Face-mask CPAP as palliative support for diffuse tracheobronchomalacia from ring agenesis in an infant: When sleep becomes therapeutic.

Congenital absence or deficiency of tracheal rings is exceptionally rare and may cause diffuse tracheobronchomalacia (TBM) with life-threatening airway collapse. A 6-month-old girl had persistent stridor from birth, cyanotic spells during feeding, and nocturnal desaturation. Cervicothoracic CT suggested severe airway collapse, and rigid bronchoscopy showed a flattened trachea and main bronchi without visible cartilaginous rings, consistent with ring agenesis and diffuse TBM. Curative surgery was not feasible. After in-hospital titration during a nap, face-mask CPAP with heated humidification was prescribed during sleep and postprandial windows. A minimal effective pressure of 6 cmH2O stabilized oxygenation and reduced cyanotic episodes, with good tolerance and device-recorded adherence. Despite transient clinical benefit, the infant died at one and a half years of age (18 months) due to disease progression. In extensive congenital TBM from ring agenesis not amenable to reconstruction, targeted face-mask CPAP can provide palliative/bridging support by counteracting dynamic airway collapse at times of highest vulnerability (sleep and after feeding). This case highlights practical aspects of titration, interface choice, and monitoring in infants.