Congenital laryngomucocoele: a rare cause for CHAOS

• Author(s): Cunha, M ; Janeiro, P ; Fernandes, R ; Carreiro, H ; Laurini, R
• Date: 2009
• Persistent ID: http://hdl.handle.net/10400.10/397
• Origin: Repositório do Hospital Prof. Doutor Fernando Fonseca
• Subject(s): Criança; Obstrução das vias respiratórias; Doenças da laringe; Laryngomucocoele; Congenital high airway obstruction syndrome; CHAOS
• Document Type: Article
• Language: English

Description

Congenital high airway obstruction syndrome (CHAOS) is a rare but life-threatening condition that results from the obstruction of the upper airways. We describe a female newborn, from a Grávida II, Para 0, 36-year-old woman, with a routine ultrasound at 30 weeks’ gestation that showed polyhydramnios. She delivered a live-born female baby at 36 weeks without any dismorphic features but with respiratory distress. Attempts at endotracheal intubation were unsuccessful due to the presence of a mass obstructing the larynx. The reanimation process was stopped after 20 minutes. Post-mortem examination demonstrated the presence of a total occlusion of the larynx by a laryngomucocoele. Laryngocele, a congenital cyst of the larynx, occurs rarely and hardly ever as a cause of CHAOS. What is more, laryngomucocoele has not been previously reported as a cause of CHAOS. These conditions represent a neonatal emergency with reserved prognosis unless diagnosed antenatally allowing for a programmed ex utero intrapartum treatment (EXIT) by performing tracheostomy while maintaining the placental circulation.