Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature.

Autor: Borges AL; Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal.; Faculdade de Ciências da Saúde, Universidade da Beira Interior, Av. Infante D. Henrique, Covilhã, Portugal., Sanha N; Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal., Pereira H; Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal., Martins A; Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal., Costa C; Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal.
Jazyk: angličtina
Zdroj: Radiology case reports [Radiol Case Rep] 2023 Jun 06; Vol. 18 (8), pp. 2771-2784. Date of Electronic Publication: 2023 Jun 06 (Print Publication: 2023).
DOI: 10.1016/j.radcr.2023.05.024
Abstrakt: Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.
(© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)
Databáze: MEDLINE