Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare anomaly of the urogenital system. The characteristic triad of this syndrome, which was initially reported in 1950, is didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (Embrey1). The prevalence of congenital Müllerian duct anomalies is reported to be 1%.2 A 24-year-old girl got admission to MGH on 15.3.2019 with lower abdominal pain and difficulty in the passing of urine. Her menarche was established 6 months back with a regular cycle and associated with dysmenorrhea. Magnetic resonance imaging (MRI) established the diagnosis of OHVIRA. She underwent surgery for drainage of the hematocolpos and excision of the vaginal septum and urethral dilatation with cystoscopy followed by an uncomplicated recovery and the patient had normal menstrual cycles after surgery.
Embrey MP. A case of uterus didelphys with unilateral gynatresia. Br Med J 1950;1(4657):820. DOI: 10.1136/bmj.1.4657.820.
Ashton D, Amin HK, Richart RM, et al. The incidence of asymptomatic uterine anomalies in women undergoing transcervical tubal sterilization. Obstet Gynecol 1988;72(1):28–30.
Kimble RM, Khoo SK, Baartz D, et al. The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad. Aust NZ J bstet Gynaecol 2009;49(5):554–557. DOI: 10.1111/j.1479-828X.2009.01063.x.
Shih CL, Hung YC, Chen CP, et al. Resectoscopic excision of the vaginal septum in a virgin with uterus didelphys and obstructed unilateral vagina. Taiwan J Obstet Gynecol 2010;49(1):109–111. DOI: 10.1016/S1028-4559(10)60022-8.
Nigam A, Raghunandan C, Yadav R, et al. OHVIRA syndrome: Rare cause of chronic vaginal discharge in an unmarried female. Congenit Anom (Kyoto) 2011;51(3):153–155. DOI: 10.1111/j.1741-4520.2010.00293.x.
Aveiro AC, Miranda V, Cabral AJ, et al. Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls. BMJ Case Rep 2011;2011(jul14 1):41–47. DOI: 10.1136/bcr.04.2011.4147.
Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87(4):918–922. DOI: 10.1016/j.fertnstert.2006.11.015.
Ma I, Williamson A, Rowe D, et al. OHVIRA with a twist: obstructed hemivagina ipsilateral renal anomaly with urogenital sinus in 2 patients. J Pediatr Adolesc Gynecol 2014;27(2):104–106. DOI: 10.1016/j.jpag.2013.06.015.
Muraoka A, Tsuda H, Kotani T, et al. Severe hemoperitoneum during pregnancy with obstructed hemivagina and ipsilateral renal anomaly syndrome: a case report. J Reprod Med 2016;61(5–6):290–294.
Mizutamari E, Honda T, Ohba T, et al. Spontaneous rupture of an unscarred gravid uterus in a primigravid woman at 32 weeks of gestation. Case Rep Obstet Gynecol 2014;2014:209585. DOI: 10.1155/2014/209585. https://www.hindawi.com/journals/criog/2014/209585/.
Del Vescovo R, Battisti S, Di Paola V, et al. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 2012;12(1):4. DOI: 10.1186/1471-2342-12-4.
Gholoum S, Puligandla PS, Hui T, et al. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg 2006;41(5):987–992. DOI: 10.1016/j.jpedsurg.2006.01.021.
Heinonen PK. Gestational hypertension and preeclampsia associated with unilateral renal agenesis in women with uterine malformations. Eur J Obstet Gynecol Reprod Biol 2004;114(1):39–43. DOI: 10.1016/j.ejogrb.2003.10.030.
Tanaka YO, Kurosaki Y, Kobayashi T, et al. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imag 1998;23(4):437–441. DOI: 10.1007/s002619900375.
Park NH, Park HJ, Park CS, et al. Herlyn-Werner-Wunderlich syndrome with unilateral hemivaginal obstruction, ipsilateral renal agenesis, and contralateral renal thin GBM disease: a case report with radiological follow up. J Korean Soc Radiol 2010;62(4):383–388. DOI: 10.3348/jksr.2010.62.4.383.