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Objectives: Previous studies have shown rates of surgical resection of up to 41% in stricturing pediatric Crohn's disease (CD). In this retrospective multicenter study, our aims were to identify clinical risk factors and magnetic resonance enterography (MRE) features of small bowel strictures associated with surgery., Methods: Pediatric patients with symptomatic stricturing small bowel CD (defined as obstructive symptoms or proximal dilatation on MRE) confirmed by MRE between 2010 and 2020 were recruited from 12 French tertiary hospitals. Patient characteristics were compared by surgical outcome multivariable Cox regression., Results: Fifty-six patients (61% boys) aged 12.2 ± 2.7 years at diagnosis of CD were included. Median duration of CD before diagnosis of stricture was 11.7 months (interquartile range [IQR]: 25-75: 1.2-29.9). Nineteen (34%) patients had stricturing phenotype (B2) at baseline. Treatments received  before stricture diagnosis included MODULEN-IBD (n = 31), corticosteroids (n = 35), antibiotics (n = 10), anti-TNF (n = 27), immunosuppressants (n = 28). Thirty-six patients (64%) required surgery, within 4.8 months (IQR: 25-75: 1.8-17.3) after stricture diagnosis. Parameters associated with surgical resection were antibiotic exposure before stricture diagnosis (adjusted odds ratio [aOR]: 15.62 [3.35-72.73], p = 0.0005), Crohn's disease obstructive symptoms score (CDOS) > 4 (aOR: 3.04 [1.15-8.03], p = 0.02) and dilation proximal to stricture >28 mm (aOR: 3.62 [1.17-11.20], p = 0.03)., Conclusion: In this study, antibiotic treatment before stricture diagnosis, intensity of obstructive symptoms, and diameter of dilation proximal to small bowel stricture on MRE were associated with risk for surgical resection., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Ustekinumab is known to be efficient in adult patients suffering from moderate to severe Crohn disease (CD) and ulcerative colitis (UC) resistant to anti-tumor necrosis factor-alpha (TNF-α). Here, we described the clinical course of treatment with ustekinumab in French pediatric inflammatory bowel disease (IBD) patients treated with ustekinumab., Methods: This study includes all pediatric patients treated by ustekinumab injection for IBD (CD and UC), between January 2016 and December 2019., Results: Fifty-three patients were enrolled, 15 males and 38 females. Forty-eight patients (90%) had a diagnosis of CD and 5 (9.4%) had UC. Sixty-five percent of CD patients presented an ileocolitis. Perineal disease was observed in 20 out of 48 CD patients (41.7%), among them 9 were treated surgically. All patients included were resistant to anti-TNF-α treatment. Fifty-one percent had presented side effects linked to anti-TNF-α, including psoriasis and anaphylactic reaction. The average Pediatric Crohn Disease Activity Index (PCDAI) at induction was 28.7 (5-85), 18.7 (0-75) at 3 months of treatment and 10 (0-35) at the last follow-up. The average Pediatric Ulcerative Colitis Activity Index at induction was 47 (25-65), 25 (15-40) at 3 months of treatment and 18.3 (0-35) at the last follow-up. No severe side effects were observed., Conclusion: In this retrospective, multicentral study, ustekinumab proved to be efficient in pediatric patients resistant to anti-TNF-α. PCDAI has been significantly improved in patients with severe disease, treated with ustekinumab., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Introduction: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD., Methods: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale., Results: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication., Conclusion: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Crohn disease (CD) can affect patient's quality of life (QOL) with physical, social, and psychological impacts. This study aimed to investigate the QOL of children with CD and its relationship with patient and disease characteristics., Methods: Children ages from 10 to 17 years with diagnosed CD for more than 6 months were eligible to this cross-sectional study conducted in 35 French pediatric centers. QOL was assessed by the IMPACT-III questionnaire. Patient and disease characteristics were collected., Results: A total of 218 children (42% of girls) were included at a median age of 14 years (interquartile range [IQR]: 13--16). Median duration of CD was 3.2 years (IQR: 1.7-5.1) and 63% of children were in clinical remission assessed by wPCDAI. Total IMPACT-III score was 62.8 (±11.0). The lowest score was in "emotional functioning" subdomain (mean: 42.8 ± 11.2). Clinical remission was the main independent factor associated with QOL of children with CD (5.74 points higher compared with those "with active disease", 95% confidence interval [CI] 2.77--8.70, P < 0.001). Age of patient at the evaluation was found negatively correlated with QOL (-0.76 per year, 95% CI: -1.47 to -0.06, P = 0.009). Presence of psychological disorders was associated with a lower QOL (-9.6 points lower to those without, 95% CI: -13.34 to -5.86, P < 0.0001). Total IMPACT-III and its subdomains scores were not related to sex, disease duration, or treatments., Conclusions: These results not only confirm that clinical remission is a major issue for the QOL of patients, but also highlights the importance of psychological care., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: This multicentric study aimed to evaluate the quality of life (QOL) in children with Hirschsprung's disease (HD)., Methods: HD patients aged from 6 to 18 years and followed-up in 2 French pediatric surgery centers were included in this study. QOL was assessed using the HAQL questionnaires according to age (6-11 and 12-18), filled by patients and their parents (proxy reports) and correlated with initial disease characteristics, nutritional status, and functional score of Krickenbeck., Results: Sixty-three patients were included. The acquisition of satisfactory voluntary bowel movements was found in only 50% of the 6 to 11 years old and 68% of the teenagers. Seventy percentage of the children and 55% of teenagers had soiling issues. The overall HAQLproxy6--11 score was 528/700; best scores were found for "fecal continence" (94/100), "social functioning" (94/100), and "urinary continence" (92/100) whereas the worst scores were for "general well-being" (64/100) and "diurnal fecal continence" (58/100). The overall HAQLproxy12--16 score was 607/700; best scores were for "urinary continence" (96/100) and "social functioning" (93/100). In a multivariate analysis, soiling was the only factor significantly associated with low QOL (P = 0.03)., Conclusions: Soiling remains frequent in children operated on for HD and negatively affects their QOL. Assessment and treatment of soiling should be the priority for medical teams in the follow-up of these children.

Background and Objective: Eosinophilic esophagitis (EoE) is an increasingly recognized childhood disease. Esophageal atresia (EA) is the most frequent congenital malformation of the esophagus. Recently, cases of EoE occurring in patients with EA have been reported, although the exact prevalence of EoE in EA remains unknown. The aim is to investigate the prevalence of EoE among EA in adolescents and to describe these patients' characteristics., Methods: Systematic upper gastrointestinal endoscopies with multistage esophageal biopsies were prospectively performed in 63 adolescents with EA. A standardized form was used to collect clinical and endoscopic data. Diagnosis of EoE was made as ≥15 intraepithelial eosinophils/high power field, whatever the response on proton pump inhibitors therapy., Results: Six patients (9.5%) presented an EoE (17-100 eosinophils/high power field). An atopic condition was reported more frequently in the eosinophil ≥15 group than in patients with no EoE (66% vs 16%; P = 0.014). Except for chest pain, symptoms and endoscopic features were similar in patients with EoE and patients with no EoE., Conclusion: In our series of 63 patients born with EA, mainly distal tracheoesophageal fistula, the prevalence of EoE is increased, and therefore should be considered in adolescents with EA.

Background and Aims: An expanding number of monogenic defects have been identified as causative of severe forms of very early-onset inflammatory bowel diseases [VEO-IBD]. The present study aimed at defining how next-generation sequencing [NGS] methods can be used to improve identification of known molecular diagnosis and to adapt treatment., Methods: A total of 207 children were recruited in 45 paediatric centres through an international collaborative network [ESPGHAN GENIUS working group] with a clinical presentation of severe VEO-IBD [n = 185] or an anamnesis suggestive of a monogenic disorder [n = 22]. Patients were divided at inclusion into three phenotypic subsets: predominantly small bowel inflammation, colitis with perianal lesions, and colitis only. Methods to obtain molecular diagnosis included functional tests followed by specific Sanger sequencing, custom-made targeted NGS, and in selected cases whole exome sequencing [WES] of parents-child trios. Genetic findings were validated clinically and/or functionally., Results: Molecular diagnosis was achieved in 66/207 children [32%]: 61% with small bowel inflammation, 39% with colitis and perianal lesions, and 18% with colitis only. Targeted NGS pinpointed gene mutations causative of atypical presentations, and identified large exonic copy number variations previously missed by WES., Conclusions: Our results lead us to propose an optimised diagnostic strategy to identify known monogenic causes of severe IBD., (© The Author(s) 2018. Published by Oxford University Press on behalf of European Crohn’s and Colitis Organisation.)

Unlabelled: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients. Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy., Conclusion: Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence., What Is Known: • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.

Objective: This study evaluated the Trap-door button use (Cook Medical, Bloomington, IL) for antegrade enemas in children., Methods: Since 2002, patients with fecal incontinence or encopresis and constipation underwent percutaneous cecostomy under laparoscopy using a button. Technical details are described. Age at surgery, operative time, hospital stay, diagnosis, indications for cecostomy, and duration of follow-up were recorded. A survey was proposed via a questionnaire that was sent to the patients. Patients wearing the button for less than 1 month were excluded from this evaluation. The survey concerned volume and frequency of enemas, difficulties encountered, benefits and disadvantages of this method, and assessment of the antegrade enemas in continence., Results: Twenty-nine patients, 18 males and 11 females, aged 3 to 21 years (mean, 8.5 years) underwent laparoscopic Trap-door button placement. The indications for all the patients were intractable fecal incontinence in 24 cases and constipation with encopresis in 5 cases. Incontinence was because of myelomeningocele (n = 10), anorectal malformations (n = 11), caudal regression syndrome (n = 1), 22q11 syndrome (n= 1), and Hirschsprung disease with encephalopathy with convulsions (n = 1). Constipation with encopresis was because of sacrococcygeal teratoma (n = 1), cerebral palsy (n = 1), and acquired megarectum with psychiatric and social disorders (n = 3). A total of 26 cecostomy button placements and 3 sigmoidostomy button placements were successful with no intraoperative complication. The mean operative time was 25 minutes (10-40 minutes), and the hospital stay was 2.5 days (1-4 days). Twenty-two parents or patients answered the questionnaire. At the time of this survey, 2 patients had improved their fecal continence and had had the button removed. A mean of 4 weekly enemas was enough to improve fecal continence troubles (range, 1 daily to 1 for 2 weeks). The volume for enemas was 250 to 1000 mL (mean, 700 mL). The time required for the irrigation of the bowel by gravity took from 5 to 60 minutes (mean, 25 minutes) for 20 patients. Before surgery, 14 patients needed a diaper, day and night, and 6 needed sanitary protection. Soiling was a very significant inconvenience for all the patients. After surgery, only 5 patients needed a diaper (cerebral palsy, 22q11, cloacal malformation, myelomeningocele, bladder exstrophy) because of moderate results or urinary incontinence and continued soiling. Patients were asked to give an assessment (null = 0, bad = 1, fair = 2, good = 3, very good = 4). None of the patients felt there had been no changes or a bad result. There were 5 patients who felt they had an average result, 5 a good result, and 12 a very good result. The mean grade was 3.44 (17.2/20). A total of 3 patients had hypertrophic granulation tissue formation around the cecostomy button, and 12 had tiny leakage., Conclusion: Percutaneous placement of a cecostomy button under laparoscopic control is an easy and major complication-free procedure. The use of the Trap-door device by the patients or with the help of the parents for antegrade enemas is effective and satisfactory. It improves the quality of life and is reversible.