Korean J Anesthesiol.  2019 Aug;72(4):375-380. 10.4097/kja.d.18.00118.

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

• Choi Y
• Lee S
• Lee J
• Woo S

• KMID: 2458323
• DOI: http://doi.org/10.4097/kja.d.18.00118

Abstract

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.

Keyword

Autonomic dysregulation; Congenital central hypoventilation syndrome; Hypoxemia; Ondine's curse; Spinal anesthesia

MeSH Terms

Analgesia, Epidural*
Anesthesia
Anesthesia, Conduction
Anesthesia, Spinal*
Anoxia
Carbon Dioxide
Humans
Hypoventilation*
Oxygen
Respiration, Artificial
Respiratory Insufficiency
Ventilation
Carbon Dioxide
Oxygen