1. Three Cases of Didelphys Uterus, Including Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome) AKA Herlyn-Werner-Wunderlich Syndrome), with a Systematic Review
- Author
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Cass, Paul N Fuller, Shadi Rezai, er C Hughes, Celia Ligorski, ra E Henderson, and Alex
- Subjects
Gynecology ,medicine.medical_specialty ,Hysterectomy ,business.industry ,Uterine fibroids ,Obstetrics ,medicine.medical_treatment ,Endometriosis ,medicine.disease ,Uterus didelphys ,medicine.anatomical_structure ,Menstrual cramps ,Pelvic inflammatory disease ,medicine ,Vaginal septum ,medicine.symptom ,business ,Cervix - Abstract
Background Herlyn Werner Wunderlich HWW syndrome also known as obstructed hemivagina ipsilateral renal agenesis OHVIRA is characterized by the presence of a didelphys uterus obstructed hemivagina and ipsilateral renal agenesis OHVIRA most commonly presents with symptoms of menarche pelvic pain dysmenorrhea and an abdominal mass Surgical intervention is often required to correct malformations and reduce the incidence of pelvic endometriosis infection and adhesions and infertility for these patients We present three cases MDA with various presentations and clinical courses Objective Review of mullerian and other uterine abnormalities relationship to reproductive performance and pregnancy Outcome Presentation of Cases Case year old Hispanic female G P presented with Primary dysmenorrhea mullerian anomaly and solitary kidney Ultrasound and Magnetic Resonance Imaging MRI showed uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis OHVIRA Syndrome AKA Herlyn Werner Wunderlich Syndrome Case A years old Hispanic female Gravida Para with history of two previous cesarean deliveries presented with retained intrauterine device IUD The IUD strings could not be visualized on the exam but ultrasound and MRI revealed IUD in the right endocervical canal in the lower portion of the uterus Diagnostic hysteroscopy confirmed the diagnosis of didelphys uterus with a horizontal vaginal septum The retained IUD was clearly visible on the lower cervix under the vaginal septum and was easily removed with ring forceps Case year old Hispanic Female G P at weeks gestational age presented for her first prenatal visit Bedside ultrasound showed a second mass next to the uterus official ultrasound revealed duplicated uterine horns and cervices Differential diagnosis includes uterine didelphys and bicornuate bicollis uterus Single live intrauterine pregnancy within the right uterine horn noted Patient declined pelvic MRI for further evaluation of didelphys uterus despite counseling that MRI was safe imaging tool during pregnancy Discussion Mullerian duct anomalies MDA encompass a large range of defects resulting from an abnormal fusion or failure of fusion of paramesonephric ducts during embryogenesis Patient and were all diagnosed with didelphys uteri However for each patient the clinical symptomatology diagnostic imaging and management was different MDAs are present in a small percentage of the population However incidence increases among women with a history of recurrent pregnancy loss and many are incidental findings such as occurred in patients two and three If ultrasound suggests MDA it can be confirmed by magnetic resonance imaging MRI which is the gold standard for diagnosis A new classification system for HWW was created in at the Peking Union Medical College Hospital by Zhu et al Symptomatic treatment of these malformations can be hormonal or surgical The patient in case was placed on hormonal therapy to improve dysmenorrhea and pelvic pain However surgical intervention was ultimately needed to correct her anomaly thereby lowering the risk of infection adhesions endometriosis miscarriages and preterm labor Conclusion Mullerian duct anomalies encompass a range of uterine abnormalities that occur because of interrupted development and fusion of the paramesonephric ducts during embryogenesis Diagnosis may occur as a consequence of clinical symptoms fertility complications or as an incidental finding Mullerian duct abnormalities of a specific uterine anomaly and renal findings characterize OHVIRA HWW Ultrasound and MRI play an important role in prompt diagnosis of MDAs and HWW often being discovered during prenatal care Prompt diagnosis and treatment is needed to ensure relief of symptoms prevention of infection and preservation of fertility
- Published
- 2017