Author(s)
Michael Nguyen, BAchelors of Science, Medical Student
Anatoli Karas, Assistant Professor, Pediatric Otolaryngology (MD)
Rachel Akers, MS
Affiliation(s)
Otolaryngology, Rush University Medical Center, Chicago, Illinois, USA
Abstract:
Background
Acquired central hypoventilation syndrome (ACHS) is a rare disorder of autonomic control of breathing. Congenital CHS (CCHS) is characterized by adequate wakeful respiratory function with hypoventilation during sleep due to impaired automatic drive. Tracheostomy with mechanical ventilation is traditional management for CCHS but recent literature supports the use of non-invasive ventilation (NIV). Data on NIV in ACHS is not well documented due to the rarity of the condition.
Hypothesis/Aim
Unique case of hypoventilation in a pediatric patient with ACHS
Methods
This case report describes a pediatric patient with acquired central hypoventilation syndrome (ACHS) following brainstem hemorrhage and the successful transition from tracheostomy dependence to non-invasive ventilation (NIV).
Results/Discussion
A six-year-old female with acquired CHS caused by traumatic bilateral medullary subarachnoid hemorrhage at eight months old underwent tracheostomy and had been managed with mechanical ventilation via tracheostomy overnight for 5 years. She then presented to otolaryngology and sleep medicine for potential decannulation. After mask conditioning, she underwent decannulation and transitioned to nocturnal BiPAP. The patient tolerated non-invasive ventilation without desaturations or respiratory distress. At follow-up, she remained stable on BiPAP at night with no daytime or nocturnal respiratory issues.
This case demonstrates that non-invasive ventilation is a safe and effective alternative to tracheostomy in the setting of acquired CHS.