Herlyn—Werner—Wunderlich Syndrome: Premenarche

Anita David, Shobha Gudi, R Shankar
In: Journal of South Asian Federation of Obstetrics and Gynaecology · 2017 · vol. 9(2) , pp. 207–210 · doi:10.5005/jp-journals-10006-1496 · W2613289036
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This case report details the surgical management of a 10-year-old premenarcheal girl with Herlyn-Werner-Wunderlich syndrome, a rare Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.

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Abstract

ABSTRACT Aim To increase awareness about this rare condition in order to aid early diagnosis and effective treatment which can eventually prevent complications and infertility issues in the future. Materials and methods A case of a 10 year old pre-menarchal girl, who presented with this syndrome, was managed surgically. After this, a literature search was done and similar case reports were reviewed. Results The Herlyn-Werner-Wunderlich syndrome (HWW) is a rare Müllerian anomaly with an incidence of 0.1-3.8%. It is characterised by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. This condition can be treated with surgery. Since delay in diagnosis may lead to complications, early diagnosis and treatment is beneficial. When Müllerian anomalies are encountered, a screening should also be made for congenital renal abnormalities and vice versa. This condition usually presents postmenarche with abdominal pain due to hematocolpos or dysmenorrhoea. However, there are rare occasions when this condition can present even before menarche. Conclusion The clinical presentation of HWW syndrome requires a high index of clinical suspicion. Though computed tomography or ultrasound can be used to confirm the diagnosis, magnetic resonance imaging is considered to be the preferred imaging modality. Early diagnosis and surgical management is important in order to prevent complications like retrograde tubal reflux, leading to endometriosis and infertility. How to cite this article David A, Gudi SN, Shankar R. Herlyn— Werner—Wunderlich Syndrome: Premenarche. J South Asian Feder Obst Gynae 2017;9(2):207-210.

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endometriosisdysmenorrheainfertility

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