General anesthesia for dental procedures in patients with fibrodysplasia ossificans progressiva: a review of 42 cases in 30 patients.

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a rare genetic condition characterized by progressive heterotopic ossification of skeletal muscle and soft connective tissues, leading to progressive ankylosis of all joints of the axial and appendicular skeleton. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks.
METHODS: We conducted a retrospective chart review at 1 institution of patients with FOP who underwent general anesthesia (GA) for dental procedures.
RESULTS: Thirty patients underwent 42 general anesthetics. In 35 of 42 cases, GA was induced after the airway was secured by an awake fiberoptic intubation. In 4 of 42 cases, all of them pediatric, GA was first induced with maintenance of spontaneous ventilation, and the trachea was then intubated using a fiberoptic scope. In 2 cases, 1 adult and 1 pediatric, GA was first induced, and the trachea was then intubated using a GlideScope. In 1 case, the patient had a cuffed tracheostomy device in place that was accessed for GA. In 36 of 42 cases, the patients were discharged home on the same day as their dental procedure. No significant postoperative complications were encountered.
CONCLUSIONS: GA can be administered safely to patients with FOP for dental procedures with attention to perioperative and airway management using a multidisciplinary approach. An awake nasal fiberoptic intubation should be considered the first choice for airway management. Most patients can be discharged home on the same day as their dental procedure.