Emergent airway management in a case of fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressiva (FOP), or Stone man syndrome, is rare and one of the most disabling genetic conditions of the connective tissue due to progressive extraskeletal ossification. It usually presents in the first decade of life as painful inflammatory swellings, either spontaneously or in response to trauma, which later ossify and lead to severe disability. Progressive spinal deformity including thoracolumbar kyphoscoliosis leads to thoracic insufficiency syndrome, increasing the risk for pneumonia and right sided heart failure. We present the airway management in a 22-year-old male, diagnosed with FOP with severe disability, who required urgent airway intervention as a result of respiratory failure from pnuemonia. Tracheostomy triggers ossification and consequent airway obstruction at the tracheostomy site and laryngoscopy triggers temporomandibular joint ankylosis. Therefore, awake fiber-optic endotracheal intubation is recommended in these patients. Use of an airway endoscopy mask enabled us to simultaneously maintain non-invasive ventilation and intubate the patient in a situation where tracheostomy needed to be avoided.