Ｗｉｌｌｉａｍｓ症候群患者に発生した下顎骨嚢胞の１例

Abstract

Williams' syndrome is a congenital disease which mainly shows various heart disorders, the unique face and undergrowth of the mind or the body. In addition this disease shows some disorders of the mouth, for example, hyperplasia, abnormal arrangement of the teeth and high palate.We experienced a case of mandibular cyst in the patient diagnosed as Williams' syndrome, and describe outline of this case.The patient was a 10-year-old male. He visited our hospital with the chief complaint of the swelling of gingiva around 6. At birth he showed the unique face, but the chromosome examination indicated normalcy.The general examination revealed short height and the unique so called, elfin face. Oral examination revealed diffuse swelling of buccal gingiva around 6 with no fluctuation, no spontaneous pain or oppsessive pain. Moreover it revealed many caries, abnormal teeth eruption, tooth defects and high palate.X-ray findings revealed a discrete radiolucent picture about pigeon egg size around impacted 6 impacted supernumerary teeth and the lack of dental germ.The echocardiogram and cardiac catheterization examinations revealed valvular aortic stenosis, but supra-valvular aortic stenosis was not observed.Concerning heart disorders, we judged it better to observe the progress. The cyst was extirpated completely under local anesthesia for the purpose of preventing pathologic fracture of the mandible.The progress is favorable with no abnormality.