P049: PERIOPERATIVE MANAGEMENT AND PREEMPTIVE ECMO CANNULATION OF A PARTURIENT WITH CYSTIC FIBROSIS UNDERGOING CESAREAN DELIVERY
Danielle Rioux, MD; Reine Zbeidy, MD; Department of Anesthesiology, University of Miami, Jackson Memorial Hospital
Introduction: Physiologic changes of pregnancy and cystic fibrosis (CF) pathology provide a unique set of circumstances. Pulmonary disease accounts for the vast majority of morbidity and mortality associated with CF patients. These abnormalities create anesthetic challenges due to multiple organ systems being affected including the respiratory, gastrointestinal, cardiovascular, and genitourinary tracts, where patients present with chronic respiratory failure, pancreatic insufficiency, poor nutrition, and cardiac manifestations. One of the most important goals in anesthetic management is to prevent respiratory decompensation.
Case Description: A 21-year-old parturient with cystic fibrosis presented with multiple predictors of a poor outcome, including a severely decreased FEV1 meeting requirements for lung transplantation, on home oxygen and steroids, frequent pulmonary infections, diabetes mellitus, and pancreatic insufficiency. A multidisciplinary team was organized to devise an anesthetic plan. The decision was made for the patient to undergo preterm cesarean delivery at 34 weeks under neuraxial anesthesia with preemptive ECMO cannulation via bilateral femoral sheaths.
Results: The parturient delivered a healthy neonate with Apgar scores of 9 at 1, 5, and 10 minutes. The use of backup respiratory support therapy in the form of nasal cannula and subsequently Venturi mask were required during delivery, however intraoperative arterial blood gas sample demonstrated adequate oxygenation. Following delivery, postoperative pain was controlled without opioids, chest physiotherapy resumed, and bilateral femoral sheaths removed. On post-operative day 5 the patient no longer had oxygen requirements on a 6-minute walk test with a normal appearing CXR, and was discharged home.
Discussion: Anesthetic challenges are amplified in pregnant patients with cystic fibrosis, especially as more CF patients are living until reproductive age. An enlarged uterus in pregnancy displaces the diaphragm upward, which decreases total lung capacity and residual volume. Poor lung function may prohibit vaginal delivery, while intubation should be avoided if possible to prevent prolonged mechanical ventilation. Multidisciplinary planning is needed for successful outcome in these patients, including the option for preemptive ECMO cannulation.