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2014, Number 4

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Cir Cir 2014; 82 (4)

Pelvic inflammatory disease due to Herlyn-Werner-Wunderlich syndrome

Alumbreros-Andújar MT, Aguilar-Galán EV, Pérez-Parra C, Céspedes-Casas C, Ramírez-Gómez M, González-López A
Full text How to cite this article

Language: Spanish
References: 9
Page: 448-452
PDF size: 433.80 Kb.


Key words:

Herlyn-Wener-Wunderlich, OHVIRA, hematocolpos.

ABSTRACT

Background: Herlyn-Werner-Wunderlich syndrome is a congenital urogenital malformation that is associated with a uterus didelphys and a longitudinal vaginal septum, resulting in a blind hemivagina and ipsilateral renal agenesis. Clinical presentation is highly variable, delaying diagnosis and leading to important complications.
Clinical case: We present the case of a 13-year-old female who was diagnosed with Herlyn-Werner-Wunderlich syndrome following an acute abdomen due to a right tubo-ovarian abscess. She had a vaginal septum giving rise to a right blind hemivagina. It was microperforated, causing intermittent genital bleeding. This hematocolpos was colonized by microorganisms that ascended to the pelvic cavity, causing right tuboovarian abscess. Nuclear magnetic resonance imaging provided the Wernermost diagnostic information. We performed a vaginal septum resection, and both hemiuteros communicated with a single vagina, resulting in an asymptomatic patient.
Conclusion: Herlyn-Werner-Wunderlich syndrome is a little known entity and can be presented atypically, resulting in diagnostic difficulty and treatment delay. It is important to be aware of this syndrome in order to avoid irreversible complications.


REFERENCES

  1. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87(4):918-922.

  2. Vercellini P, Daguati R, Somigliana E, Viganň P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steri 2007;87(4):719-724.

  3. 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(5):987-992.

  4. Zurawin RK, Dietrich JE, Heard MJ, Edwards CL. Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of literature. J Pediatr Adolesc Gynecol 2004;17(2):137-141.

  5. Mandava A, Prabhakar RR, Smitha S. OHVIRA Syndrome (obstructed hemivagina and ipsilateral renal anomaly) with Uterus Didelphys, and Unusual Presentation. J Pediatr Adolesc Gynecol 2012;25:23-25.

  6. Rastogi A, Khamesra A. Herlyn-Werner-Wunderlich syndrome: a rare urogenital anomaly masquerading as acute abdomen. Indian J Pediat 2010;77(8):917.

  7. Cox D, Ching BH. Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos. J Radiol Case Rep 2012;6(3):9-15.

  8. Güdücü N, Gönenç G, Işçi H, Yiğiter AB, Dünder I. Herln- Werner-Wunderlich Syndrome – Timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol 2012;25(5):e111-112.

  9. Kaba M, Gurgor T, Baser E, Ozdal B, Sirvan L. Cervical cancer in a patient with uterus didelphys and obstructive hemivagina, ipsilateral renal anomaly (OHVIRA) syndrome. Arch Gynecol Obstet. Publicado online el 20 de diciembre de 2012. (consultado 2013 Abril 8). Disponible en http://rd.springer.com/ content/pdf/10.1007%2Fs00404-012-2690-y




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Cir Cir. 2014;82