January 15th, 2021

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Herlyn-Werner-Wunderlich Syndrome: A Diagnostic Challenge: A Case Report
Authors:  Anastasia Vatopoulou, M.D., Ph.D., and Grigoris Grimbizis, M.D., Ph.D.
BACKGROUND: Herlyn-Werner-Wunderlich syndrome represents a very rare congenital abnormality of the female urogenital system. It is characterized by didelphys uterus, blind hemivagina, and ipsilateral renal agenesis and is due to partial embryonic agenesis of both the paramesonephric (Mùˆllerian) and mesonephric (Wolffian) ducts. Symptoms and signs are frequently nonspecific, resulting in delayed diagnosis and in potentially serious acute and long-term complications. Therefore, awareness of this syndrome is important to prevent the associated morbidity.

CASE: We report the case of an adolescent girl with Herlyn-Werner-Wunderlich syndrome.

CONCLUSION: Although rare, this syndrome should be considered in the differential diagnosis of dysmenorrhea, particularly during early adolescence. Magnetic resonance imaging is the imaging method of choice for establishing the diagnosis, and prompt surgical management results in an excellent short- and long-term outcome.
Keywords:  adolescents, female; didelphys uterus; diagnosis, differential; dysmenorrhea; Herlyn-Werner-Wunderlich syndrome; kidney/abnormalities; magnetic resonance imaging; mesonephric ducts; Mullerian ducts; paramesonephric ducts; renal agenesis; urogenital abnormalities; uterus/abnormalities; Wolffian ducts
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