OHVIRA syndrome with Orofacial clefts: a rare association

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dc.contributor.authorSambednaen_US
dc.contributor.authorKumar, Amiten_US
dc.contributor.authorAgarwal, Muktaen_US
dc.contributor.authorAgrawal, Nimishaen_US
dc.contributor.authorSinha, Sudwitaen_US
dc.date.accessioned2020-10-16T08:03:13Z
dc.date.available2020-10-16T08:03:13Z
dc.date.issued2020-03
dc.description.abstractObstucted hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome or Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital anomaly consist of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We are reporting an adolescent girl with orofacial defect who presented with lower abdominal pain. She attained menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. On abdominal examination a firm, mobile tender mass extending from left iliac fossa up to umbilicus (24 weeks size) was found.  Lower border of mass could not be approached. Further evaluation with ultrasound showed enlarged uterus with collection with internal echoes and non-visualization of the left kidney. CECT showed absent left kidney and didelphys uterus with large left hematocolpometra with left complex adenexal cyst. Patient was posted for hematocolpos drainage and vaginoplasty. An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to severe complications such as endometriosis and infertility.en_US
dc.identifier.affiliationsDepartment of Obstetrics and Gynecology, AIIMS, Patna, Bihar, Indiaen_US
dc.identifier.affiliationsDepartment of Pediatric Surgery, AIIMS, Patna, Bihar, Indiaen_US
dc.identifier.citationSambedna, Kumar Amit, Agarwal Mukta, Agrawal Nimisha, Sinha Sudwita. OHVIRA syndrome with Orofacial clefts: a rare association. International Journal of Reproduction, Contraception, Obstetrics and Gynecology. 2020 Mar; 9(3): 1306-1309en_US
dc.identifier.issn2320-1770
dc.identifier.issn2320-1789
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/207553
dc.languageenen_US
dc.publisherMedip Academyen_US
dc.relation.issuenumber3en_US
dc.relation.volume9en_US
dc.source.urihttps://dx.doi.org/10.18203/2320-1770.ijrcog20200923en_US
dc.subjectHerlyn-Werner-Wunderlich syndromeen_US
dc.subjectObstructed hemivaginaen_US
dc.subjectOrofacial defecten_US
dc.subjectVaginoplastyen_US
dc.titleOHVIRA syndrome with Orofacial clefts: a rare associationen_US
dc.typeJournal Articleen_US
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