OHVIRA Syndrome: A Müllerian Anomaly as a Cause of Abdominal Pain in an Adolescent

  • Joana Antunes Hospital de Cascais, Dr. José de Almeida
  • Mariana Barros
  • Marta Ezequiel
  • Rita Calado
  • Mafalda Martins


OHVIRA (Obstructed Hemi Vagina with Ipsilateral Renal Agenesis) or Herlyn-Werner-Wunderlich Syndrome is a rare congenital disease, caused by a Mullerian anomaly, characterized by a didelphys uterus, obstruction of the hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive abdominal pain and pelvic mass secondary to hematocolpus. When the hemivagina obstruction is incomplete, the diagnosis may be delayed by the reduction of the severity of symptoms and possible regularity of the menstrual cycle. Rarely, it presents in the adult age as the cause of primary infertility. We describe a case of a 15-year-old girl with prenatal diagnosis of right renal agenesis who


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