A RARE CASE OF HERLYN-WERNER-WUNDERLICH SYNDROME : HOW, WHEN & WHY

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Dr. Shiralee Runwal
Dr. Sudha Karnawat

Abstract

OHVIRA syndrome (Obstructed hemivagina with ipsilateral renal agenesis), eponymously termed as Herlyn-Werner-Wunderlich syndrome is an extremely rare disorder characterized by congenital birth defect of lower abdominal and pelvic organs. Although the true incidence is unknown, it is reported to be 0.1 to 3%. It usually presents as progressively increasing pelvic pain during menstruation or a pelvic mass or rarely as primary or secondary infertility in later years. Whenever a multicystic dysplastic kidney or renal agenesis is noted in a new born female, it is recommended to search for concomitant uterovaginal anomalies. Thus, the role of paediatric nephrologist cannot be overemphasized in collaboration with the gynaecologist. This case report also aims at unleashing the relatively new theory of urogenital development by Acien, as OHVIRA syndrome refutes the traditional concept of the same.



Keywords: OHVIRA syndrome; Congenital birth defect; Urogenital development; Mullerian anomalies

Published Jan 10, 2022

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