Cesarean delivery with low-dose combined spinal epidural in a patient with congenital central hypoventilation syndrome: a case report

Congenital central hypoventilation syndrome (CCHS), also known as Ondine’s Curse, is an autonomic disorder resulting in an inadequate respiratory response to hypercapnia and hypoxia, especially during periods of decreased wakefulness. Patients with CCHS are particularly sensitive to the effects of anesthetic medications, with increased risk for intraoperative events including hypotension, bradycardia, and hypoxemia. The current literature on the anesthetic management for patients with CCHS mainly described the use of general anesthesia, with few case reports describing neuraxial anesthesia. To our knowledge, this is the first case describing the anesthetic care for cesarean delivery of a patient with CCHS, with low-dose combined spinal epidural anesthesia. The patient had two previous cesarean deliveries with spinal anesthesia, complicated by hypotension and syncope secondary to autonomic dysfunction and/or neural-mediated syncope. This case highlights low-dose combined spinal epidural as a possible anesthetic approach in patients with CCHS, reducing the risk of hypotension and respiratory depression.