Objective: To outline two cases of congenital tracheocutaneous fistula and discuss the potential pathogenesis of this previously unreported developmental abnormality.
Methods: Two cases of tracheocutaneous fistula evaluated at John Peter Smith Hospital (Fort Worth, TX) from May to October 2001 were reviewed. The surgical treatment of one infant is described.
Results: Two infants were evaluated with a congenital fistula extending from the suprasternal region of the neck dorsally to the trachea in the midline. The infants were otherwise developmentally normal with unremarkable prenatal histories. Primary surgical closure of a fistula was accomplished without complication.
Conclusions: Congenital tracheocutaneous fistula appears to be an isolated developmental abnormality not associated with the same degree of morbidity as acquired tracheocutaneous fistula. The development of a congenital tracheocutaneous fistula may be the result of abnormal epidermal migration secondary to a localized midline mesodermal defect. Congenital tracheocutaneous fistula may be successfully treated with primary closure. Observation and close follow-up of asymptomatic fistulas may be reasonable.