Louisiana State University Health Sciences Center Shreveport, Louisiana
Introduction: Tracheoesophageal fistula (TEF) constitutes one of the commonest congenital anomalies with an incidence of roughly 1 in 3500 births with treatment consisting of surgical correction in very early life. Chronic complications can include esophageal stricture, dysphagia, gastroesophageal reflux disease (GERD), Barrett’s esophagus, and esophageal cancer. While rare, these patients do have an increased risk of esophageal carcinoma attributable to chronic GERD leading to ongoing mucosal disruption. Sparce data on esophageal cancer after TEF repair appears to show an increased risk of this event occurring after 40 years of age.
Case Description/Methods: The patient is a 32-year-old man with a past medical history of Cystic Fibrosis, GERD, osteoporosis, and tobacco use, and a past surgical history significant for Tracheoesophageal fistula repair. The patient presented to a primary care physician with complaints of dysphagia. He reported dysphagia only to liquids that started 2 months before presentation. The dysphagia was gradually progressed to solids. He was referred to gastroenterology, and an EGD was performed showing a single traversable mass measuring 5 cm in the esophagus (20 cms. from incisors) covering one-quarter of the circumference, and cold forceps biopsy was performed. The EGD also revealed signs of the previous tracheoesophageal fistula repair and mild generalized atrophic mucosa. Biopsies taken from the esophagus and stomach were sent for pathology. Pathology from the esophageal biopsy came back positive for moderately differentiated Squamous cell carcinoma and gastric biopsy revealed chronic gastritis with complete intestinal metaplasia. The patient was referred to oncology and underwent a PET scan which revealed the esophageal malignancy and bilateral pulmonary nodules. The patient was started on chemoradiation therapy and follows up with oncology regularly.
Discussion: This case describes the importance of continuity of care during the transition from pediatrics to adult care in the field of gastroenterology in patients with a history of TEF repair, given the patient’s young age. While it appears esophageal cancer after TEF repair is rare, there should be an investigation into guidelines for adequate screening in this disease process.
