{"orpha":519392,"name":"Isolated iridoschisis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare disorder of the anterior segment of the eye characterized by spontaneous separation of the anterior layer of the iris stroma from the posterior stroma and muscle layers. The anterior layer then splits into strands, and the free ends float freely in the anterior chamber. The condition usually affects patients in the seventh decade of life and is often associated with glaucoma. It may begin on one side but is typically a bilateral disease. The inferior part of the iris is most commonly involved.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-519392/","orphanet_url":"https://www.orpha.net/en/disease/detail/519392","names":{"fr":{"name":"Iridoschisis isolé","definition":"Maladie rare du segment antérieur de l'oeil caractérisée par une dissociation spontanée entre la couche antérieure du stroma de l'iris et les couches du stroma postérieur et des muscles. La couche antérieure se dissocie alors en fibrilles dont les extrémités flottent dans la chambre antérieure. Souvent associée à un glaucome, la maladie touche les patients dans la septième décennie de vie. Elle est généralement bilatérale, mais se manifeste dans un premier temps d'un seul côté. La partie le plus souvent touchée est la partie inférieure de l'iris."},"de":{"name":"Iridoschisis, isolierte","definition":"Eine seltene Erkrankung des vorderen Augenabschnitts, bei der sich die vordere Schicht des Irisstromas spontan von der hinteren Stroma- und Muskelschicht ablöst. Die vordere Schicht teilt sich in Stränge, deren freie Enden frei in der vorderen Augenkammer schwimmen. Die Erkrankung tritt meist im siebten Lebensjahrzehnt auf und geht häufig mit einem Glaukom einher. Sie kann auf einer Seite beginnen, ist aber typischerweise beidseitig. In den meisten Fällen ist der untere Teil der Regenbogenhaut betroffen."},"es":{"name":"Iridosquisis aislada","definition":"Es un trastorno poco frecuente del segmento anterior del ojo caracterizado por la separación espontánea de la capa anterior del estroma del iris del estroma posterior y de las capas musculares. Posteriormente, la capa anterior se divide en hebras y los extremos flotan libremente en la cámara anterior. Esta entidad suele afectar a pacientes en la séptima década de la vida y se asocia a menudo con glaucoma. Puede comenzar en un ojo pero, por lo general, es una enfermedad bilateral. La parte inferior del iris es la más comúnmente afectada."},"it":{"name":"Iridoschisi isolata","definition":"Si tratta di una malattia rara del segmento anteriore dell'occhio, caratterizzata dalla separazione spontanea dello strato anteriore dello stroma dell'iride dallo stroma posteriore e dagli strati muscolari. Lo strato anteriore si separa in diversi filamenti e le estremità libere fluttuano nella camera anteriore. Di solito la malattia esordisce nella settima decade di vita e spesso si associa al glaucoma. Può manifestarsi dapprima in un solo occhio, ma in genere è bilaterale. La porzione inferiore dell'iride è maggiormente interessata."},"nl":{"name":"Geïsoleerde iridoschisis","definition":"Een zeldzame aandoening van voorste oogsegment, gekarakteriseerd door spontane loslating van anterieure laag van stroma van iris van posterieur stroma en spierlagen. De voorste laag splitst vervolgens in strengen waarvan de vrije uiteinden vrij rondzweven in anterieure oogkamer. De aandoening treft meestal patiënten in het zevende levensdecennium en is vaak geassocieerd met glaucoom. De aandoening kan aanvangen aan één zijde maar is doorgaans bilateraal. In de meeste gevallen is het inferieure deel van iris betrokken."},"pt":{"name":"Iridosquisis isolada"},"pl":{"name":"Izolowane rozwarstwienie tęczówki"},"cs":{"name":"Izolovaná iridoschíza"},"uk":{"name":"Ізольований іридошизис","definition":"A rare disorder of the anterior segment of the eye characterized by spontaneous separation of the anterior layer of the iris stroma from the posterior stroma and muscle layers. The anterior layer then splits into strands, and the free ends float freely in the anterior chamber. The condition usually affects patients in the seventh decade of life and is often associated with glaucoma. It may begin on one side but is typically a bilateral disease. The inferior part of the iris is most commonly involved."},"zh":{"name":"孤立性虹膜劈裂","definition":"一种罕见的眼前节疾病，其特征是虹膜基质前层与后层基质和肌肉层自发分离。前层随即分裂成股，游离端在前房中自由漂浮。本病通常影响七旬患者，常与青光眼有关。可起于一侧，但一般为双侧性疾病。虹膜的下半部最常受累。"},"tr":{"name":"İzole iridoskizis"}},"xrefs":[{"source":"ICD-10","code":"H21.2","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"LA11.61","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1870961422"},{"source":"MONDO","code":"0033810","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0154919","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":98634,"name":"Anterior segment developmental anomaly without extraocular manifestations","classifications":[147,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}