The challenging airway: Tracheal agenesis in the newborn.

A preterm female presented with severe respiratory distress in the delivery room and was found to have tracheal agenesis with a tracheoesophageal fistula and a congenital heart defect. Tracheal agenesis is uncommon and is often associated with other congenital abnormalities. Although there are surgical options for repair, mortality remains high.

Esophageal atresia/tracheoesophageal fistula and proximal symphalangism in a patient with a NOG nonsense mutation.

Esophageal atresia and tracheoesophageal fistula (EA/TEF) are relatively common malformations of the human foregut. The etiology remains incompletely understood with genetic causes identified in a small minority of affected patients. We present the case of a newborn with type C EA/TEF along with proximal symphalangism found to have a de novo NOG nonsense mutation. Patients with chromosome 17q deletions including the NOG gene have previously been reported to have EA/TEF but mutations in the gene have not been identified in patients with this malformation. This case provides evidence that haploinsufficiency for NOG may be the cause for EA/TEF in the 17q deletion syndrome and suggests that the clinical spectrum of NOG-related symphalangism spectrum disorders may include EA/TEF.

Thoracoscopic management of non-type C esophageal atresia and tracheoesophageal atresia.

PURPOSE: This study evaluates the results of thoracoscopic management of complex, non-type C, EA and TEF in infants. METHODS: From March 2000 to February 2017, 23 patients were treated for Type A N=13, Type B N=4, and Type E N=6. Patients diagnosed with EA had G-tube feeds for a period of 4-9weeks. All procedures were performed thoracoscopically. EA gaps were between 4 and 7 1/2 vertebral bodies. RESULTS: All surgeries were completed thoracoscopically. Average operative time was 95min for Type A, 115min for Type B, and 50min for Type E. Two patients with long gaps had small leaks which resolved with conservative management. One patient with an H-type was re-intubated causing a partial disruption of the tracheal repair. This required thoracoscopic re-exploration with repair and placement of an intercostal muscle flap. No patient has any clinical evidence of fused ribs, chest wall asymmetry, shoulder girdle weakness, or winged scapula. CONCLUSION: Thoracoscopic repair of complex EA and TEF is safe and effective. The excellent visualization of the thoracic inlet allows for extensive mobilization creating sufficient length for long gaps and safely managing high fistulas. This may limit injury to adjacent structures and avoid a neck incision and chest wall deformity. LEVEL OF EVIDENCE: IV.

Treatment outcomes for eosinophilic esophagitis in children with esophageal atresia.

Eosinophilic esophagitis (EoE) has been reported to be more prevalent in patients with esophageal atresia/tracheoesophageal fistula (EA-TEF). To date, there is limited data on the management of EoE in this group of patients. The aim of this study is to evaluate the treatment outcomes of EoE in children with EA-TEF. A retrospective chart review was performed on all EA-TEF children who were diagnosed with and treated for EoE between January 2000 and September 2013 at the Sydney Children's Hospital. Data collected included details of the patient's treatment, post-treatment endoscopy, symptoms and nutrition. Twenty patients were included in the study. Median age at diagnosis was 26 months (8-103 months), and median time from diagnosis to last follow-up was 23 months (2-132 months). Patients were treated with budesonide slurry, swallowed fluticasone, elimination diet alone or in combination. All patients were on proton pump inhibitors at time of diagnosis of EoE which was continued. Six out of seven patients who had furrowing/exudate in endoscopy at diagnosis had complete resolution at a median follow-up period of 26 months (P = 0.031). Median peak intraepithelial eosinophil count reduced significantly from 30/high-powered field (HPF) (19-80/HPF) to 8/HPF (0-85/HPF) (median time for improvement = 24 months) (P = 0.015). There was a significant reduction in symptoms of dysphagia and reflux post-treatment (P < 0.001). Prevalence of strictures significantly decreased (P = 0.016), as did need for dilatations (P = 0.004). In four out of six patients with gastrostomies at baseline, the feeding improved on treatment of EoE and the gastrostomy could be closed. There was also a nonsignificant trend towards improvement in weight and height 'z scores' of the patients. Treatment of EoE in children with EA-TEF was found to significantly reduce intraepithelial eosinophil count, symptoms, strictures and need for dilatations.

The application of a new cyanoacrylate glue in pediatric surgery for fistula closure.

Surgical glues have been used in pediatric surgery because of the fragility of tissue, and to prevent major surgeries. The present report describes our experience with using a new cyanoacrylate Glubran 2 (Viareggio, Italy) in the treatment of five cases of tracheoesophageal atresia with fistula (one fistula protection, three recurrent fistula, and one unstable patients), two cases of hypospadias, one case of vesicutanouse fistula after bladder extrophy, and one case of cloacal extrophy from January-December 2008. Three cases of recurrent tracheoesophageal atresia with fistula were treated by bronchoscpic glue injection. The other two cases benefited from glue through its ability to plug the fistula and to act as a protecting layer on anastomosis. In two cases with hypospadias excessive use of the glue caused skin necrosis, which was repaired. The wounds of cloacal extrophy were protected from nearby colostomy contamination and infection, and the vesicocutanouse fistula was closed by deepithelialization and sealing with glue. Based on the outcomes of the cases, it may be possible to suggest that Glubran 2 may be used safely in Pediatric Surgery as a sealant for the prevention and treatment of fistulas.