{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T02:20:51Z","timestamp":1773886851243,"version":"3.50.1"},"reference-count":14,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2021,12,30]],"date-time":"2021-12-30T00:00:00Z","timestamp":1640822400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>The aim of this study was to present a new case of congenital Herlyn\u2013Werner\u2013Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.<\/jats:p>","DOI":"10.3390\/diagnostics12010083","type":"journal-article","created":{"date-parts":[[2021,12,30]],"date-time":"2021-12-30T21:41:21Z","timestamp":1640900481000},"page":"83","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["A New Case of Herlyn\u2013Werner\u2013Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney"],"prefix":"10.3390","volume":"12","author":[{"given":"Jin-Hee","family":"Yu","sequence":"first","affiliation":[{"name":"Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7890-8348","authenticated-orcid":false,"given":"Sa-Ra","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heayeon","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kun-Suk","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Urology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Byung-Moon","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,12,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.crad.2007.04.025","article-title":"MRI in the assessment of congenital vaginal anomalies","volume":"63","author":"Humphries","year":"2008","journal-title":"Clin. Radiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1016\/j.fertnstert.2006.11.015","article-title":"Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: Management and follow-up","volume":"87","author":"Smith","year":"2007","journal-title":"Fertil. Steril."},{"key":"ref_3","first-page":"340","article-title":"Das gemeinsame Vorkommen von effener Gartner-Gang-Zyste, gleichseitiger Nierenaplasie und Uterusdoppelmissbildung als typisches Missbildungssyndrom Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities","volume":"31","author":"Herlyn","year":"1971","journal-title":"Geburtshilfe Frauenheilkd."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"480.e17","DOI":"10.1016\/j.crad.2020.01.016","article-title":"Herlyn-Werner-Wunderlich syndrome: Diverse presentations and diagnosis on MRI","volume":"75","author":"Zhang","year":"2020","journal-title":"Clin. 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Reprod."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1093\/humupd\/dmv048","article-title":"The presentation and management of complex female genital malformations","volume":"22","year":"2016","journal-title":"Hum. Reprod. Update"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.ejogrb.2004.01.042","article-title":"Complex genital malformation: Ectopic ureter ending in a supposed mesonephric duct in a woman with renal agenesis and ipsilateral blind hemivagina","volume":"117","author":"Susarte","year":"2004","journal-title":"Eur. J. Obs. Gynecol. Reprod. Biol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.jmig.2012.03.017","article-title":"Laparoscopic-assisted uterovaginal anastomosis in congenital atresia of uterine cervix: Follow-up study","volume":"19","author":"Kriplani","year":"2012","journal-title":"J. Minim. 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Steril."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Kim, D.Y., Nam, G., Lee, S.R., Kim, S.H., Chae, H.D., and Kang, B.M. (2021). Congenital Obstructive M\u00fcllerian Anomaly: The Pitfalls of a Magnetic Resonance Imaging-Based Diagnosis and the Importance of Intraoperative Biopsy. J. Clin. 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