Abstract
Aim To evaluate the clinical features, diagnostic routine, treatment, and prognosis of patients with double uterus with obstructed hemivagina and ipsilateral renal agenesis at a University Hospital. Methods A retrospective study analyzing the medical charts of outpatients with similar complex genital malformations seen at the University Hospital of the Ribeirão Preto Medical School from 1994 to 2015. Results Fourteen patients were included in this retrospective study, all presenting with double uterus with obstructed hemivagina and ipsilateral renal agenesis. The main symptom was dysmenorrhea occurring shortly after menarche, and pelvic ultrasound was the examination of choice. The treatment consisted of resection of the vaginal septum, complemented by an abdominal approach in 5 cases. Complications of the syndrome observed in this case series included severe endometriosis, pelvic abscess, need for hysterectomy, and salpingectomy. Conclusions Severe dysmenorrhea shortly after menarche is a typical symptom of this kind of malformation, even though the diagnosis of patients who present with fistulization of the vaginal septum can be delayed due to milder clinical features. Pelvic ultrasound can be considered the first-choice examination in diagnostic routine. Relief of pain and prevention of complications can be achieved successfully in most cases by resection of the vaginal septum.
Highlights
The presence of double uterus with obstructed hemivagina and ipsilateral renal agenesis, referred to as HerlynWerner-Wunderlich (HWW) syndrome, or OHVIRA syndrome, is a type of complex anomaly associated with changes in the fusion of the mullerian ducts, with different degrees of uterine duplicity [1, 2], in addition to obstruction of the hemivagina and ipsilateral renal agenesis [3], corresponding to a concomitant Wolffian abnormality
Whatever the type of malformation, in all cases there is obstruction of the unilateral hemivagina and ipsilateral renal agenesis, so that the condition is known as obstructed hemivagina and ipsilateral renal agenesis (OHVIRA)
This was a retrospective study based on the survey of the medical records of patients seen from 1994 to 2015 at the Human Reproduction, Gynecological Endoscopy and Children’s and Pubertal Outpatient Clinics of the Department of Gynecology and Obstetrics of FMRP-USP with a diagnosis of double uterus with obstructed hemivagina and ipsilateral renal agenesis, according to diagnostic criteria established in the scientific literature [1,2,3,4]
Summary
The presence of double uterus with obstructed hemivagina and ipsilateral renal agenesis, referred to as HerlynWerner-Wunderlich (HWW) syndrome, or OHVIRA syndrome, is a type of complex anomaly associated with changes in the fusion of the mullerian ducts, with different degrees of uterine duplicity (class III of Buttran and Gibbons) [1, 2], in addition to obstruction of the hemivagina and ipsilateral renal agenesis [3], corresponding to a concomitant Wolffian abnormality. The induction of the Mullerian by the mesonephric ducts is disrupted, resulting in nonabsorption defects as the uterine duplicate [5, 6]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
