436 - Dental Management of a Child with TANGO2-Related Metabolic Disorder

TANGO2-related metabolic encephalopathy and arrhythmias is a rare genetic disorder resulting from variations in the TANGO2 gene. Clinical manifestations include metabolic crises characterized by rhabdomyolysis, cardiac arrhythmias, neurodegeneration, and hypoglycemia. This presentation discusses a 4-year-old female who presented to Geisinger Medical Center with severe early childhood caries and acute situational anxiety to the dental setting. The patient’s medical history is significant for gross motor delay, microcephaly, hypotonia, seizures, and homozygous deletions within the TANGO2 gene in the 22q11.2 region. Due to the patient’s age and medical history, full mouth oral rehabilitation under general anesthesia was the selected treatment modality. This case report details the clinical findings associated with TANGO2-related disease, medical management of our patient for general anesthesia, as well as comprehensive dental treatment rendered.