Successful EXIT (ex utero intrapartum treatment) procedure in a fetus diagnosed prenatally with congenital high-airway obstruction syndrome due to laryngeal atresia

Congenital high-airway obstruction syndrome (CHAOS) is due to rare malforma tions and has been reported previously in only few cases. If the diagnosis can be made prenatally, the ex utero intrapartum treatment (EXIT) procedure may be life-saving. A healthy 28-year old nulli-para was referred because of isolated ascites f ound at gestational week 16 during routine ultrasound scan. Repeated scans showed overdistended hyperechogenic lungs with inverted diaphragm and a dil ated trachea, which was interpreted as a CHAOS resulting from laryngeal atr esia. The ascites eventually disappeared. An EXIT procedure was performed a t 35 weeks of gestation. Anesthesia of the mother was induced with thiopental, succinylcholine and f entanyl followed by intubation, and maintained with isoflurane and nitrous oxide. A low abdominal midline incision was performed followed by a low tra nsverse incision of the uterus. The fetal head, right arm and shoulder were delivered and intramuscular anesthesia was administered to the fetus. Imme diate laryngoscopy confirmed the diagnosis and a tracheostomy was therefore performed. Surfactant was given after a few minutes of ventilation. Compli ance improved and when the fetus was easy to ventilate, it was delivered. T he baby is developing normally at 18 months of age. Surgical correction of the malformation will be performed after two years of age. It is concluded that some fetuses with a prenatal diagnosis of CHAOS can be nefit from the EXIT procedure at delivery. This necessitates a multidiscipl inary management team.