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.

CASE PRESENTATION: A 13-year-old postmenarchal girl was referred with persistent brown vaginal discharge following menstruation and recent right lower abdominal pain. She reported regular menstrual cycles for one year without dysmenorrhea. Examination revealed normal external genitalia with an intact hymen. Pelvic ultrasonography demonstrated uterus didelphys, hematocolpos, and non-visualization of the left kidney, raising suspicion of OHVIRA syndrome. Pelvic magnetic resonance imaging confirmed uterus didelphys with duplicated cervices and left-sided hematocolpos. According to the Zhu classification, the case was classified as Class 2 OHVIRA syndrome, indicating incomplete vaginal obstruction. According to the ESHRE/ESGE classification, the uterine, cervical, and vaginal anomalies were described as U3b-C2-V2, corresponding to a complete bicorporeal uterus, double cervix, and longitudinal obstructing vaginal septum, respectively. The patient underwent genitoscopy, transverse incision and complete excision of an oblique vaginal septum, drainage of retained collections, vaginal irrigation, and hymenorrhaphy. Approximately 50 mL of purulent material mixed with retained menstrual blood was evacuated, consistent with pyocolpos associated with chronic incomplete obstruction. Microbiological culture was not performed; therefore, the causative organism could not be identified. Cefazolin was administered preoperatively and postoperatively. Postoperative ultrasonography demonstrated complete resolution of the hematocolpos without residual pelvic fluid. During 6 months of telephone follow-up, the patient reported no recurrent symptoms; in-person reassessment was limited by school commitments and distance from the hospital.

CONCLUSION

Incomplete OHVIRA syndrome should be considered in postmenarchal adolescents with persistent postmenstrual brown vaginal discharge, even when menstruation is regular and dysmenorrhea is absent. Preserved menstrual flow does not exclude obstructive Müllerian anomalies. Pelvic ultrasonography, renal evaluation, magnetic resonance imaging, and endoscopic assessment are important for diagnosis and surgical planning. Early recognition and complete vaginal septum excision can relieve obstruction and treat retained collections; long-term reproductive outcomes cannot be determined from the available follow-up.