{"orpha":209956,"name":"Idiopathic uveal effusion syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare panuveitis characterized by uni- or bilateral abnormal fluid accumulation within the suprachoroidal space, resulting in internal choroidal elevation, in the absence of any known cause, such as decreased intraocular tension, intraocular tumor, intraocular inflammation or nanophtalmos. Patients typically present a protracted, relapsing-remitting course of visual acuity loss and fundus examination shows annular celio-choroidal detachment and shifting, serous retinal detachment.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-209956/","orphanet_url":"https://www.orpha.net/en/disease/detail/209956","names":{"fr":{"name":"Syndrome d'effusion uvéale idiopathique","definition":"Maladie ophtalmique acquise rare caractérisée par une accumulation anormale uni- ou bilatérale de liquide dans l'espace suprachoroïdien, entraînant une élévation de la couche interne de la choroïde, en l'absence de toute cause connue, telle qu'une baisse de la pression intraoculaire, une tumeur intraoculaire, une inflammation intraoculaire ou une nanophtalmie. Les patients présentent généralement une perte d'acuité visuelle prolongée et récurrente, et l'examen du fond d'oeil met en évidence un décollement et un déplacement cilio-choroïdien annulaire et un décollement séreux de la rétine."},"de":{"name":"Uveales Effusions-Syndrom, idiopathisches"},"es":{"name":"Síndrome de efusión uveal idiopático","definition":"El síndrome de efusión uveal idiopático es una enfermedad ocular adquirida poco frecuente caracterizada por un acúmulo uni- o bilateral anómalo de líquido dentro del espacio supracoroideo que produce una elevación coroidea interna, en ausencia de cualquier causa conocida, como disminución de la presión intraocular, tumor intraocular, inflamación intraocular o nanoftalmia. Los afectados suelen presentar pérdida de agudeza visual de curso remitente-recurrente y prolongado y el examen de fondo de ojo muestra desprendimiento cilio-coroideo anular y un desprendimiento seroso de retina variable."},"it":{"name":"Sindrome idiopatica da effusione uveale","definition":"La sindrome da versamento uveale idiopatico è una malattia acquisita rara dell'occhio, caratterizzata dall'accumulo anomalo mono- o bi-laterale di liquido nello spazio sopracoroideo, che causa uno slivellamento nella parte interna della coroide, in assenza di cause evidenti, come la diminuzione della tensione intraoculare, la presenza di un tumore o di infiammazione intraoculare, oppure il nanoftalmo. Di solito i pazienti perdono per lunghi periodi l'acuità visiva con un andamento recidivante-remittente. L'esame del fondo oculare evidenzia un distacco cilio-coroideale anulare e un distacco sieroso della retina che risulta dislocata."},"nl":{"name":"Idiopathische uveale effusie-syndroom","definition":"Idiopathische uveale effusie-syndroom is een zeldzame, verworven oogziekte die gekarakteriseerd wordt door uni- of bilaterale abnormale vochtophoping in de suprachoroïdale ruimte, wat leidt tot interne choroïdale elevatie, in afwezigheid van een gekende oorzaak, zoals verminderde intraoculaire spanning, intraoculaire tumor, intraoculaire inflammatie of nanoftalmie. Patiënten vertonen doorgaans een langdurig, recidiverend-remitterend verloop van verlies van visus, en fundusonderzoek toont ringvormige celio-choroïdale loslating en verplaatsing, en sereuze netvliesloslating."},"pt":{"name":"Síndrome de derrame uveal idiopática"},"pl":{"name":"Idiopatyczny zespół wysiękowy naczyniówki"},"cs":{"name":"Syndrom idiopatické uveální efuze"},"uk":{"name":"Синдром ідіопатичного увеального випоту","definition":"A rare panuveitis characterized by uni- or bilateral abnormal fluid accumulation within the suprachoroidal space, resulting in internal choroidal elevation, in the absence of any known cause, such as decreased intraocular tension, intraocular tumor, intraocular inflammation or nanophtalmos. Patients typically present a protracted, relapsing-remitting course of visual acuity loss and fundus examination shows annular celio-choroidal detachment and shifting, serous retinal detachment."},"zh":{"name":"特发性葡萄膜积液综合征","definition":"特发性葡萄膜渗漏综合征是一种罕见的获得性眼病，其主要临床表现为无已知病因（如眼内压降低、眼内肿瘤、眼内炎症或小眼球）情况下，脉络膜上腔内单侧或双侧异常积液，导致脉络膜突出。患者通常表现为视力下降，呈复发-缓解-复发循坏，眼底检查显示环状脉络膜脱落和转移性浆液性视网膜脱落。"},"tr":{"name":"İdiyopatik uveal efüzyon sendromu"}},"xrefs":[{"source":"ICD-10","code":"H31.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9B6Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1481963661"},{"source":"MONDO","code":"0016206","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4755300","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":280898,"name":"Panuveitis","classifications":[189]},{"orpha":716213,"name":"Rare isolated developmental choroidal disorder","classifications":[147,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-07-01","source":"22218051[PMID]_6676971[PMID]_8762565[PMID]_17458802[PMID]_20159229[PMID]","terms":[{"hpo":"HP:0000591","label":"Abnormal sclera morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0004328","label":"Abnormal anterior eye segment morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012231","label":"Exudative retinal detachment","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030823","label":"Scleral thickening","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031526","label":"Subretinal fluid","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025339","label":"Superficial episcleral hyperemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012508","label":"Metamorphopsia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000568","label":"Microphthalmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008052","label":"Retinal fold","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001123","label":"Visual field defect","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012632","label":"Abnormal intraocular pressure","frequency":"Excluded (0%)","criteria":null}]}}