OHVIRA Syndrome with Incomplete Vaginal Obstruction, Hematocolpos, and Pyocolpos Despite Regular Menstruation in an Adolescent: A Case Report

Background

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Patients with complete vaginal obstruction typically present shortly after menarche with severe dysmenorrhea and cyclic pelvic pain. Incomplete obstruction may permit partial menstrual drainage, resulting in atypical manifestations and delayed diagnosis. We report incomplete OHVIRA syndrome presenting with preserved menstrual flow despite hematocolpos and pyocolpos. The Zhu classification distinguishes complete obstruction (Class 1) from incomplete obstruction (Class 2), in which some menstrual outflow may occur despite persistent obstruction. In this case, findings supported incomplete obstruction, although a specific small septal opening was not documented.