Management of CHAOS by intact cord resuscitation: case report and literature review

Background: Congenital high airway obstruction syndrome (CHAOS) is a near fatal condition, except when the ex utero intrapartum treatment (EXIT) procedure is performed as rescue. After antenatal diagnosis of the condition, counseling regarding prognosis and outcome needs to be provided. Case: We describe here a case with CHAOS due to isolated fetal laryngeal atresia, presented at our center at 33-week gestation. After counseling regarding the uncertain outcome, consent for elective caesarean was not given. Intact cord resuscitation (ICR) was done as a rescue by a well-coordinated team during delivery. Tracheostomy was performed successfully under local anesthesia within five minutes, while the cord was still attached to the placenta. The baby had supraglottic stenosis on CT scan. Reconstructive surgery is planned after 8 months. The literature review showed 24 reports of 28 cases with intrinsic airway obstruction managed by EXIT, laryngeal atresia was the most common cause (18/28). The outcome was poor in tracheal agenesis (1/4 survived) whereas those having laryngeal web or small communication (4/4 survived) had better outcome. Tracheal reconstruction was done in 3/28 cases only. Conclusions: The case emphasizes that ICR and tracheostomy during vaginal delivery can rescue the baby. The literature reviewed provided insight into the outcome of CHAOS cases in world literature.