True uterus didelphys in she-camel: a case report and review of literature.

Background: Uterus didelphys is a rare congenital anomaly of the female reproductive tract characterized by a divided uterine cervix and body. It occurs due to abnormal development of the paramesonephric (Müllerian) duct. Different forms of uterus didelphys have been reported in several animal species, including bovine, equine, ewe, goat, swine, and bitch. However, there is no previous report that has documented a completely divided female genital tract in she-camel. Moreover, there is a lack of literature regarding this anomaly in animals. Therefore, the present study reports, for the first time, a rare case of a completely divided female genital tract in a she-camel. In addition, the existing relevant literature on uterus didelphys in different animal species is reviewed. Case presentation: A female reproductive tract of she-camel, approximately 10 years old, with a history of previous successful pregnancy, was brought to the anatomy department following the slaughtering of the animal. Initial examination revealed a normal reproductive tract consisting of two ovaries, two fallopian tubes, a uterus, and a vagina. A closer examination revealed a completely divided vagina, with an external os opened into each part of the vagina, as well as a divided uterine body and cervix. Intrauterine infusion of saline through one external os confirmed complete separation of uterine body and cervix. Conclusion: To the authors' knowledge, this is the first reported case of a completely divided female genital tract in a she-camel. This review summarizes the previous reports about uterus didelphys in farm animals.

An Incidental Diagnosis of Herlyn-Werner-Wunderlich Syndrome in a Young Female: A Case Report of a Rare Entity.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.

Herlyn–Werner–Wünderlich syndrome: A case series / Síndrome de Herlyn-Werner-Wünderlich: una serie de casos

Introduction: Herlyn–Werner–Wünderlich syndrome is a uterine malformation characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Clinical findings: The manifestation of the disease is widely diverse; it is usually diagnosed after menarche, with dysmenorrhea and abnormal uterine bleeding; it is also associated with infertility. Main diagnosis: Four clinical cases, their diagnosis are reported here. Therapeutic interventions and results: The treatment and results of these four patients are described here. Conclusion: When studying uterine malformation it is important to consider this rare disease to avoid possible complications and giving the patient a correct diagnose and treatment. The hysteroscopy resection of the longitudinal vaginal septum in those symptomatic patients with hematocolpos should be considered as a good option for treatment.(AU)

Introducción: El síndrome de Herlyn-Werner-Wünderlich es una malformación uterina que asocia útero didelfo, hemivagina obstruida total o parcialmente y agenesia renal ipsilateral. Hallazgos clínicos: La clínica que presenta este síndrome es muy diversa; se suele diagnosticar después de la menarquia cursando con dismenorrea y sangrado uterino anómalo; así mismo se asocia a infertilidad. Diagnósticos principales: Se presentan a continuación 4 casos clínicos, su diagnóstico y tratamiento mediante diversas técnicas. Intervenciones terapéuticas y resultados: Se describen en este manuscrito los tratamientos aplicados a estas pacientes y sus resultados. Conclusión: Ante el hallazgo de una malformación uterina es importante tener en cuenta esta entidad infrecuente, para evitar posibles complicaciones y proporcionar a la paciente un diagnóstico y tratamiento correctos. La resección histeroscópica del tabique vaginal longitudinal en aquellas pacientes sintomáticas con hematocolpos debe ser considerada como una buena opción de tratamiento.(AU)

Laparoscopic Uterine Preservation and Vaginal Reconstruction in a 13-Year-Old Girl with Uterus Didelphys, Bilateral Vesicovaginal Fistulas, and Transverse Vaginal Septum.

BACKGROUND: Complex female genital tract malformations account for 1.2% of all female genitourinary malformations. Although exceedingly rare, they can cause severe gynecologic symptoms in young women and lead to fertility problems. CASE: We present the case of a 13-year-old girl with primary amenorrhea referred for cyclic abdominal lower pain and menouria. Detailed diagnostics revealed uterus didelphys, transverse vaginal septum, and bilateral vesicovaginal fistulas. Laparoscopic left hemi-hysterectomy and salpingectomy were performed. The vesicovaginal fistula on the right side was excised, and the proximal vagina was anastomosed with the distal dimple. Since the operation, the patient has been pain-free and menstruating regularly from the right uterus. SUMMARY AND CONCLUSION: Preservation of the uterus should be considered in any case of complex female genital tract malformation and, as successful laparoscopic treatment advocates, a minimally invasive approach is feasible.

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review.

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13-year-old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi-uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post-procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.

Undiagnosed uterus didelphys with unicavitary twin gestation in a district hospital in Ghana: a case report.

Uterus didelphys is a congenital anomaly of the female reproductive tract which arises from the abnormal fusion of the Mullerian ducts. We present, the first case to the best of our knowledge, of uterus didelphys with a unicavitary twin gestation to be documented in Ghana, a low-middle income country. A 24-year-old woman, gravida 3, para 0+2 miscarriages, was seen and admitted to our maternity ward due to elevated blood pressure with ++ proteinuria at 36 weeks of gestation. She attended an antenatal clinic regularly during the pregnancy but was mainly seen by midwives. Apart from multiple pregnancy, two 2D ultrasound examinations (one at 25 weeks gestation and another during admission) did not reveal any uterine malformations. At 37 weeks+2 days, she underwent emergency cesarean section on account of pre-eclampsia and a twin pregnancy with the leading twin in breech presentation. After delivering both babies and the placenta, the uterus was exteriorized and inspected, during which a non-gravid bulky left uterus was first found. Each uterus had a normal ovary and fallopian tube on its lateral end. Further postoperative examination revealed a normal-looking vulva, two vaginas, and two cervices. Both babies weighed 1.9 kg, each below the fifth percentile of weight for age. The elevated blood settled postoperatively and the postoperative period was uneventful. The patient and twins were found in a stable condition on review two weeks after delivery and the twins were healthy at 5 years. Despite being a rare presentation, we wish to create awareness among health workers in rural and low-resource settings of such cases and highlight the need to improve prenatal diagnostic capabilities, as this is key to determining the mode of delivery and achieving favorable maternal and fetal outcomes.

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

Successful management of Herlyn-Werner-Wunderlich syndrome in a 17-year-old virgin girl in the challenging socio-cultural-logistic setting of Syria: a case report.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation: A 17-year-old girl presented to the authors' department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion: The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion: Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.

Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management.

Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions.

Case Report: From epilepsy and uterus didelphys to Turner syndrome-associated dysgerminoma.

Dysgerminoma is a rare occurrence in Turner syndrome patients without Y chromosome mosaicism or hormone therapy during puberty. We present a unique case of a 33-year-old nulliparous Chinese woman with intermittent epilepsy and Mullerian anomalies carrying a double uterus, cervix, and vagina. The patient is also characterized as having Turner syndrome accompanied by 46,X, del(Xp22.33-11.23) and del(2)(q11.1-11.2). MRI exhibited a 17.0 cm × 20.0 cm × 10.5 cm solid ovarian lesion. Radical surgery and pathology revealed dysgerminoma at stage IIIc with lymphatic metastases and a KIT gene mutation identified in exon 13. Furthermore, the tumor microenvironment (TME) displayed robust expression of CD4+ T lymphocytes and PD-1, whereas the distribution of CD8+ T lymphocytes and PDL-1 was sporadic. Despite the administration of enoxaparin to prevent thromboembolism, the patient experienced multiple cerebral infarctions during chemotherapy. Subsequently, the patient chose to decline further treatment and was discharged. This exceptional case imparts several noteworthy lessons. First, the coexistence of Mullerian anomalies, although rare, is not incompatible with Turner syndrome. Second, screening for KIT mutations is imperative to reduce the risk of dysgerminoma in Turner syndrome, especially for patients with Y mosaicism who are recommended for hormone replacement therapy. Lastly, comprehensive anticoagulation therapy is crucial for Turner syndrome patients undergoing cisplatin-based chemotherapy.

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.

The Impact of Uterus Didelphys on Fertility and Pregnancy.

Uterus didelphys occurs as a result of abnormal fusion of the paramesonephric ducts and is characterized by complete duplication of uterine horns, cervix, and very often also the vagina or presence of longitudinal vaginal septum. Most women with a uterus didelphys are asymptomatic; some cases may coincide with dyspareunia or dysmenorrhea. The anomaly is associated with a higher risk of miscarriage, preterm labor, breech delivery, and decreased live births. We present the case of a 26-year-old woman (primigravida) who was known to have uterus didelphys. The diagnosis was made when the patient was 23 years old using ultrasound and hysteroscopy. The patient became pregnant after 18 months of efforts. The patient was referred to prenatal care in the 13th week of pregnancy with vaginal bleeding. In the 23rd week of pregnancy, gestation cholelithiasis was diagnosed. The pregnancy progressed without obstetric complications and the fetus developed normally. Due to the vaginal septum and fact that the patient felt stressed, the pregnancy was terminated at term by cesarean section. We concluded that uterus didelphys can be asymptomatic making an early diagnosis difficult. A pregnancy belongs to a high-risk group and more attention should be paid to this case. Cesarean section should be considered, especially in case of the presence of vaginal septum.

Herlyn-Werner-Wunderlich syndrome: A case report in a young woman, with literature review.

Herlyn-Werner-Wunderlich syndrome is a rare complex congenital disorder, with combined Müllerian and mesonephric duct anomalies, presenting with uterus didelphys, unilateral blind hemivagina and ipsilateral renal agenesis. Hemivaginal obstruction usually leads to impairment of normal menstrual flow, resulting in symptoms after menarche, namely dysmenorrhea, pelvic pain or infertility. Age of presentation depends on the anatomical features of this anomaly. We report a case of a 21-year-old female presenting with few symptoms and incidental findings on transvaginal ultrasound, with typical findings of this disorder on magnetic resonance imaging, which remains the gold standard imaging technique for thorough assessment of Herlyn-Werner-Wunderlich syndrome, allowing for a correct diagnosis and adequate surgical management. Our case also highlights some unusual features, such as the presence of a blind ectopic ureter, with hematic content, and an incomplete septum within the obstructed hemivagina.

A rare variant of obstructed hemivagina and ipsilateral renal agenesis and its improvement of classification.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital malformation of the female reproductive organs, including uterine dysplasia, blind hemivagina, and ipsilateral renal dysplasia. It has a serious impact on patients' quality of life and fertility. We report a rare case of OHVIRA with a complete septate uterus, ectopic dysplasia of the left kidney, left ectopic ureter, and three oblique vaginal septa. The dysplastic left kidney and the ectopic ureter were removed during laparoscopy. In addition, the two layers of vaginal septa, which were ignored in the past 10 years, were removed by hysteroscopy. As the detection rate of OHVIRA has gradually increased, manifestations that previous classification systems could not recapitulate have emerged. By retrieving the relevant cases, we have tried to propose a new classification system, which would allow clinicians to have a complete understanding of the manifestations of OHVIRA and would be helpful in the treatment of patients.

Uterus didelphys with double vagina diagnosed during third cesarean section: A case report.

Uterus didelphys is a rare uterine anomaly. It is associated with fetal breech presentation, preterm delivery, and cesarean section. Longitudinal vaginal septum may be associated with uterus didelphys, which may cause dyspareunia and labor obstruction. We report a case of 28-year-old woman whose uterus didelphys and longitudinal vaginal septum were diagnosed during third cesarean section. This case report highlights the importance of routine examination of adnexal structures during cesarean section, so that any unsuspected pathologies in the uterus, tubes, and ovaries are diagnosed.

Pregnancy outcome after in-vitro fertilization/intracytoplasmic sperm injection in women with congenital uterus didelphys.

OBJECTIVE: To investigate the pregnancy and obstetric outcomes of patients with congenital uterus didelphys who achieved clinical pregnancy after in-vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI). METHODS: This was a retrospective matched-cohort study of 83 infertile patients with uterus didelphys who underwent IVF/ICSI and achieved clinical pregnancy from January 2005 to December 2018 at our center. For each patient in the study group, three control patients with normal uterine morphology who underwent IVF/ICSI in 2018 were selected randomly. Patients in the two groups were matched for number of gestational sacs, maternal age, infertility type, cause of infertility, fertilization method, endometrial thickness 1 day before embryo transfer and number of embryos transferred. The classification of congenital uterine anomalies was based on the American Fertility Society system (1988). The pregnancy and obstetric outcomes of the didelphic and control groups were compared separately for singleton and twin pregnancies, and for all pregnancies combined. RESULTS: In singleton pregnancies, women with uterus didelphys had increased risk of preterm birth (odds ratio (OR), 4.68; rate difference (RD), 0.14; P < 0.001), Cesarean section (OR, 2.80; RD, 0.17; P = 0.016) and birth weight < 2500 g (OR, 4.06; RD, 0.10; P = 0.017) compared to women with normal uterine morphology. In twin pregnancies, the presence of uterus didelphys was associated with increased risk of preterm delivery (OR, 4.79; RD, 0.37; P = 0.006), perinatal mortality (OR, 3.16; RD, 0.19; P = 0.043) and birth weight < 2500 g (OR, 9.57; RD, 0.35; P = 0.001). CONCLUSIONS: The presence of uterus didelphys was associated with significantly increased risk of some adverse pregnancy outcomes compared to pregnancies with normal uterine morphology in women who underwent IVF/ICSI. A twin pregnancy in women with uterus didelphys was associated with worse perinatal outcome. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Torsion of Uterus Didelphys at Term: Once-in-a-Lifetime Experience for an Obstetrician.

Torsion of the gravid uterus is very rare in obstetric practice. We report a case of torsion in the uterus didelphys at term which is rare and a lifetime experience for an obstetrician. The patient, a 25-year-old gravida 2 para 1 was admitted to the labor ward at 37 weeks and six days of gestation with abdominal pain. Her previous delivery was a caesarean section performed four years back. She was taken to the operating room for an emergency caesarean section for fetal distress and the lie was transverse. On entering the peritoneal cavity, we found an engorged infundibulopelvic ligament with the fallopian tube and ovary covering the lower segment of the uterus. The baby was successfully delivered by breech extraction. Due to uterine torsion of more than 180 degrees, the posterior surface of the uterus was placed anteriorly, and the incision was made on the posterior surface of the uterus. There was a hemi uterus on the left side of the pelvic cavity with the fallopian tube and ovary attached to it; a diagnosis of uterus didelphys was made. The diagnosis of uterine torsion is intraoperative and prompt and timely decision by surgeons is crucial. We had favorable maternal and fetal outcomes in this rare and interesting case. The diagnosis, though rare, should be kept in mind in all cases of abdominal pain during pregnancy, especially in those with malpresentation.