Complete bicorporeal uterus, double cervix, longitudinal obstructing vaginal septum: an integrated approach for one-stop diagnosis and ultrasound-guided endoscopic hymen-sparing treatment.

Abstract

Background: Complete bicorporeal uterus, double cervix and obstructive longitudinal vaginal septum (classified as U3bC2V2 according to ESHRE/ESGE classification) is a rare congenital anomaly of the genital tract. This condition is typically associated with ipsilateral renal agenesis and is known as Herlyn-Werner-Wunderlich syndrome or OHVIRA (Obstructed HemiVagina and Ipsilateral Renal Anomaly) syndrome. The primary symptoms include dysmenorrhea and pelvic pain, which usually manifest after menarche due to haematocolpos in the obstructed hemivagina. Diagnosis is often challenging and frequently delayed. Early detection and surgical drainage of the haematocolpos are essential for symptom relief and prevention of complications. Various surgical approaches have been described, with vaginoplasty and septal resection being the recommended treatment.

Objective: To propose a step-by-step demonstration with narrated video footage of an integrated approach for one-stop diagnosis and ultrasound-guided endoscopic hymen-sparing treatment in a patient with OHVIRA syndrome.

Materials and methods: We present the case of a 17-year-old virgo-intacta female who was referred to our institution due to dysmenorrhea, abnormal uterine bleeding and a right presumed ovarian endometrioma. A preoperative evaluation, including pelvic ultrasound and MRI, suspected a U3bC2V2 malformation, associated to a right haematocolpos and ipsilateral renal agenesis. The patient underwent a complete minimally invasive vaginoscopic resection of the obstructive longitudinal vaginal septum under transabdominal ultrasound guidance, using a 15Fr bipolar mini-resectoscope. The procedure successfully drained the haematocolpos and allowed visualisation of the right cervix, confirming the preoperative diagnosis.

Results: The procedure was performed in our Digital Hysteroscopic Clinic (DHC) - CLASS Hysteroscopy -under general anaesthesia (with laryngeal mask), according to an ambulatory model of care. No complications occurred and the patient was discharged three hours after the procedure.

Main outcomes: After 40 days, hysteroscopic office control revealed a normal vagina with double cervix and complete bicorporeal uterus (classified as U3bC2V0 according to ESHRE/ESGE classification), and the patient had complete relief of symptoms.

Conclusion: The combined approach of one-stop diagnosis and ultrasound-guided minimally invasive vaginoscopic resection of the obstructive longitudinal vaginal septum, using a 15Fr bipolar mini-resectoscope, has proven to be an effective strategy. This approach leads to optimal surgical results without complications.