%) and 31 patients had postoperative HAEC (21%). Median preoperative HAEC free interval was 112 days (IQR 182 days). Length of hospital stay due to readmissions was longer for patients with a history of postoperative HAEC compared to patients without a history of postoperative HAEC (9.5 vs 16 days, U = 1872.5, p = 0.047). Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up., Conclusions: HAEC incidence was relatively low in our population. No patient or clinical characteristics were associated with the risk of postoperative HAEC., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Patients with Hirschsprung disease may have obstructive symptoms after resection of the aganglionic segment. Botulinum toxin (BT) injections can help improve faecal passage by relaxing the internal anal sphincter. This study assess effect of BT injections and aims to identify factors associated with receiving BT injections and favourable response to the first BT injection., Methods: A retrospective study was performed in a cohort of consecutive patients treated for Hirschsprung disease in our centre between 2003 and 2017. The indication for BT injections was obstructive defecation problems that were non-responsive to high-dose laxatives or rectal irrigation, or an episode of Hirschsprung-associated enterocolitis (HAEC). Effectiveness of BT injections was measured in terms of clinical improvement. Relationships between factors associated with receiving BT injections and with response to the first BT injection were tested with group comparison and logistic regression., Results: Forty-one out of 131 patients received BT injections (31%) with a median of two injections (range 1-11). All patients had obstructive defecation problems non-responsive to high-dose laxatives or rectal irrigation, two patient also had an episode of HAEC. Twenty-five out of 41 patients (61%) had clinical improvement after first injection. In 29 of the 41 patients (71%) spontaneous defecation or treatment with laxatives only was achieved. Adverse effects were seen in 12 out of 41 patients (29%) after 14 injections (16%), and consisted of anal pain, temporary loss of stools and dermatitis. Patients who received BT injections more often had long segment disease, more often required laxatives or rectal irrigation and had longer length of hospital stay, both after corrective surgery and in follow-up. None of the tested factors was associated with clinical improvement after first BT injection., Conclusion: Our findings show that BT injections effectively treat obstructive defecation problems in the majority of patients with Hirschsprung disease with mild adverse effects., Level of Evidence: Level III., (Copyright © 2020. Published by Elsevier Inc.)

Purpose: Patients with Hirschsprung disease (HD) can have persistent obstructive symptoms after resection of the aganglionic segment. If obstructive symptoms are treated inadequately, this may lead to recurrent faecal stasis and impaction, and may result in severe distension of the bowel. A permanently distended bowel which not responds to conservative treatment may be an indication for redo surgery. The aim of this study is to describe our experiences and the short-term results of a novel technique: longitudinal antimesenteric resection with a longitudinal anastomosis., Methods: We reviewed the medical records of our three patients who underwent longitudinal resection of severe distended bowel. This technique aims to improve defecation by improving faecal passage and is characterized by resection of the antimesenteric side of the distended intestinal segment, followed by plication with a longitudinal anastomosis. In this paper, this novel technique is described in detail, as well as short-term outcomes., Results: All patients had an uneventful recovery after longitudinal antimesenteric resection. During follow-up, the functional outcomes were excellent, with a large improvement of bowel function. All patients were continent for faeces, and treated with low-dose laxatives or occasional preventive irrigation in one patient. There were no more complaints of persistent constipation or soiling., Conclusion: Longitudinal resection is a surgical redo-procedure offering large benefits for patients with Hirschsprung disease with distended bowel after primary surgery.

Background: A large proportion of patients with Hirschsprung disease experience persistent obstructive symptoms after corrective surgery. Persistent obstructive symptoms may result in faecal stasis that can develop into Hirschsprung-associated enterocolitis, a potential life-threatening condition. Important treatment to improve faecal passage is internal anal sphincter relaxation using botulinum toxin injections., Aim: To give an overview of all empirical evidence on the effectiveness of botulinum toxin injections in patients with Hirschsprung disease., Methods: A systematic review and meta-analysis was done by searching PubMed, EMBASE and the Cochrane Library, using entry terms related to: (1) Hirschsprung disease; and (2) Botulinum toxin injections. 14 studies representing 278 patients met eligibility criteria. Data that were extracted were proportion of patients with improvement of obstructive symptoms or less enterocolitis after injection, proportion of patients with adverse effects and data on type botulinum toxin, mean dose, average age at first injection and patients with associated syndromes. Random-effects meta-analysis was used to aggregate effects and random-effects meta-regression was used to test for possible confounding factors., Results: Botulinum toxin injections are effective in treating obstructive symptoms in on average 66% of patients [event rate (ER) = 0.66, P = 0.004, I 2 = 49.5, n = 278 patients]. Type of botulinum toxin, average dose, average age at first injections and proportion of patients with associated syndromes were not predictive for this effect. Mean 7 duration of improvement after one botulinum toxin injections was 6.4 mo and patients needed on average 2.6 procedures. There was a significant higher response rate within one month after botulinum toxin injections compared to more than one month after Botulinum toxin injections (ER = 0.79, vs ER = 0.46, Q = 19.37, P < 0.001). Botulinum toxin injections were not effective in treating enterocolitis (ER 0.58, P = 0.65, I 2 = 71.0, n = 52 patients). There were adverse effects in on average 17% of patients (ER = 0.17, P < 0.001, I 2 = 52.1, n = 187 patients), varying from temporary incontinence to mild anal pain., Conclusion: Findings from this systematic review and meta-analysis indicate that botulinum toxin injections are effective in treating obstructive symptoms and that adverse effects were present, but mild and temporary., Competing Interests: Conflict-of-interest statement: All authors have no conflicts of interest to disclose.

Aim: Total colonic aganglionosis (TCA) is a severe form of Hirschsprung's disease (HD) associated with a high morbidity. This study assessed long-term functional outcome and quality of life (QoL) of patients with TCA in a national consecutive cohort., Methods: Surgical and demographic characteristics in the medical records of all patients (n = 53) diagnosed with TCA between 1995 and 2015 were reviewed. Functional outcome of all nonsyndromal patients, aged ≥ 4 years (n = 35), was assessed using a questionnaire and in medical records. Generic and disease-specific QoL were assessed using standardized validated questionnaires., Results: Of 35 patients eligible for follow-up, 18 (51%) responded to the questionnaires. They were aged 4-19 years. A Duhamel procedure was performed in 67% of these patients and a Rehbein procedure was performed in 33%. In the questionnaire, 65% of the patients reported constipation, 47% faecal incontinence and 53% soiling. Moreover, 18% of patients used bowel management (flushing or laxatives) and 29% had an adapted diet only. Children and adolescents with TCA had worse perception of their general health and were more limited by bodily pain and discomfort compared with healthy peers. Their quality of life is influenced most by frequent complaints of diarrhoea and other physical symptoms., Conclusion: Children and adolescents with TCA report lower health-related QoL compared with healthy peers, especially in the physical domain. We suggest standardized follow-up and prospective longitudinal future research on functionality and QoL of these patients., (Colorectal Disease © 2018 The Association of Coloproctology of Great Britain and Ireland.)