DENTAL CARE TO PATIENTS WITH RARE CLINICAL FIDINGS OF GOLTZ SYNDROME—CASE REPORT

Goltz syndrome or focal dermal hypoplasia is a rare hereditary genetic disease of the dominant X chromosome. Multisystemic manifestations of a wide phenotypic variety with involvement of the maxillomandibular region are observed. A 6-year-old female patient with the syndrome sought a specialized dental service, presenting as main complaint the need for dental treatment for extensive carious lesions. Physical and intraoral examination showed delayed tooth eruption, agenesis, enamel hypoplasia, and atypical anatomy of the teeth as well as unusual clinical findings of the syndrome, such as abnormal tongue morphology and cleft lip and palate. Occlusal and panoramic radiographies were performed. The care plan began with health promotion, followed by instruction in oral hygiene for parents and children, prophylaxis, topical application of fluoride, restorative treatments, exodontia, and laser therapy. Management techniques such as positive reinforcement, voice control, say-show-making, and awards were used. Currently, the patient is being followed up.