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Herlyn-Werner-Wunderlich syndrome: Case report
Síndrome de Herlyn-Werner-Wunderlich. Reporte de caso
DOI:
https://doi.org/10.15446/revfacmed.v69n4.83840Palabras clave:
Congenital Abnormalities, Mullerian Ducts, Hematocolpos, Solitary Kidney (en)Anomalías congénitas, Conductos paramesonéfricos, Hematocolpos, Riñón único (es)
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Introduction: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation of the urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, which is usually diagnosed after menarche. It is treated by resecting the vaginal septum and draining the obstructed hemivagina and has a favorable postoperative prognosis.
Case presentation: This is the case of 22-year-old woman with a 9-year history of dysmenorrhea, pelvic pain, and irregular menstrual cycles that started after she had her first menstruation, who visited the gynecology service of a secondary care hospital in Sogamoso, Boyacá (Colombia). The patient reported having visited multiple specialists and being treated with vitamin E, metformin, and contraceptives, without experiencing an improvement of symptoms and signs. After being assessed, and taking into account ultrasound and MRI findings, she was diagnosed with HWW syndrome. She underwent a colpotomy plus resection of right paracervical mass of approximately 60x60mm and vaginal septum, achieving complete resolution of symptoms. Finally, 10 months after the surgery, she reported being pregnant without complications.
Conclusion: HWW syndrome is a rare malformation that should be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract, since its early diagnosis and timely treatment considerably improve the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.
Introducción. El síndrome de Herlyn-Werner-Wunderlich (SHWW) es una malformación congénita rara del tracto urogenital que se caracteriza por la triada útero didelfo, hemivagina obstruida y anomalía renal ipsilateral, y que suele diagnosticarse después de la menarquia. Su tratamiento consiste en resección del tabique vaginal y drenaje de la hemivagina obstruida, con un buen pronóstico postquirúrgico.
Presentación del caso. Mujer de 22 años con un cuadro clínico de 9 años de evolución que inició cuando tuvo su primera menstruación, consistente en dismenorrea, dolor pélvico y ciclos menstruales irregulares, quien asistió al servicio de ginecología de un hospital de segundo nivel en Sogamoso, Boyacá (Colombia). La paciente reportó haber visitado múltiples especialistas y recibido tratamiento con vitamina E, metformina y anticonceptivos, sin mejoría de los síntomas y signos. Luego de ser valorada, y teniendo en cuenta los hallazgos en ecografía y resonancia magnética, fue diagnosticada con SHWW, por lo que se le realizó colpotomía más resección de masa paracervical derecha de aproximadamente 60x60mm y de tabique vaginal, lográndose la resolución completa de los síntomas. Finalmente, 10 meses después de la cirugía, la joven refirió encontrarse en estado de embarazo, sin presentar complicaciones.
Conclusión. El SHWW es una malformación poco común que debe considerarse como diagnóstico diferencial en mujeres de cualquier edad con anomalías de los conductos paramesonéfricos, dolor pélvico, dismenorrea y masa en el tracto genital, pues su diagnóstico temprano y un tratamiento oportuno mejoran considerablemente la calidad de vida de estas pacientes al reducir la severidad de los síntomas, disminuir la incidencia de complicaciones y mejorar el pronóstico obstétrico.
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