開口障害を伴った進行性骨化性線維異形成症の1例
Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary disease characterized by heterotopic ossification of skeletal muscles, tendons, and ligaments. In the oral and maxillofacial region, FOP leads to eating disorders caused by a limited range of mouth opening. At a late stage, respiratory...
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| Published in | 日本口腔外科学会雑誌 Vol. 61; no. 4; pp. 241 - 245 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
社団法人 日本口腔外科学会
20.04.2015
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0021-5163 2186-1579 |
| DOI | 10.5794/jjoms.61.241 |
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| Abstract | Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary disease characterized by heterotopic ossification of skeletal muscles, tendons, and ligaments. In the oral and maxillofacial region, FOP leads to eating disorders caused by a limited range of mouth opening. At a late stage, respiratory function is impaired by limited movement of the thorax. We reported a rare case of FOP with trismus and discuss the related literature. A 28-year-old woman was referred to our hospital because of trismus in December 2011. She had heterotopic ossification in the left scapula and was given a diagnosis of FOP at the age of 17 years. Extraoral examination showed temporary pain extending from the left temporomandibular joint to the buccal region when she opened the mouth. A maximal mouth opening of 3 mm was confirmed. Intraoral examination showed an inflammatory reaction at the gingiva of the maxillary left third molar. Magnetic resonance imaging showed high intensity of the left lateral pterygoid muscle. Nonsteroidal anti-inflammatory drugs and injectable bisphosphonates were administered, followed by mouth opening training. Her maximal mouth opening markedly improved to 22 mm 1 year after initial presentation, without any recrudescence. |
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| AbstractList | Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary disease characterized by heterotopic ossification of skeletal muscles, tendons, and ligaments. In the oral and maxillofacial region, FOP leads to eating disorders caused by a limited range of mouth opening. At a late stage, respiratory function is impaired by limited movement of the thorax. We reported a rare case of FOP with trismus and discuss the related literature. A 28-year-old woman was referred to our hospital because of trismus in December 2011. She had heterotopic ossification in the left scapula and was given a diagnosis of FOP at the age of 17 years. Extraoral examination showed temporary pain extending from the left temporomandibular joint to the buccal region when she opened the mouth. A maximal mouth opening of 3 mm was confirmed. Intraoral examination showed an inflammatory reaction at the gingiva of the maxillary left third molar. Magnetic resonance imaging showed high intensity of the left lateral pterygoid muscle. Nonsteroidal anti-inflammatory drugs and injectable bisphosphonates were administered, followed by mouth opening training. Her maximal mouth opening markedly improved to 22 mm 1 year after initial presentation, without any recrudescence. |
| Author | 高橋, 哲 櫻井, 拓真 森山, 雅文 中村, 誠司 宮本, 郁也 吉賀, 大午 |
| Author_xml | – sequence: 1 fullname: 高橋, 哲 organization: 東北大学大学院歯科研究科口腔病態外科学講座顎顔面口腔外科学分野 – sequence: 1 fullname: 宮本, 郁也 organization: 九州歯科大学生体機能学講座口腔内科学分野 – sequence: 1 fullname: 櫻井, 拓真 organization: 九州歯科大学生体機能学講座口腔内科学分野 – sequence: 1 fullname: 中村, 誠司 organization: 九州大学大学院歯学研究院口腔顎顔面病態学講座顎顔面 – sequence: 1 fullname: 吉賀, 大午 organization: 九州歯科大学生体機能学講座顎顔面外科学分野 – sequence: 1 fullname: 森山, 雅文 organization: 九州大学大学院歯学研究院口腔顎顔面病態学講座顎顔面 |
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| References | 13) 松崎 哲, 片桐岳信, 他：進行性骨化性線維異形成症患者の歯科治療. 歯界展望 115: 556-557, 2010. 8) Michael P W, Deborah W, et al : Fibrodysplasia ossificans progressive. middle-age onset of heterotopic ossfication from a unique missense mutation (c.974G>c, p.G325A) in ACVR1. J Bone Miner Res 27: 729-737, 2012. 11) 片桐岳信：進行性骨化性線維異形成症 (FOP) の病院と病態. 内分泌・糖尿病科 27: 270-276, 2008. 16) Liu JX, Hu R, et al : General anesthesia in fibrodysplasia ossificans progressive. Int J Clin Exp Med 7: 1474-1479, 2014. 9) Shore EM, Xu M, et al : A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressive. Nat Genet 38: 525-527, 2006. 1) Nakashima Y, Haga N, et al : Deformity of the great toe in fibrodysplasia ossficans progressive. J Orthop Sci 15: 804-809, 2010. 7) 古谷博和：進行性骨化性線維異形成症の病態と治療への試み. IRYO 65: 193-198, 2011. 5) 大湾一郎：FOPの臨床的所見と具体的事例. 難病と住宅ケア13: 43-46, 2008. 10) 片桐岳信：進行性骨化性線維異形成症 (FOP) の発症メカニズムの解明と治療法. 日未熟児新生児会誌 22: 30-32, 2010. 14) 松崎 哲：難病進行性骨化性線維異形成症 (FOP) の患者さんへの口腔ケアにおける歯科衛生士の役割. デンタルハイジーン 30: 1151-1153, 2010. 15) Kaplan FS, Pignolo RJ, et al : From mysteries to medicines. drug development for fibrodysplasia ossificans progressive. Expert Opin Orphan Drugs 1: 637-649, 2014. 4) Vashisht R and Prosser D: Anesthesia in a child with fibrodysplasia ossificans progressiva. Paediatr Anaesth 16: 684-688, 2006. 2) Smith R : Fibrodysplasia (myositis) ossificans progressive. clinical lessons from a rare disease. Clin Orthop 346: 7-14, 1998. 6) Kitterman JA, Kantanie S, et al : Iatrogenic harm caused by diagnostic errors in fibrodysplasia ossificans progressive. Pediatrics 116: 654-661, 2005. 17) Fukuda T, Kohda M, et al : Constitutively activated ALK2 and increased SMAD1/5 cooperatively induce bone morphogenetic protein signaling in fibrodysplasia assificans progressiva. J Biol Chem 284: 7149-7156, 2009. 12) Chakkalakal SA, Zhang D, et al : An Acvr1 R206H knock-in mouse has fibrodysplasia ossificans progressiva. J Bone Miner Res 27: 1746-1756, 2012. 3) Connor JM and Evans DA: Fibrodysplasia ossificans progressiva. the clinical features and natural history of 34 patients. J Bone Joint Surg Br 64: 76-83, 1982. |
| References_xml | – reference: 5) 大湾一郎：FOPの臨床的所見と具体的事例. 難病と住宅ケア13: 43-46, 2008. – reference: 1) Nakashima Y, Haga N, et al : Deformity of the great toe in fibrodysplasia ossficans progressive. J Orthop Sci 15: 804-809, 2010. – reference: 9) Shore EM, Xu M, et al : A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressive. Nat Genet 38: 525-527, 2006. – reference: 10) 片桐岳信：進行性骨化性線維異形成症 (FOP) の発症メカニズムの解明と治療法. 日未熟児新生児会誌 22: 30-32, 2010. – reference: 2) Smith R : Fibrodysplasia (myositis) ossificans progressive. clinical lessons from a rare disease. Clin Orthop 346: 7-14, 1998. – reference: 13) 松崎 哲, 片桐岳信, 他：進行性骨化性線維異形成症患者の歯科治療. 歯界展望 115: 556-557, 2010. – reference: 6) Kitterman JA, Kantanie S, et al : Iatrogenic harm caused by diagnostic errors in fibrodysplasia ossificans progressive. Pediatrics 116: 654-661, 2005. – reference: 7) 古谷博和：進行性骨化性線維異形成症の病態と治療への試み. IRYO 65: 193-198, 2011. – reference: 4) Vashisht R and Prosser D: Anesthesia in a child with fibrodysplasia ossificans progressiva. Paediatr Anaesth 16: 684-688, 2006. – reference: 11) 片桐岳信：進行性骨化性線維異形成症 (FOP) の病院と病態. 内分泌・糖尿病科 27: 270-276, 2008. – reference: 16) Liu JX, Hu R, et al : General anesthesia in fibrodysplasia ossificans progressive. Int J Clin Exp Med 7: 1474-1479, 2014. – reference: 17) Fukuda T, Kohda M, et al : Constitutively activated ALK2 and increased SMAD1/5 cooperatively induce bone morphogenetic protein signaling in fibrodysplasia assificans progressiva. J Biol Chem 284: 7149-7156, 2009. – reference: 8) Michael P W, Deborah W, et al : Fibrodysplasia ossificans progressive. middle-age onset of heterotopic ossfication from a unique missense mutation (c.974G>c, p.G325A) in ACVR1. J Bone Miner Res 27: 729-737, 2012. – reference: 3) Connor JM and Evans DA: Fibrodysplasia ossificans progressiva. the clinical features and natural history of 34 patients. J Bone Joint Surg Br 64: 76-83, 1982. – reference: 12) Chakkalakal SA, Zhang D, et al : An Acvr1 R206H knock-in mouse has fibrodysplasia ossificans progressiva. J Bone Miner Res 27: 1746-1756, 2012. – reference: 15) Kaplan FS, Pignolo RJ, et al : From mysteries to medicines. drug development for fibrodysplasia ossificans progressive. Expert Opin Orphan Drugs 1: 637-649, 2014. – reference: 14) 松崎 哲：難病進行性骨化性線維異形成症 (FOP) の患者さんへの口腔ケアにおける歯科衛生士の役割. デンタルハイジーン 30: 1151-1153, 2010. |
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| SubjectTerms | 摂食障害 異所性骨化 進行性骨化性線維異形成症 開口障害 |
| Title | 開口障害を伴った進行性骨化性線維異形成症の1例 |
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