Esophageal Atresia

Oesophageal atresia (OA) encompasses a group of congenital anomalies comprising of an interruption of the continuity of the oesophagus with or without a persistent communication with the trachea. In 86% of cases there is a distal tracheooesophageal fistula, in 7% there is no fistulous connection, while in 4% there is a tracheooesophageal fistula without atresia. OA occurs in 1 in 2500 live births. Infants with OA are unable to swallow saliva and are noted to have excessive salivation requiring repeated suctioning. Associated anomalies occur in 50% of cases, the majority involving one or more of the VACTERL association (vertebral, anorectal, cardiac, tracheooesophageal, renal and limb defects). The aetiology is largely unknown and is likely to be multifactorial, however, various clues have been uncovered in animal experiments particularly defects in the expression of the gene Sonic hedgehog (Shh). The vast majority of cases are sporadic and the recurrence risk for siblings is 1%. The diagnosis may be suspected prenatally by a small or absent stomach bubble on antenatal ultrasound scan at around 18 weeks gestation. The likelihood of an atresia is increased by the presence of polyhydramnios. A nasogastric tube should be passed at birth in all infants born to a mother with polyhydramnios as well as to infants who are excessively mucusy soon after delivery to establish or refute the diagnosis. In OA the tube will not progress beyond 10 cm from the mouth (confirmation is by plain X-ray of the chest and abdomen). Definitive management comprises disconnection of the tracheooesophageal fistula, closure of the tracheal defect and primary anastomosis of the oesophagus. Where there is a "long gap" between the ends of the oesophagus, delayed primary repair should be attempted. Only very rarely will an oesophageal replacement be required. Survival is directly related to birth weight and to the presence of a major cardiac defect. Infants weighing over 1500 g and having no major cardiac problem should have a near 100% survival, while the presence of one of the risk factors reduces survival to 80% and further to 30-50% in the presence of both risk factors.

Background and aimsWe aimed to provide a contemporaneous assessment of outcomes at one-year post oesophageal atresia/tracheoesophageal fistula (OA-TOF) repair, focussing particularly on post-operative complications. It is generally accepted that oesophageal stricture is the most common complication and causes significant morbidity. We also aimed to assess the efficacy of prophylactic anti-reflux medication (PARM) in reducing stricture formation.MethodA prospective, multi-centre cohort study of all infants live-born with oesophageal atresia in the United Kingdom and Ireland in 2008/9 was performed, recording clinical management and outcomes at one year. The effect of PARM on stricture formation in infants with the type-c anomaly was assessed using logistic regression analysis.Results151 infants were live-born with oesophageal atresia in the defined reporting period, 126 of whom had the type-c anomaly. One-year follow-up information was returned for 105 infants (70%); the mortality rate was 8.6% (95% CI 4.7-14.3%). Post-operative complications included anastomotic leak (5.4%), recurrent fistula (3.3%) and oesophageal stricture (39%). Seventy-six (60%) of those with type-c anomaly were alive at one-year with returned follow-up, 57(75%) of whom had received PARM. Of these, 24 (42%) developed a stricture, compared to 4 (21%) of those who had not received PARM (adjusted odds ratio 2.60, 95% CI 0.71-9.46, p = 0.147).ConclusionsThis study provides a benchmark for current outcomes and complication rates following OA-TOF repair, with oesophageal stricture causing significant morbidity. The use of PARM appeared ineffective in preventing strictures. This study creates enough doubt about the efficacy of PARM in preventing stricture formation to warrant further investigation of its use with a randomised controlled trial.

ObjectiveTo describe the evolution from delayed management of long gap esophageal atresia to thoracoscopic treatment directly after birth without the placement of a gastrostomy.BackgroundLong gap esophageal atresia remains a challenge for pediatric surgeons. Over the years, several techniques have been described to deal with the problem of the distance between the proximal and distal esophagus. More recently, a traction technique has been advocated. With the advent of minimal invasive surgery, the thoracoscopic elongation technique has been developed.MethodsRetrospective description of a single-center experience with the thoracoscopic treatment of patients with long gap esophageal atresia over a 7-year period.ResultsBetween 2007 and May 2014, 10 children with long gap esophageal atresia were treated by thoracoscopic elongation technique. In two children, the procedure failed. Eight children successfully underwent thoracoscopic traction with delayed primary anastomosis. Initially, all patients had a gastrostomy. During the course, the technique evolved into delayed primary anastomosis directly after birth without the use of a gastrostomy.ConclusionThoracoscopic elongation technique in long gap esophageal atresia not only is feasible, but can nowadays also be performed directly after birth without the use of a gastrostomy. With this development, we have entered a new era in the management of long gap esophageal atresia.

BackgroundEA survivors face long-term morbidity after thoracotomy with adult outcomes largely uninvestigated. This study assessed musculoskeletal and pulmonary outcomes in adults who underwent neonatal thoracotomy for EA using real-time magnetic resonance imaging (rt-MRI).MethodsBetween 09/2022 and 02/2023, 30 adult EA patients and 22 healthy controls underwent rt-MRI to evaluate chest wall motion, lung volumes, and anatomical deformities. Thoracic cross-sectional area (CSA), lung volumes, and rib/spinal abnormalities were analyzed using a 3 Tesla MRI system.ResultsRib adhesions and fusions were significantly more frequent in EA patients (90%) than in controls (p ConclusionThis study demonstrates the significant long-term musculoskeletal and pulmonary sequelae in adults following open EA repair, with the pioneering application of rt-MRI. These findings emphasize the risks associated with open surgery for EA, underscoring the need for long-term, multidisciplinary follow-up and preventive strategies.ImpactThis is the first study to use real-time MRI to assess dynamic thoracic function in adults after open repair of esophageal atresia (EA), revealing significant long-term musculoskeletal and pulmonary impairments. Findings include high rates of scoliosis, rib anomalies, and reduced chest wall motility and lung volume on the operated side. Results underscore the need for long-term, multidisciplinary follow-up and inform future strategies to reduce morbidity after neonatal thoracotomy.