WOFAPS 2025 8th World Congress of Pediatric Surgery

Emine Burcu Çığşar Kuzu ¹, Dilnur Sevinç ², Bade Toker Kurtmen ¹

¹ Department of Pediatric Surgery, İzmir Katip Çelebi University School of Medicine, İzmir, Turkey
² Izmir City Hospital Pediatric Surgery Clinic

Introduction

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare condition within the spectrum of Müllerian anomalies. Most cases present after puberty with cyclic pelvic pain or dyspareunia. However, with the increased use of detailed antenatal ultrasonographic (USG) evaluations, prepubertal diagnoses have become more frequent. Despite this, reported infant cases remain limited, and no gold standard surgical approach has been defined for this age group.

Case Report

A 9-month-old girl with antenatally diagnosed right renal agenesis was referred due to a cystic mass compressing the rectum on postnatal USG. Abdominal and external genital exams were normal. In lithotomy position, rectal examination revealed a mass on the anterior wall. USG showed a 38×39 mm cystic lesion posterior to the bladder. Pelvic MRI revealed a well-defined, purely cystic, non-enhancing 60×50×40 mm lesion in the presacral area, displacing the rectum posteriorly and bladder anteriorly. The uterus appeared normal. Tailgut cyst and cystic sacrococcygeal teratoma were considered. Laboratory results were unremarkable. Due to progressive fluid accumulation, laparotomy revealed a cystic structure inseparable from the vagina and a uterus didelphys. The cyst was marsupialized to the abdominal wall. Fluoroscopy via a catheter inserted into the vaginal introitus revealed a separate second vaginal canal, confirming OHVIRA diagnosis. In a second session, the vaginal septum was excised with electrocautery, uniting both hemivaginas. Postoperative USG was normal.

Conclusion

Although OHVIRA syndrome occurs in 0.1–3.8% of cases, prepubertal diagnosis is rare. Maternal estrogen-induced vaginal secretions may cause obstructive symptoms in infancy. OHVIRA should be considered in the differential diagnosis of pelvic cystic masses. Conservative surgery involving vaginal septum excision relieves symptoms and prevents long-term complications (e.g., endometriosis, infections, infertility). In uncertain intraoperative diagnoses, a tissue-sparing approach followed by definitive surgery after further evaluation is a safe option.