Marfan syndrome is an autosomal dominant disorder of connective tissue primarily characterized with anomalies affecting the musculoskeletal system, the
cardiovascular system and the eyes. It has been suggested that early diagnosis of the syndrome is important, because of the risk of infective endocarditis. A
7-year-old female was referred to our clinic, with a chief complaint of dental crowding in the anterior region of mandible. It was observed that the patient needed
multiple treatments based on detailed clinical and radiographic examinations. The treatment was carried out with antibiotic prophylaxis an hour prior to her
appointment considering her profound caries. The patient was advised to visit regularly for follow up and she was referred for orthodontic evaluation.
Loeys-Dietz syndrome (LDS) is a connective tissue disease which develops due to the mutations in the genes synthesizing TFG-beta receptors. Hypertelorism, cleft palate with bifid uvula and arterial/aortic aneurysms are the 3 basic elements of the disease forming a triad. Considering oral and maxillofacial findings in individuals with Loeys-Dietz syndrome, the responsibility of dentist is very important both in diagnosis of syndrome and in treatment. In the literature, there has been no publication of a dental approach to LDS and it is expected that this publication may be a guide to dentists for the syndrome.
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