alexa Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrom
Reproductive Medicine

Reproductive Medicine

Gynecology & Obstetrics

Author(s): Mandava A, Prabhakar RR, Smitha S

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BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly with uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with pelvic pain and/or a mass and rarely, in later years, with primary infertility. Strong suspicion and knowledge of this anomaly are essential for a precise diagnosis.

CASE: A 14-year-old female presented with acute retention of urine and was diagnosed as a case of OHVIRA syndrome with uterus didelphys. Acute retention of urine as the initial clinical presentation has been rarely reported in this syndrome. She was treated with hemivaginal septal resection.

SUMMARY AND CONCLUSION: OHVIRA syndrome should be considered among the differential diagnoses in young females with renal anomalies presenting with pelvic mass, symptoms of acute abdomen, and acute urinary retention.

This article was published in J Pediatr Adolesc Gynecol. and referenced in Gynecology & Obstetrics

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