{"orpha":180086,"name":"Didelphys uterus","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Bicervical bicornuate uterus"],"definition":"A rare non-syndromic uterovaginal malformation characterized by two separate uterine cavities and cervices, due to failure of the Müllerian ducts to fuse. A longitudinal vaginal septum of variable thickness and elasticity is also present. Patients may be asymptomatic or experience dyspareunia or dysmenorrhea. There is increased frequency of endometriosis, as well as fertility and gestational issues with significantly reduced chances of seeing a pregnancy to term. The condition may be associated with renal agenesis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180086/","orphanet_url":"https://www.orpha.net/en/disease/detail/180086","names":{"fr":{"name":"Utérus didelphe","synonyms":["Utérus bicorne bicervical"],"definition":"Malformation utérovaginale non syndromique caractérisée par deux cavités et deux cols utérins distincts, en raison de l'absence de fusion des canaux müllériens. On observe un septum vaginal de forme longitudinale d'épaisseur et d'élasticité variables. Les patientes peuvent être asymptomatiques ou souffrir de dyspareunie ou de dysménorrhée. On observe une fréquence plus élevée d'endométriose, ainsi que des problèmes de fertilité et de gestation, avec des chances sensiblement réduites de mener une grossesse à terme. La maladie peut être associée à une agénésie rénale."},"de":{"name":"Uterus Didelphys","synonyms":["Uterus bicornis, bizervikaler"],"definition":"Eine seltene nicht-syndromale uterovaginale Fehlbildung, die durch zwei getrennte Gebärmutterhöhlen und Cerven gekennzeichnet ist, da die Müllerschen Gänge nicht miteinander verschmelzen können. Außerdem besteht ein verlängertes Vaginalseptum von unterschiedlicher Dicke und Elastizität. Die Patientinnen können asymptomatisch sein oder an Dyspareunie oder Dysmenorrhoe leiden. Es besteht eine erhöhte Inzidenz von Endometriose sowie von Fertilitäts- und Schwangerschaftsproblemen mit deutlich reduzierter Chance auf eine Schwangerschaft. Die Erkrankung kann mit einer Nierenagenesie einhergehen."},"es":{"name":"Útero didelfo","synonyms":["Útero bicorne bicervical"],"definition":"Es una malformación uterovaginal no sindrómica poco frecuente caracterizada por dos cavidades y cuellos uterinos separados, debido a un defecto en la fusión de los conductos de Müller, así como por la presencia de un tabique vaginal longitudinal de grosor y elasticidad variables. Las pacientes pueden permanecer asintomáticas o experimentar dispareunia o dismenorrea. Asocia una mayor frecuencia de endometriosis, así como problemas de fertilidad y gestacionales con una reducción significativa de la probabilidad de llegar a término con el embarazo. La entidad puede estar asociada a agenesia renal."},"it":{"name":"Utero didelfo","synonyms":["Utero bicorne bicervicale"],"definition":"Si tratta di una malformazione utero-vaginale rara non sindromica, caratterizzata dalla presenza di due cavità uterine e due cervici separate, dovuta alla mancata fusione dei dotti mülleriani. È presente anche un setto vaginale longitudinale di spessore ed elasticità variabile. Le pazienti possono essere asintomatiche o presentare dispareunia o dismenorrea. È aumentata la frequenza dell'endometriosi. Le pazienti presentano problemi di fertilità e della gestazione, con una probabilità significativamente ridotta di portare a termine una gravidanza. La malattia piò associarsi all'agenesia renale."},"nl":{"name":"Uterus didelphys","synonyms":["Bicervicale tweehoornige baarmoeder"],"definition":"Een zeldzame niet-syndromale uterovaginale malformatie, gekarakteriseerd door twee aparte baarmoederholtes en baarmoederhalzen, ten gevolge van gefaalde fusie van gangen van Müller. Een longitudinaal vaginaal septum met variabele dikte en elasticiteit is ook aanwezig. Patiënten kunnen asymptomatisch zijn, of dyspareunie of dysmenorroe ervaren. Er is verhoogde frequentie van endometriose, alsook problemen op vlak van fertiliteit en zwangerschap met significant gereduceerde kans op een voldragen zwangerschap. De aandoening kan geassocieerd zijn met agenesie van nier."},"pt":{"name":"Útero didelfo"},"pl":{"name":"Macica podwójna","synonyms":["Macica dwurożna dwuszyjkowa"]},"cs":{"name":"Uterus duplex didelphys","synonyms":["Bicervical bicornuate uterus"]},"uk":{"name":"Матка Didelphys","synonyms":["Біцервікальна дворога матка"],"definition":"A rare non-syndromic uterovaginal malformation characterized by two separate uterine cavities and cervices, due to failure of the Müllerian ducts to fuse. A longitudinal vaginal septum of variable thickness and elasticity is also present. Patients may be asymptomatic or experience dyspareunia or dysmenorrhea. There is increased frequency of endometriosis, as well as fertility and gestational issues with significantly reduced chances of seeing a pregnancy to term. The condition may be associated with renal agenesis."},"zh":{"name":"双子宫","synonyms":["双宫颈双角子宫"],"definition":"双子宫是一种罕见的非综合征性子宫输卵管畸形，由于苗勒管融合不全导所致，其主要临床表现为双子宫和双宫颈。不同患者的阴道纵隔的厚度和弹性不同。患者可能无症状，或表现为性交痛或痛经。子宫内膜异位症的发生率增加，妊娠和分娩问题增多，足月妊娠的可能性降低。该病与肾发育不全有关。"},"tr":{"name":"Didelfis uterus","synonyms":["Bikervikal bicornuat uterus"]}},"xrefs":[{"source":"ICD-10","code":"Q51.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"LB44.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1691111436"},{"source":"MONDO","code":"0015834","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000093642","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0266393","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10012770","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":180134,"name":"Bicornuate uterus","classifications":[147,185,205,233]}],"children":[{"orpha":180106,"name":"Bicervical bicornuate uterus and blind hemivagina","type":"Clinical subtype"},{"orpha":180111,"name":"Bicervical bicornuate uterus with patent cervix and vagina","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}