Adolescent abdominal pain due to rare mullerian duct anomaly

Abdominal pain is a common presenting complaint to the Emergency Department (ED). Often, rare etiologies can be discovered in the work up of this common complaint. Here we present the case of an adolescent female who presented with abdominal pain and was found to have obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) or Herlyn-Werner-Wunderlich Syndrome. A 12 year old femalewith known renal agenesis presented with 5 days of left sided abdominal pain that then developed into right lower quadrant pain. She had regular menses for the last 2 years. Ultrasound (US) showed a fluid collection in the lower uterine segment and a complex cystic structure anterior to the uterus. Magnetic resonance imaging (MRI) showed the patient to have didelphys uterus with severe dilatation of the cervix/vaginal canal. extending from the right uterine horn and left-sided ovarian and Fallopian tube torsion. She was taken to the operating room where she underwent vaginal septum excision and a left salpingo-oopherectomy. OHVIRA includes the triad of obstructed hemivagina, uterine didelphys, and ipsilateral renal agenesis. This occurs due to embryologic arrest of themullerian and mesonephric ducts at 8weeks of gestation. Most abnormalities are right sided which leads to right lower abdominal and pelvic pain approximately 4 months post-menarche. Diagnosis of OHVIRA is made utilizing US and CT scans. MRI can also be useful to further delineate specific anatomy. It is important for the emergency physician to be aware of this entity as most patients don't present to care until acute, severe symptoms develop. This makes it more likely for themto seek care in the ED as opposed to the outpatient setting. (c) 2022 Elsevier Inc. All rights reserved.

Automated summary

Abdominal pain is a common complaint in the Emergency Department, and one rare cause is obstructed hemivagina and ipsilateral renal anomaly. This article presents a case of a 12-year-old girl with didelphys uterus and obstructed hemivagina. OHVIRA is a triad of developmental arrest involving the mullerian and mesonephric ducts.