Abstract
Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome is a rare obstructive uterovaginal anomaly involving both mesonephric and paramesonephric ducts. It usually presents after menarche with non-specific symptoms like pelvic pain, dysmenorrhea, or paravaginal mass and examination findings of paravaginal or pelvic mass. Because of non-specific symptoms and signs, the diagnosis is usually overlooked, which leads to complications like endometriosis, tubo-ovarian abscess compromising patient’s fertility, and quality of life. Therefore, in presence of these nonspecific clinical features along with imaging findings of uterine didelphys and unilateral renal agenesis, this syndrome should be considered in the diagnosis. We present a case of a 17-year-old lady with Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome, diagnosed by finding of paravaginal mass on examination and uterine didelphys with ipsilateral renal agenesis in USG and managed successfully by resection of vaginal septum. Keywords: ipsilateral renal anomaly;paravaginal mass;uterovaginal anomaly.
Highlights
Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome is a rare obstructive uterovaginal anomaly involving both mesonephric and paramesonephric ducts
The syndrome is characterized by the presence of uterine didelphys, blind hemivagina, and renal anomalies, the most common renal anomaly being renal agenesis.[2]
Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) constitutes 2-3% of all Mullerian anomalies.[3]. This condition usually presents after menarche,typically presentswith non-specific symptoms of pelvic pain or dysmenorrhea and a paravaginal bulge on examination in an adolescent girl
Summary
Urine with a small urinary stream, intermittent flow, Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA), known as Herlyn-Werner-Wunderlich (HWW) syndrome is a rare combined paramesonephric and mesonephric duct anomaly.[1] The syndrome is characterized by the presence of uterine didelphys, blind hemivagina, and renal anomalies, the most common renal anomaly being renal agenesis.[2] OHVIRA constitutes 2-3% of all Mullerian anomalies.[3] This condition usually presents after menarche,typically presentswith non-specific symptoms of pelvic pain or dysmenorrhea and a paravaginal bulge on examination in an adolescent girl It can present with an acute abdomen and tubo-ovarian mass.[1,4] The nonspecific presenting complaints and heterogeneity of presentation can make the diagnosis a challenge. The MRI pelvis was planned it was not afforded by the patient Instead, she followed up with a CT scan of the pelvis which showed two widely separated endometrial myometrial complexes with two cornua, body, and cervix of the uterus (uterine didelphys) with mild fluid collection in the right endometrial cavity and vagina on the right side (unilateral hematometrocolpos) (Figure 2A). She was reassured of the normal findings and asked to follow up if she had any problems
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