Caffeine Use in the Anesthetic Management of a Patient With Congenital Central Hypoventilation

Congenital central hypoventilation syndrome (CCHS) is a rare neurological disease affecting the brain's response to carbon dioxide levels, resulting in dysregulation of respiration. CCHS is characterized by a diminished effort to breathe during sleep despite hypoxia and hypercapnia. Ventilation is adequate during wakeful periods but diminished during sleep. Alterations in ventilation pose a challenge to anesthesiologists in their attempts to wean these patients from ventilatory support. We describe a patient with CCHS and a complicated history of prolonged tracheal intubation, who was treated with intravenous (IV) caffeine and was able to resume adequate spontaneous ventilation and baseline mental status immediately post-procedure.