OHVIRA syndrome with Orofacial clefts: a rare association

OHVIRA syndrome with Orofacial clefts: a rare association DOI: https://doi.org/10.18203/2320-1770.ijrcog20200923Keywords: Herlyn-Werner-Wunderlich syndrome, Obstructed hemivagina, Orofacial defect, VaginoplastyAbstract Obstucted hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome or Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital anomaly consist of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We are reporting an adolescent girl with orofacial defect who presented with lower abdominal pain. She attained menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. On abdominal examination a firm, mobile tender mass extending from left iliac fossa up to umbilicus (24 weeks size) was found. Lower border of mass could not be approached. Further evaluation with ultrasound showed enlarged uterus with collection with internal echoes and non-visualization of the left kidney. CECT showed absent left kidney and didelphys uterus with large left hematocolpometra with left complex adenexal cyst. Patient was posted for hematocolpos drainage and vaginoplasty. An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to severe complications such as endometriosis and infertility. Metrics References Aveiro AC, Miranda V, Cabral AJ, Nunes S, Paulo F, Freitas C. Herlyn-Werner-Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls. BMJ Case Rep. 2011;2011:bcr0420114147. Piccinini PS, Doski J. Herlyn-Werner-Wunderlich syndrome: a case report. Rev Bras Ginecol Obstet. 2015;37(4):192-6. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87(4):918-22. Purslow C. A case of unilateral haematokolpos, haematometra and haematosalpinx. BJOG Int J Obstet Gynaecol. 1922;29(4):643. Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007;37:657-65. Grimbizis GF, Campo R. Congenital malformations of the female genital tract: The need for a new classification system. Fertil Steril. 2010;94:401-7. Gungor Ugurlucan F, Dural O, Yasa C, Kirpinar G, Akhan S. Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome. Clin Imag. 2020;59(2):172-8. Tong J, Zhu L, Lang J. Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome. Inter J Gynecol Obstet. 2013;121(2):173-5. Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol. 2012;25:e23-5. Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. State of the art. Radiol. 2004;233:19-34. Capito C, Echaieb A, Loratat-Jcob S, Thibaud E, Sarnacki S, Nihoul-Fekete C. Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: A series of 32 cases. Pediatr. 2008;122:e891-e897. Takagi H, Matsunami K, Imai A. Uterovaginal duplication with blind hemivagina and ipsilateral renal agenesis: Review of unusual presentation. J Obstet Gynaecol. 2010;30:350-3. Prada Arias M, Muguerza Vellibre R, Montero Sanchez M, Vázquez Castelo JL, Arias González M, Rodríguez Costa A. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg. 2005;15:441. Dhar H, Razek YA, Hamdi I. Uterus didelphys with obstructed hemivagina, Ipsilateral renal agenesis and right pyocolpos: a case report. Oman Med J. 2011;26(6):447-50. Kim TE, Lee GH, Choi YM, Jee BC, Ku SY, Suh CS, et al. Hysteroscopic resection of the vaginal septum in uterus didelphys with obstructed hemivagina: a case report. J Korean Med Sci. 2007;22(4):766-9. Sharma D, Janu MK, Gaikwad R, Usha MG. Herlyn-Werner-Wunderlich syndrome complicated with pyocolpos: An unusual cause of postabortal sepsis. J Gynecol Endosc Surg. 2011;2:105-8.