Herlyn-Werner-Wunderlich syndrome (HWWS) or (OHVIRA): A late diagnosis

Yee Kyaing, Yee and Poh Ching, Lee Herlyn-Werner-Wunderlich syndrome (HWWS) or (OHVIRA): A late diagnosis. [Non Article]

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Abstract

Introduction: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn-Werner-Wunderlich syndrome (HWWS), is an abnormal development of the Mullerian and the Wolffian duct resulting in uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. Its incidence is 1 out of 1,000, 000 people. Case Description: A 33-year-old lady was on investigations and treatment for a few years on primary infertility. Her magnetic resonance imaging (MRI) abdomen and pelvis showed that there was bicornuate uterus with two separate endometrial cavities separated by a thin septum. The right uterine cavity led to cervical os but the left was a blind end. The left kidney was absent, and the right kidney was normal. Laparoscopic hysterotomy on the left side of uterus and left vaginal septectomy with vaginoplasty were done. The right salpingectomy was done as it was dilated. She had her menarche at 11 years old and cycles were regular. She had laparotomies twice at the age of 18 years and 21 years for tubo-ovarian abscess. Discussion: Diagnosis of OHVIRA syndrome can be misinterpreted as vagina obstruction is unilateral and menstruation is there. Sometimes symptoms are non-specific like dysmenorrhea, abdominal pain, foul smelling vaginal discharge, acute urinary retention, fever and vomiting. Delayed diagnosis may cause complications and adverse fertility outcomes. So, healthcare professionals must be aware of the possibility of OHVIRA syndrome to get early diagnosis and timely management.

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