Dysmenorrhea due to undiagnosed obstructed hemi-vagina and ipsilateral renal anomaly syndrome can become a cause of suicide

BACKGROUND@#A Japanese woman in her early twenties had committed suicide, jumped from a 25-meter high bridge into a lake. She had been suffering from severe dysmenorrhea and general fatigue monthly.@*RESULTS@#A forensic autopsy revealed indications of a bicorporeal uterus, obstructed hemi-vagina, and ipsilateral renal agenesis, which lead to a diagnosis of obstructed hemi-vagina and ipsilateral renal anomaly (OHVIRA) syndrome. On the right side of the uterus, an enclosed cavity composed of black clots was observed. Histological findings suggested that her endometrium was in the early proliferative phase, implying that she was in the menstrual phase just before her death. She may have been suffering from severe lower abdominal pain from the increased pressure of the closed uterus cavity.@*CONCLUSIONS@#This case indicates that dysmenorrhea from undiagnosed OHVIRA syndrome can possibly lead to a suicide attempt. In Japan, because suicide was the leading cause of death for people aged 15 to 39 in 2019, preventive measures for suicide should be promoted. The present case also suggests that intervention for dysmenorrhea may prevent this in adolescent woman.

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.

Herlyn-Werner-Wunderlich Syndrome with Central Precocious Puberty: A Case Report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

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.

Herlyn-Werner-Wunderlich Syndrome: A Mini-review

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

Complete septate uterus, obstructed hemivagina, and ipsilateral adnexal and renal agenesis in pregnancy

Most cases of double uterus with obstructed hemivagina and ipsilateral renal agenesis were diagnosed at adolescents after menarche. This is the first reported case of complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis in addition to ipsilateral agenesis of fallopian tube and ovary in which the diagnosis was delayed until pregnancy. The pregnancy was uneventful in spite of intermittent vaginal spotting. During the cesarean section, the septum of the uterus was resected and about a 3-cmx3-cm window was made on the vaginal septum to allow an opening for the obstructed vaginal discharge. We followed the patient up for one and half years, and she has not had symptoms such as dysmenorrhea or abnormal vaginal bleeding.

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.

Síndrome de Herlyn Werner Wünderlich. Reporte de tres casos / Herlyn Wunderlich Werner Syndrome. Report of three cases

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 Wünderlich, 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.

Diagnosis and treatment of uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis / 대한산부인과학회지

Uterine malformations consist of a group of miscellaneous congenital anomalies of the female genital system, and these anomalies are the result of major disturbances in the development, such as formation or fusion of the Mullerian or paramesonephric ducts during fetal life. The Mullerian anomalies have been estimated to occur in up to 0.001~10%, and these anomalies may be associated with numerous congenital anomalies, and ipsilateral renal anomalies especially are the most common. The uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis clinically may result in dysmenorrhea, low abdominal pain, endometriosis or pelvic mass after menarche. Furthemore the delayed diagnosis causes infertility and poor pregnancy outcome due to continued retrograde menstruation which leads to endometriosis and distorted pelvic anatomy. These anomalies could be diagnosed with ultrasonography and MRI that have the high sensitivity and specificity. Excision of vaginal septum and marsupialization are appropriate treatments to relieve symptoms and reserve fertility. We report 7 cases of uterine didelphys with obstructed hemivagina and ipsi-unilateral renal agenesis with a brief review of relevant literatures to help understand these anomalies.

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.

Magnetic resonance imaging in the evaluation of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis: a case report / 대한산부인과학회지

Uterus didelphys with unilateral obstructed hemivagina is indeed a very rare congenital anomaly. Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis usually presents after menarche with progressive abdominal pain during menses secondary to hematocolpos. The method of choice for diagnosis is magnetic resonance imaging (MRI). MRI can do much for the early diagnosis and the prevention against further complications of this condition because it can demonstrate the mullerian duct anomaly complicated with obstructed hemivagina in detail and even ipsilateral renal agenesis. A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity. Early accurate diagnosis after menarche followed by excision and marsupialization of the blind hemivagina offers complete relief of symptoms and preserves reproductive potential. We report a case of uterine didelphys with obstructed hemivagina and ipsilateral renal agensis with a brief review of the literature.

A Case of the Uterus Didelphys with Unilateral Obstructed Hemivagina / 대한산부인과학회잡지

Uterus didelphys with unilateral obstructed hemivagina is indeed a very rare congenital anomaly due to M llerian duct malformation. The most common clinical presentation is pelvic pain and dysmenorrhea shortly after menarche, in associated with the finding of a vaginal or pelvic mass. An accurate and prompt diagnosis is of importance to permit treatment and to assure the future fertility of the patient. The simple and adequate treatment of the condition is incision of the obstructed vaginal septum providing adequate drainage of the retained blood. We report a case of uterus didelphys with obstructed hemivagina with brief review of the literature.

A Case of Hematometrocolpos due to Complete Septate Uterus and Obstructed Hemivagina associated with Ipsilateral Renal Agenesis / 대한산부인과학회잡지

We report a case of a 13-year-old girl who was noted to have combined malformation of complete septate uterus, obstructed hemivagina and ipsilateral renal agenesis with complaints of dysmenorrhea. Hematometrocolpos was developed in the result of retained menstrual blood since menarche. This rare congenital anomaly is supposed to be derived from disorders of mesonephric and M llerian developement in embryogenesis of female genitouinary tract. An accurate preoperative diagnosis of this syndrome is critical because of the difference in therapeutic strategy and prognosis. Simple and definite treatment of the condition is excision of the obstructed vaginal septum with prompt exit of retained blood. We present here a interesting case of M llerian anomaly in attempt to alert gynecologist to the possible occurrence of such a malformation with a brief review of concerned literatures.

3 Cases of Uterus Didelphys with an Obstructed Hemivagina and Ipsilateral Renal Agenesis / 대한산부인과학회잡지

Associated congenital anomalies of both reproductive and urinary tracts are frequent, because wolffian and mullerian developments are closely related. The combination of uterus didelphys, obstructive hemivagina, ana ipsilateral renal agenesis is a rare but specific syndrome. The most common clinical presentation is pelvic pain and/or dysmenorrhea shortly after menarche, in association with the finding of a vaginal or pelvic mass. An accurate and prompt diagnosis is of importance to permit treatment and to assure the future fertility of the patients. The simple and curative treatment of the condition is incision of the obstructing vaginal septum providing adequate drainage of the retained blood. We report three cases of uterus didelphys with an obstructed bemivagina and ipsilateral renal agenesis with a brief review of concerned literatures.

A Case of Successful Gestational Outcome in the Presence of Preterm-labor Due to Hematometro-colpos of Obstructed Hemivagina and Uterus Didephys / 대한주산의학회잡지

Uterine didelphys with unilateral hematometra, hemicolpos, and ipsilateral renal agenesis is a rare m01lerian duct malformation. Uterine didelphys with an obstructed hemivagina is frequently associated with renal agenesis, mostly ipsilateral to the blind vaginal pouch. Accurate early diagnosis and septal resection elevate pregnancy rate and birth rate but it is difficult to find these malfomations until the complication associated with pregnancy was developed. We report a case of successful gestational outcome in the presence of preterm labor due to hematometrocolpora caused by uterine didelphys with unilateral imperforated vagina and ipsilateral renal agenesis with brief review of the literatures.