Embarazo triple espontáneo en una paciente con útero didelfo: reporte de un caso y revisión de la bibliografía / Spontaneous triplet pregnancy in a patient with uterus didelphys: report of a case and review of the literature

Resumen ANTECEDENTES: Las anomalías müllerianas implican la desviación de la anatomía normal del aparato reproductor femenino debido a un desarrollo embriológico alterado. Padecer alguna de estas anomalías, en cualquier escenario de su amplio espectro, suele asociarse con infertilidad o desenlaces obstétricos adversos, como la pérdida gestacional temprana o el parto pretérmino. CASO CLÍNICO: Paciente de 21 años, sin antecedentes patológicos relevantes, con dos embarazos finalizados: el primero por cesárea y el segundo por parto (la cesárea se indicó por la presentación podálica, a las 32 semanas). Acudió a un centro de referencia de la ciudad de Medellín, Colombia (Clínica Universitaria Bolivariana) a la primera consulta prenatal a las 17 semanas y 4 días de embarazo, motivada por un cuadro clínico de cólico biliar, sin requerimiento de intervenciones. Se trataba de embarazo triple, monocorial, triamniótico, con diagnóstico de útero didelfo a las 17 semanas y 1 día, con ambos cuellos uterinos reportados en 38 mm. La gestación finalizó a las 28 semanas y 4 días con el nacimiento de tres niños, quienes luego de superar los retos de la prematurez, en la actualidad llevan una vida normal. CONCLUSIONES: Las anomalías müllerianas, en su amplio espectro de manifestación clínica, son una condición que se asocia con una mayor frecuencia de desenlaces adversos materno-perinatales que se incrementan cuando la anomalía se relaciona con un embarazo múltiple. En la bibliografía actual no abundan los reportes de casos que combinen ambas condiciones, ni con recomendaciones oficiales estandarizadas para la atención médica de las pacientes, sobre todo para el escenario de embarazo triple y útero didelfo.

Abstract BACKGROUND: Müllerian anomalies involve deviation from the normal anatomy of the female reproductive tract due to altered embryological development. Having any of these anomalies, in any of their broad spectrum, is often associated with infertility or adverse obstetric outcomes, such as early gestational loss or preterm delivery. CLINICAL CASE: 21-year-old female patient, with no relevant pathological history, with two pregnancies terminated: the first by cesarean section and the second by delivery (the cesarean section was indicated due to breech presentation, at 32 weeks). She attended a referral center in the city of Medellín, Colombia (Clínica Universitaria Bolivariana) for the first prenatal consultation at 17 weeks and 4 days of pregnancy, motivated by a clinical picture of biliary colic, without requiring interventions. The pregnancy was triple, monochorionic, triamniotic, with a diagnosis of didelphic uterus at 17 weeks and 1 day, with both cervix reported at 38 mm. The gestation was terminated at 28 weeks and 4 days with the birth of three infants, who after overcoming the challenges of prematurity, are now living normal lives. CONCLUSIONS: Mullerian anomalies, in their broad spectrum of clinical presentation, are a condition that is associated with an increased frequency of adverse maternal-perinatal outcomes that are increased when the anomaly is associated with multiple pregnancy. The current literature does not abound with case reports combining both conditions, nor with standardized official recommendations for patient care, especially in the setting of triple pregnancy and didelphic uterus.

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.

“Antipodal asymmetry” – The undiagnosed twin in the opposite uteri: A case report of dicavitary twin pregnancy in uterine didelphys / Philippine Journal of Obstetrics and Gynecology

@#Muüllerian duct anomalies (MDAs) are congenital defects arising from probable teratogenic assault at 6–22 weeks of gestation.Uterine didelphys arises from complete lack of fusion of Muüllerian ducts, resulting in two entirely separate hemiuteri, cervices and vaginas.The incidence of MDA is <5% and the frequency of uterine didelphys is 1 in 1000–30,000 women and twin pregnancy in uterus didelphys is only 1 in a million. This is a case of a gravida 1 para 0, with uterine didelphys and unilateral renal agenesis diagnosed of single live intrauterine pregnancy in the left half of uterine didelphys who had a spontaneous passage of meaty material from the right hemiuterus which were histopathologically consistent with degenerating products of conception, hence confirming a dicavitary twin pregnancy. Management should be wholistic encompassing preconception, prenatal, intrapartum, and postpartum period as any complications may arise at any point.

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.

Young or old, this condition may unfold: The use of ultrasound in the diagnosis of a wide spectrum of Herlyn Werner Wunderlich Syndrome, a case series / Philippine Journal of Obstetrics and Gynecology

@#Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct anomaly. This case series shows a wide spectrum of the condition, one presenting in an adolescent, managed conservatively and the other in the perimenopausal age group given a more definitive management. The first case is an 18-year- old nulligravid who manifested with progressive dysmenorrhea and foul smelling vaginal discharge a few years after menarche. She subsequently underwent vaginal septotomy followed by diagnostic hysteroscopy. On the other hand, the second case is a 46- year-old nulligravid whose chief complaint is a foul smelling vaginal discharge and consequently went through a total abdominal hysterectomy with salpingo-oophorectomy. To our knowledge, the second case is the only patient diagnosed in the perimenopausal stage and underwent a total hysterectomy. Ultrasound is the first-line imaging modality used in both cases and its merits are highlighted to prove its importance and diagnostic value in the workup of this condition.

Uterine didelphys with unilateral cervicovaginal agenesis: A case report

@#<p style="text-align: justify;">Unilateral cervicovaginal agenesis in a didelphic uterus is an extremely rare congenital anomaly. Women born with this anomaly present with menarche associated with progressive dysmenorrhea and symptoms of worsening endometriosis. Presented here is a case of a 12 year old nulligravid who presented with progressive cyclic abdominal pain. Physical examination revealed a tender large right adnexal mass and presence of a vaginal canal and cervix tilted to the left. Sonography showed the presence of uterine didelphys, right hematosalpinx and hematocolpos. Preoperative impression was outflow tract obstruction probably due to Herlyn Werner-Wunderlich syndrome. However, intraoperatively, it was discovered this was a case of uterine didelphys with outflow tract obstruction of the right hemiuterus due to unilateral cervicovaginal agenesis and ipsilateral renal agenesis.</p>

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.

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: 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.

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.

A Case of Successful Unilateral Singleton Pregnancy in Incidentally Detected Uterine Didelphys.

Uterine didelphys or duplication of uterus is a rare congenital anomaly, which constitutes approximately 5% of mullerian duct anomalies. There is failure of the fusion of mullerian ducts resulting in the duplication of uterus and cervix. We report a case of uterine didelphys, which was incidentally detected in a 23 years old G2P1 patient who came for ultrasound with complaints of bleeding per vaginum. MRI was done and uterine didelphys was confirmed. Six months later that patient came again with 6 weeks pregnancy in the right uterine body. We did all antenatal follow up scans in this patient till 36 weeks. Lower segment cesarean section (LSCS) was done at 38 weeks and patient delivered a normal baby of 2.9-kilogram weight.

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.

Malformaciones uterinas en el Hospital Central de Maracay: revisión de 1999 a 2007 / Uterine malformations in the Hospital Central of Maracay: review of 1999-2007

Conocer la incidencia de malformaciones uterinas y describir las características clínicas asociadas. Estudio descriptivo, retrospectivo y epidemiológico de 24 pacientes egresadas con el diagnóstico de malformación uterina entre los años de 1999 a 2007. Departamento de Obstetricia y Ginecología del Hospital Central de Maracay. Las pacientes en 70,8 por ciento tenían edades comprendidas entre 16 y 25 años. El 50 por ciento eran primigestas. El 33,3 por ciento presentaba diagnóstico de ingreso por presentación fetal anómala. En el 66,6 por ciento de los casos el diagnóstico se realizó por visualización directa de la estructura. En el 62,5 por ciento la malformación más frecuente fue el útero didelfo. En 50 por ciento de los casos el resultado obstétrico fue la cesárea segmentaria. En cuanto al diagnóstico de ingreso el útero didelfo registra el 62,5 por ciento de los casos ingresados. Las mujeres con anomalías uterinas se reproducen normalmente. Las malformaciones uterinas por defectos de fusión de los ductos müllerianos son las más frecuentes. Esporádicamente pueden ser sintomáticas ameritando diagnóstico diferencial y tratamiento

To know the incidence of congenital uterine anomalies and the clinical presentations. Retrospective, epidemiological, descriptive study of 24 cases of patients with congenital uterine anomalies. Department of Obstetric and Gynecology, Hospital Central de Maracay. In the 70.8 percent the age was between 16 and 25 years. The 50 percent were primipara. The 33.3 percent had personal history of abnormal fetal positions. The 66.6 percent were detected by visualization of the uterus. In 62.5 percent the most frequent malformation was the uterus didelphys. In 50 percent the obstetric performance was the cesarean section. The reproductive performance didelphic reported 62.5 percent of patients. Women’s with Müllerian anomalies demonstrate no problems with conception. However comes to medical attention when become symptomatic because of poor obstetric performance

Diagnosis and treatment of uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis / 대한산부인과학회지

Uterine malformations consist of a group of miscellaneous congenital anomalies of the female genital system, and these anomalies are the result of major disturbances in the development, such as formation or fusion of the Mullerian or paramesonephric ducts during fetal life. The Mullerian anomalies have been estimated to occur in up to 0.001~10%, and these anomalies may be associated with numerous congenital anomalies, and ipsilateral renal anomalies especially are the most common. The uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis clinically may result in dysmenorrhea, low abdominal pain, endometriosis or pelvic mass after menarche. Furthemore the delayed diagnosis causes infertility and poor pregnancy outcome due to continued retrograde menstruation which leads to endometriosis and distorted pelvic anatomy. These anomalies could be diagnosed with ultrasonography and MRI that have the high sensitivity and specificity. Excision of vaginal septum and marsupialization are appropriate treatments to relieve symptoms and reserve fertility. We report 7 cases of uterine didelphys with obstructed hemivagina and ipsi-unilateral renal agenesis with a brief review of relevant literatures to help understand these anomalies.

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.