Nuss procedure for combined pectus excavatum and carinatum in a patient with a history of congenital esophageal atresia repair surgery

Cardiothoracic surgery usually causes tissue adhesion on the operation site which increases the risk of complications in the subsequent thoracic surgery including Nuss procedure. Disorders that require cardiothoracic surgery include chest wall deformities such as pectus excavatum, congenital heart diseases, lung diseases such as congenital cystic adenomatiod malformation and bronchopulmonary dysplasia, and congenital diaphragmatic hernia. Recently, we encountered a rare case of combined pectus excavatum and carinatum in a patient with a history of congenital esophageal atresia repair surgery. Commendably, despite tissue adhesion from the previous surgery, a modified Nuss procedure was performed successfully with no complications. We agree that the Nuss procedure is feasible for thoracic deformities in patients with a surgical history of cardiothoracic surgery.

First-in-human autologous oral mucosal epithelial sheet transplantation to prevent anastomotic re-stenosis in congenital esophageal atresia

BackgroundCongenital esophageal atresia postoperative anastomotic stricture occurs in 30-50% of cases. Patients with severe dysphagia are treated with endoscopic balloon dilatation (EBD) and/or local injection of steroids, but many patients continue to experience frequent stricture. In this study, we investigated the transplantation of autologous oral mucosa-derived cell sheets (epithelial cell sheets) as a prophylactic treatment for congenital esophageal atresia postoperative anastomotic stricture.MethodsEpithelial cell sheets were fabricated from a patient’s oral epithelial tissue, and their safety was confirmed by quality control tests. The epithelial cell sheets were transported under controlled conditions from the fabrication facility to the transplantation facility and successfully transplanted onto the lacerations caused by EBD using a newly developed transplantation device for pediatric patients. The safety of the transplantation was confirmed by follow-up examinations over 48 weeks.ResultsThe number of EBDs required after transplantation and the number of days between EDBs were recorded. Before transplantation, EBDs were performed approximately every two weeks, whereas after transplantation, the interval was extended to a maximum of four weeks. The patient was also aware of a reduction in dysphagia.ConclusionsThis study suggests that cell sheet transplantation might be effective in preventing anastomotic stricture after surgery for congenital esophageal atresia. We chose this very severe case for the first clinical study in humans. Future studies are needed to identify cases in which cell sheet transplantation is most effective and to determine the appropriate timeframes for transplantation.

DOZ047.58: The familial occurrence of congenital esophageal atresia with or without a fistula is an extremely rare pathology

We report on two siblings (boy and girl), which were treated for congenital esophageal atresia with the fistula, at the Department of Pediatric Surgery and Urology in Wroclaw, at an interval of two years. These children have two healthy siblings. Both newborns were born on time (38 and 42 week of pregnancy). Prenatally hydramnios was observed in younger child. They were postnatally diagnosed with congenital esophageal atresia with a distal fistula. Newborns were operated on the second day of life. Bronchoscopy and right-sided thoracoscopy were performed. Both patients had a short-gap esophageal atresia with a distal fistula. The fistula was proximally closed with sutures and divided distally. Subsequently, the one-layered esophageal anastomosis was performed. There were no early postoperative complications. Both patients underwent two esophageal dilatations. Siblings are tolerating oral feeding. Occasionally, parents observe choking in the older child. Patients remain under the constant care of a pediatric surgery clinic. Moreover, children were evaluated by clinical geneticist and exome sequencing (WES) study on the base of DNA isolated from lymphocytes and esophageal tissues were performed in both siblings.