Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Diagnóstico y tratamiento del síndrome de Herlyn-Werner-Wünderlich: reporte de dos casos

Las malformaciones müllerianas son anomalías congénitas que suelen manifestarse después de la menarquia. Entre ellas, el síndrome de Herlyn-Werner-Wünderlich (SHWW) se caracteriza por útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica del SHWW suele ser asintomática hasta la menarquia; la mayoría es diagnosticada entre 2 meses y 1 año después de la primera menstruación. Los síntomas que se presentan son dismenorrea, dolor abdominal cíclico recurrente, menstruación irregular y, a veces, masa pélvica palpable. Ocasionalmente, el hematocolpos o la hematometra pueden desarrollar piocolpos, piosálpinx y pelviperitonitis, y algunas pacientes solo manifiestan infertilidad o pérdida recurrente del embarazo. Presentamos los casos de dos pacientes de 11 y 13 años que acudieron con dolor abdominal cíclico y describimos la historia clínica, las imágenes y el tratamiento específico que se realizó en cada una de las pacientes.

Müllerian malformations are congenital anomalies that usually manifest after menarche. Among them, Herlyn-Werner-Wünderlich syndrome (SHWW) is characterized by didelphic uterus, obstructed hemivagina and ipsilateral renal agenesis. The clinical presentation of SHWW is usually asymptomatic until menarche; most are diagnosed 2 months to 1 year after first menstruation. Presenting symptoms are dysmenorrhea, recurrent cyclic abdominal pain, irregular menses, and sometimes palpable pelvic mass. Occasionally, hematocolpos or hematometra may develop pyocolpos, pyosalpinx and pelviperitonitis, and some patients manifest only infertility or recurrent pregnancy loss. We report the cases of two patients aged 11 and 13 years who presented with cyclic abdominal pain and describe the clinical history, imaging and specific treatment performed in each patient.

Síndrome de Herlyn-WernerWunderlich: Reporte de un caso / Herlyn-Werner-Wunderlich syndrome: A case report

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract caused by defective fusion of the Müllerian ducts, which gives rise to obstructed hemivagina, uterus didelphys and ipsilateral renal agenesis. We report the case of a 16-year-old girl who underwent resection of the vaginal septum and drainage of the secondary hematocolpos, thus preserving her reproductive function.

El síndrome de Herlyn-Werner-Wunderlich es una anomalía congénita rara del tracto urogenital, causada por un fallo de la fusión de los conductos müllerianos, que se caracteriza por presentar una hemivagina obstruida, útero didelfo y anomalía renal ipsilateral. Presentamos el caso de una paciente de 16 años a quien se le realizó resección del tabique vaginal y drenaje del hematocolpos secundario, para así preservar su función reproductiva.

Síndrome de Herlyn-Werner-Wünderlich: dos modalidades diferentes de manifestación. Reporte de dos casos / Herlyn-Werner-Wunderlich syndrome: two different presentation modalities. Report of two cases

Resumen ANTECEDENTES: El síndrome de Herlyn-Werner-Wünderlich es una anomalía congénita mülleriana rara, que se caracteriza por la asociación entre útero didelfo, obstrucción del tabique vaginal y agenesia renal. PRIMER CASO: Paciente de 13 años, con dolor pélvico severo de 8 días de evolución. Durante el examen físico refirió dolor abdominal generalizado. La ecografía pélvica abdominal mostró: útero bicorne con hematocolpos y la resonancia magnética nuclear: útero único con hematocolpos y agenesia renal derecha. En la laparoscopia se evidenció el útero didelfo, con hematosalpinx, síndrome adherencial moderado de epiplón y focos de endometriosis. Se efectuaron la incisión del tabique vaginal y su resección. Con esto se consiguió el drenaje completo del hematocolpos y fue posible la vaginoplastia; la paciente tuvo buena evolución. SEGUNDO CASO: Paciente de 30 años, con dismenorrea, dolor pélvico y ciclos regulares. En el examen físico se observaron dos cuellos uterinos: el derecho de aspecto normal y el izquierdo puntiforme. La ecografía mostró: útero didelfo con colección líquida en el medio. La resonancia magnética reportó: útero didelfo y agenesia renal izquierda. En la histeroscopia se evidenció, en el cuello derecho y a la mitad del útero una cavidad tubular. El endometrio y ostium derecho normal. El cuello izquierdo era rudimentario y puntiforme, con salida de material purulento. La laparoscopia evidenció dos hemiúteros con trompas y ovarios normales. CONCLUSIÓN: El diagnóstico temprano de esta anomalía congénita disminuye la morbilidad a largo plazo, la posibilidad de endometriosis, deterioro de la fertilidad y las complicaciones obstétricas. El tratamiento es con técnicas de mínimo acceso que ofrecen más ventajas: magnificación del campo quirúrgico, menor dolor posoperatorio, recuperación más rápida y mejor resultado estético.

Abstract BACKGROUND: The Herlyn-Werner-Wünderlich syndrome is a rare Müllerian congenital anomaly, characterized by the association between didelphus uterus, obstruction of the vaginal septum and renal agenesia. FIRST CASE: Patient of 13 years, with severe pelvic pain of 8 days of evolution. During the physical examination he reported generalized abdominal pain. Abdominal pelvic ultrasound showed: bicornuate uterus with hematocolpos and nuclear magnetic resonance: single uterus with hematocolpos and right renal agenesis. In laparoscopy the didelphus uterus was evidenced, with hematosalpinx, moderate adherent adhesion syndrome and foci of endometriosis. The incision of the vaginal septum and its resection were made. With this, complete drainage of the hematocolpos was achieved and vaginoplasty was possible; the patient had a good evolution. SECOND CASE: Patient of 30 years, with dysmenorrhea, pelvic pain and regular cycles. In the physical examination two uterine necks were observed: the right one of normal aspect and the left one punctate. The ultrasound showed: uterus didelphy with liquid collection in the middle. Magnetic resonance reported: didelphys uterus and left renal agenesis. The hysteroscopy revealed a tubular cavity in the right neck and in the middle of the uterus. The endometrium and normal right ostium. The left neck was rudimentary and punctate, with leakage of purulent material. The laparoscopy showed two hemiutero with normal tubes and ovaries. CONCLUSION: Early diagnosis of this congenital anomaly decreases long-term morbidity, the possibility of endometriosis, impaired fertility and obstetric complications. The treatment is with minimal access techniques that offer more advantages than open surgery.

Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome / Piometria e gravidez com síndrome de Herlyn-Werner-Wunderlich

ABSTRACT We describe a Herlyn-Werner-Wunderlich syndrome (HWWS) patient with previous history of infertility who got pregnant without treatment and presented a pyometra in the contralateral uterus throughout the gestational period, despite multiple antibiotic treatments. Due to the uterus' congenital anomaly and the possibility of ascending infection with subsequent abortion, this pregnancy was classified as high-risk. We believe that the partial horizontal septum in the vagina may have contributed to the closure of the gravid uterus cervix, thus ensuring that the pregnancy came to term, with an uneventful vaginal delivery.

RESUMO Os autores descrevem uma paciente com síndrome de Herlyn-Werner-Wunderlich (SHWW) e história prévia de infertilidade, que engravidou espontaneamente. Durante todo o período gestacional apresentou, apesar da instituição de antibioticoterapia, um piometra localizado ao útero não gravídico. Devido à anomalia congênita uterina e ao risco de infeção ascendente, com possível desfecho obstétrico desfavorável, esta gravidez foi classificada de alto risco. O septo vaginal horizontal e parcial poderá ter contribuído para ausência de disseminação da infecção, permitindo que a gravidez tenha chegado a termo, com um parto vaginal, sem intercorrências.