Adaptive Servo‐Ventilation for Glottic Closure in a Patient With Adult‐Onset Congenital Central Hypoventilation Syndrome: A Case Report

ABSTRACT Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterised by autonomic dysfunction and typically presents in infancy, although adult‐onset disease is increasingly recognised. We report a genetically confirmed case of adult‐onset CCHS in a 69‐year‐old man whose condition became clinically manifest following transsphenoidal surgery for a pituitary microadenoma, complicated by high‐grade atrioventricular block and recurrent postoperative type 2 respiratory failure. Polysomnography demonstrated severe sleep‐related hypoventilation, with glottic closure repeatedly impairing ventilation across spontaneous, spontaneous‐timed and intelligent volume‐assured pressure support modes of non‐invasive ventilation. This pattern of glottic‐closure–associated NIV failure has not previously been described in adult‐onset CCHS. Notably, adaptive servo‐ventilation uniquely restored stable ventilation and resolved associated respiratory events, representing the first documented successful use of this modality in this context. This case highlights the need to consider CCHS in adults with unexplained postoperative hypercapnia and suggests a potential role for adaptive servo‐ventilation when conventional NIV is ineffective.