RESUMO
INTRODUCTION: Herlyn-Werner-Wunderlich syndrome , a rare Müllerian ducts congenital disease, is characterized by a diphtheritic uterus, blind hemivagina, and ipsilateral renal agenesis. Diagnosis is at young age by ultrasound and magnetic resonance imaging, and the prognosis is good. Usually, complications evolve endometriosis and secondary pelvic inflammation. CASE REPORT: A 40-year-old female patient, Brazilian, white, primigravida, diagnosed at 30 years with a didelphic uterus on ultrasound, and 4 years later, with a left ovarian endometrioma, multiple ovarian cysts, and left renal agenesis on magnetic resonance imaging. Subsequently, due to dyspareunia and a feeling of swelling, the patient underwent transvaginal ultrasound with bowel preparation, and a hematocolpos was found and Herlyn-Werner-Wunderlich syndrome was suspected; 10 years after the diagnosis she had a planned pregnancy. She presented frequent contractions following the 15th week of pregnancy and fortunately there were no complications or premature labor. Labor was inducted at 40 weeks and 6 days without progress and a cesarean section was indicated and performed without complications. Herlyn-Werner-Wunderlich syndrome often goes unnoticed, leading to inadequate treatment. Individuals with Herlyn-Werner-Wunderlich syndrome commonly face fertility issues, such as high miscarriage rate (21-33%), and obstetric complications, such as spontaneous abortions (40% risk), intrauterine growth restriction, postpartum hemorrhage, increased fetal mortality, preterm delivery (21-29%), and elevated rates of cesarean sections. In addition, there is higher susceptibility of developing endometriosis, especially with hemivaginal obstruction, and pelvic adhesions. CONCLUSION: Early diagnosis enables timely treatment and, consequently, fewer complications. Still, when these factors are absent, vaginal birth may still be possible. The true prevalence and incidence of complications related to Herlyn-Werner-Wunderlich syndrome are still unknown.
Assuntos
Endometriose , Humanos , Feminino , Adulto , Endometriose/complicações , Gravidez , Útero/anormalidades , Útero/diagnóstico por imagem , Cesárea , Rim/anormalidades , Ductos Paramesonéfricos/anormalidades , Anormalidades Múltiplas , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/diagnóstico por imagem , Vagina/anormalidades , Complicações na Gravidez , Nefropatias/congênito , Nefropatias/diagnósticoRESUMO
Müllerian duct anomalies are a group of uncommon and underdiagnosed entities, which cause specific symptoms in adolescent females and may be associated with infertility as well as adverse pregnancy outcomes. These malformations occur as a result of an arrest or abnormal development of the Müllerian ducts in different stages of the female reproductive tract during gestation. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA), formerly known as the Herlyn-Werner-Wunderlich syndrome, is a rare entity characterized by the presence of a uterus didelphys with an obstructed hemivagina cause by a vaginal septum and the association of a renal anomaly (most commonly renal agenesis) ipsilateral to the obstruction. This syndrome may remain undiagnosed during childhood and usually becomes symptomatic after menarche, causing obstructive symptoms. Occasionally it may be identified after the evaluation of a patient with infertility or recurrent pregnancy loss. The clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan. Opportune diagnosis and treatment achieve complete improvement of symptoms, adequate reproductive prognosis and avoid major complications such as endometriosis, pelvic adhesions and infertility. The purpose of this review is to demonstrate the pathophysiology, clinical manifestations, diagnostic methods and treatment of the obstructed hemivagina and ipsilateral renal anomaly syndrome.
Las malformaciones de los conductos de Müller son un grupo de entidades raras y poco diagnosticadas que ocasionan síntomas inespecíficos en adolescentes y pueden acompañarse de problemas de infertilidad y RESULTADOS obstétricos adversos. Estas malformaciones ocurren durante la gestación como consecuencia del desarrollo anormal de los conductos de Müller en diferentes etapas del proceso de formación del aparato reproductor femenino. El síndrome de hemivagina obstruida y anomalía renal ipsilateral, antes conocido como el síndrome de Herlyn-Werner-Wünderlich, es un padecimiento poco común, caracterizado por útero didelfo con una hemivagina obstruida por un tabique vaginal, y la asociación de una anomalía renal (agenesia renal principalmente) ipsilateral a la obstrucción. Este síndrome rara vez se identifica durante la niñez y se vuelve sintomático posterior a la menarquia, ocasionado por los síntomas obstructivos. A veces se identifica posterior a la evaluación de una paciente con problemas de infertilidad o pérdidas gestacionales recurrentes. El diagnóstico clínico es difícil, por eso se requieren estudios de imagen en los que el ultrasonido y la resonancia magnética desempeñan un papel decisivo para el diagnóstico, clasificación y plan terapéutico. El diagnóstico y tratamiento oportunos logran la desaparición de los síntomas, con pronóstico reproductivo adecuado, y se evitan las principales complicaciones: endometriosis, adherencias pélvicas e infertilidad. OBJETIVO: mostrar la fisiopatología, las manifestaciones clínicas, los métodos diagnósticos y terapéuticos del síndrome de hemivagina obstruida y anomalía renal ipsilateral.
Assuntos
Anormalidades Múltiplas , Rim/anormalidades , Ductos Paramesonéfricos/anormalidades , Útero/anormalidades , Vagina/anormalidades , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/embriologia , Anormalidades Múltiplas/epidemiologia , Anormalidades Múltiplas/fisiopatologia , Anormalidades Múltiplas/cirurgia , Adolescente , Diagnóstico por Imagem , Diagnóstico Precoce , Endometriose/etiologia , Feminino , Hematocolpia/etiologia , Humanos , Infertilidade Feminina/etiologia , Rim/embriologia , Ductos Paramesonéfricos/embriologia , Ductos Paramesonéfricos/patologia , Gravidez , Prognóstico , Síndrome , Útero/embriologia , Vagina/embriologia , Ductos Mesonéfricos/embriologiaRESUMO
Caso Clínico 1. J.A, 21 años, nuligesta. Acude por hipermenorragia y síndrome doloroso abdominal. Ecografía informa útero didelfo, hematocolpo en hemivagina ciega, tumoraciones anexiales y agenesia renal derecha conformándose el diagnostico de HWW, es sometida a neosalpingostomia con fimbrioplastia por hematosalpinx derecho por vía laparoscópica. A quince días del postoperatorio presenta cuadro de abdomen agudo. Ecografía informa quiste de ovario con hemorragia intraquística y liquido libre abdominal. Se realiza histerectomía subtotal derecha y drilling del quiste por vía laparotómica. Caso Clínico 2. T.A, 53 años, diagnostico conocido de HWW, cirugías previas histerectomía subtotal y resección del tabique vaginal 38 años antes. Acude por hipermenorrea por miomatosis uterina. Se realiza histerectomía total con resección del 2º cérvix. Caso Clínico 3. S.S, 35 años, diagnostico conocido de HWW, cirugías previas anexectomía izquierda por endometrioma 14 años antes, e histerectomía subtotal, sin resección del tabique en otro hospital 6 años antes. Acude a la consulta donde se diagnostica cervicitis mucopurulenta. Actualmente en planes de resección del tabique vaginal que no se realizo por el cuadro infeccioso presente. Discusión: Hay aproximadamente 200 casos reportados de HWW. La clínica más frecuente es dolor por hematocolpo. Esta patología presenta estrecha relación con infertilidad y endometriosis. Palabras Claves: Síndrome de Herlyn Werner WuÌnderlich, útero didelfo, hemivagina, agenesia renal.
Objective: To report three cases of diagnosis of Werner Wünderlich Herlyn syndrome (HWWS) in the Central Hospital of Social Security Institute of Paraguay (HC-IPS). Materials and Methods: Data from the records of patients of the Gynecology Service of HC-IPS between January/2010 to enero/2012 diagnosed with HWW. Case 1. J.A., female, 21, nulígesta. Presents abdominal pain and hipermenorragia, ultrasound reports didelphys uterus, hematocolpo, adnexal tumors and right renal agenesia making the diagnosis of HWW, is treated by fimbrioplasty and neosalpingostomy for right hematosalpinx by laparoscopy. Postoperative presents acute abdominal pain, ultrasound reports intracystic hemorrhage and abdominal free fluid. Subtotal hysterectomy is done and drilling of right cyst by laparotomy. Case 2. TA, female, 53, HWW known diagnosis, previous surgery: subtotal hysterectomy and vaginal septum resection 38 years earlier. Presents menorrhagia for uterine fibroids. Total hysterectomy is performed with resection of the second cervix. Case 3. SS, female, 35, known diagnosis of HWW, previous surgeries: left adnexectomy for endometrioma 14 years earlier, and subtotal hysterectomy with out removal of the vaginal septum in another hospital 6 years earlier. Attending the clinic where they diagnosed mucopurulent cervicitis. Currently in plans of vaginal septum resection that was not performed by the infection present. Discussion: There are approximately 200 reported cases of HWW. The most frequent symptom is pain by hematocolopo. Keep in mind the strong association with infertility and endometriosis of this disease. Keywords: Werner Syndrome Wünderlich Herlyn, uterus didelphys, hemivagina, renal agenesis.