Ohvira síndrome: Case Report

Background: OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome is a rare congenital condition characterized by uterine malformations, such as didelphic uterus with blind hemivagina and ipsilateral renal agenesis. It usually presents in adolescence with primary dysmenorrhea, cyclic pelvic pain and hematocolpos or hematometra. Its incidence is low, estimated between 1 in 2,000 to 1 in 28,000 women, and the available literature on this syndrome is limited. Case report: 15-year-old female patient with a history of right renal agenesis diagnosed in childhood, who consulted for intense dysmenorrhea and recurrent vaginal bleeding, requiring hospitalization. The imaging studies, ultrasound and pelvic MRI, showed a didelphic uterus with obstructed hemivagina, compatible with OHVIRA syndrome. During laparoscopy, endometriosis foci were also identified. Combined laparoscopic and hysteroscopic surgical management was performed to resolve the obstruction and resect endometriotic lesions. The patient evolved with regular menstrual cycles and without symptoms. Discussion: OHVIRA syndrome is an underdiagnosed pathology, associated with complications such as endometriosis, infertility and chronic pelvic pain. Its timely diagnosis by 3D ultrasound and magnetic resonance imaging is essential to plan the appropriate treatment and prevent long-term sequelae. Conclusions: In patients with menstrual abnormalities and renal agenesis, the presence of Müllerian malformations, such as OHVIRA syndrome, should be suspected in order to make an early diagnosis and treatment, avoid future complications and preserve fertility.