ORAL MANIFESTATIONS AND CRANIOFACIAL CHARACTERISTICS OF HUTCHINSON-GILFORD PROGERIA SYNDROME: A CASE REPORT

Abstract

Introduction: Hutchinson-Guilford progeria syndrome (HGPS) is a rare genetic disease with a characteristic phenotype of premature aging in young children caused by a mutation in the LMNA gene and consequent accumulation of progerin in the cell. Aim: Describe oral manifestations of Hutchinson-Guilford progeria
syndrome. Case Report: This is a case report of a six-year-old female patient with Hutchinson-Guilford Progeria syndrome. The physical examination revealed skin atrophy, lipodystrophy, hair rarefaction, prominent blood vessels of the scalp, craniofacial disproportion, perioral cyanosis and enlarged knee joints. The intraoral exam revealed limited mouth opening, mixed dentition with normal tooth anatomy and anteroinferior crowding. The eruption sequence and chronology were abnormal. The treatment plan included professional prophylaxis, the topical application of fluoride as well as both oral hygiene and dietary
counselling. Monitoring the development of dentition and an early and timely dental intervention contributed to the maintenance of child’s oral health. Conclusion: Early clinical and educational interventions can help patients with HGPS maintain adequate oral health status and improve their quality of life.