Objective: To describe the unique presentation and surgical management of a complete uterovaginal septum.
Design: Video case report.
Setting: Tertiary care academic medical center.
Patient(s): A 25-year-old woman, gravida 2, para 0-0-2-0, referred for evaluation after imaging and clinical examination revealed conflicting information. She was initially seen by her local provider for menorrhagia. Locally an ultrasound revealed a septate uterus, and examination under anesthesia with hysteroscopy noted a single vagina and cervix with a unicornuate uterus. Due to incongruous findings, she was referred for evaluation.
Intervention(s): Magnetic resonance imaging (MRI), examination under anesthesia, vaginal surgery, and operative hysteroscopy.
Main Outcomes And Measure(s): The MRI identified a complete uterovaginal septum with a single septate cervix. Vaginal gel was used to define vaginal anatomy, and the gel was noted to fill the right hemivagina with none noted on the left. Examination under anesthesia revealed an imperforate hymen with a small opening on the left as the cause for confusion in the clinical presentation. A hymenectomy was performed followed by guided surgical management of a complete uterovaginal septum, unicollis.
Result(s): The patient was discharged home the same day of surgery.
Conclusion(s): Presentation of müllerian anomalies are often complex, and anatomic variations in commonly described anomalies make misdiagnoses common. Advanced imaging with use of MRI with vaginal gel or three-dimensional ultrasonography and detailed examination are often helpful. Differentiating between unicollis and bicollis presentations in complete uterovaginal septum cases is an important distinction during surgical management.
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http://dx.doi.org/10.1016/j.fertnstert.2020.08.020 | DOI Listing |
Med J Malaysia
August 2024
Universitas Gadjah Mada, Faculty of Medicine, Public Health and Nursing, Department of Obstetric and Gynecology, Yogyakarta, Indonesia.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche.
View Article and Find Full Text PDFInt J Gynaecol Obstet
October 2024
Kaiser Permanente Bernard J. Tyson School of Medicine, Pasadena, California, USA.
Objective: To describe the incidence of uterovaginal anomalies and histologic findings in transgender and nonbinary (TGNB) patients seeking hysterectomies.
Methods: All patients receiving gender-affirming hysterectomies between 2013 and 2023 were retrospectively reviewed. Primary outcomes included uterovaginal anomalies and histological findings.
Ann Med Surg (Lond)
June 2023
Department of Radiology and Imaging, Tribhuvan University Teaching Hospital.
Unlabelled: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome also known as the Herlyn-Werner-Wunderlich Syndrome is a rare embryological disorder associated with Mullerian and mesonephric duct abnormality.
Case Presentation: The cases presented describe the imaging (ultrasound and MRI) findings of four young females who presented with dysmenorrhea and urinary complaints. All of them had solitary kidneys with a didelphic uterus and unilateral hematometrocolpos.
Ann Med Surg (Lond)
October 2022
Department of Gynecology and Obstetrics, Lumbini Provincial Hospital, Lumbini, 32914, Nepal.
Introduction: Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is a rare congenital disorder with an incidence of 1 in 5000 females. It is characterized by uterovaginal aplasia with normal secondary sexual characteristics and genetic karyotype 46XX. The exact etiology of MRKH syndrome is not known.
View Article and Find Full Text PDFInt J Gynaecol Obstet
March 2023
Department of Pediatric Surgery, Aghia Sophia Children's Hospital, Athens, Greece.
Obstructive vaginal and uterine anomalies including imperforate hymen, transverse vaginal septum, and vaginal and/or cervical atresia or aplasia, might rarely present in infancy or childhood with hydrocolpos and/or hydrometra but they usually go unrecognized until presentation with amenorrhea and hematocolpos and/or hematometra in puberty. They should always be included in the differential diagnosis of a suprapubic and/or introital mass; in the latter case, vaginal vascular malformations and vaginal tumors should also be considered. Uterovaginal aplasia typically manifests with amenorrhea in puberty and needs to be differentiated from complete androgen insensitivity syndrome and gonadal dysgenesis of genetic males.
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