JAPANESE JOURNAL OF ORAL & MAXILLOFACIAL SURGERY

Vol. 53 No. 9       2007

ISSN: 0021-5163      UBIC: 151-J

ABSTRACT

Marfan´s syndrome is a congenital disorder that affects connective tissue. It is characterized by arachnodactyly, aortic incompetence, mitral valve prolapse, and dissecting aneurysm throughout the body. Patients with this syndrome show craniofacial abnormalities such as a high palatal vault, cleft palate, mandibular prognathism, malalignment, and morphological abnormalities of the teeth. We report a case of jaw deformity associated with Marfan's syndrome that was treated by Le Fort 1 osteotomy and sagittal splitting ramus osteotomy. The patient was a 29-year-old man with complications throughout his entire body, including hardness of hearing, pneumothorax, aortic valve enhancing syndrome, and aortic regurgitation. We therefore closely consulted with his heart surgeon and anesthesiologist before the operation. We performed surgery, taking me following precautions: (1) Antibiotics were administered preoperatively to prevent bacterial endocarditis. (2) Anticoagulants were discontinued to reduce the risk of hemorrhage. (3) An appropriate circulatory volume was maintained to reduce the burden on the circulatory organs. (4) An excessive rise in airway pressure was avoided to prevent pneumothorax. An excellent result was obtained, with no complications. Key words: Marfan's syndrome, Le Fort I osteotomy, sagittal splitting ramus osteotomy, orthognathic surgery.

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