Dental Management of Tatton-Brown-Rahman Syndrome: A Case Report

Tatton-Brown-Rahman syndrome (TBRS) is a rare autosomal dominant overgrowth disorder caused by pathogenic variants in the DNMT3A gene and characterized by intellectual disability, behavioral abnormalities, craniofacial dysmorphism, and systemic comorbidities. Dental manifestations and management strategies in affected individuals remain poorly documented. This report is aimed at describing the dental findings and multidisciplinary management of a pediatric patient with TBRS, highlighting comprehensive dental rehabilitation under general anesthesia (GA). An 11-year-old Saudi female diagnosed with TBRS presented for dental clearance prior to planned transcatheter atrial septal defect (ASD) device closure. The patient exhibited intellectual disability, autism spectrum disorder, severe communication difficulties, and definitely negative behavior, making routine dental care impossible. Clinical and radiographic examinations revealed multiple carious teeth, congenitally missing maxillary incisors, ectopic tooth eruption, abnormal root morphology, and short clinical crowns. Comprehensive dental rehabilitation, including restorative treatment, extraction, and preventive care, was successfully performed under GA following multidisciplinary medical consultation. Dental rehabilitation under GA can be safely and effectively performed in children with TBRS when careful preoperative assessment and interdisciplinary coordination are ensured.