(1543) Emergent Airway Management and Vascular Access in Fibrodysplasia Ossificans Progressiva

Introduction: Obtaining vascular access and securing an airway are always priorities in cases of shock. Here we present a challenging case in which efforts to stabilize a decompensating patient were compromised by the presence of an extremely rare connective tissue disease and distorted anatomy.

Description: A 40-year-old female with large, recurrent ovarian cysts presented to the ICU in suspected hemorrhagic shock after an elective CT-guided cyst drainage. Past medical history was also significant for fibrodysplasia ossificans progressiva, a rare genetic disorder in which fibrous connective tissue ossifies into bone. Due to this condition, her hips were in a fixed flexion, her upper extremities were contracted, and she had significant lymphedema. She additionally had limited range of neck motion, and her jaw was in a fixed position with minimal mouth opening. When attempting vascular access to administer emergent blood products for a suspected bleed, the femoral veins were unable to be safely visualized on ultrasound due to edema and the flexed position of the hips. When attempting to place the patient in Trendelenburg position to engorge the internal jugular veins, the patient had intolerable hip pain due to the fixed nature of the joints. Subclavian vein cannulation was not attempted due to chest wall calcifications overlying the veins. Her only option for emergent vascular access was an intraosseous (IO) cannula, thus placed in the humerus. Once resuscitated, she was taken for CT scans and found to have active intra-abdominal bleeding. She was eventually intubated via awake fiberoptic intranasal approach given limited neck and mandible mobility. She subsequently underwent laparotomy and bleeding was controlled.

Discussion: Although rare, FOP causes anatomic challenges with airway and vascular access that can be seen in more common conditions like cerebral palsy and neck cancer. IO cannulation is fast and requires minimal changes to patient position. Fiberoptic intubation (FOI) can achieve airway management with minimal mouth or neck movement. However, it is technically difficult and often only performed by anesthesiologists. Critical care physicians should be prepared for such airways with skills to perform either FOI or cricothyrotomy in emergent settings.