von Recklinghausen病 (NF1) を伴う骨格性下顎前突症に対して下顎枝垂直骨切り術を施行した1例

• Published in: 日本口腔外科学会雑誌 Vol. 69; no. 10; pp. 464 - 469
• Main Authors: 齊藤, 志都, 野上, 晋之介, 奥山, 喬介, 鈴木, 飛佳理, 森島, 浩允, 山内, 健介
• Format: Journal Article
• Language: Japanese
• Published: 公益社団法人 日本口腔外科学会 20.10.2023
• Subjects: レックリングハウゼン病，NF1; 下顎枝垂直骨切り術，IVRO; 顎変形症
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.69.464

NF1 is characterized by café-au-lait spots and neurofibromas and occurs in about 1 in 3000 people. This disease is often associated with various lesions, and it is necessary to evaluate the risk factors for surgery. In this report, we describe a case of NF1 treated with orthognathic surgery. The patient, a 42-year-old male, had been aware of facial asymmetry and malocclusion since his twenties and was referred to Tohoku University Hospital. His facial appearance was skeletal class Ⅲ , concave, and the chin was deviated to the right. He had right temporomandibular joint noise, with an opening of 54mm. CT showed that the right mandibular branch was buccolingually narrow and a diffuse neurofibroma was close to the posterior border of the right mandible. Difficulties associated with surgery included the narrow width of the mandibular branch, which makes splitting difficult if a sagittal splitting ramus osteotomy (SSRO) is chosen, and the possibility of vascular collapse and the formation of an arteriovenous aneurysm due to his medical history, so IVRO was chosen. He was operated on under general anesthesia and progressed well without postoperative bleeding. He was discharged 14 days after the surgery and has been stable for four years.