tracheoesophageal fistula
Summary
A tracheoesophageal fistula (TEF) is an abnormal connection between the trachea and esophagus, most commonly occurring as a congenital anomaly associated with esophageal atresia. The most common variant is esophageal atresia with distal TEF (type C, ~85% of cases), presenting with polyhydramnios, excessive drooling, choking with feeds, and inability to pass a nasogastric tube.
Detail
Pathophysiology: TEF results from incomplete separation of the foregut into respiratory and digestive tracts during the 4th-6th week of embryonic development (failure of tracheoesophageal septum formation). Esophageal atresia (EA) often coexists. Classification (Gross classification): Type A - pure EA without fistula (~8%); Type B - EA with proximal TEF (~2%); Type C - EA with distal TEF (~85%, most common); Type D - EA with both proximal and distal TEF (~2%); Type E (H-type) - TEF without EA (~4%), often presents later in life with recurrent aspiration pneumonia.
Clinical presentation: Prenatally, polyhydramnios (fetus cannot swallow amniotic fluid). Postnatally, excessive drooling, choking, coughing, cyanosis with feeding, and regurgitation. In distal TEF, air enters the stomach causing abdominal distension; reflux of gastric contents into the trachea causes chemical pneumonitis. Diagnosis is suspected when a nasogastric/orogastric tube cannot be passed into the stomach (coils in the proximal esophageal pouch, visible on X-ray). Associated with VACTERL syndrome (Vertebral defects, Anal atresia, Cardiac defects, TEF, Renal anomalies, Limb defects) - should prompt workup for these anomalies.
Management: Surgical repair - primary anastomosis of the esophagus with ligation of the fistula. NPO status, gastric decompression, and IV fluids preoperatively. Complications include anastomotic leak, stricture, recurrent fistula, and gastroesophageal reflux disease (GERD) which is common long-term.
H-type fistula: Since there's no atresia, an NG tube CAN pass into the stomach, making diagnosis more challenging. Presents later in childhood/adulthood with recurrent respiratory infections/pneumonia due to aspiration, and may have a chronic cough associated with feeding.
Sources
- First Aid for the USMLE Step 1
- Blueprints Pediatrics
- Sabiston Textbook of Surgery
- UpToDate: Esophageal atresia and tracheoesophageal fistula
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