OBJECTIVES:Inflammatory bowel disease (IBD) and chronic nonbacterial osteomyelitis (CNO) is a rare association. Its prevalence is probably underestimated due to low awareness among clinicians. The aim of this study was to describe the French pediatric national cohort and to estimate the incidence of CNO among IBD. METHODS:We retrospectively collected all reports of IBD-CNO association from pediatric gastroenterological and rheumatologic units between 2000 and 2024. We analyzed demographic data; age of the onset; IBD type; CNO localizations; dermatological lesions; explorations results; medications; follow-up. Incidence was estimated using French regional inception population-based cohort EPIMAD data. RESULTS:Thirty-seven patients were identified. Median age at the onset was 13.5 years (interquartile range [IQR] 11.5-14.5). CNO diagnosis preceded IBD in 11 cases, followed it in 13 cases, while 13 IBD-CNO cases were diagnosed simultaneously (median interval 1 year). IBD included 25 Crohn's disease, 10 ulcerative colitis, 2 IBD-unclassified. Bone localizations predominated in lower limbs and pelvis (75.3%). Fourteen children (37.8%) had dermatological lesions. At last follow-up (median 3 years, IQR 1.25-4.75), 28 patients (75.7%) were in IBD-CNO remission, having mostly anti-tumor necrosis factor (TNF)-α (p = 0.041, 82.1% remission, 44.4% relapse) in monotherapy (60.7%). We estimate the incidence of this association among French pediatric IBD at 1 case for 20,000 patients-year. CONCLUSION:This study describes a large national cohort of pediatric IBD-CNO association. Gastroenterologist should request a whole body-magnetic resonance imaging when IBD patients present chronic bone pain. Multidisciplinary management involving rheumatologists and dermatologists is recommended. Most patients responded to anti-TNF-α antibodies, which could avoid bone complications.

OBJECTIVES:Primary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein-losing enteropathy. There is little published data about treatments efficacy and outcomes. Our main objective was to describe the clinical profile, response to therapy, and outcomes of children with PIL. METHODS:We conducted a national retrospective study including children with PIL followed in French university hospitals between 2010 and 2022. Response to treatment was defined as clinical remission and no need for albumin infusion. Response was considered complete if albuminemia was normal (>35 g/l) during follow-up and partial if less than 35 g/l. RESULTS:Thirty-four children (22 males) were included; median age at diagnosis was 7 (3-29,5) months. The median follow-up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty-one patients received a low long-chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second-line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (p  < 0.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow-up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet. CONCLUSIONS:Most children with PIL responded to diet therapy and about a quarter of the cohort had a good prognosis with complete remission even after discontinuation of the diet. Presence of lymphedema or a genetic variant was associated with a chronic condition.

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.

Although central venous catheter (CVC)-related thrombosis (CRT) is a severe complication of home parenteral nutrition (HPN), the amount and quality of data in the diagnosis and management of CRT remain low. We aimed to describe current practices regarding CVC management in French adult and pediatric HPN centers, with a focus on CVC obstruction and CRT. Current practices regarding CVC management in patients on HPN were collected by an online-based cross-sectional survey sent to expert physicians of French HPN centers. We compared these practices to published guidelines and searched for differences between pediatric and adult HPN centers’ practices. Finally, we examined the heterogeneity of practices in both pediatric and adult HPN centers. The survey was completed by 34 centers, including 21 pediatric and 13 adult centers. We found a considerable heterogeneity, especially in the responses of pediatric centers. On some points, the centers’ responses differed from the current guidelines. We also found significant differences between practices in adult and pediatric centers. We conclude that the management of CVC and CRT in patients on HPN is a serious and complex situation for which there is significant heterogeneity between HPN centers. These findings highlight the need for more well-designed clinical trials in this field.

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. This study of patients from the French national network oesophageal atresia (OA) registry used a standardised questionnaire to investigate the frequency of feeding disorders. These were diagnosed in 42%, of whom over a third had failed to thrive. The authors advocate systematic feeding assessment in follow up of children operated on for OA.

Introduction The aims of this retrospective multicenter study were to assess the technical success and adverse events of endoscopic retrograde cholangiopancreatography (ERCP) procedures in children in French and Belgian centers. Methods All children aged 1 day to 17 years who underwent ERCP between January 2008 and March 2019 in 15 tertiary care hospitals were retrospectively included. Results 271 children underwent 470 ERCP procedures. Clinical long-term follow-up was available for 72 % of our patients (340/470 procedures). The median age at intervention was 10.9 years. ERCP was therapeutic in 90 % (423/470) and diagnostic in cases of neonatal cholestasis in 10 % of the patients. The most common biliary indication was choledocholithiasis; the most common pancreatic indication was chronic pancreatitis. Biliary cannulation was successful in 92 % of cases (270/294); pancreatic cannulation in 96 % of cases (169/176); and planned therapeutic procedures in 92 % of cases (388/423). The overall complication rate was 19 % (65/340). The most common complication was post-ERCP pancreatitis (PEP) in 12 % of cases (40/340) and sepsis in 5 % (18/340). On univariate analyses, pancreatic stent removal was protective against PEP (odds ratio [OR] 0.1, 95 % confidence interval [CI] 0.01 – 0.75; P = 0.03), and sepsis was associated with history of liver transplantation (OR 7.27, 95 %CI 1.7 – 31.05; P = 0.01). Five patients had post-ERCP hemorrhage and two had intestinal perforation. All complications were managed with supportive medical care. There was no procedure-related mortality. Conclusion Our cohort demonstrates that ERCP can be performed safely with high success rates in many pancreaticobiliary diseases of children. The rate of adverse events was similar to that in previous reports.

Objectives: The use of semielemental diets concerns a small proportion of children on enteral nutrition whose characteristics have never been reported. Our aim was to describe a cohort of patients on home enteral nutrition with Peptamen Junior, including the tolerance and nutritional efficacy of this product. Methods: We performed a retrospective multicenter survey on a cohort of patients receiving this semielemental diet at home between 2010 and 2015 in 14 tertiary pediatric French centers. We recorded at baseline, 3, 6, and 12 months, and then every year the anthropometric characteristics of the patients, indications and modalities of administration of the diet, and the tolerance and adverse events. Results: We recruited 136 patients ages 9.8 +/- 4.4 years at baseline. Mean body mass index z score was -1.0 +/- 1.8; mean height z score was -1.1 +/- 1.9. The main underlying diseases were digestive (35.3%), neurological (33.1%), and hematological (19.9%). The indications for a semielemental diet were failure of another diet in 70 patients (51.9%), severe malnutrition in 19 (14.1%), cystic fibrosis in 11 (8.1%), and switch from parenteral nutrition in 11 (8.1%). Side effects were observed in 39.2% of the patients, and required medical attention in 8.2%. Body mass index improved or remained normal in 88.3% of children. Conclusions: This semielemental diet seems to be well tolerated and efficient in the setting of home enteral nutrition in children with complex diseases featuring malabsorption and/or after failure of polymeric diet.