{"orpha":180079,"name":"Pseudounicornuate uterus","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Incomplete unilateral Müllerian aplasia","Incomplete unilateral aplasia of the Müllerian ducts","Unicornuate uterus with rudimentary horn"],"definition":"A rare, non-syndromic uterovaginal malformation characterized by a crescent-shaped, small-sized uterus containing a single horn and fallopian tube associated with a rudimentary second horn (which can be solid or contain a cavity with functioning endometrium and be communicating or non-communicating). Urinary tract anomalies are frequently associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180079/","orphanet_url":"https://www.orpha.net/en/disease/detail/180079","names":{"fr":{"name":"Utérus pseudo-unicorne","synonyms":["Aplasie müllerienne unilatérale incomplète","Aplasie unilatérale incomplète des canaux de Müller","Utérus unicorne avec utérus rudimentaire controlatéral"],"definition":"Une malformation utéro-vaginale rare, non syndromique, caractérisée par un utérus de petite taille en forme de croissant, contenant une corne utérine et une trompe de Fallope unitaires, associées à une deuxième corne rudimentaire (elle peut être solide ou contenir une cavité avec un endomètre fonctionnel, et peut ou non être communicante). Des anomalies des voies urinaires sont fréquemment associées."},"de":{"name":"Pseudo-Uterus unicornis","synonyms":["Aplasie des Müller-Ganges, inkomplette unilaterale","Uterus unicornis mit rudimentärem Horn"]},"es":{"name":"Útero pseudo-unicorne","synonyms":["Aplasia mulleriana unilateral incompleta","Aplasia unilateral incompleta de los conductos de Müller","Útero unicorne con cuerno rudimentario"],"definition":"Es una malformación uterovaginal no sindrómica poco frecuente caracterizada por un útero de tamaño pequeño y en forma de media luna con un solo cuerno y una trompa de Falopio asociada a un segundo cuerno rudimentario (que puede ser sólido o contener una cavidad con endometrio funcional, tanto comunicante como no comunicante). Con frecuencia se asocian anomalías en el tracto urinario."},"it":{"name":"Utero pseudo-unicorne","synonyms":["Aplasia mülleriana unilaterale incompleta","Aplasia unilaterale incompleta del dotto mülleriano","Utero unicorne con corno rudimentale"],"definition":"L'utero pseudo-unicorne è una malformazione utero-vaginale rara non sindromica, caratterizzata da un utero di piccole dimensioni, a forma di mezzaluna, contenente un solo corno e una sola tuba di Falloppio, associato ad un secondo corno rudimentale, che può essere compatto oppure contenere una cavità, comunicante o non comunicante, con un endometrio funzionante. Spesso le pazienti presentano anomalie delle vie urinarie."},"nl":{"name":"Pseudo-eenhoornige uterus","synonyms":["Eenhoornige baarmoeder met rudimentaire hoorn","Onvolledige unilaterale Mülleriaanse aplasie","Onvolledige unilaterale aplasie van gang van Müller","Pseudo-eenhoornige baarmoeder"],"definition":"Een zeldzame, niet-syndromale uterovaginale malformatie, gekarakteriseerd door een halvemaanvormige, kleine baarmoeder met één enkele hoorn en eileider, geassocieerd met een rudimentaire tweede hoorn (die solide kan zijn of een holte met functionerend baarmoederslijmvlies kan bevatten, en waarmee al dan niet communicatie mogelijk is). Anomalieën van het urinewegstelsel zijn frequent geassocieerd."},"pt":{"name":"Útero pseudo-unicórneo"},"pl":{"name":"Rzekoma macica jednorożna","synonyms":["Macica jednorożna z rogiem szczątkowym","Niecałkowita jednostronna aplazja przewodu Müllera"],"definition":"*Rzekoma macica jednorożna to rzadka, izolowana wada macicy, która charakteryzuje się małą macicą w kształcie półksiężyca zawierającą pojedynczy róg i jajowód, z szczątkowym drugim rogiem (który może być lity lub zawierać jamę z funkcjonującym endometrium, połączoną lub nie z jamą macicy). Często towarzyszą wady układu moczowego."},"cs":{"name":"Nekompletní jednostranná aplázie Müllerových vývodů","synonyms":["Neúplná jednostranná Müllerova aplázie","Uterus unicornis cum cornu rudimentario","Uterus pseudounicornis"]},"uk":{"name":"Псевдооднорога матка","synonyms":["Неповна одностороння аплазія мюллерових проток","Неповна одностороння аплазія Мюллера","Однорога матка з рудиментарним рогом"],"definition":"A rare, non-syndromic uterovaginal malformation characterized by a crescent-shaped, small-sized uterus containing a single horn and fallopian tube associated with a rudimentary second horn (which can be solid or contain a cavity with functioning endometrium and be communicating or non-communicating). Urinary tract anomalies are frequently associated."},"zh":{"name":"假性单角子宫","synonyms":["单角子宫伴残角"],"definition":"假性单角子宫是一种罕见的非综合征性子宫输卵管畸形，其主要临床表现为偏小的新月形单角子宫和伴有残角子宫的输卵管（可以是实心的或是含有功能性子宫内膜的空腔，相通或封闭），常伴有泌尿道异常。"},"tr":{"name":"Psödounikollis uterus","synonyms":["Eksik unilateral Müllerian aplazisi","İlkel boynuz ile seyreden unikollis uterus","Müllerian kanallarının eksik unilateral aplazisi"]}},"xrefs":[{"source":"ICD-10","code":"Q51.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB44.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1698505883"},{"source":"MONDO","code":"0015833","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4749300","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":180071,"name":"Unilateral aplasia of the Müllerian ducts","classifications":[147,156,185,205,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}