title: Imaging Diagnosis of Herlyn-Werner-Wunderlich Syndrome- An Extremely Rare Urogenital Anomaly.
abstract: Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract resulting from maldevelopment of both Mullerian and Wolffian ducts. It is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It generally presents at puberty shortly following menarche with the symptom of acute pelvic pain. Management of these cases is surgical and consists mainly of vaginoplasty with excision of the vaginal septum in order to release the obstruction and prevent the long term complication of recurrent pyocolpos and infertility. We report here a case of Herlyn-Werner-Wunderlich syndrome in a 13-year-old adolescent girl, emphasizing the role of imaging in the accurate and prompt diagnosis of this rare developmental urogenital anomaly. Only a few hundred such cases have been reported in literature till date.
authors.affiliation: Associate Professor, Department of Radiodiagnosis, Dr Ram Manohar Lohia Hospital & PGIMER , New Delhi, India .
authors.forename: Shibani
authors.initials: S
authors.lastname: Mehra
authors.affiliation: Senior Resident, Department of Radiodiagnosis, Dr Ram Manohar Lohia Hospital & PGIMER , New Delhi, India .
authors.forename: Komal
authors.initials: K
authors.lastname: Chamaria
authors.affiliation: Professor and Head of Department, Department of Radiodiagnosis, Dr Ram Manohar Lohia Hospital & PGIMER , New Delhi, India .
authors.forename: U C
authors.initials: UC
authors.lastname: Garga
authors.affiliation: Senior Resident, Department of Radiodiagnosis, Dr Ram Manohar Lohia Hospital & PGIMER , New Delhi, India .
authors.forename: Ankur
authors.initials: A
authors.lastname: Kataria
authors.affiliation: Senior Resident, Department of Radiodiagnosis, Dr Ram Manohar Lohia Hospital & PGIMER , New Delhi, India .
authors.forename: Ashim
authors.initials: A
authors.lastname: Ahuja
keywords: Hematometra; MRI; Obstructed hemivagina; USG; Uterus Didelphys; Vaginal atresia; Vaginal septum