Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (original) (raw)
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Uterus Didelphys with Obstructed Hemivagina: A Report of Three Cases
Journal of Clinical Case Studies, 2017
We present the cases of three young women (aged 11, 14, and 16 years) who had uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly syndrome. In all cases, we evaluated the anomaly by performing clinical, ultrasonography or computed tomography, and magnetic resonance imaging. Assessment was based on the standard Müllerian classification system. Although obstructed hemivagina and ipsilateral renal anomaly syndrome is a rare anomaly of paramesonephric and mesonephric ducts, it should be considered in the differential diagnosis of young women presenting with cyclic abdominal pain after menarche and a pelvic mass. Magnetic resonance imaging is a useful diagnostic tool for early and accurate classification of Müllerian duct anomalies, such as obstructed hemivagina and ipsilateral renal anomaly syndrome, and can reduce the need for surgical diagnostic methods in these young women.
Journal of Radiology Case Reports, 2011
Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case. It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.
Journal of Gynecologic Surgery, 2012
Background: The unique clinical syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly is very rare and can be quite difficult to recognize because of the enormous heterogeneity in its clinical presentation. There are few long-term reports of the reproductive performance of women with this syndrome following treatment, or about the location of subsequent pregnancies. Case: A case in which two spontaneous pregnancies occurred alternatively in both hemiuteri: one despite a previous ipsilateral large hematometra and hematocolpos and the other, 8 years after, simultaneously with contralateral hematometra and hematocolpos (because of vaginal restenosis), is reported. Drainage of hematocolpos was performed at 14 weeks of pregnancy with immediate pain relief. Results: Pregnancy proceeded without complications. Eight month after delivery, a vaginoplasty was performed by excising the longitudinal vaginal septum, and marsupializing the vaginal cuff. Conclusions: This case highlights the importance of a correct and early diagnosis of developmental anomalies of the urogenital tract, as well as how a conservative approach in a Mü llerian anomaly with unilateral obstruction led to two successful pregnancies occurring alternatively in the unaffected and in the previously blocked side. This is additional information supporting that every effort should be made to preserve the obstructed uterus.
Oman medical journal, 2011
Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome) is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional referral hospital presented with history of dysmenorrhoea and foul vaginal discharge with right cystic pelvic mass. She was diagnosed as a case of double uterus with obstructed right hemivagina and right pyocolpos with ipsilateral renal agenesis after routine ultrasonography in the clinic followed by MRI. Excision of the right vaginal septum with drainage of 200 ml of purulent discharge was performed. She was relieved of her symptoms and conceived promptly after the surgical excision of the partia...
Uterus didelphys with obstructed hemivagina with chronic presentation: a case report
International Journal of Research in Medical Sciences
This is a case report of a 24-year patient who had uterus didelphys with obstructed hemivagina. Clinical manifestations of this case were of progressive dysmenorrhea, abdominal pain after menarche, cyclic difficulty in urination and constipation, with the existing paravaginal tumor indicated this rare anomaly. The diagnosis was by clinical examination and ultrasonography. The patient underwent successful transvaginal septoplasty and drainage of the hematocolpos and hematometra, which is the appropriate mode of treatment in such cases.
The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad
Australian and New Zealand Journal of Obstetrics and Gynaecology, 2009
Study Objective: To compare a case series of the obstructed hemivagina, ipsilateral renal anomaly and uterine didelphys triad with the literature, with a focus on a subset of patients with cervical aplasia. Design, Setting, and Participants: A retrospective case series was conducted of all patients with the triad managed between 2005-2013 at a tertiary center for adolescent gynecology. Results: Similarities in this cohort, compared to the literature, included heterogeneity of presentation, presence of endometriosis, and asymmetry of affected side. Notable differences included 1 patient with a 16p11.2 microdeletion and 2 patients with subsequent unilateral cervical aplasia. All patients underwent magnetic resonance imaging for diagnosis. Vaginal septum division was performed in 8 cases and excision in 1 case. Both cases with cervical aplasia ultimately underwent hemi-hysterectomy, and highlight the implications of this rare variant in regards to its existence, limitations of magnetic resonance imaging in this context, and suggestions for improvement in diagnosis and management. Conclusion: The complexity of these cases, especially the evolving manifestation of cervical aplasia postoperatively, illustrates the need to recognize limitations in imaging and divergence in definitive management.
Uterus Didelphys with Gynatresic Right Vagina, Associated Hematopyocolpos, and Right Renal Agenesis
Journal of Gynecologic Surgery, 1994
We report a case of a patient with uterus didelphys, obliteration of the right cervical os, and septate vagina, with associated right hematopyocolpos and right renal agenesis. This 19-year-old woman with menarche at age 12 and regular periods every 28 days reported 2 years of noncyclic, dull, nonradiating, right lower quadrant pain. This case is reported for two reasons. The first is to call attention to the usefulness of radiologie modalities, such as ultrasound and MRI, in demonstrating both external contour and internal arrangement of the organs. The second reason is the interest of the case itself. The only finding that prevented this from being associated with a right hematometra was the apparent closure at a point in the past of the right internal cervical os and retrograde menstruation through the fallopian tube on the right. Associated renal anomalies are discussed.