#112 : Obstructed Hemivagina with Ipsilateral Renal Anomaly (OHVIRA) with Ureteric Remnant in an Adolescent Female

Abstract

Background and Aims: Müllerian duct anomalies are a group of uncommon entities, which cause specific symptoms in adolescent females. OHVIRA is a very rare congenital anomaly of the urogenital tract characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. This paper describes a case of OHVIRA with ectopic ureteral insertion to the obstructed hemivagina and non-functional urinary bladder who was diagnosed and treated early. This study aimed to increase awareness regarding mullerian anomalies, discuss the ideal diagnostic tools and management of OHVIRA, and promote early diagnosis and treatment to avoid long-term complications. Method: A case of 12-year-old came for a consult with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and non-functional urinary bladder. The patient underwent resection of the non-communicating longitudinal vaginal septum, drainage of hematocolpos, diagnostic hysteroscopy, and cystoscopy, which are the main treatment of patients with OHVIRA. Results: One week after discharge, the patient followed up with no hypogastric pain, dysuria, and no vaginal bleeding. One month after, she had a menstrual period soaking 2-3 pads per day with no associated hypogastric pain nor recurrence of a pelvic mass with on digital rectal examination. Her subsequent menses were unremarkable occurring every month. Conclusion: OHVIRA should be suspected in cases with cyclic pelvic pain in adolescents and also in neonatal cases with any renal malformations. The delay in its diagnosis and management is inevitable due to its rarity; however, the complications can be devastating. Overall, timely surgical removal of the obstructing vaginal septum can provide rapid relief of symptoms, prevention of complications, and preservation of fertility in the majority of cases.