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Anesthetic Management of a Parturient with “Ondine’s Curse”
Abstract Number: SA-61
Abstract Type: Case Report/Case Series
Ondine’s Curse or Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder resulting from mutations in the PHOX2B gene, characterized by impaired ventilatory response to hypercapnia and hypoxemia (1). Voluntary breathing is intact while the patient is awake, but during periods of sleep there is no central control of ventilation resulting in alveolar hypoventilation (2). We report a case of a parturient presenting with CCHS for spontaneous vaginal delivery.
A 29 year old primigravida presented for consultation regarding anesthetic management for labor and delivery due to a history of CCHS. She had been managed since birth via tracheostomy with ventilator support needed only when sleeping. She denied any other medical problems and her pregnancy was uncomplicated.
Consultation involved a multidisciplinary approach to determine how the anesthesia, obstetric, and nursing team would manage the patient’s condition and ventilator requirement. The patient and her family expressed a desire for as normal a delivery process possible preferably on the labor and delivery unit.
After discussion including, obstetrics anesthesia, maternal-fetal medicine, nursing, respiratory therapy and critical care medicine, it became evident there were multiple systems-based challenges to overcome which would require an intensive care unit environment. Critical care nursing with intermittent mechanical ventilator support for her intended delivery and postpartum care would be necessary for adequate patient safety.
The patient presented at 37 weeks gestational age in active labor. She was admitted to our surgical intensive care unit where a labor and delivery nurse, as well as critical care nurse, were designated for her care.
We will discuss the anesthetic concerns in caring for a patient with CCHS, the anesthetic plan intended for her proposed vaginal delivery, an alternative plan for possible surgical intervention as well as possible concern for the newborn. We will also examine the multiple systems based challenges involving the care of this parturient while providing a safe but family centered care environment.
1. Visser, et al. Thoracic paravertebral block for awake breast surgery in a patient with congenital central hypoventilation syndrome (Ondine's Curse). Journal of Clinical Anesthesia. 2013; 25(7): 604-605.
2. Prottengeier, et al. Anesthesia for orphan disease. Eur J Anaesthesiol. 2014; 31: 338-340.