Tracheal atresia with a cephalically developed lung bud

Abstract

Rationale: Tracheal atresia (TA) involves complete or partial tracheal absence below the larynx. It involves failure of complete separation of the laryngotracheal diverticulum from the foregut at the 4th gestational week. In TA, the trachea and main bronchi generally proceed in the normal caudal directions. Patient concerns: At the gestational age of 34 weeks and 6 days, a male baby weighing 2290 g was born via cesarean section. A brisk bag was used, and mask ventilation was performed, but was not effective. Intubation was attempted; however, the endotracheal tube did not advance below the vocal cord. Diagnosis: Tracheal atresia. Interventions: An emergent open neck exploration could not reveal the trachea. On computed tomography, the tracheoesophageal fistula (TEF) started from the lower esophagus just above the gastroesophageal junction. The trachea arose upward to form the main bronchus. Outcomes: His parents signed the “Do not resuscitate” form due to poor outcome. On the 23rd day of birth, the baby expired. Lessons: Our case involved TA with tracheoesophageal fistula forming between the trachea and main bronchi in a cephalic direction as detected on computed tomography. Further, the trachea arose from the gastroesophageal junction; thus, it does not belong to any Floyd classification. Herein, we report a TA case with a cephalically developed lung bud.

Esophageal Trachea, a Unique Foregut Malformation Requiring Multistage Surgical Reconstruction: Case Report

“…He described 96 types of foregut malformations, which were classified with 10 groups, including the large spectrum of esophageal atresia, with or without tracheo-esophageal fistula, as well as laryngo-tracheo-esophageal clefts, CBPFMs, and tracheal atresia (3). The malformation detected in our case was not recognizable among any of those described in the atlas and, to the best of our knowledge, has not been presented in further published case reports and small series (1,4,(14)(15)(16)(17)(18)(19)(20). Congenital absence of tracheal or bronchial rings, sometimes associated with esophageal atresia and trifurcated carina, has been described in very few cases, but none of them presented a segmental fusion between esophagus and distal trachea (21,22).…”

“…The lower respiratory tract, which includes the area between the larynx and lungs, embryologically originates from the foregut, firstly through the formation of the respiratory bud, and then by the action of the tracheo-esophageal septum (12)(13)(14). Consequently, the alteration of this embryological process can result in communicating malformations that might involve not only the esophagus and trachea, but also the pharynx, stomach, and biliary system, with the larynx, bronchi, and lungs (15)(16)(17)(18)(19).…”