A case of successful slide tracheoplasty for long-segment congenital tracheal stenosis in a neonate with a congenital diaphragmatic hernia and Fallot’s tetralogy

Abstract

Congenital tracheal stenosis (CTS) is a rare and life-threatening airway disorder, which is often associated with cardiac malformations. Among them, neonatal symptomatic CTS with cardiac malformations has an extremely poor prognosis. In contrast to cardiac malformation, congenital diaphragmatic hernia (CDH) has rarely been associated with CTS. We report a neonatal case in which slide tracheoplasty and intracardiac repair were performed simultaneously for CTS and Fallot’s tetralogy (TOF). An infant with left CDH and Fallot's tetralogy (TOF) was born by cesarean section at 38 weeks of gestation. At the time of resuscitation, a 2.5 mm (ID) endotracheal tube could only be inserted just below the vocal cords. After repairing the CDH at 3 days of age, planned extubation was performed at 7 days of age. However, the patient required re-intubation due to life-threatening episodes after 2 days of the extubation. Enhanced CT revealed a long segment CTS from the upper trachea to the right bronchus (length of stenosis: 40 mm, minimum inner diameter: 2 mm). At 24 days of age, veno-arterial extracorporeal membrane oxygenation (ECMO) was introduced due to severe respiratory failure. At 28 days of age, slide tracheoplasty and palliative right ventricular outflow tract reconstruction (RVOTR) was performed with cardiopulmonary bypass (CPB). After tracheoplasty, a 3.5 mm tracheal (ID) tube could be placed in the reconstructed trachea in a patient with CTS. ECMO was completed 7 days after the operation. On the 17th day after the operation, he was extubated successfully. He was discharged 5 months after birth with home oxygenation therapy. We reported the successful simultaneous correction of slide tracheoplasty and palliative RVOTR for a neonate with CDH. ECMO was used for respiratory management before and after surgery.