Primary tracheocutaneous fistula closure with immediate transition to nocturnal noninvasive positive pressure ventilation in two children with Congenital Central Hypoventilation Syndrome.

Transitioning children with Congenital Central Hypoventilation Syndrome (CCHS) from nocturnal invasive ventilation via tracheostomy to noninvasive positive pressure ventilation (NIPPV) is challenging due to the leak caused by the tracheocutaneous fistula (TCF), resulting in insufficient ventilation. Decannulation and primary closure of the TCF with immediate transition to nocturnal NIPPV was performed in two children with CCHS at a tertiary care children's hospital. Neither child developed significant adverse effects such as pneumomediastinum or pneumothorax. This technique is a novel approach that may improve decannulation outcomes and aid transition to NIPPV in this patient population.