Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Usually, such patients present with dysmenorrhea shortly after menarche, increasing pelvic pain and a palpable mass due to the obstructed hemivagina. Interestingly in the present case, the patient had her menarche seven years ago, but dysmenorrhea started only 1-year back. She never sought medical help previously as she was mostly asymptomatic all through the years. It was only after she conceived and got investigated for antenatal concerns that she was found to have HWWS. A tortuous history and an unusual clinical presentation made this case an interesting one.
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http://dx.doi.org/10.4103/tcmj.tcmj_13_19 | DOI Listing |
Front Vet Sci
December 2024
Department of Veterinary Surgery, College of Veterinary Medicine, Konkuk University, Seoul, Republic of Korea.
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), also called Herlyn-Werner-Wunderlich syndrome, is an extremely rare Müllerian duct anomaly accompanied by Wolffian duct anomalies. A 10-year-old intact female Yorkshire Terrier weighing 3.35 kg was presented with anorexia, depression, vomiting, and abdominal pain.
View Article and Find Full Text PDFMedicina (Kaunas)
November 2024
Department of Anatomy and Embryology, "Iuliu Hatieganu" University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania.
Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome.
View Article and Find Full Text PDFJ Clin Med
December 2024
Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany.
: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. : A literature search was conducted in PubMed and Web of Science, using the following search terms: "endometriosis and uterine malformation, endometriosis and Herlyn-Werner-Wunderlich syndrome", "endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome" and "uterus didelphys, renal agenesis and endometriosis". : We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA.
View Article and Find Full Text PDFBackground: Complete bicorporeal uterus, double cervix and obstructive longitudinal vaginal septum (classified as U3bC2V2 according to ESHRE/ESGE classification) is a rare congenital anomaly of the genital tract. This condition is typically associated with ipsilateral renal agenesis and is known as Herlyn-Werner-Wunderlich syndrome or OHVIRA (Obstructed HemiVagina and Ipsilateral Renal Anomaly) syndrome. The primary symptoms include dysmenorrhea and pelvic pain, which usually manifest after menarche due to haematocolpos in the obstructed hemivagina.
View Article and Find Full Text PDFWorld J Clin Oncol
October 2024
Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education; West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare Müllerian duct anomaly, characterized by a combination of urogenital abnormalities. The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is exceptionally rare, posing significant challenges for screening, early diagnosis, and effective management.
Case Summary: We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.
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