Key clinical message: OHVIRA syndrome can be misdiagnosed due to its rarity, resulting in the need for more invasive interventions than vaginoscopy. Also, delayed diagnosis of OHVIRA syndrome can affect patient's quality of life by leading to chronic gynecological diseases such as endometriosis and pelvic inflammatory disease.
Abstract: Obstructive hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is one of the infrequent congenital Mullerian duct anomalies characterized by obstructed hemivagina and ipsilateral renal agenesis. This study presents a 20-year-old virgin female who was diagnosed with OHVIRA syndrome and treated by vaginoscopy using the hymen preservation technique. Also, she was misdiagnosed with non-communicating rudimentary uterine horn 4 years ago. Late or misdiagnosis of OHVIRA syndrome can affect fertility and pregnancy outcomes. Therefore, early diagnosis and management are crucial. OHVIRA syndrome's misdiagnosis is possible with other Mullerian duct anomalies, such as a rudimentary uterine horn. Also, patients with misdiagnosis undergo unnecessary interventions.
Keywords: Herlyn–Werner–Wunderlich syndrome; OHVIRA syndrome; bicornuate uterus; hymen preservation; vaginoscopy.
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