Herlyn–Werner–Wunderlich syndrome, 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.
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Herlyn–Werner–Wunderlich syndrome, 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–either bicornuate or didelphys– 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
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