(1033) Bridge to Extubation: Extracorporeal Membrane Oxygenation in Klippel-Feil Syndrome

Introduction: Klippel-Feil syndrome (KFS) is a rare congenital bone disorder characterized by cervical spine fusion. In patients with the syndrome, limited head flexion and extension pose unique challenges for airway management. Our case describes the use of extracorporeal membrane oxygenation (ECMO) as a bridge to tracheal extubation in a patient with KFS and challenging anatomy for emergent reintubation or placement of a surgical airway.

Description: The patient is a 37-year-old male with a past medical history of KFS with fusion of thoracic and cervical vertebrae, kyphoscoliosis with restrictive lung disease, previous jaw surgery, and congenital aortic valve stenosis who underwent a redo aortic valve replacement, aortic root enlargement, and replacement of ascending aorta. Before induction of anesthesia, the airway was successfully secured via awake fiberoptic intubation with flexible bronchoscopy. The patient's postoperative course was complicated by difficult weaning from mechanical ventilation. Exit strategies for failed extubation were limited; CT head and neck demonstrated a neck circumference too large for safe placement of a surgical airway. On postoperative day four, the patient was electively placed on venous-venous ECMO and safely extubated. After intense pulmonary rehabilitation, the patient was successfully liberated from ECMO on postoperative day seven.

Discussion: ECMO has many applications for patients with heart or lung disease and is often used as a lifesaving emergent procedure. The use of ECMO has expanded in recent years and should be considered in patients with severe airway compromise and "at-risk" tracheal extubation. In our patient with KFS and restrictive pulmonary disease, liberation from mechanical ventilation was difficult, and the ability to oxygenate and ventilate after tracheal extubation was uncertain. Due to unfavorable anatomy, the patient was not a candidate for a surgical airway, and the risk of an emergency reintubation was prohibitively high. The use of venovenous ECMO allowed for safe extubation. A multidisciplinary approach focused on intense pulmonary rehabilitation was crucial for gradual weaning from ECMO and discharge from the intensive care unit.