UTERINE DIDELPHYS WITH OBSTRUCTED HEMIVAGINA AND IPSILATERAL RENAL ANOMALY: OHVIRA SYNDROME – A CASE REPORT

Shamrao Ramji Wakode and Sakshi Pramod Sharma*

ABSTRACT

Introduction: OHVIRA syndrome is a rare abnormality of the urogenital tract characterized by the triad of obstructed hemivagina, uterine didelphys, and ipsilateral renal agenesis, which is generally diagnosed after menarche. It often creates a diagnostic dilemma as most of the patients with OHVIRA syndrome are diagnosed late due to the rare incidence and the non- specific clinical presentation. Another reason for delay in diagnosing this syndrome are due to usual onset of puberty and menstruation as there are different phenotypic variables. Case presentation: A 13-year-old female approached with complain of dull pain in lower abdomen since last 7 to 8 months. Patient gave history of menarche 10 months ago and her menstrual cycle was irregular and was associated with progressively increasing dysmenorrhea. Her USG abdomen and pelvis was suggestive of haematocolpos with agenesis of right kidney. CT scan report showed uterus didelphys with right renal agenesis and obstructed right hemivagina secondary to vagina. Vertical incision was made over the right vaginal section and the dark collected blood was drained. Post operatively patient was stable and repeat USG showed reduction in size of hematocolpos. Patient was discharged on day 5 after the procedure and was counselled regarding the need for septoplasty and further implications of her diagnosis. Discussion: The causes of OHVIRA syndrome are not fully understood so far, but it is considered as a result of multiple factors, including genetics, environment, and endocrine influences. When patients approach with dysplastic or absent kidneys, an attempt to diagnose uterovaginal anomalies must be made to allow early recognition and management of this rare entity. It must be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract. Conclusion: It’s early diagnosis and timely treatment considerably improves the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.

Keywords: OHVARIA Syndrome, Uterine didelphys, obstructed hemivagina, ipsilateral renal anomaly.