Embarazo gemelar espontáneo en útero bicorpóreo: una contribución a la experiencia / Spontaneous twin pregnancy in a bicorporeal uterus, a contribution to the experience

Resumen ANTECEDENTES: Las anomalías uterinas congénitas son consecuencia de la formación anormal, fusión o reabsorción de los conductos de Müller durante el desarrollo embriológico. El útero bicorne, unicorne y didelfo (U1, U2, U3 ESHRE-ESGE) son defectos en la unificación con una baja prevalencia en la población general y en la que tiene antecedente de aborto e infertilidad; se estima en alrededor de 0.4 a 1.1%, respectivamente. La presentación del caso puede aportar información útil relacionada con la evolución del embarazo y los desenlaces perinatales cuando aparece esta anomalía. CASO CLÍNICO: Paciente de 35 años, con amenorrea secundaria y prueba inmunológica de embarazo positiva, con antecedentes de dismenorrea primaria severa y anomalía uterina congénita compatible con útero bicorpóreo (U3 completo ESHRE-ESGE), bicorne completo (4a-AFS) y didelfo. El embarazo trascurrió sin complicaciones hasta la semana 37, cuando finalizó mediante cesárea, con hipotonía uterina que no ameritó transfusión de hemoderivados. CONCLUSIÓN: Los embarazos gemelares con útero bicorne son excepcionales y casi todos se logran espontáneamente; los que llegan a término finalizan mediante cesárea electiva.

Abstract BACKGROUND: Congenital uterine anomalies are the result of abnormal formation, fusion, or resorption of the Müllerian ducts during embryological development. The bicornuate, unicornuate and didelphic uterus (U1, U2, U3 ESHRE/ESGE), are unification defects with a low prevalence both in the general population and in the population with a history of abortion and infertility, is estimated at around 0.4 to 1.1%, respectively. The presentation of the case can provide useful information related to with the evolution of the pregnancy and the perinatal outcomes when this anomaly appears. CLINICAL CASE: A 35-year-old patient with secondary amenorrhea and a positive immunological pregnancy test with a history of severe primary dysmenorrhea and previous magnetic resonance imaging with a congenital uterine anomaly compatible with a bicorporeal uterus (U3 complete ESHRE-ESGE), complete bicornuate (4a /AFS) , uterus didelphys (ASRM). The pregnancy was uncomplicated until 37 weeks, when it was terminated by cesarean section, with uterine hypotonia that did not require transfusion of blood products. CONCLUSION: Twin pregnancy with bicornuate uterus is a rare entity, most of them have been achieved spontaneously and those that reach term have been resolved by elective cesarean section.

Útero bicorporeo - doble cérvix con embarazo gemelar en una sola cavidad uterina: reporte de caso / Bicorporeo uterus - double cervix with twin pregnancy in a single uterine cavity: case report

El embarazo gemelar en una de las cavidades uterinas del útero bicorpóreo es una rara presentación clínica. La viabilidad de este tipo de embarazo es de baja probabilidad, dado que esta malformación puede condicionar parto prematuro, placentación anormal, restricción del crecimiento fetal o progresión anormal del trabajo de parto. Se presenta un caso de un útero bicorpóreo con doble cérvix y una gestación gemelar en unas de las cavidades uterinas, con muerte fetal de uno de los fetos y parto pretérmino de 28 semanas del otro gemelo. Dada la rareza de la presentación se describen el caso y los hallazgos imagenológicos representativos.

Twin pregnancy in one of the uterine cavities of the bicoporous uterus is a rare clinical presentation. The viability of this type of pregnancy is unlikely since this malformation can lead to premature delivery, abnormal placentation, fetal growth restriction or abnormal progression of labor. We present a case of bicoporous uterus with double cervix with twin pregnancy in one of the uterine cavities, with stillbirth of one of the fetuses and preterm delivery of the other twin at 28 weeks. Given the rarity of the presentation, the case and representative imaging findings are described.

Obstructed hemivagina with ipsilateral renal anomaly with ureteric remnant in a prepubertal female / Philippine Journal of Obstetrics and Gynecology

@#Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12‑year‑old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.

Ovarian dysgerminoma with Müllerian anomaly: a case report

Uterus didelphys with a successful term pregnancy

Mullerian duct anomalies are congenital defects of female genital system that arise from abnormal embryological development of mullerianducts. Uterusdidelphys also known as double uterus is one of the least mulleriananomalies. Here we present a case of a woman with uterus didelphys and her successful full pregnancy. Patient came to our hospital with diagnosis of G1P0L0 with 39 weeks with antepartum hemorrhage with active bleeding. On ultrasound she was found to be type iv placenta previa, in view of same we had done her lower segment cesarean section with delivery of a 2.5 kg female baby with intraoperative findings of two uterus and two cervix i.e uterus didelphys in which pregnancy was present in right uterus, placenta covering os. Post operative period was uneventful. Patient who have uterus didelphys due to mullerian defect not associated with difficulty in conception but there is increase incidence of preterm labour, malpresentation and malposition. Howeverwith appropriate antenatal and intrapartum surveillance pregnancy outcome are good.

Herlyn-Werner-Wunderlich Syndrome with Central Precocious Puberty: A Case Report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

Anorectal Malformation with Associated Duodenal Obstruction and Uterus Didelphys / Malformación anorrectal asociada con obstrucción duodenal y útero didelfo

ABSTRACT Anorectal malformations have long been associated with multiple other anomalies, commonly referred to as the VACTERL complex. We present a case of a newborn baby girl with an unusually high number of associated anomalies, including the rarely encountered condition of uterus didelphys.

RESUMEN Las malformaciones anorrectales se han asociado por mucho tiempo con otras anomalías, conocidas comúnmente como asociación VACTERL. Presentamos el caso de una niña recién nacida con un número inusualmente alto de anomalías asociadas, incluyendo una condición raramente encontrada conocida como útero didelfo.

Herlyn-Werner-Wunderlich Syndrome: A Mini-review

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

Radio-diagnosis of Herlyn Werner Wunderlich Syndrome a rare case of urogenital abnormality

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare urogenital abnormality, which is characterized by Mullerian duct anomaly with associated mesonephric duct anomaly. This syndrome is also known as OHVIRA (Obstructed hemivagina with Ipsilateral Renal Anomaly). The incidence of Mullerian Duct Anomaly is 2-3%, and incidence of OHVIRA is 0.16 to 10% of that. The three important components of Herlyn-Werner-Wunderlich (HWW) syndrome are didelphys uterus, obstructed hemivagina and renal agenesis on the same side. Typically presents with pelvic pain in young females after menarche. Presentation in the reproductive age group is unusual. The most common renal anomaly associated with this condition is renal agenesis. Other renal anomalies include renal dysplasia, duplication of kidneys and ureters, crossed fused ectopia and ectopic ureter. Ultrasound and Magnetic resonance imaging are considered as the vital modalities for detection. MRI is the gold standard modality for assessing the anatomical characteristics of the uterus, cervix, and vagina. Early diagnosis helps to prevent complications such as endometriosis, pyocolpos, adhesions, infertility. The unique feature of our case is a presentation in the reproductive age group. Only very few case reports of OHVIRA with a presentation in the reproductive age group are available in the literature. Whenever there is a renal anomaly or renal agenesis, screening of pelvic organs is important in a female child for early detection of Mullerian duct anomalies and prevention of further complications.

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.

Síndrome de Herlyn Werner Wünderlich. Reporte de tres casos / Herlyn Wunderlich Werner Syndrome. Report of three cases

Caso Clínico 1. J.A, 21 años, nuligesta. Acude por hipermenorragia y síndrome doloroso abdominal. Ecografía informa útero didelfo, hematocolpo en hemivagina ciega, tumoraciones anexiales y agenesia renal derecha conformándose el diagnostico de HWW, es sometida a neosalpingostomia con fimbrioplastia por hematosalpinx derecho por vía laparoscópica. A quince días del postoperatorio presenta cuadro de abdomen agudo. Ecografía informa quiste de ovario con hemorragia intraquística y liquido libre abdominal. Se realiza histerectomía subtotal derecha y drilling del quiste por vía laparotómica. Caso Clínico 2. T.A, 53 años, diagnostico conocido de HWW, cirugías previas histerectomía subtotal y resección del tabique vaginal 38 años antes. Acude por hipermenorrea por miomatosis uterina. Se realiza histerectomía total con resección del 2º cérvix. Caso Clínico 3. S.S, 35 años, diagnostico conocido de HWW, cirugías previas anexectomía izquierda por endometrioma 14 años antes, e histerectomía subtotal, sin resección del tabique en otro hospital 6 años antes. Acude a la consulta donde se diagnostica cervicitis mucopurulenta. Actualmente en planes de resección del tabique vaginal que no se realizo por el cuadro infeccioso presente. Discusión: Hay aproximadamente 200 casos reportados de HWW. La clínica más frecuente es dolor por hematocolpo. Esta patología presenta estrecha relación con infertilidad y endometriosis. Palabras Claves: Síndrome de Herlyn Werner Wünderlich, útero didelfo, hemivagina, agenesia renal.

Objective: To report three cases of diagnosis of Werner Wünderlich Herlyn syndrome (HWWS) in the Central Hospital of Social Security Institute of Paraguay (HC-IPS). Materials and Methods: Data from the records of patients of the Gynecology Service of HC-IPS between January/2010 to enero/2012 diagnosed with HWW. Case 1. J.A., female, 21, nulígesta. Presents abdominal pain and hipermenorragia, ultrasound reports didelphys uterus, hematocolpo, adnexal tumors and right renal agenesia making the diagnosis of HWW, is treated by fimbrioplasty and neosalpingostomy for right hematosalpinx by laparoscopy. Postoperative presents acute abdominal pain, ultrasound reports intracystic hemorrhage and abdominal free fluid. Subtotal hysterectomy is done and drilling of right cyst by laparotomy. Case 2. TA, female, 53, HWW known diagnosis, previous surgery: subtotal hysterectomy and vaginal septum resection 38 years earlier. Presents menorrhagia for uterine fibroids. Total hysterectomy is performed with resection of the second cervix. Case 3. SS, female, 35, known diagnosis of HWW, previous surgeries: left adnexectomy for endometrioma 14 years earlier, and subtotal hysterectomy with out removal of the vaginal septum in another hospital 6 years earlier. Attending the clinic where they diagnosed mucopurulent cervicitis. Currently in plans of vaginal septum resection that was not performed by the infection present. Discussion: There are approximately 200 reported cases of HWW. The most frequent symptom is pain by hematocolopo. Keep in mind the strong association with infertility and endometriosis of this disease. Keywords: Werner Syndrome Wünderlich Herlyn, uterus didelphys, hemivagina, renal agenesis.

Conduta frente às malformações genitais uterinas: revisão baseada em evidências / Medical care for uterine genital malformations: evidence-based review

Este trabalho de revisão foi idealizado para analisar as malformações dos ductos de Müller, que, devido à sua frequência de 3 a 7,3% na população em geral, justificam uma análise mais profunda do tema. O objetivo foi avaliar, de acordo com a literatura por meio de metodologia adequada? Os aspectos mais relevantes dessas anomalias, com destaque para a etiopatogenia, classificação, diagnóstico e tratamento. Os resultados obtidos nesta revisão apontaram as melhores evidências, até o momento, de como conduzir as mulheres portadoras dessas malformações.

This review paper was organized in order to analyse Müllerian anomalies, because their frequency from 3 to 7,3% in people in general justify a better evaluation about these malformations. The objective of the study was to evaluate, according to literature - by a proper methodology - the main aspects of these malformations, with special attention to etiology, classification, diagnostic and treatment. The results of this review showed the best evidences up till now of how to manage women with these genital malformations.

Acute renal failure associated with ureteral stone of the unilateral kidney and uterus didelphys with hemivaginal obstruction / 대한산부인과학회지

The unique clinical syndrome of the uterus didelphys, a unilateral partially or completely obstructed vagina, and ipsilateral renal agenesis is quite rare, and observed on the right side in greater frequency than on the left. The case of fifteen year old girl with uterus didelphys, unilateral partially obstructed vagina and acute renal failure associated with ureteral stone in unilateral kidney is described. Extracorporealshock wave lithotripsy (ESWL) and evacuation of the hematocolpos and excision of the vaginal septum were done. Aware of this relatively rare condition is mainstay of prompt diagnosis which will prevent permanent renal failure and unnecessary and destructive surgery.

A Case of the Uterus Didelphys with Unilateral Obstructed Hemivagina / 대한산부인과학회잡지

Uterus didelphys with unilateral obstructed hemivagina is indeed a very rare congenital anomaly due to M llerian duct malformation. The most common clinical presentation is pelvic pain and dysmenorrhea shortly after menarche, in associated with the finding of a vaginal or pelvic mass. An accurate and prompt diagnosis is of importance to permit treatment and to assure the future fertility of the patient. The simple and adequate treatment of the condition is incision of the obstructed vaginal septum providing adequate drainage of the retained blood. We report a case of uterus didelphys with obstructed hemivagina with brief review of the literature.

Uterus didelphys

1. Three new cases of uterus didelphys admitted in the Maternity and Childrens Hospital were recorded2. The obstetrical complications observed during delivery were stated and discussed3. Experiences of foreign authors mentioned. (Summary)

3 Cases of Uterus Didelphys with an Obstructed Hemivagina and Ipsilateral Renal Agenesis / 대한산부인과학회잡지

Associated congenital anomalies of both reproductive and urinary tracts are frequent, because wolffian and mullerian developments are closely related. The combination of uterus didelphys, obstructive hemivagina, ana ipsilateral renal agenesis is a rare but specific syndrome. The most common clinical presentation is pelvic pain and/or dysmenorrhea shortly after menarche, in association with the finding of a vaginal or pelvic mass. An accurate and prompt diagnosis is of importance to permit treatment and to assure the future fertility of the patients. The simple and curative treatment of the condition is incision of the obstructing vaginal septum providing adequate drainage of the retained blood. We report three cases of uterus didelphys with an obstructed bemivagina and ipsilateral renal agenesis with a brief review of concerned literatures.