Abstract
The 22q11.2 deletion syndrome (SD22q11), known as Digeorge syndrome, has a wide phenotypic spectrum with more than 180 clinical signs described in the literature, related to both physical and behavioral aspects, which makes the diagnosis challenging in relation to the condition. The 22q11.2 microdeletion is detected only by molecular genetic techniques such as comparative hybridization of 2 DNA samples. A 13-year-old female patient diagnosed with SD22q11 was referred for specialized dental care for special patients. During anamnesis, heart disease; immunologic, hematologic, neurocognitive, psychiatric disorders; and craniofacial malformation were reported, characteristics consistent with the syndrome in question. Dental procedures, such as extractions and endodontic accesses, were made possible through the medical report of the analysis of systemic conditions, associated with periodic complementary exams. Neurologic management through the administration of carbamazepine and valproic acid was essential to reduce hallucinations while in the dental chair.
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