RESUMO
The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.
RESUMO
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.