Congenital Tracheoesophageal Fistulas in Adults

Abstract

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.

Achalasia-like findings in a case with delayed diagnosis of H-type tracheoesophageal fistula

“…Heitmiller et al [12] reported detailed esophageal manometric findings as uncoordinated, lowamplitude peristalsis of the esophageal body; both low and normal LES pressures in an adult with congenital Htype TEF, with persisting manometric findings after fistula repair. Esophageal dysmotility has also been reported in a child and few adults with H-type TEF [3,4,7]. Additionally, it is possible that an esophagus which has been periodically distended with air for long time may show dysmotility [4].…”

“…1 a Chest X-ray demonstrating dilated esophagus (arrows) and bronchiectatic areas. b HRCT revealing bronchiectatic areas adulthood [1][2][3][4][5]. The classical triad of symptoms is paroxysmal choking and cough precipitated by feeds, abdominal distention due to passage of air from the trachea to the esophagus and recurrent aspiration pneumonia [1,7,9].…”

“…Air easily passes from trachea to the esophagus, producing air-filled dilated esophagus and dysmotility which may mimic achalasia [3,7]. Only three cases of H-type TEF and associated achalasia have been reported to date and management of these cases seemed a bit confusing [3,4,7]. Additionally, this association has not been reported in large congenital tracheoesophageal anomalies or H-type TEF series and large childhood achalasia series including our series [1,8].…”

“…Although most of the patients have been symptomatic since birth, some rare cases diagnosed in adulthood have also been reported [3][4][5]. This anomaly has few specific symptoms and it can be very difficult to diagnose.…”

H-Type Congenital Tracheoesophageal Fistula in a Saudi Child

“…It is known that some of these cases remain undiagnosed until adulthood, the oldest reported case being 63 years of age. [22][23] The diagnosis of these fistulas should be made by barium swallow with cinefluoroscopy and video recording, as they can be missed by the conventional methods such as esophagography. 24 Tracheobronchoscopy in conjunction with instillation of methylene blue into the esophagus to identify the tracheoesophageal fistula was recommended by Abbott.…”