GP40 OHVIRA syndrome- an unusual variant in an infant

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare Mullerian duct anomaly. The classical description is that of a uterus didelphys, unilateral obstructed hemivagina caused by a longitudinal sagittal vaginal septum and ipsilateral renal agenesis. An updated classification allows for inclusion of a wide variety of renal anomalies such as dysplastic kidneys, pelvic kidneys, or ectopic ureters. We report a rare case of OHVIRA syndrome in a fourteen month old girl, with a single normal uterus, obstructed hemi-vagina with a longitudinal coronal vaginal septum and unilateral dysplastic kidney. Antenatal ultrasound scan at twenty weeks of gestation revealed a suspected hydrocolpos and dysplastic kidney. The infant was born at term via elective caesarean section (maternal request for elective repeat caesarean). Of note mum had a personal history of uterus didelphys which was diagnosed at her first delivery (emergency caesarean section). Mum had no known associated renal anomaly. The patient was reviewed at five weeks old in the paediatric and adolescent gynaecology (PAG) clinic. She was thriving, but had a small vaginal bulge on examination. Repeat abdominal ultrasound scan revealed a single uterus, a normal right kidney and dysplastic left kidney, and a small hydrocolpos. As the patient was asymptomatic this was managed conservatively, and mum was advised to contact the PAG service if any concerns. At twelve months of age mum reported copious grey non-odorous discharge into the patient’s nappy. This was thought to be the hydrocolpos draining spontaneously and the patient was called in for review in the PAG clinic, however on clinical examination a large mass was palpable to the umbilicus. Repeat ultrasound suggested a persistent large hydrocolpos (10 × 6 × 4 cm). Examination under anaesthesia was performed. This demonstrated an elongated vagina with no cervix palpable at the superior aspect. A bulging septum in the coronal plane was then noted and a small punctum identified just inferior to the urethral orifice. This was opened draining 300mls of fluid. A single cervix was located in this obstructed anterior hemi- vagina. Clinical and sonographic review at six weeks post-operative confirmed a single uterus, unilateral dysplastic kidney and no recurrence of hydrocolpos or discharge and the infant was thriving. Cases of OHVIRA syndrome with variations in the classically described anatomical anomalies found in OHVIRA have been reported, however our patients variant with a single normal uterus, and a coronal septum may be a new variant not previously identified.