OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
Journal Title: Revista Científica do CRO-RJ / Rio de Janeiro Dental Journal - Year 2019, Vol 4, Issue 2
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 clinicalexamination 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 andother 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.
Authors and Affiliations
Marina Fernandes Sena, Paôla Caroline da Silva Mira, Thais Tedeschi dos Santos, Carolina Paes Torres, Francisco Wanderley Garcia Paula Silva, Fábio Lourenço Romano, Sandra Maria Garcia da Silveira, Karina Mayra de Queiroz Brito, Alexandra Mussolino de Queiroz
Keywords
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