A Rare Variant of Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome with a Complete Septate Uterus

Regta  L. Pichay,; Bernadette  L. Dela Cruz,

A Rare Variant of Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome with a Complete Septate Uterus

Bernadette L. Dela Cruz | Regta L. Pichay

Abstract:

Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rare Müllerian congenital anomaly. An adolescent will usually experience recurrent pelvic pain or dysmenorrhea after menarche. It is seen in the triad of Herlyn-Werner-Wünderlich Syndrome which is composed of an obstructed hemivagina, ipsilateral renal agenesis and a didelphys uterus. Although more often associated with a didelphys uterus, rare variants of Herlyn-Werner-Wünderlich syndrome have been reported where OHVIRA syndrome is seen with other Müllerian anomalies. This is a case of a 22-year-old, nulligravid, who is regularly menstruating, with a history of progressive, cyclic dysmenorrhea, which affected the quality of her life. She consulted in our institution due to persistence of symptoms. Ultrasound revealed a bicornuate uterus, hematocolpos on the right hemivagina and absent right kidney. She underwent diagnostic hysteroscopy with ultrasound guided incision and drainage of right hematocolpos, diagnostic laparoscopy with adhesiolysis and chromotubation. The procedure revealed a complete septate uterus and patent left fallopian tube. After one month, she had her menses with improvement of symptoms.

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