Behavioral and dental management of a patient with Tatton-Brown-Rahman syndrome: Case report

Objective: To present a clinical case of a patient with Tatton-Brown-Rahman syndrome, to provide evidence of the importance of supplying patients with appropriate dental care, emphasizing in behavioral management.

Study design: Clinical case report.

Results: This 7-year-old child, had a history of persistent ductus arteriousus, autism spectrum behavior, language disorders, dyslalias, hearing disorder, hypotonic musculature, and joint hyperlaxity. The main facial and oral diagnoses were dolichocephaly, convex profile, atypical swallowing, mixed breathing, class II malocclusion, mandibular retrognathism, maxillary prognathism, caries lesions, and biofilm associated gingivitis. A comprehensive treatment was carried out from the stage of adaptation to dental care, control of dental biofilm, motivation, and teaching of oral hygiene with appropriate strategies to the child's age and cognitive abilities. Also, resin restorations, habits management and malocclusion with the use of the modified upper and lower Sanders orthopedic device. The child began with a definitely negative behavior at dental appointments, and evolved to negative on Frankl's Behavior Rating Scale.

Conclusion: Dentists must manage behavior management protocols, in order to avoid situations of rejection of treatment in patients with TBRS, and thus avoid sedation or general anesthesia. Prevention is the priority for these patients supported by recreational-educational strategies.

Keywords: DNMT3A; Tatton-Brown-Rahman Syndrome; behavioral management; dental treatment; intellectual disability; overgrowth.