{"orpha":284460,"name":"Acute annular outer retinopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AAOR"],"definition":"A rare, acquired retinal disorder characterized by unilateral, acute onset, rapidly progressive visual field loss. Sometimes patients have photopsia and complain of floaters. Typical ophthalmoscopic finding is a unilateral, yellowish-white annular intraretinal line, splitting the retinal field to affected outer retina with thinning, and normal retina. Gradual spontaneous visual recovery has been observed.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-284460/","orphanet_url":"https://www.orpha.net/en/disease/detail/284460","names":{"fr":{"name":"Rétinopathie externe annulaire aiguë","synonyms":["AAOR"],"definition":"Maladie rétinienne rare et acquise caractérisée par une perte unilatérale, aiguë et rapidement progressive du champ visuel. Les patients présentent parfois une photopsie et se plaignent de la présence de corps flottants. L'observation ophtalmoscopique typique est une ligne intrarétinienne unilatérale, de coloration blanche-jaunâtre, divisant le champ rétinien en une rétine externe affectée et amincie et une rétine normale. Une récupération visuelle progressive et spontanée a été observée."},"de":{"name":"Retinopathie, akute äußere ringförmige","synonyms":["AAOR"]},"es":{"name":"Retinopatía externa anular aguda","synonyms":["AAOR"],"definition":"Es un trastorno retinal adquirido poco frecuente caracterizado por una pérdida unilateral de campo visual, de inicio agudo y rápidamente progresiva. En ocasiones, los afectados presentan fotopsia y refieren \"partículas flotantes\". El hallazgo oftalmoscópico típico es una línea intrarretinal anular unilateral, de color blanco amarillento, dividiendo el campo retiniano en retina externa afectada con adelgazamiento y retina normal. Se ha observado recuperación visual gradual espontánea."},"it":{"name":"Retinopatia esterna anulare acuta","synonyms":["AAOR"],"definition":"La retinopatia esterna anulare acuta è una retinopatia acquisita rara, caratterizzata da perdita monolaterale del campo visivo ad esordio acuto e progressione rapida. Occasionalmente i pazienti presentano fotopsia e miodesopsie. Il segno oculare caratteristico è la presenza di una linea anulare intraretinica bianco-giallastra monolaterale, che suddivide il campo retinico nella retina esterna interessata, che è assottigliata, e nella retina normale. Sono stati documentati casi di recupero graduale spontaneo della vista."},"nl":{"name":"Acute ringvormige perifere retinopathie","synonyms":["AAOR","Acute annulaire perifere retinopathie"],"definition":"Een zeldzame, verworven aandoening van retina, gekarakteriseerd door unilateraal, acuut aanvangend, snel progressief gezichtsveldverlies. Soms hebben patiënten fotopsie en klagen ze over myodesopsie. De typische oftalmoscopische bevinding is een unilaterale, geelwitte, ringvormige intraretinale lijn die het netvlies verdeeld in een getroffen perifere retina met verdunning, en normaal netvlies. Gradueel spontaan visueel herstel werd reeds waargenomen."},"pt":{"name":"Retinopatia externa anular aguda"},"pl":{"name":"Ostra zewnętrzna retinopatia pierścieniowa","synonyms":["AAOR"]},"cs":{"name":"Akutní anulární zevní retinopatie","synonyms":["AAOR"]},"uk":{"name":"Гостра кільцева зовнішня ретинопатія","synonyms":["AAOR"],"definition":"A rare, acquired retinal disorder characterized by unilateral, acute onset, rapidly progressive visual field loss. Sometimes patients have photopsia and complain of floaters. Typical ophthalmoscopic finding is a unilateral, yellowish-white annular intraretinal line, splitting the retinal field to affected outer retina with thinning, and normal retina. Gradual spontaneous visual recovery has been observed."},"zh":{"name":"急性环状外视网膜病变","synonyms":["AAOR"],"definition":"一种罕见的获得性视网膜病，特征为单侧、急性发作、快速进行性视野丧失。有时病人有闪光感，并诉有漂浮物。典型眼底镜检查结果呈单侧黄白色视网膜环状线，使视网膜视野分裂为受影响的变薄的外视网膜和正常视网膜。也可观察到逐渐自发性视力恢复。"},"tr":{"name":"Akut anüler dış retinopati","synonyms":["AAOR"]}},"xrefs":[{"source":"ICD-10","code":"H35.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B71.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0017299","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5191002","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":716432,"name":"Rare isolated progressive predominantly macular disorder","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":12.0,"area":"Worldwide","status":"Validated","source":"16181293[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}