Hysteroscopic versus laparoscopic management of two different variants of OHVIRA syndrome with preservation of hymen integrity

OHVIRA syndrome is a rare mesonephric duct-induced mullerian anomaly characterised by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly.
I present here 2 cases of OHVIRA syndrome. One with the mentioned classic features and another rare variant with a high/proximal vaginal septum which made the diagnosis and management even more challenging.
The first case was a 12-year-old girl who presented with dysmenorrhea in the last 2 months since menarche. Ultrasound and MRI scans confirmed the diagnosis of right haematocolpos, double uterus and right renal agenesis. Trans-hymenal vaginoscopic septoplasty was performed using a hook and loop of a bipolar resectoscope.
The second case was a 14-year-old girl who was referred to us with left iliac fossa pain, absent left kidney and a pelvic cystic structure. After full assessment and investigations including MRI, the differential diagnosis was unicornuate uterus with functioning non-communicating horn versus OHVIRA syndrome with cervicovaginal atresia (class 1.2). However, the final diagnosis of OHVIRA syndrome (class 1.1) with a high/proximal vaginal septum was confirmed by combined hysteroscopy and laparoscopy. She was managed by laparoscopic ipsilateral hemihysterectomy and septoplasty. The integrity of the hymen was preserved as per the patient and her parents’ wishes.
OHVIRA syndrome is generally a very rare condition. However, there are even more rare forms and variants of this anomaly which need more vigilance to reach timely accurate diagnosis and more experience and skills to perform proper management.