Atresia of esophagus with tracheo-esophageal fistula

This code signifies a congenital malformation where the esophagus is discontinuous (atresia) and has an abnormal connection to the trachea (tracheo-esophageal fistula). This condition prevents normal passage of food to the stomach and can lead to aspiration of gastric contents or saliva into the lungs. It is a serious birth defect requiring surgical intervention.

Assign this code for newborns or infants diagnosed with both esophageal atresia and a tracheo-esophageal fistula. Documentation should clearly state the presence of both conditions, often identified through imaging studies like esophagrams or bronchoscopy, and clinical signs such as choking, coughing, and respiratory distress during feeding.

• Always confirm the presence of both esophageal atresia and a tracheo-esophageal fistula; if only one is present, a different code is required.
• Do not confuse this with isolated esophageal atresia (Q39.0) or isolated tracheo-esophageal fistula (Q39.2).
• Code any associated congenital anomalies, such as VACTERL association components, in addition to this code.