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.

Dental care for patients who are unable to open their mouths.

There are a number of diseases and conditions that prevent the sufferer from adequately opening the mouth. The danger of inanition, malnutrition, chronic periodontal disease, caries, and abscessed teeth are very real to this population. Dental treatment issues include inadequate access to the oral cavity, inability to locally anesthetize mandibular posterior teeth, inability to gain access for traditional operative dentistry, and lack of clearance for most oral surgery procedures. The purpose of this article is to provide the reader with a discussion of the various conditions and then discuss the dental and anesthesia issues for this unique population.

Oral management in a patient with fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is an autosomal dominant disorder of connective tissue. FOP results in debilitating heterotopic ossification of the axial and appendicular skeleton. Permanent ankylosis of the temporomandibular joint (TMJ) is a common late finding, but is usually preceded by specific inciting trauma. Extreme care must be exercised when carrying out routine dental care to prevent soft tissue trauma. Oral health care for patients with FOP is usually complex. There are additional considerations when oral surgical procedures, such as extractions, are needed. Maintaining a balance between oral health and disease progression in these patients is challenging, as they frequently present with advanced oral disease. Fiber optic-assisted placement of the nasoendotracheal tube is the standard of care. Intramuscular injections including mandibular blocks must be avoided. Permanent fusion of the TMJ leads to malnutrition, inanition, and aspiration of food. In patients with complete TMJ ankylosis, dental extractions can be safely and effectively performed while accessing teeth from the buccal aspect. This approach has successfully resulted in decreased morbidity in these patients as well as others with limited oral opening.