Congenital tracheoesophageal fistulas in adults

Congenital tracheoesophageal fistula without esophageal atresia is commonly known as "H" type. This is an extremely rare anomaly in infants and accounts for approximately 1 1/2-4% of all congenital tracheoesophageal malformations. An anomaly should be described by its anatomical defect, not by a number or letter. There are five main anatomical categories with 85-95% being of the esophageal atresia and distal tracheoesophageal fistula type. About 1 1/2% are of the "H" type. Although today tracheoesophageal fistula is a well-recognized entity, a few of the "H" type have passed through the pediatric period without diagnosis. Those undetected fistulas have successfully masqueraded as chronic lung disease of unknown etiology. Congenital "H" type tracheoesophageal fistulas assume an oblique orientation with the growth of the host to adulthood. This helps to explain the difficulty in diagnosis plus the ability of the host to survive to adult life. Treatment is surgical, consisting of simple ligation via cervical or thoracic approach depending upon location. The 12 previously reported cases in the English literature are reviewed, and a 13th case has been added.