The Obstructed Hemivagina, Ipsilateral Renal Anomaly, Uterus Didelphys Triad

17 08 2009

To the Editor:

We read with interest the paper by Asha and Manila published in September 2008. (1) Here the authors described a case of the triad of obstructed hemivagina, uterus didelphys and ipsilateral renal anomaly. In this article the authors state that this set of anomalies is also known as the Herlyn-Werner-Wunderlich Syndrome. The authors are correct in what they say; however we would like to take this opportunity to disagree with the triad being named thus, and clarify the history of reports of this condition.

The combination of obstructed hemivagina and uterus didelphys was first reported in 1922;(2) however, the triad of obstructed hemivagina and uterus didelphys as well as an ipsilateral renal anomaly was not reported until 1950.(3) Since 1983 (4) the triad has been called the Herlyn-Werner-Wunderlich syndrome in at least 10 papers. This eponymous syndrome was most likely derived from a paper in German by Gazárek et al in 1979 (5) where the terms Herlyn-Werner and Wunderlich syndromes were used as two separate entities from the original descriptions in German by Herlyn and Werner in 1971 and Wunderlich in 1976, respectively. (6) Unfortunately, none of the case descriptions in these later two papers describe the triad of obstructed hemivagina, uterus didelphys and ipsilateral renal anomaly. Following Gazárek’s paper the triad was then termed Herlyn-Werner-Wunderlich syndrome in three successive Bulgarian language papers between 1986-90. (7-9) The first English language paper to call it thus was in 2006.(10)

More recently the acronym OHVIRA has been used to describe patients with an obstructed hemivagina and ipsilateral renal anomaly.(11) By including two out of the three components of the triad, it enables inclusion of uterine anomalies, other than a uterus didelphys, such as a completely septate uterus, which is present in the OHVIRA syndrome in up to 22% of cases.(12)

Rebecca M.N. Kimble, MBBSa
Roy M. Kimble, MBChBb

aObstetric Services and Paediatric and Adolescent Gynaecology
Royal Brisbane and Women’s Hospital
Discipline of Obstetrics and Gynaecology
University of Queensland
Brisbane, Australia
b Department of Paediatrics & Child Health
University of Queensland Royal Children’s Hospital
Brisbane, Australia

References:
1. Asha B, Manila K. An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis. Fertil Steril. 2008; 90: 849.e9-10.

2. Purslow CE. A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp. 1922; 29: 643

3. Embrey MP. A case of uterus didelphys with unilateral gynatresia. Br Med J. 1950; 1: 820-1

4. Karagozov I. Herlyn-Werner-Wunderlich syndrome. Akush Ginekol 1983; 22: 70 – 6.

5. Gazárek F, Kudela M, Zenisek L, Nevrla F. Herlyn-Werner and Wunderlich syndromes. Zentralbl Gynakol. 1979; 101:1411-5.

6. Herlyn U, Werner H. Simultaneous occurrence of an open Gartner duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd 1971; 31: 340 – 7.

7. Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol 1976;98:559 – 62.

8. Nalbanski B, Sirakov M, Veltova L, Karag’ozov I. A report of 2 cases of the Herlyn-Werner-Wunderlich syndrome. Akush Ginekol 1990; 29: 47-51

9. Novachkov L, Sirakov M. Pregnancy and labor in a woman with Herlyn-Werner-Wunderlich syndrome. Akush Ginekol 1986;25(1):91-4

10. 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 (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg. 2006; 41: 987–92

11. Smith N, Laufer M. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertility and Sterility 2007; 87: 918-922

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.

Published online in Fertility and Sterility doi:10.1016/j.fertnstert.2009.08.046

The Authors Respond:

We thank Doctors Roy and Rebecca Kimble for reading and showing interest in our article. We would take opportunity in responding to their view.

It is true that recently the acronym OHVIRA has been used to describe patients with an obstructed hemivagina and ipsilateral renal anomaly by including two out of the three components of the triad known as the Herlyn-Werner-Wunderlich Syndrome. It was interesting to know how this eponymous syndrome got its name. However many recent articles have mentioned Herlyn-Werner-Wunderlich syndrome as a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly (1-4). OHVIRA would include other müllerian anomalies like septate, arcuate and bicornuate uterus, which are commonly found. Our case report was unique as the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly is rarely seen, and causes diagnostic difficulty as women menstruate regularly.

Manila Kaushal, M.S.
Disha Fertility and Surgical Center
Indore, Madhya Pradesh, India

References
1. Suad G, Pramod S. Puligandlaa, Thomas H, Wendy S, Elsa Q et al. Management and outcome of patients combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). 2006; 41:987-92.

2. Orazi C, Lucchetti MC, Schingo PMS 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.

3. Sarac A, Demir MK. Herlyn-Werner-Wunderlich syndrome: a rare cause of infertility Eur Radiol. 2009;19:1306-8. Epub 2009 Apr 1

4. Rana R, Pasrija P, Manju. Herlyn-Werner-Wunderlich syndrome with pregnancy: A rare presentation Congenital Anomalies.2008; 48:142-3.

Published online in Fertility and Sterility doi:10.1016/j.fertnstert.2009.08.044

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One response

20 10 2010
elsie

Good day! I would like to ask if there are cases of pregnancy in Herlyn, Werner & Wunderlich on the obstructed hemivagina? Or maybe in a partial hemivaginal obstruction? Because we have a case in our hospital where pregnancy occured on the obstructed vagina.

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