Case report, or case study
Vol. 22 No. 3 (2020)
A case of OHVIRA syndrome
Programa de Pediatría, Facultad de Ciencias de la Salud, Universidad del Cauca. Popayán, Colombia
Programa de Pediatría, Facultad de Ciencias de la Salud, Universidad del Cauca. Popayán, Colombia - Hospital Universitario San José. Popayán, Colombia
Programa de Pediatría, Facultad de Ciencias de la Salud, Universidad del Cauca. Popayán, Colombia - Hospital Universitario San José. Popayán, Colombia
Abstract
Introduction: The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as the Herlyn-Werner-Wunderlich syndrome, is a rare müllerian anomaly characterized by didelphic uterus, hemivaginal obstruction, and ipsilateral renal anomaly. The incidence of müllerian malformations is 2% - 3%, OHVIRA being the least common of these cases. Objective: To describe an exceptional case of a müllerian disorder. Case presentation: A 14-year-old adolescent with a history of pelvic pain, dysmenorrhea, and recurrent urinary infections. Renal gammagraphy analysis revealed right renal agenesis, and pelvic magnetic resonance imaging showed a didelphic uterus with right hematocolpos. These findings were confirmed by diagnostic laparoscopy. Conclusions: This syndrome is not well known. Renal anegesis, chronic pelvic pain, and menstrual abnormalities in post-pubertal patients must be presumed as indicators of OHVIRA, whose treatment requires a multidisciplinary approach.
References
- Bermejo N, Isusi M, Diez F. Solución del caso 19: Síndrome de Herlyn-Werner-Wunderlich. Radiología. 2010;52(4):366-8. DOI: 10.1016/j.rx.2008.10.016
- Saltzman A, Roth C. A Curious Case of Continuous Incontinence. Urology. 2016;92:113-6. DOI: 10.1016/j.urology.2016.02.005
- Mandava A, Prabhakar R, Smitha S. OHVIRA Syndrome (obstructed hemivagina and ipsilateral renal anomaly) with Uterus Didelphys, an Unusual Presentation. J Pediatr Adolesc Gynecol. 2012;25(2):e23-5. DOI: 10.1016/j.jpag.2011.11.004
- González Y, Delgado R, Siles A, Izquierdo B, Rihuete M. Diagnóstico prepuberal del síndrome de OHVIRA: ¿es posible?. Anales de Pediatría. 2019;90(4). p. 244-245. DOI: 10.1016/j.anpedi.2018.04.010
- Aswani Y, Varma R, Choudhary P, Gupta R. Wolffian Origin of Vagina Unfolds the Embryopathogenesis of OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome and Places OHVIRA as a Female Counterpart of Zinner Syndrome in Males. Pol J Radiol. 2016;81:549-56. DOI: 10.12659/PJR.898244
- Santos X, Dietrich J. Obstructed Hemivagina with Ipsilateral Renal Anomaly. J Pediatr Adolesc Gynecol. 2016;29(1):7-10. DOI: 10.1016/j.jpag.2014.09.008
- Goswami D, Sharma A, Vyas V, Vasudevan B. Association of OHVIRA syndrome with aortic stenosis and block vertebra: A case report and anaesthetic management. J Obstet Gynaecol. 2016;36(6):764-6. DOI: 10.3109/01443615.2016.1157156
- Han J, Lee Y, Im Y, Kim S, Lee M, Han S. Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group. PLOS ONE. 2016;11(11):e0166776. DOI: //doi.org/10.1371/journal.pone.0166776
- Grant C, Bayne C, Kern N, Peters C. Atypical presentation of obstructed hemivagina and ipsilateral renal anomaly. Urol Case Rep. 2018;19:70-1. DOI: 10.1016/j.eucr.2018.05.011
- Tsai J, Tsai S. Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome. Iran Red Crescent Med J. 2016;18(11):e40267 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5294448/
- Friedman M, Aguilar L, Heyward Q, Wheeler C, Caldamone A. Screening for Mullerian anomalies in patients with unilateral renal agenesis: Leveraging early detection to prevent complications. J Pediatr Urol. 2018;14(2):144-9. DOI: 10.1016/j.jpurol.2018.01.011
- Echebarria O, Landin A, Canales V, López A, Morales P, de Pablo J. Síndrome de Herlyn-Werner-Wunderlich. Prog Obstet Ginecol. 2012;55(8):405-7. DOI: 10.1016/j.pog.2011.06.017
- Fuentes A, Gómez M, López E, Belmonte L, González G. Síndrome de Herlyn-Werner-Wunderlich. Prog Obstet Ginecol. 2015;58(1):20-4. DOI: 10.1016/j.pog.2014.07.001
- Gungor F, Bastu E, Gulsen G, Kurek M, Akhan S. OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature. Clin Imaging. 2014;38(3):357-9. DOI: 10.1016/j.clinimag.2013.12.011
- Al Ghafri A, Fida A, Al-Gharras A. Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome. Oman Med J. 2018;33(1):69-71. DOI: 10.5001/omj.2018.13
- Mishra N, Ng S. Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases. Australas J Ultrasound Med. 2014;17(4):153-8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5024931/
- Bhoil R, Ahluwaila A, Chauhan N. Herlyn Werner Wunderlich Syndrome with Hematocolpos: An Unusual Case Report of Full Diagnostic Approach and Treatment. Int J Fertil Steril. 2016; 10(1): 136–140 DOI: 10.22074/ijfs.2016.4779
- Tomlin K, Barnes C, Van Leeuwen K, Williamson A. Three-Dimensional Technology to Diagnose Unilateral Cervical Atresia in Obstructive Hemivagina with Ipsilateral Renal Anomaly: A Case Report and Review of the Literature. J Pediatr Adolesc Gynecol. 2018;31(1):67-70. DOI: 10.1016/j.jpag.2017.07.003
- Kamio M, Nagata C, Sameshima H, Togami S, Kobayashi H. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with septic shock: A case report. J Obstet Gynaecol Res. 2018;44(7):1326-9. DOI: 10.1111/jog.13656
- Cox D, Ching B. Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos. J Radiol Case Rep. 2012; 6(3):9-15. DOI: 10.3941/jrcr.v6i3.877
Downloads
Download data is not yet available.