Treatment of dental caries in a patient with Joubert syndrome without the use of sedatives: A case study.

BACKGROUND

Joubert syndrome (JS) is a rare autosomal recessive inherited ciliopathy caused by gene mutation. Manifestations can include intermittent dyspnea, apnea, ataxia, and other nervous system abnormalities.

CASE PRESENTATION: The patient was a 21-year-old female with JS, severe intellectual disability, cerebral palsy, and epilepsy. Dental caries in both mandibular lower first molars was diagnosed by a local dentist, and the patient was referred to us for further treatment. Although her oral hygiene was good, the dental caries had reached the vicinity of the dental pulp. The caries had developed symmetrically on both first molars and was completely covered with the gingiva. As she was hesitant to receive treatment without anxiolysis, we considered using general anesthesia or intravenous sedation. However, we were recommended against it by the patient's family doctor because of the risk of apneic episodes in JS. Accordingly, dental treatment was performed over multiple days through physical behavior adjustment, while providing oxygenation (3 L/min) in preparation for an apneic episode. The dental treatment was successfully completed with a good postoperative outcome.

CONCLUSIONS

Dental treatment can be safely performed in patients with JS through physical behavior adjustment, thereby minimizing the risk of an apnea attack.