C43-22 Staying Ahead of the Curve: Multidisciplinary Management of Pregnancy With Acute-on-Chronic Respiratory Failure From Multiple Pterygium Syndrome and Kyphoscoliosis

Introduction Multiple pterygium syndrome (MPS) is a rare genetic disorder characterized by skin webbing, contractures, and kyphoscoliosis that can result in restrictive lung disease and chronic respiratory failure. Pregnancy in restrictive lung disease is uncommon and challenging, as the physiologic changes of pregnancy can worsen pulmonary mechanics and respiratory failure. We present a case of pregnancy in restrictive lung disease from MPS, where acute-on-chronic respiratory failure was successfully managed with a multidisciplinary approach involving maternal-fetal medicine, pulmonary and critical care, anesthesiology, and neonatology. Case A 29-year-old female, gravida 1, with restrictive lung disease (forced vital capacity 0.7L, 26% predicted; diffusing capacity of the lung for carbon monoxide 4.84 mL/min/mmHg, 26% predicted; total lung capacity 1.61L, 40% predicted) and severe kyphoscoliosis from MPS on home oxygen, and obstructive sleep apnea on nocturnal bi-level positive pressure ventilation (BPAP) presented at 22 weeks gestation with dyspnea, presyncope, and worsening hypoxemia. She was admitted to the intensive care unit for acute-on-chronic hypoxemic and hypercarbic respiratory failure requiring high-flow supplemental oxygen and increased inspiratory pressures with nocturnal BPAP. In planning for possible maternal decompensation requiring extracorporeal support, computed tomography angiography confirmed bifemoral cannulation was anatomically possible. After extensive and repeated multidisciplinary discussions, the decision was made to offer venovenous extracorporeal membrane oxygenation as a bridge to maternal recovery only if respiratory failure could not be managed with conventional mechanical ventilation strategies. Ultimately, a scheduled cesarean delivery was performed at 27 weeks 3 days under spinal anesthesia with BPAP. Maternal postpartum course was uncomplicated and she was discharged home on postpartum day five on her baseline oxygen. Neonatal course included anticipated prolonged stay due to gestational age without other complications of prematurity. Discussion Limited data are available to guide management of pregnant patients with severe restrictive lung disease. A multidisciplinary approach to delivery timing, route, anesthesia modality, ventilatory support, and contingency planning is essential. In this case, shared decision-making also played a role in delivery timing, balancing maternal respiratory status with fetal survival benefit of delivery after 26-28 weeks. Neuraxial anesthesia may be limited by kyphoscoliosis in this patient population, however, if feasible, avoids the need for general anesthesia. Intubation carries greater risk in pregnancy, and while peripartum BPAP is generally well tolerated, perioperative use requires planning. As pregnant patients with restrictive lung disease face a high risk of respiratory decompensation, individualized planning should include discussions regarding pre-conception counseling and contingency planning in the peripartum period. This abstract is funded by: none