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.

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>

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.

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.

Magnetic resonance imaging in the evaluation of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis: a case report / 대한산부인과학회지

Uterus didelphys with unilateral obstructed hemivagina is indeed a very rare congenital anomaly. Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis usually presents after menarche with progressive abdominal pain during menses secondary to hematocolpos. The method of choice for diagnosis is magnetic resonance imaging (MRI). MRI can do much for the early diagnosis and the prevention against further complications of this condition because it can demonstrate the mullerian duct anomaly complicated with obstructed hemivagina in detail and even ipsilateral renal agenesis. A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity. Early accurate diagnosis after menarche followed by excision and marsupialization of the blind hemivagina offers complete relief of symptoms and preserves reproductive potential. We report a case of uterine didelphys with obstructed hemivagina and ipsilateral renal agensis with a brief review of the literature.

A Case of Successful Gestational Outcome in the Presence of Preterm-labor Due to Hematometro-colpos of Obstructed Hemivagina and Uterus Didephys / 대한주산의학회잡지

Uterine didelphys with unilateral hematometra, hemicolpos, and ipsilateral renal agenesis is a rare m01lerian duct malformation. Uterine didelphys with an obstructed hemivagina is frequently associated with renal agenesis, mostly ipsilateral to the blind vaginal pouch. Accurate early diagnosis and septal resection elevate pregnancy rate and birth rate but it is difficult to find these malfomations until the complication associated with pregnancy was developed. We report a case of successful gestational outcome in the presence of preterm labor due to hematometrocolpora caused by uterine didelphys with unilateral imperforated vagina and ipsilateral renal agenesis with brief review of the literatures.