Spinal anesthesia and postoperative epidural analgesia in a patient with congenital central hypoventilation syndrome -a case report

• Main Authors: Yongjoon Choi, Sunam Lee, Jiyeon Lee, Seongwon Woo
• Format: Article
• Language: English
• Published: Korean Society of Anesthesiologists 2019-08-01
• Series: Korean Journal of Anesthesiology
• Subjects: autonomic dysregulation; congenital central hypoventilation syndrome; hypoxemia; ondine’s curse; spinal anesthesia
• Online Access: http://ekja.org/upload/pdf/kja-d-18-00118.pdf

Background Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by alveolar hypoventilation and autonomic dysregulation. Patients with CCHS have adequate ventilation while awake but exhibit hypoventilation while asleep. More severely affected patients exhibit hypoventilation both when awake and when asleep. Case Here, we report a case of successful spinal anesthesia and postoperative epidural analgesia in a patient with CCHS who underwent orthostatic surgery. Conclusions In patients with CCHS, anesthesia is used with the goal of minimizing respiratory depression to avoid prolonged mechanical ventilation. Regional anesthesia should be considered where appropriate. Continuous oxygen saturation and end-tidal carbon dioxide monitoring must be available.