{"orpha":180157,"name":"Longitudinal vaginal septum","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare vaginal malformation characterized by the presence of a complete or incomplete septum dividing the vagina into two parallel cavities, resulting from failure of reabsorption of the midline uterine septum between the two fused Müllerian ducts during embryogenesis. Patients are often asymptomatic, but may present with menorrhagia, dysmenorrhea, dyspareunia, infertility, or spontaneous abortion. The condition may occur as an isolated malformation or in association with other Müllerian duct anomalies (such as septate uterus or uterus didelphys) or renal abnormalities.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180157/","orphanet_url":"https://www.orpha.net/en/disease/detail/180157","names":{"fr":{"name":"Vagin cloisonné longitudinal","definition":"Malformation vaginale rare caractérisée par la présence d'une cloison complète ou incomplète divisant le vagin en deux cavités parallèles, résultant d'un défaut de résorption de la cloison intermülerienne fusionnée pendant l'embryogenèse. Les patientes sont souvent asymptomatiques, mais peuvent présenter des ménorragies, des dysménorrhées, des dyspareunies, une infertilité ou un avortement spontané. La maladie peut se présenter sous la forme d'une malformation isolée ou en association avec d'autres malformations du canal Müllérien (telles que, l'utérus septal ou l'utérus didelphe) ou des malformations rénales."},"de":{"name":"Vaginalseptum, longitudinales","definition":"Eine seltene vaginale Fehlbildung, die durch ein vollständiges oder unvollständiges Septum gekennzeichnet ist, das die Vagina in zwei parallele Hohlräume teilt. Dies ist auf eine fehlgeschlagene Reabsorption des Uterusseptums in der Mittellinie zwischen den beiden verschmolzenen Müllerschen Gängen während der Embryogenese zurückzuführen. Die Patientinnen sind oft asymptomatisch, können aber auch Menorrhagie, Dysmenorrhoe, Dyspareunie, Infertilität oder Spontanaborte aufweisen. Die Erkrankung kann als isolierte Fehlbildung oder in Kombination mit anderen Anomalien der Müller'schen Gänge (z.B. Uterus septus oder Uterus didelphysus) oder Nierenanomalien auftreten."},"es":{"name":"Septo vaginal longitudinal","definition":"Es una malformación vaginal poco frecuente caracterizada por la presencia de un septo completo o incompleto que divide la vagina en dos cavidades paralelas, como resultado de un defecto de reabsorción del septo uterino en la línea media entre los dos conductos de Müller fusionados durante la embriogénesis. Las pacientes suelen permanecer asintomáticas pero pueden presentar menorragia, dismenorrea, dispareunia, infertilidad o aborto espontáneo. La entidad puede ocurrir como una malformación aislada o en asociación con otras anomalías de los conductos de Müller (como útero septado o útero didelfo) o renales."},"it":{"name":"Setto vaginale longitudinale","definition":"Si tratta di una malformazione vaginale rara, caratterizzata dalla presenza di un setto completo o incompleto che suddivide la vagina in due cavità parallele, a seguito del mancato riassorbimento del setto uterino mediano tra i due dotti mülleriani fusi durante l'embriogenesi. Le pazienti sono spesso asintomatiche, ma possono presentare menorragia, dismenorrea, dispareunia, infertilità o aborti spontanei. La malformazione può essere isolata oppure associarsi ad altre anomalie dei dotti mülleriani (ad es. l'utero setto o l'utero didelfo) o ad anomalie renali."},"nl":{"name":"Longitudinaal vaginaal septum","definition":"Een zeldzame malformatie van vagina, gekarakteriseerd door aanwezigheid van een volledig of onvolledig septum dat de vagina in twee parallelle holtes verdeelt, als gevolg van gefaalde reabsorptie van mediaan uterusseptum tussen de twee gefuseerde gangen van Müller tijdens de embryogenese. Patiënten zijn vaak asymptomatisch, maar vertonen mogelijk menorragie, dysmenorroe, dyspareunie, infertiliteit, of spontane abortus. De aandoening kan voorkomen als geïsoleerde malformatie, of in associatie met andere anomalieen van gang van Müller (zoals uterus bilocularis of uterus duplex) of nieranomalieën."},"pt":{"name":"Septo vaginal longitudinal"},"pl":{"name":"Przegroda podłużna pochwy"},"cs":{"name":"Longitudinální poševní septum"},"uk":{"name":"Поздовжня перегородка піхви","definition":"A rare vaginal malformation characterized by the presence of a complete or incomplete septum dividing the vagina into two parallel cavities, resulting from failure of reabsorption of the midline uterine septum between the two fused Müllerian ducts during embryogenesis. Patients are often asymptomatic, but may present with menorrhagia, dysmenorrhea, dyspareunia, infertility, or spontaneous abortion. The condition may occur as an isolated malformation or in association with other Müllerian duct anomalies (such as septate uterus or uterus didelphys) or renal abnormalities."},"zh":{"name":"阴道纵膈"},"tr":{"name":"Boyuna vajinal septum"}},"xrefs":[{"source":"ICD-10","code":"Q52.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB42.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1594393492"},{"source":"MONDO","code":"0015849","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1841680","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":180154,"name":"Septate vagina","classifications":[147,185,201,205,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}