Herlyn-Werner-Wunderlich syndrome: a very rare urogenital anomaly in a teenage girl

Leyla Karaca, Berhan Pirimoglu, Berhan Pirimoğlu, Ümmügülsüm Bayraktutan, Ummugulsum Bayraktutan, Hayri Ogul, Hayri Oğul, Şükrü Oral, Akgun Oral, Mecit Kantarci, Mecit Kantarcı
The Journal of emergency medicine · 2014 · vol. 48(3) , pp. e73–e75 · doi:10.1016/j.jemermed.2014.09.064 · PMID:25499400 · W1975085906
Case report OA: closed CC0 ⤵ 4 in-corpus citations
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This case report describes the presentation and management of a teenage girl diagnosed with Herlyn-Werner-Wunderlich syndrome, a rare urogenital anomaly characterized by didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis.

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Abstract

BACKGROUND: Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon variant of Müllerian duct anomalies, consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in a post-pubertal adolescent or adult woman in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. CASE REPORT: We report the case of a 13-year-old girl who presented to the emergency radiology department with sudden onset of severe pain at the right lower quadrant of the abdomen; imaging confirmed the diagnosis of HWW syndrome. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: When unilateral renal agenesis and uterus didelphys coexist, the first thing that the physician should remember is to confirm or refute the presence of a blind vagina for diagnosis of HWW syndrome.

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MeSH descriptors

Abnormalities, Multiple Congenital Abnormalities Hydrocolpos Kidney Kidney Diseases Uterus Vagina Abdomen, Acute Abdomen, Acute Abnormalities, Multiple Adolescent Congenital Abnormalities Female Humans Hydrocolpos Hydrocolpos Kidney Kidney Diseases Kidney Diseases Magnetic Resonance Imaging

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License: CC0 · commercial use OK