PT - JOURNAL ARTICLE AU - Khademian, Majid AU - Saneian, Hosein AU - Zare, Narges AU - Famouri, Fatemeh AU - Nasri, Peiman AU - Moghadasi, Mehri AU - Kelishadi, Roya TI - Treatment methods for oesophageal strictures in paediatric patients with epidermolysis bullosa: a systematic review AID - 10.1136/bmjpo-2024-002689 DP - 2024 Oct 01 TA - BMJ Paediatrics Open PG - e002689 VI - 8 IP - 1 4099 - http://bmjpaedsopen.bmj.com/content/8/1/e002689.short 4100 - http://bmjpaedsopen.bmj.com/content/8/1/e002689.full SO - BMJ Paediatrics Open2024 Oct 01; 8 AB - Background Epidermolysis bullosa (EB) is a collection of rare, inherited disorders that require treatment in specialised centres by multidisciplinary teams knowledgeable about the unique features and challenges of EB manifestations and complications.A major gastrointestinal complication in patients with EB is oesophageal strictures. Effective management of oesophageal strictures can significantly improve patients’ quality of life. This study systematically reviews the current literature on treatment options for oesophageal strictures in paediatric patients with EB.Methods In September 2023, we conducted a systematic search for articles on the treatment of oesophageal stricture in patients with EB. We searched PubMed, Scopus, Embase and Ovid database without language or publication date restrictions. We screened 1042 articles, 15 of them were included in the current review. We extracted the following data from these studies: patient demographics, stricture characteristics, procedural details, clinical outcomes, complications and recurrences.Results Overall, in the reviewed papers, strictures were located mostly in cervical oesophagus followed by thoracic lesions. Moreover, in most of the cases only a single stricture was reported, but multiple strictures were not uncommon. Stricture treatment approaches included medical management, bougienage, as well as fluoroscopic and endoscopic balloon dilation or a combination of these methods. In most studies, fluoroscopic dilation was used as the primary treatment method in 756 procedures. They commonly used general anaesthesia for the procedure, only one study used sedation. Hospital stays were usually brief, with an average duration of 1 day, and in one study patients were discharged after just 4 hours. Most patients experienced symptom relief, could resume oral intake and gained weight soon after the procedure. However, recurrence rates had large variations from 12% to 83%. Studies reported median recurrence intervals ranging from 7 to 18 months. This review showed that complications such as perforation, fever and odynophagia were relatively uncommon, and were controlled by conservative treatment.Conclusions Both fluoroscopic and endoscopic balloon dilation are widely used methods for the management of oesophageal strictures in patients with EB. Each technique presents its own set of advantages and potential complications. Although the current evidence is notably limited, practical clinical decision-making may favour the fluoroscopic technique over endoscopic balloon dilation due to a comparatively reduced risk of procedural trauma. To ascertain the most effective approach, high-quality randomised controlled trials are imperative to delineate the superiority of one technique over the other.Data are available in a public, open access repository. In accordance with the guidelines provided, we require that the data generated from our research on this article be made openly and publicly available upon publication of our article. We understand the importance of transparency and accessibility in academic research and are committed to supporting this initiative. In cases where it is not feasible to make this data openly available, we will ensure that the data is shared through a controlled access repository. This approach will allow us to protect sensitive information while still making our findings accessible to the broader research community. We affirm our commitment to adhering to these standards, ensuring that our research contributes to the ongoing dialogue and advancements in the treatment of paediatric oesophageal strictures associated with epidermolysis bullosa.