Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Double the trouble: A case report on uterine didelphys with unilateral cervical atresia / Philippine Journal of Obstetrics and Gynecology

@#Genetic errors and teratogenic events during embryonic development can lead to congenital abnormalities of the female reproductive tract. Many patients are asymptomatic, while some have major abnormalities that can cause severe impairment of menstrual and reproductive functions. This case report focuses on Müllerian anomaly Class III, uterine didelphys, along with obstructed hemivagina and ipsilateral renal agenesis (the Herlyn–Werner–Wunderlich syndrome [HWWs]). The patient presented with cyclic pelvic pain due to the hematometra and hematocolpos. She initially underwent resection of the vaginal septum but had a recurrence of obstructive symptoms more than a year after vaginal surgery was performed. This prompted further evaluation with magnetic resonance imaging revealing cystic dilatation of the right uterine horn and a hypoplastic right vagina appearing to end blindly. The patient subsequently underwent hemihysterectomy. The right hemiuterus was noted to have a depression on its thickened inferior aspect, but no cervix was identified. This case is a variant of the classic HWWS, as there was cervical atresia on the right uterine corpus. A thorough preoperative evaluation and accurate intraoperative assessment of patients with Müllerian anomalies can decrease misdiagnoses, guide appropriate intervention, and decrease the risk of future reproductive complications.

Herlyn-Werner-Wunderlich Syndrome: A Case Report.

Herlyn-Werner-Wunderlich syndrome is a rare congenital malformation of the Mullerian ducts characterized by uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis. Commonly, such patients present with pelvic pain, dysmenorrhea following menarche, and an abdominal mass secondary to hematometrocolpos. In this report, a case of a 14-year-old female presented with abdominal pain, back pain and acute urinary retention. She attained menarche at the age of 10 years; however, symptoms of dysmenorrhea only appeared 4 years later. She was eventually diagnosed with the help of ultrasound and computed tomography urogram. She was managed conservatively with an incision and drainage procedure and was also advised for resection of vaginal septum. The nonspecific nature of symptoms such as regular menstruation with cyclical abdominal pain impedes the diagnosis which can lead to an array of complications. Awareness of this syndrome can help avoid misdiagnosis and allow for early surgical intervention. Keywords: case reports; dysmenorrhea; uterine didelphys.

Embarazo multiple en utero didelfo y bicollis.. Reporte de caso / Multiple pregnancy in uterus didelphys and bicollis. Case report

El útero didelfo forma parte del grupo de anomalías müllerianas. Este se produce posterior a una falla en la fusión de los conductos müllerianos, resultando dos cavidades uterinas diferentes y dos cérvix que se unen en el extremo uterino inferior. En la mayoría de los casos esta malformación se asocia a un tabique vaginal longitudinal. Todo esto debido a deficiencias en el proceso de formación de los conductos müllerianos. Los embarazos gemelares en úteros didelfos se estiman en uno por cada millón de embarazos, pero en la actualidad se encuentran reportados pocos casos en el mundo. Esta revisión relata el caso de una paciente con útero didelfo, quién presentó un embarazo múltiple con un feto en cada uno de los hemiúteros. Sin ningún método de apoyo para la concepción del embarazo, se obtuvieron dos recién nacidos sanos, tras cesárea de emergencia por iniciar trabajo de parto y ruptura de membranas, con distocia de partes blandas ocasionada por el tabique vaginal

The didelphys uterus is part of the group of Müllerian anomalies. This occurs after a failure in the fusion of the Müllerian ducts, resulting in two different uterine cavities and two cervix that unite at the lower uterine end. In most cases this malformation is associated with a longitudinal vaginal septum. All this due to deficiencies in the process of formation of the Müllerian ducts. Twin pregnancies in didelphys uteri are estimated at one per million pregnancies, but few cases are currently reported in the world. This review reports the case of a patient with a didelphic uterus, who presented a multiple pregnancy with a fetus in each of the hemi-uteri. Without any support method for the conception of pregnancy, two healthy newborns were obtained, after emergency cesarean section due to initiation of labor and rupture of membranes, with soft tissue dystocia caused by the vaginal septum

The wandering twin: A case of a uterine didelphys with the obstructed hemiuteri in the anterior abdominal wall

@#Mullerian duct anomalies (MDAs) are congenital defects of the female genital system that arise from abnormal embryological development of the Mullerian ducts. A didelphys uterus, also known as a "double uterus," is one of the least common amongst the MDAs. Reported here is a case of a 16 year old female with a uterus didelphys with the obstructed left hemiuteri adherent in the anterior abdominal wall, and an endometriotic cyst on the same side. She underwent hysteroscopy-guided vaginoscopy, laparoscopic left hemihysterectomy, left oophorocystectomy. Cases such as these require careful preoperative planning and diagnostic imaging for more accurate diagnosis and, hence, for the most appropriate surgical procedure to be carried out. 3D ultrasonography and Magnetic Resonance Imaging have been the most widely used imaging techniques. The goals of management are to relieve the symptoms of obstruction and to restore the normal anatomy as much as possible in order to provide the best chance for future fertility.

A second look at the embryogenesis of the mullerian duct system in the background of purulent vaginal discharge in wunderlichhherlyn-werner syndrome

Wunderlich-Herlyn-Werner syndrome is an uncommon but already well-established mullerian anomaly consisting of uterine didelphys, hemivaginal septum and ipsilateral renal agenesis. The discussion here would concentrate on the occurrence of foul-smelling vaginal discharge in some of these cases, its pathogenesis and its impact on generally-accepted assumptions on the embryogenesis of the mullerian system. Reported here are three such cases with discussions on the goals of therapy being relief of the obstructive symptoms, restoration of normal menstrual egress and sexual function, with preservation of reproductive potential.

Uterine didelphys and a blind hemivagina in a 6 year-old: A case report

A six year-old presented with a 10-month history of vaginal discharge. On initial gynecologic evaluation, a vaginal mass was noted. A thorough investigation included pelvic sonogram, KUB sonogram, pelvic examination under anesthesia. A close team-up with a pediatrician and a urologist was established. Intra-operative findings revealed uterine didelphys and a blind hemivagina. This report discusses the approach to the investigation of vaginal discharge in association with a vaginal mass in a 6-year old.