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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.)

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.)