遺伝性血管性浮腫の小児に対して下顎の含歯性嚢胞摘出術を行った1例

• Published in: 日本口腔外科学会雑誌 Vol. 59; no. 3; pp. 192 - 196
• Main Authors: 山城, 正司, 鵜澤, 成一, 山口, 聰, 原田, 清, 園田, 格
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.03.2013
• Subjects: C1インヒビター; 喉頭浮腫; 遺伝性血管性浮腫
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.59.192

We report the case of an 8-years-old child with hereditary angioedema who required oral surgery. Hereditary angioedema (HAE) is a rare autosomal dominant hereditary disease caused by a functional disorder of C1 inhibitor (C1-INH) or a low level of C1- INH protein. HAE is characterized by repeated localized edema of submucocutaneous tissues. Attacks are caused by trauma, drugs, infection, or mental stress. Dental treatment and oral surgery in patients with HAE can trigger fatal laryngeal edema. To prevent angioedema attacks, we injected C1-INH before surgery in a child with HAE. She underwent tooth extraction and cystectomy of the left side of the mandible under general anestethia. No HAE-related complications occurred during or after surgery, and perioperative management went well.