著しい開口障害を呈したRett syndromeの1例

• Published in: 日本口腔外科学会雑誌 Vol. 59; no. 4; pp. 250 - 253
• Main Authors: 細原, 政俊, 式守, 道夫, 玄, 景華, 江原, 雄一, 田中, 四郎, 住友, 伸一郎
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.04.2013
• Subjects: Rett 症候群; 筋突起過形成症; 開口障害
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.59.250

Rett syndrome is found exclusively in young females, and is a progressive neurologic disease characterized by mental retardation, repeated hand-rubbing behavior, and related symptoms. We report a case of trismus that was treated in a patient with Rett syndrome. A 14-year-old girl (height, 120 cm; weight 20 kg) presented at Asahi University Hospital in December 2006 because of trismus. Her medical history included epilepsy, patent ductus arteriosus, microcephaly, hypertension, and suspected Rett syndrome since 6 years of age. Trismus had been present for several years. Examination revealed mental retardation, scoliosis, manual stereotyped behavior, developmental disease of the feet, and difficulty in independently walking. Her face was symmetric, with a developed mandible angle. Flaredout maxillary front teeth with attrition and jaw opening of 15 mm were confirmed. Radiologic examination showed hypertrophy of both coronoid processes and slight adhesion in the left temporomandibular joint. Hypertrophy of both coronoid processes was diagnosed clinically, and both were removed. The intraoperative jaw opening was 45 mm. The jaw opening has been maintained at 32 mm as of 5 years 9 months after surgery. The case was considered to involve trimus caused by coronoid process enlargement due to development of the masseter and temporal muscles secondary to bruxism.