{"orpha":521127,"name":"Osteoradionecrosis of the mandible","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["MORN"],"definition":"A rare radiation-induced disorder characterized by exposed irradiated bone that fails to heal over a period of three months without evidence of persisting or recurrent tumor. Patients present with pain, dysesthesia, dysgeusia, fetor oris, trismus, ulceration or necrosis of the mucosa with exposure of necrotic bone, and local suppuration. Complications include pathological fractures, formation of intra- or extra-oral fistulae, and infection. MRI shows cortical destruction, abnormal bone marrow signal, and irregular contrast enhancement.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-521127/","orphanet_url":"https://www.orpha.net/en/disease/detail/521127","names":{"fr":{"name":"Ostéoradionécrose mandibulaire","definition":"Maladie rare induit par la radiothérapie, caractérisée par l'exposition de l'os irradié qui ne parvient pas à guérir sur une période de trois mois, en l'absence de toute tumeur persistante ou récurrente. Les patients présentent une douleur, une dysesthésie, une dysgueusie, une halitose, un trismus, une ulcération ou une nécrose de la muqueuse avec exposition de l'os nécrosé, et une suppuration locale. Les complications sont des fractures pathologiques, la formation de fistules intra- ou extra-orales et des infections. L'IRM révèle une nécrose corticale, un signal anormal de la moelle osseuse et un rehaussement de contraste irrégulier."},"de":{"name":"Osteoradionekrose der Mandibula","synonyms":["MORN"],"definition":"Die Osteoradionekrose der Mandibula ist eine seltene Störung des Unterkiefersknochens nach Behandlung mit hochenergetischer Strahlung. Typische klinische Merkmale sind freiliegender Kieferknochen, der über einen Zeitraum von drei Monaten nicht heilt, ohne Anzeichen eines persistierenden oder rezidivierenden Tumors. Die Patienten präsentieren sich mit Schmerzen, Dysästhesie, Dysgeusie, Fetor oris, Trismus, Ulzeration oder Nekrose der Schleimhaut mit freiliegendem nekrotischem Knochen und lokaler Eiterbildung. Komplikationen sind pathologische Frakturen, intra- oder extraorale Fistelbildung und Infektionen. Die MRT zeigt eine Zerstörung der Kortikalis, ein abnormes Knochenmarkssignal und eine unregelmäßige Kontrastmittelanreicherung."},"es":{"name":"Osteorradionecrosis de la mandíbula","synonyms":["MORN"],"definition":"Es un trastorno poco frecuente inducido por radiación caracterizado por hueso irradiado que no cicatriza durante un período de tres meses sin evidencia de tumor persistente o recurrente. Los pacientes presentan dolor, disestesia, disgeusia, halitosis, trismo, ulceración o necrosis de la mucosa con exposición de hueso necrótico y supuración local. Las complicaciones incluyen fracturas patológicas, formación de fístulas intra- o extraorales e infección. La RM muestra destrucción cortical, señal anómala en médula ósea y realce irregular del contraste."},"it":{"name":"Osteoradionecrosi della mandibola","synonyms":["MORN"],"definition":"Si tratta di una malattia rara indotta dalle radiazioni, caratterizzata dalla mancata guarigione, per un periodo di tre mesi, di un osso esposto alle radiazioni, in assenza di un tumore persistente o ricorrente. I pazienti presentano dolore, disestesia, disgeusia, alitosi, trisma, ulcerazione o necrosi della mucosa con esposizione dell'osso necrotico e suppurazione locale. Le complicanze comprendono le fratture patologiche, la formazione di fistole intra- o extraorali e le infezioni. La RMN evidenzia una distruzione corticale, anomalie del segnale del midollo osseo e un rinforzo irregolare dopo la somministrazione del mezzo di contrasto."},"nl":{"name":"Osteoradionecrose van mandibula","synonyms":["MORN","Osteoradionecrose van onderkaak"],"definition":"Een zeldzame aandoening door straling, gekarakteriseerd door blootgesteld bestraald bot dat niet is geheeld na een periode van drie maanden zonder bewijs van persisterende of recidiverende tumor. Patiënten presenteren zich met pijn, dysesthesie, dysgeusie, halitose (slechte adem), trismus, ulceratie of necrose van mucosa met blootstelling van necrotisch bot, en locale etterige ontsteking. Complicaties zijn onder meer pathologische fracturen, vorming van intra- of extra-orale fistels, en infectie. MRI toont corticale destructie, abnormaal signaal van beenmerg, en onregelmatige contrastversterking."},"pt":{"name":"Osteoradionecrose da mandíbula"},"pl":{"name":"Martwica popromienna żuchwy","synonyms":["MORN"]},"cs":{"name":"Osteoradionekróza mandibuly","synonyms":["MORN"]},"uk":{"name":"Остеорадіонекроз нижньої щелепи","definition":"A rare radiation-induced disorder characterized by exposed irradiated bone that fails to heal over a period of three months without evidence of persisting or recurrent tumor. Patients present with pain, dysesthesia, dysgeusia, fetor oris, trismus, ulceration or necrosis of the mucosa with exposure of necrotic bone, and local suppuration. Complications include pathological fractures, formation of intra- or extra-oral fistulae, and infection. MRI shows cortical destruction, abnormal bone marrow signal, and irregular contrast enhancement."},"zh":{"name":"下颌骨放射性骨坏死","definition":"一种罕见的放射线引起的疾病，其特征是暴露的受辐射骨在三个月内不能愈合，但没有证据表明肿瘤持续存在或复发。患者表现为疼痛、感觉迟钝、味觉异常、口臭、牙关紧闭、溃疡或黏膜坏死伴坏死骨外露及局部化脓。并发症包括病理性骨折、口内或口外瘘管的形成和感染。MRI显示皮质破坏、骨髓信号异常、不规则对比度增强。"},"tr":{"name":"Mandibulanın osteoradiyonekrozu"}},"xrefs":[{"source":"ICD-10","code":"K10.2","qualifier":"NTBT","icd_relation":"inclusion","validated":false},{"source":"ICD-11","code":"DA06.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"677862301"},{"source":"MONDO","code":"0033839","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1290728","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93419,"name":"Rare bone disease"},"parents":[{"orpha":399158,"name":"Osteonecrosis","classifications":[196,199]},{"orpha":521132,"name":"Radiation-induced disorder","classifications":[204]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}