{"orpha":180126,"name":"Complete septate uterus","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Total septate uterus"],"definition":"Complete septate uterus is a rare, non-syndromic uterovaginal malformation characterized by a uterus that has a longitudinal septum which elongates from the uterine fundus to the internal or external cervical os. Most often women are asymptomatic, however dysmenorrhea, unilateral obstruction, and endometriosis could be observed. Unlike urinary tract abnormalities, which are very rarely associated, poor reproductive outcome is frequent.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180126/","orphanet_url":"https://www.orpha.net/en/disease/detail/180126","names":{"fr":{"name":"Utérus cloisonné total","definition":"L'utérus cloisonné total est une malformation utéro-vaginale rare, non syndromique caractérisée par un utérus possédant un septum longitudinal qui s'étend du fond de l'utérus à l'orifice interne ou externe cervical. Les femmes sont le plus souvent asymptomatiques. Cependant, une dysménorrhée, une obstruction unilatérale et une endométriose peuvent s'observer. La fonction reproductrice est souvent affectée. Les anomalies des voies urinaires sont très rarement associées à la maladie."},"de":{"name":"Uterus, komplett septierter","definition":"Ein kompletter Uterus septus ist eine seltene, nicht-syndromale uterovaginale Fehlbildung, die durch einen Uterus gekennzeichnet ist, der ein longitudinales Septum aufweist, das sich vom Uterusfundus bis zum inneren oder äußeren Muttermund erstreckt. Meistens sind die Frauen asymptomatisch, jedoch können Dysmenorrhoe, einseitige Obstruktion und Endometriose beobachtet werden. Im Gegensatz zu Harnwegsanomalien, die nur sehr selten auftreten, sind schlechte reproduktive Ergebnisse häufig."},"es":{"name":"Útero septado completo","synonyms":["Útero septado total","Útero tabicado"],"definition":"El útero septado completo es una malformación uterovaginal no sindrómica poco frecuente caracterizada por un útero con un tabique longitudinal que se extiende desde el fondo uterino hasta el orificio cervical interno o externo. Más frecuentemente, las mujeres son asintomáticas, aunque puede observarse dismenorrea, obstrucción unilateral y endometriosis. A diferencia de las anomalías del tracto urinario, que se asocian muy raras veces, el resultado reproductivo adverso es frecuente."},"it":{"name":"Utero settato totale","definition":"L'utero setto completo è una malformazione utero-vaginale rara, non sindromica; l'utero presenta un setto longitudinale che si estende dal fondo dell'utero fino all'orifizio cervicale interno o esterno. La maggior parte delle donne è asintomatica, ma può essere presente dismenorrea, ostruzione monolaterale ed endometriosi. A differenza delle anomalie delle vie urinarie, che raramente si associano a questo difetto, l'infertilità è comune."},"nl":{"name":"Baarmoeder met volledig septum","definition":"Een zeldzame, niet-syndromale uterovaginale malformatie, gekarakteriseerd door een uterus met een longitudinaal septum dat zich uitstrekt van uteriene fundus tot de inwendige of uitwendige opening van baarmoederhals. Meestal zijn vrouwen asymptomatisch, maar dysmenorroe, unilaterale obstructie, en endometriose kunnen waargenomen worden. In tegenstelling tot afwijkingen van urinewegen, die maar zeer zelden zijn geassocieerd, komt een ongunstige uitkomst wat betreft voortplanting geregeld voor."},"pt":{"name":"Útero totalmente septado"},"pl":{"name":"Macica z przegrodą całkowitą","synonyms":["Total septate uterus"]},"cs":{"name":"Uterus septus - kompletní","synonyms":["Total septate uterus"]},"uk":{"name":"Повністю перегороджена матка","synonyms":["Тотальна роздвоєна матка"],"definition":"Complete septate uterus is a rare, non-syndromic uterovaginal malformation characterized by a uterus that has a longitudinal septum which elongates from the uterine fundus to the internal or external cervical os. Most often women are asymptomatic, however dysmenorrhea, unilateral obstruction, and endometriosis could be observed. Unlike urinary tract abnormalities, which are very rarely associated, poor reproductive outcome is frequent."},"zh":{"name":"完全纵膈子宫","synonyms":["完全纵膈子宫"],"definition":"完全纵隔子宫是一种罕见的非综合征性子宫阴道畸形，其临床表现为子宫纵隔从子宫底延伸至宫颈内外。大多数患者无症状，也可能出现痛经、单侧梗阻和子宫内膜异位症。生殖系统异常较常见的，但泌尿道异常很少发生。"},"tr":{"name":"Tam septat uterus","synonyms":["Total septat uterus"]}},"xrefs":[{"source":"ICD-10","code":"Q51.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB44.4","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2105450595"},{"source":"MONDO","code":"0015840","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2957116","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":180122,"name":"Septate uterus","classifications":[147,185,201,205,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}