Adult Congenital H-Type Tracheoesophageal Fistula

Abstract

SESSION TITLE: Imaging SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Congenital tracheoesophageal fistula (TEF) is an anomaly of the respiratory tract with incidence of 1 in 3,500 live births, whereas acquired type is rare. Congenital TEF usually occurs with esophageal atresia (EA) and is classified based on anatomy. CASE PRESENTATION: A 52 year-old man was referred for computed tomography (CT) of his chest for lung cancer screening. He had a history of chronic obstructive pulmonary disease, chronic kidney disease with congenital solitary kidney, and gastroesophageal reflux disease. He reported no history of esophageal trauma, esophageal or trachea procedures, and no history of thoracic radiation. Childhood history included recurrent pulmonary infections and abdominal distention. Past surgical history was significant for spinal fusion and left-sided arthroscopic shoulder repair. He denied any family history of congenital abnormalities. On initial evaluation, patient complained of occasional choking episodes after drinking liquids. Lung exam was normal. CT revealed TE fistula and prominent gaseous distention of the thoracic esophagus. Bronchoscopic evaluation revealed a posterior TE fistula with dynamic patency upon inspiration and collapse upon expiration. The location of TEF was approximately 8 centimeters distal from the vocal cords. The maximal size of the opening was 10 millimeters in diameter. Visualization of the esophagus was noted with increase inspiratory effort. The rest of the lung parenchyma was mildly hyperemic but was otherwise normal. DISCUSSION: Discovery of presence of congenital H-type TEF in adulthood is rare with less than 20 cases described in the English literature. TEF is usually diagnosed in infancy with fifty percent of infants born as non-syndromic (without other anomalies) while the rest have associated anatomic anomalies, most often associated with the VACTERL(vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) syndrome. CONCLUSIONS: Our patient was incidentally found to have TEF. By history, this abnormality was most likely a congenital deformity, discovered by CT performed for lung cancer screening. Discovery of congenital TE fistula in adults is exceedingly rare. Patients without all associated VACTERL findings and/or with small TE fistulas may not be diagnosed until later in adulthood. Reference #1: Depaepe A, Dolk H, Lechat MF. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group. Archives of Disease in Childhood. 1993;68(6):743-748. Reference #2: Keckler SJ, St. Peter SD, Valusek PA, et al. VACTERL anomalies in patients with esophageal atresia: An updated delineation of the spectrum and review of the literature. Pediatric Surgery International. 2007;23(4):309-313. Reference #3: Al-Salem AH, Mohaidly MA, Al-Buainain HMH, Al-jadaan S, Raboei E. Congenital h-type tracheoesophageal fistula: A national multicenter study. Pediatric Surgery International. 2016;32(5):487-491. DISCLOSURE: The following authors have nothing to disclose: John Bradshaw, Yasir Usman, Kellie Jones No Product/Research Disclosure Information