Global InformationOHVIRA information
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| (a) Coronal Single shot T2W image shows absence of the right kidney. The distended hemivagina (asterisk) is seen on the right side and the normal collapsed left hemivagina with minimal fluid is seen adjacent to it (black arrow). The distended hemivagina ends above the introitus and its contents are hypointense to fat. (b) Coronal Single shot T2W image shows right and left uterine horns (white arrows). The right uterine horn cavity is seen to communicate with the upper end of the fluid collection in right hemivagina (small black arrow) |
Herlyn-Werner-Wunderlich syndrome,[1] also known as OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. It is a type of abnormality of the Müllerian ducts.
In most cases, OHVIRA presents as a double uterus with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis (or renal anomalies). It can also affect the urethra, urethral sphincter, ureters, bladder and spleen.
Although the true incidence is unknown, it has been reported to be between 0.1% and 3%.[2]
- ^ Mandava, Anitha; Prabhakar, R. R.; Smitha, S. (2012-04-01). "OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation". Journal of Pediatric and Adolescent Gynecology. 25 (2): e23–25. doi:10.1016/j.jpag.2011.11.004. ISSN 1873-4332. PMID 22421561.
- ^ Aveiro, Ana Cristina; Miranda, Victor; Cabral, António Jorge; Nunes, Sidónia; Paulo, Filomeno; Freitas, Conceição (2011-07-15). "Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls". BMJ Case Reports. 2011: bcr0420114147. doi:10.1136/bcr.04.2011.4147. ISSN 1757-790X. PMC 3139160. PMID 22689557.