CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2013; 23(03): 243-246
DOI: 10.4103/0971-3026.120283
Abdominal Radiology

Herlyn-Werner-Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis

Zohra Ahmad
Department of Radiology, All India Institute of Medical Sciences, New Delhi, India
,
Ankur Goyal
Department of Radiology, All India Institute of Medical Sciences, New Delhi, India
,
Chandan J Das
Department of Radiology, All India Institute of Medical Sciences, New Delhi, India
,
Dipika Deka
Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, India
,
Raju Sharma
Department of Radiology, All India Institute of Medical Sciences, New Delhi, India
› Author Affiliations
Financial support and sponsorship Nil.

Abstract

Herlyn-Werner-Wunderlich syndrome (HWWS), characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, is an uncommon combined Mullerian and mesonephric duct anomaly, and its presentation in adulthood is even rarer. We report here a 22-year-old female presenting with primary infertility where magnetic resonance imaging (MRI) suggested the diagnosis of HWWS with endometriosis. In a patient of infertility with endometriosis and unilateral renal agenesis, diagnosis of HWWS should be suspected and MRI is the investigation of choice for such anomalies.



Publication History

Article published online:
30 July 2021

© 2013. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 1 Wu TH, Wu TT, Ng YY, Ng SC, Su PH, Chen JY, et al. Herlyn-Werner-Wunderlich Syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol 2012;53:68-71.
  • 2 Jeong JH, Kim YJ, Chang CH, Choi HI. A case of Herlyn-Werner-Wunderlich syndrome with recurrent hematopyometra. J Womens′ Med 2009;2:77-9.
  • 3 Asha B, Manila K. An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis. Fertil Streril 2008;849:e9-10.
  • 4 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.
  • 5 Adair II L, Georgiades M, Osborne R, Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation. J Radiol Case Rep 2011;5:1-8.
  • 6 Buttram VC Jr, Gibbons WE. Mullerian anomalies: A proposed classification (an analysis of 144 cases). Fertil Steril 1979;32:40-6.
  • 7 Prada Arias M, Muguerza Vellibre R, Montero Sánchez M, Vázquez Castelo JL, Arias González M, Rodríguez Costa A. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg 2005;15:441-5.
  • 8 Heinonen PK. Clinical implications of the didelphic uterus: Long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol 2000;91:183-90.
  • 9 Tanaka YO, Kurosaki Y, Kobayashi T, Eguchi N, Mori K, Satoh Y, et al. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imaging 1998;23:437-41.
  • 10 Brody JM, Koelliker SL, Frishman GN. Unicornuate uterus: Imaging appearance, associated anomalies, and clinical implications. AJR Am J Roentgenol 1998;171:1341-7.
  • 11 Singh M, Geareart JP, Rock JA. Double urethra, double bladder, left renal agenesis, persistent hymen, double vagina and uterus didelphys. Adolesc Pediatr Gynecol 1993;6:99.
  • 12 Haddad B, Barranger E, Paniel BJ. Blind hemivagina: Long-term follow-up and reproductive performance in 42 cases. Hum Reprod 1999;14:1962-4.
  • 13 Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, et al. Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 2012;12:4.
  • 14 Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long term follow-up. Obstet Gynecol 1997;90:26-32.
  • 15 Gholoum S, Puligandla PS, Hui T, Su W, Quiros E, Laberge JM. Management and outcome of patients with combined vaginal septum, bifid uterus and ipsilateral renal agenesis (Herlin-Werner-Wunderlich syndrome. J Pediatr Surg 2006;41:987-92.
  • 16 Park NH, Park HJ, Park CS, Park SI. 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;62:383-8.