OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME

Abstract

Introduction: Robinow syndrome is a rare genetic disorder of skeletal development. It is characterized by short stature, facial dysmorphisms and orodental anomalies, underdeveloped genitalia, mesomelic brachymelia. Case Report: A 6 year old girl with Robinow Syndrome was referred in the Center for Formation of Human Resources Specialized in Dental Care to Special Needs Patients (Ribeirão Preto, São Paulo, Brazil) for evaluation. Medical history investigation and clinical examination were observed in short stature, facial dimorphism and hypoplastic genitalia. Orofacial findings showed retrognathism, a mid-line hemangioma
approximately 1 cm wide with upturned borders, bifid tongue, ankyloglossia, general gingival hypertrophy, deep bite and mild tooth crowding. Cardiac abnormality and neuromotor developmental delay consisted of systemic manifestations present which demanded individualized dental care. The dental treatment consisted of preventive and restoratives procedures to adequate the oral health condition of the patient and orthodontic treatmentwas planned. The patient has been in follow-up for six years. Conclusion: Orofacial anomalies and other alterations found in the present case contributed to complement the orofacial
findings described in the literature and to assist in diagnosis of the syndrome. In this case, amultiprofessional team and integral treatment were essential to rescue oral health and improvelife quality of the patient.