Clinical features of 102 patients with different types of Herlyn -Werner -Wunderlich syndrome / 中南大学学报(医学版)

OBJECTIVES@#Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare female genital tract malformation, and there are few large sample studies reported at home and abroad. The clinical manifestations of this syndrome are diverse, and insufficient understanding may delay the diagnosis and treatment of the patients. This study aims to analyze the clinical characteristics of different types of HWWS patients, and to improve the diagnosis and treatment of HWWS.@*METHODS@#The clinical data of patients with HWWS who were hospitalized in the Department of Obstetrics and Gynecology, Third Xiangya Hospital of Central South University from October 1, 2009 to April 5, 2022 were retrospectively analyzed. The age, medical history, physical examination, imaging examination and treatment of the patients were collected for statistical analysis. The patients were divided into an imperforate oblique vaginal septum type, a perforate oblique vaginal septum type, and an imperforate oblique vaginal septum and cervical fistula type. The clinical characteristics of different types of HWWS patients were compared.@*RESULTS@#A total of 102 HWWS patients were enrolled, with age of 10-46 years old, including 37 (36.27%) patients with type I, 50 (49.02%) type II, and 15 (14.71%) type III. All patients were diagnosed after menarche, with an average age of (20.5±7.4) years. There were significant differences in the age of diagnosis and course of disease among the 3 types of HWWS patients (both P<0.05). Patients with type I had the youngest age of diagnosis [(18.0±6.0) years] and the shortest course of disease (median course of 6 months), while patients with type III had the oldest age of diagnosis [(22.9±9.8) years] and the longest course of disease (median course of 48 months). The main clinical manifestation of type I was dysmenorrhea, and the main clinical manifestation of type II and type III was abnormal vaginal bleeding. Of the 102 patients, 67 (65.69%) patients had double uterus, 33 (32.35%) had septate uterus, and 2 (1.96%) had bicornuate uterus. The vast majority of patients had renal agenesis on the oblique septum, and only 1 patient had renal dysplasia on the oblique septum. The oblique septum located on the left side in 45 (44.12%) patients and on the right side in 57 (55.88%) patients. There were no significant differences in uterine morphology, urinary system malformation, pelvic mass, and oblique septum among the 3 types of HWWS patients (all P>0.05). Six (5.88%) patients had ovarian chocolate cyst, 4 (3.92%) patients had pelvic abscess, and 5 (4.90%) patients had hydrosalpinx. All patients underwent vaginal oblique septum resection. Among them, 42 patients underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen because they had no sexual life history, and the remaining 60 patients underwent traditional oblique vaginal septum resection. Among the 102 patients, 89 patients were followed up for 1 month to 12 years. The symptoms of vaginal oblique septum in 89 patients such as dysmenorrhea, abnormal vaginal bleeding and vaginal discharge were improved after operation. Among the 42 patients who underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen, 25 patients underwent hysteroscopies again 3 months after operation, and there was no obvious scar formation at the oblique septum incision site.@*CONCLUSIONS@#Different types of HWWS have different clinical manifestations, but all can be manifested as dysmenorrhea. The patient's uterine morphology can be manifested as double uterus, septate uterus, or bicornuate uterus. The possibility of HWWS should be considered if uterine malformation is combined with renal agenesis. Vaginal oblique septum resection is an effective treatment.

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.

OHVIRA syndrome with Orofacial clefts: a rare association

Obstucted hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome or Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital anomaly consist of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We are reporting an adolescent girl with orofacial defect who presented with lower abdominal pain. She attained menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. On abdominal examination a firm, mobile tender mass extending from left iliac fossa up to umbilicus (24 weeks size) was found.  Lower border of mass could not be approached. Further evaluation with ultrasound showed enlarged uterus with collection with internal echoes and non-visualization of the left kidney. CECT showed absent left kidney and didelphys uterus with large left hematocolpometra with left complex adenexal cyst. Patient was posted for hematocolpos drainage and vaginoplasty. An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to severe complications such as endometriosis and infertility.

Síndrome de Herlyn-Werner-Wünderlich y diagnóstico tardío: a propósito de un caso / Herlyn-Werner-WÜnderlich syndrome with late diagnosis: a propos of a case

Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly that affects the genitourinary system. It is defined by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It consists in a Mullerian anomaly. Most patients remain asymptomatic until the menarche, when they present dysmenorrhea, pelvic pain, and a pelvic mass is noticed due to hematocolpos. We present the case of a 32-year-old patient studied for infertility. 3D transvaginal ultrasound and hysterosalpingography showed uterus didelphys and a blind hemivagina. Speculoscopy during menses revealed a normal cervix and a little orifice on the lateral vagina where blood could be seen draining from a fistulized hemivagina. The abdominal ultrasound showed ipsilateral renal agenesis, confirming the suspected diagnosis.

El síndrome de Herlyn-Werner-Wünderlich es una anomalía congénita del tracto urogenital. Se caracteriza por la tríada de útero didelfo, hemivagina obstruida y agenesia renal unilateral. La mayoría de pacientes se mantienen asintomáticas hasta la menarquia, cuando debutan con dismenorrea, dolor pélvico y masa palpable debido al hematocolpos asociado. Presentamos el caso de una paciente de 32 años en estudio por problemas de fertilidad. Se realizó estudio ecográfico 3D e histerosalpingografía, donde se observó útero bicorne completo con doble canal cervical, con impresión diagnóstica de vagina homolateral ciega. A la especuloscopia durante el periodo menstrual se observó cérvix de hemivagina normal y orificio de salida en cara lateral, por donde drenaba sangre de hemivagina ciega (probablemente fistulizada). Se realizó una ecografía abdominal que constató agenesia renal izquierda, confirmándose el diagnóstico.

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.

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.

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.

Radio-diagnosis of Herlyn Werner Wunderlich Syndrome a rare case of urogenital abnormality

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare urogenital abnormality, which is characterized by Mullerian duct anomaly with associated mesonephric duct anomaly. This syndrome is also known as OHVIRA (Obstructed hemivagina with Ipsilateral Renal Anomaly). The incidence of Mullerian Duct Anomaly is 2-3%, and incidence of OHVIRA is 0.16 to 10% of that. The three important components of Herlyn-Werner-Wunderlich (HWW) syndrome are didelphys uterus, obstructed hemivagina and renal agenesis on the same side. Typically presents with pelvic pain in young females after menarche. Presentation in the reproductive age group is unusual. The most common renal anomaly associated with this condition is renal agenesis. Other renal anomalies include renal dysplasia, duplication of kidneys and ureters, crossed fused ectopia and ectopic ureter. Ultrasound and Magnetic resonance imaging are considered as the vital modalities for detection. MRI is the gold standard modality for assessing the anatomical characteristics of the uterus, cervix, and vagina. Early diagnosis helps to prevent complications such as endometriosis, pyocolpos, adhesions, infertility. The unique feature of our case is a presentation in the reproductive age group. Only very few case reports of OHVIRA with a presentation in the reproductive age group are available in the literature. Whenever there is a renal anomaly or renal agenesis, screening of pelvic organs is important in a female child for early detection of Mullerian duct anomalies and prevention of further complications.

A variant of Herlyn Werner Wunderlich Syndrome

@#Herlyn Werner Wunderlich Syndrome is a rare congenital Mullerian anomaly that commonly presents with uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. Presented here is a rare variant with contralateral dysplastic kidney with ectopic ureteral insertion instead of renal agenesis. The patient initially presented as a case of recurrent pelvic inflammatory disease who eventually underwent excision of vaginal septum with drainage of pyocolpos. Postoperatively, the patient had urinary incontinence and was eventually referred to Urology for further surgical intervention. The wide range of symptomatology and the uncommon anatomic presentation of this case led to the delay in the diagnosis and the consequent gynecologic complications.

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.

MRI in diagnosis of Herlyn-Werner-Wunderlich syndrome / 中国医学影像技术

Objective To assess the value of MRI in diagnosis of Herlyn-Werner-Wunderlich syndrome (HWWS).Methods MR images of 10 patients with HWWS confirmed surgically and pathologically were analyzed retrospectively.The classification of HWWS on MRI was discussed.Results The 10 patients were included 4 cases of type Ⅰ,3 cases of type Ⅱ and 3 cases of type Ⅲ.All of them were complicated with ipsilateral renal agenesis.Eight cases were complicated with didelphic uterus and double cervix,2 were complicated with complete septate uterus.Type Ⅰ showed the complete oblique vaginal septum,hematocele in the oblique septum cavity and contralateral uterine cavity.Type Ⅱ showed the oblique septum cavity was connected with main vaginal through the hole of septum.Type Ⅲ showed the oblique septum cavity was connected with main vaginal through the cervical fistula.The prognosis were good in 8 patients.One patient occured obvious dysmenorrhea postoperation and MRI reminded right ovarian endometriosis cyst.One patient occured gusty hypogastralgia,and MRI reminded pelvic inflammation.Conclusion MRI can definitely diagnose and classify HWWS,and can provide useful information in the treatment of HWWS.

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.

Síndrome de Herlyn-Werner-Wunderlich. Síndrome OHVIRA. Reporte de Caso / Herlyn-Werner-Wunderlich Syndrome or OHVIRA Syndrome. Case Report

Paciente Femenina de 11 años con el síndrome de Herlyn-Werner-Wunderlich, manejado multdisciplinariamente y resuelto endoscópicamente con una septotomía y dilataciones vaginales.

This case is about a female patent, 11 years old, with Herlyn-Werner-Wunderlich Syndrome, who received multdisciplinary approach and was endoscopically resolved with septotomy and vaginal dilatatons.

Two Cases of Herlyn-Werner-Wunderlich Syndrome Diagnosed in Perinatal Period

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.

Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome: Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis

Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy-proven thin glomerular basement membrane disease which is not related to the above syndrome.