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Background: Congenital biliary dilatation (CBD) is a congenital malformation of the main biliary tract usually associated with the pancreatobiliary maljunction (PBM), determining stone formation, cholangitis, pancreatitis, and cholangiocarcinoma. The role of endoscopic retrograde cholangiopancreatography (ERCP) in treatment and diagnosis has not been established yet. Therefore, the aim of our study is to define the actual role of ERCP in children with CBD., Methods: A retrospective review of consecutive patients with congenital biliary dilatation undergoing preoperative ERCP and subsequent surgical treatment at our pediatric tertiary referral center (Endoscopy and Digestive Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy) was performed between 2012 and 2023., Results: A total of 31 patients were included in the present study. Preoperative ERCP detected a PBM in 28 patients (90%). According to Todani's classification, 2 patients (6.5%) had choledochal cyst (CC) type IV, and 29 patients (93.5%) were diagnosed with CC type I. In 18 (58%) patients, ERCP was performed for treating acute pancreatitis. Sphincterotomy could be performed in 23 of 31 (74%) patients. Patients who did not undergo sphincterotomy had a higher number of acute episodes while awaiting surgery., Conclusions: The present study is supportive of an essential role of ERCP in the diagnostic and preoperative management of children with CBD with acute presentation or inconclusive magnetic resonance cholangiopancreatography findings., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2025 Rollo, Balassone, Faraci, Torroni, Dall'Oglio, De Angelis and Caldaro.)

Phosphatase and tensin homolog ( PTEN ) hamartoma tumor syndrome (PHTS) is a cancer predisposition syndrome characterized by an increased risk of developing benign and malignant tumors, caused by germline pathogenic variants of the PTEN tumour suppressor gene. PTEN gene variants often present in childhood with macrocephaly, developmental delay, and/or autism spectrum disorder while tumors and intestinal polyps are commonly detected in adults. PHTS is rarely associated with childhood brain tumors with only two reported cases of medulloblastoma (MB). We report the exceptional case of an infant carrying a germline and somatic pathogenic variant of PTEN and a germline and somatic pathogenic variant of CHEK2 who developed a MB SHH in addition to intestinal polyposis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Caroleo, Rotulo, Agolini, Macchiaiolo, Boccuto, Antonelli, Colafati, Cacchione, Megaro, Carai, De Ioris, Lodi, Tornesello, Simone, Torroni, Cinalli and Mastronuzzi.)

Current surgical options for patients requiring esophageal replacement suffer from several limitations and do not assure a satisfactory quality of life. Tissue engineering techniques for the creation of customized "self-developing" esophageal substitutes, which are obtained by seeding autologous cells on artificial or natural scaffolds, allow simplifying surgical procedures and achieving good clinical outcomes. In this context, an appealing approach is based on the exploitation of decellularized tissues as biological matrices to be colonized by the appropriate cell types to regenerate the desired organs. With specific regard to the esophagus, the presence of a thick connective texture in the decellularized scaffold hampers an adequate penetration and spatial distribution of cells. In the present work, the Quantum Molecular Resonance ® (QMR) technology was used to create a regular microchannel structure inside the connective tissue of full-thickness decellularized tubular porcine esophagi to facilitate a diffuse and uniform spreading of seeded mesenchymal stromal cells within the scaffold. Esophageal samples were thoroughly characterized before and after decellularization and microperforation in terms of residual DNA content, matrix composition, structure and biomechanical features. The scaffold was seeded with mesenchymal stromal cells under dynamic conditions, to assess the ability to be repopulated before its implantation in a large animal model. At the end of the procedure, they resemble the original esophagus, preserving the characteristic multilayer composition and maintaining biomechanical properties adequate for surgery. After the sacrifice we had histological and immunohistochemical evidence of the full-thickness regeneration of the esophageal wall, resembling the native organ. These results suggest the QMR microperforated decellularized esophageal scaffold as a promising device for esophagus regeneration in patients needing esophageal substitution., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Marzaro, Pozzato, Tedesco, Algeri, Pozzato, Tomao, Montano, Torroni, Balassone, Contini, Guerra, D’Angelo, Federici di Abriola, Lupoi, Caristo, Boškoski, Costamagna, Francalanci, Astori, Bozza, Bagno, Todesco, Trovalusci, Oglio, Locatelli and Caldaro.)

Improved surgical techniques, as well as preoperative and postoperative care, have dramatically changed survival of children with esophageal atresia (EA) over the last decades. Nowadays, we are increasingly seeing EA patients experiencing significant short- and long-term gastrointestinal morbidities. Anastomotic stricture (AS) is the most common complication following operative repair. An esophageal stricture is defined as an intrinsic luminal narrowing in a clinically symptomatic patient, but no symptoms are sensitive or specific enough to diagnose an AS. This review aims to provide a comprehensive view of AS in EA children. Given the lack of evidence-based data, we critically analyzed significant studies on children and adults, including comments on benign strictures with other etiologies. Despite there is no consensus about the goal of the luminal diameter based on the patient's age, esophageal contrast study, and/or endoscopy are recommended to assess the degree of the narrowing. A high variability in incidence of ASs is reported in literature, depending on different definitions of AS and on a great number of pre-, intra-, and postoperative risk factor influencing the anastomosis outcome. The presence of a long gap between the two esophageal ends, with consequent anastomotic tension, is determinant for stricture formation and its response to treatment. The cornerstone of treatment is endoscopic dilation, whose primary aims are to achieve symptom relief, allow age-appropriate capacity for oral feeding, and reduce the risk of pulmonary aspiration. No clear advantage of either balloon or bougie dilator has been demonstrated; therefore, the choice is based on operator experience and comfort with the equipment. Retrospective evidences suggest that selective dilatations (performed only in symptomatic patients) results in significantly less number of dilatation sessions than routine dilations (performed to prevent symptoms) with equal long-term outcomes. The response to dilation treatment is variable, and some patients may experience recurrent and refractory ASs. Adjunctive treatments have been used, including local injection of steroids, topical application of mitomycin C, and esophageal stenting, but long-term studies are needed to prove their efficacy and safety. Stricture resection or esophageal replacement with an interposition graft remains options for AS refractory to conservative treatments.

There are many causes of gastrointestinal bleeding (GIB) in children, and this condition is not rare, having a reported incidence of 6.4%. Causes vary with age, but show considerable overlap; moreover, while many of the causes in the pediatric population are similar to those in adults, some lesions are unique to children. The diagnostic approach for pediatric GIB includes definition of the etiology, localization of the bleeding site and determination of the severity of bleeding; timely and accurate diagnosis is necessary to reduce morbidity and mortality. To assist medical care providers in the evaluation and management of children with GIB, the "Gastro-Ped Bleed Team" of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) carried out a systematic search on MEDLINE via PubMed (http://www.ncbi.nlm.nih.gov/pubmed/) to identify all articles published in English from January 1990 to 2016; the following key words were used to conduct the electronic search: "upper GIB" and "pediatric" [all fields]; "lower GIB" and "pediatric" [all fields]; "obscure GIB" and "pediatric" [all fields]; "GIB" and "endoscopy" [all fields]; "GIB" and "therapy" [all fields]. The identified publications included articles describing randomized controlled trials, reviews, case reports, cohort studies, case-control studies and observational studies. References from the pertinent articles were also reviewed. This paper expresses a position statement of SIGENP that can have an immediate impact on clinical practice and for which sufficient evidence is not available in literature. The experts participating in this effort were selected according to their expertise and professional qualifications., Competing Interests: Conflict-of-interest statement: All authors declare that they have no conflict of interest. The data presented, the statements made and the views expressed are solely the responsibility of the authors.

Post-esophageal atresia anastomotic strictures and post-corrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures (ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the stricture's etiology, the availability of different tools and the operator's experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids (either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse, even if such relapses occur following the execution of well-conducted conservative strategies, surgical stricture resection and anastomosis or esophageal substitution are the only remaining options.

Aim: To assess the usefulness of the balloon assisted enteroscopy in preventing surgical intervention in patients with Peutz-Jeghers syndrome (PJS) having a small bowel large polyps., Methods: Seven consecutive asymptomatic pts (age 15-38 years) with PJS have been collected; six underwent polypectomy using single balloon enteroscopy (Olympus SIF Q180) with antegrade approach using push and pull technique. SBE system consists of the SIF-Q180 enteroscope, an overtube balloon control unit (OBCU Olympus Balloon Control Unit) and a disposable silicone splinting tube with balloon (ST-SB1). All procedures were performed under general anesthesia. Previously all pts received wireless capsule endoscopy (WCE). Prophylactic polypectomy was reserved mainly in pts who had polyps > 15 mm in diameter. The balloon is inflated and deflated by a balloon control unit with a safety pressure setting range from -6.0 kPa to +5.4 kPa. Informed consent has been obtained from pts or parents for each procedure., Results: Six pts underwent polypectomy of small bowel polyps; in 5 pts a large polyp > 15 mm (range 20-50 mm in diameter) was resected; in 1 patient with WCE negative, SBE was performed for previous surgical resection of gastrointestinal stromal tumors. In 2 pts endoscopic clips were placed due to a polypectomy. No surgical complication have been reported. SBE with resection of small bowel large polyps in PJS pts was useful to avoid gastrointestinal bleeding and emergency laparotomy due to intestinal intussusceptions. No gastrointestinal tumors were found in subsequent enteroscopic surveillance in all seven pts. In order surveillance, all pts received WCE, upper endoscopy, ileocolonoscopy every 2 years. No pts had extraintestinal malignant lesions. SBE was performed when WCE was positive for significant polyps (> 15 mm)., Conclusion: The effective of prophylactic polypectomy of small bowel large polyps (> 15 mm) could be the first line treatment for conservative approach in management of PJS patients.

Pancreatic pseudocysts (PP) arise from trauma and pancreatitis; endoscopic gastro-cyst drainage (EGCD) under endoscopic ultrasonography (EUS) in symptomatic PP is the treatment of choice. Miniprobe EUS (MEUS) allows EGCD in children. We report our experience on MEUS-EGCD in PP, reviewing 13 patients (12 children; male:female = 9:3; mean age: 10 years, 4 mo; one 27 years, malnourished male Belardinelli-syndrome; PP: 10 post-pancreatitis, 3 post-traumatic). All patients underwent ultrasonography, computed tomography and magnetic resonance imaging. Conservative treatment was the first option. MEUS EGCD was indicated for retrogastric cysts larger than 5 cm, diameter increase, symptoms or infection. EGCD (stent and/or nasogastrocystic tube) was performed after MEUS (20-MHz-miniprobe) identification of place for diathermy puncture and wire insertion. In 8 cases (61.5%), there was PP disappearance; one, surgical duodenotomy and marsupialization of retro-duodenal PP. In 4 cases (31%), there was successful MEUS-EGCD; stent removal after 3 mo. No complications and no PP relapse in 4 years of mean follow-up. MEUS EGCD represents an option for PP, allowing a safe and effective procedure.

The following recommendations for management of caustic and foreign body ingestion in children have been developed following a multicentre study performed by the Italian Society for Paediatric Gastroenterology, Hepatology and Nutrition (SIGENP). They are principally addressed to medical professionals involved in casualty. Because there is paucity of good quality clinical trials in children on this topic, many of the recommendations are currently extrapolated from adult experiences or based on experts opinions. The document represents a level 2 to 5 degree of evidence (according to the Oxford Centre for Evidence-based Medicine Levels of Evidence), gathered from clinical experience, recent studies, and expert reports discussed during a consensus conference of the Endoscopic Section of the Italian Society of Paediatric Gastroenterology Hepatology and Nutrition. This working group comprises paediatricians, endoscopists, paediatric surgeons, toxicologists, and ENT surgeons, who are all actively involved in the management of these children. Recommendations are intended to serve as an aid to clinical judgement, not to replace it and therefore do not provide answers to every clinical question; nor does adherence to them ensure a successful outcome in every case. The ultimate decision on the clinical management of an individual patient will always depend on the specific clinical circumstances of the patient, and on the clinical judgement of the health care team.