Abstract
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to HWWS. The diagnosis was established with abdominal ultrasound and Magnetic Resonance Imaging (MRI). The patient subsequently underwent surgical resection of the vaginal septum resulting in relief of obstruction. Clinical symptoms in patients with HWWS typically present after menarche with progressive hematometra causing pain and compression of localized structures. Even though ultrasound can help in the diagnosis, MRI is the best choice of imaging for the visualization of these anomalies. The diagnosis of HWWS is important to consider in young females of reproductive age presenting with symptoms of obstruction of adjacent structures. Our patient presented with acute urinary retention which is a rare symptom in this entity. A high index of clinical suspicion and awareness of the syndrome are required to make a speedy diagnosis and prevent future complications.
Highlights
We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to Herlyn-Werner-Wunderlich syndrome (HWWS)
Herlyn-Werner-Wunderlich syndrome (HWWS) known as OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis [1]
Ultrasound and Magnetic Resonance Imaging (MRI) findings can collectively aid in assessing the anatomy of the uterus, absence of the ipsilateral kidney and the nature of the fluid content in the obstructed hemivagina
Summary
Herlyn-Werner-Wunderlich syndrome (HWWS) known as OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis [1]. It is hypothesized that HWWS is caused by the anomalous development of Müllerian and Wolffian ducts [1] It is generally observed in adolescents and young women within 1-2 years post-menarche [3]. Our patient reported a recurrence of such symptoms for the past few months, around her menstrual period, but with spontaneous resolution She denied any history of fever, hematuria, blood in the stool, vomiting or abdominal pain. She had a history of urinary tract infection (UTI) five months prior to the presentation that resolved after antibiotics. She continued laxatives but had a poor appetite and did not have a bowel movement since admission She developed severe rectal pain and the rectal exam revealed a firm mass anterior to the rectal vault.
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