Case report: dental care approaches for a pediatric patient with Joubert syndrome

Background: Most dental professionals, including students, find it challenging to provide care to patients with particular medical needs, particularly in cases of rare syndromes. Methods: A 6-year-old girl with Joubert syndrome (JS) scheduled an appointment at our pediatric dentistry clinic. JS is a rare, genetically heterogeneous autosomal recessive disorder, which manifests as a neuropathological illness of the cerebellum and brainstem, along with the involvement of several organs. Various nonpharmacological interventions for behavioral management were offered by our dental office to meet her dental needs, including early morning appointments and minimizing the treatment duration to tackle her short attention span. The “tell, show and do” technique was advocated through the use of simple words and short sentences to facilitate slow language development. Due to her mild hyperactivity, she was appreciated for good behavior and rewarded with short breaks during dental treatment session. Results: These interventions resulted in a positive outcome, facilitated by the use of potent local anesthetics and strategies to maintain her attention, coupled with rewarding tactics. Maintaining the patient under local anesthesia, we achieved a positive outcome in the form of complete dental care through adequate placement of stainless-steel crowns, composite restoration, pulp therapy, and extraction. Conclusions: Managing patients with specific healthcare needs requires collaboration and consent from the patient’s physician, as well as a multidisciplinary approach. Recognizing oral health issues and emergency protocols before initiating dental treatment is crucial.

Dental treatment; Joubert syndrome; Molar tooth appearance; Bat wing appearance; Case report

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