RESUMO
Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare congenital anomalies occurring in approximately 1 in 2500 to 3500 neonates. We present a neonatal patient diagnosed with EA/TEF in conjunction with pulmonary agenesis requiring definitive repair via median sternotomy. The child was born at 33 weeks gestational age with post-delivery respiratory distress necessitating intubation. A nasogastric tube was unable to be passed. After subsequent imaging, TEF and pulmonary agenesis were diagnosed. During planned staged repair with ligation of TEF via standard right thoracotomy approach, significant ventilatory compromise was encountered. Due to concern for ventilatory compromise and anatomical variance limiting visualization, a median sternotomy approach was utilized for definitive repair. This exposure and repair were successful and may be considered for cases with complex pulmonary malformation limiting standard thoracotomy. To our knowledge, this is only the second reported case of a successful TEF/EA repair using a median sternotomy approach.