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

Date of submission: 07-08-2018 | Date of acceptance: 03-03-2019 | Published: 15-07-2019

  • Joana Antunes Serviço de Pediatria, Departamento da Criança, Hospital de Cascais Dr. José de Almeida, Cascais, Portugal
  • Mariana Barros Serviço de Pediatria, Departamento da Criança, Hospital de Cascais Dr. José de Almeida, Cascais, Portugal
  • Marta Ezequiel Serviço de Pediatria, Departamento de Pediatria, Hospital Prof. Doutor Fernando da Fonseca, Amadora, Portugal
  • Rita Calado Serviço de Pediatria, Departamento da Criança, Hospital de Cascais Dr. José de Almeida, Cascais, Portugal
  • Mafalda Martins Serviço de Pediatria, Departamento da Criança, Hospital de Cascais Dr. José de Almeida, Cascais, Portugal

Abstract

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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Published
2019-07-15
Section
Case reports