Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula

ImportanceType D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence.ObjectiveTo investigate diagnostic and treatment strategies for this rare condition.MethodsWe retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.ResultsAmong 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.InterpretationType D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF. Type D Esophageal Atresia/Tracheoesophageal Fistula (EA/TEF) is a rare condition prone to misdiagnosis. This retrospective study revealed that bronchoscopy may assist in diagnosis. The surgical strategy for proximal TEFs is depend on the specific location, influencing the choice between a one-stage thoracoscopic surgery or a two-stage repair with a cervical approach.*image