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Article Synopsis
  • The Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder characterized by a double uterus, obstruction of one side of the vagina, and absence of a kidney, usually affecting the right side, with symptoms often starting at puberty.
  • Common issues experienced by individuals include pelvic pain, heavy menstrual bleeding, and infertility due to complications such as hematocolpos or hematometra, with potential long-term problems like endometriosis and increased risk of miscarriage.
  • Diagnosis typically begins with an ultrasound, but MRI is preferred for confirming the syndrome and identifying associated anomalies; treatment often involves surgically removing the obstructed vaginal septum, as demonstrated in a case study of a 43-year
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Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Endocrinol Diabetes Nutr (Engl Ed)

April 2024

Endocrinology and Nutrition Department, Parc Taulí Hospital Universitari, Institut d'Investigació i Innovació Parc Taulí I3PT-CERCA-ISCIII, Department of Medicine, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome.

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Endometriosis is known to occur frequently in adolescents with obstructed Müllerian anomalies. Our cases emphasize that endometriosis can rapidly progress to a severe stage in obstructed hemivagina and ipsilateral renal anomaly syndrome, one of the completely obstructed Müllerian anomalies. The first patient was a 14-year-old girl who complained of cyclic abdominal pain.

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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods.

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Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations.

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