Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche. Endometriosis is a prevalent complication. Magnetic resonance imaging (MRI) helps in diagnosing OHVIRA syndrome and endometriosis due to its high contrast resolution and objectivity. We reported a 13- year-old girl who was evaluated for cyclic pelvic pain after her menarche at 12 years of age. Magnetic resonance imaging (MRI) revealed two separate uterine cavities, services and vaginae, indicating didelphys. The left uterine cavity is filled with fluid, and the left hemivagina is dilated and filled with hyperintense and hypointense fluid on T1 and T2, respectively, indicating blood products. Left hemivagina dilatation implicated the presence of an obstructing vaginal septum. A single left adnexal cyst lesion with blood products was suggestive of an endometriotic cyst. Additionally, the left kidney was absent. A uterine didelphys with left hemivagina obstruction, hematometra, hematocolpos, and the ipsilateral ovarian endometriotic cyst was diagnosed. A final diagnosis of OHVIRA syndrome or HWWS was made, considering that she had no left kidney.MRI is a suitable diagnostic tool for precise anatomical delineation of the uterus, cervix, and vagina in uterovaginal disorders such as OHVIRA syndrome. MRI can also properly evaluate endometriosis and adhesion.
Persistent urogenital sinus (PUGS) is a rare anomaly whereby the urinary and genital tracts fail to separate during embryonic development. We report a three-year-old female child who was referred to the Sabah Women & Children Hospital, Sabah, Malaysia, in 2016 with a pelvic mass. She had been born prematurely at 36 gestational weeks via spontaneous vaginal delivery in 2013 and initially misdiagnosed with neurogenic bladder dysfunction. The external genitalia appeared normal and an initial sonogram and repeat micturating cystourethrograms did not indicate any urogenital anomalies. She therefore underwent clean intermittent catheterisation. Three years later, the diagnosis was corrected following the investigation of a persistent cystic mass posterior to the bladder. At this time, a clinical examination of the perineum showed a single opening into the introitus. Magnetic resonance imaging of the pelvis revealed gross hydrocolpos and a genitogram confirmed a diagnosis of PUGS, for which the patient underwent surgical separation of the urinary and genital tracts.