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Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), also called Herlyn–Werner–Wunderlich syndrome, is an extremely rare Müllerian duct anomaly accompanied by Wolffian duct anomalies. A 10-year-old intact female Yorkshire Terrier weighing 3.35 kg was presented with anorexia, depression, vomiting, and abdominal pain. Radiography, ultrasonography, and computed tomography revealed uterine didelphys, obstructed hemivagina, a cystic structure around the right uterus, and right renal agenesis, leading to the diagnosis of OHVIRA syndrome. An ovariohysterectomy and decompression of the obstructed right hemivagina were performed, and the cystic structure near the right uterine horn was removed by en bloc resection, along with the right uterus and ovary. After the surgical intervention, the patient's symptoms including abdominal pain, anorexia, and depression were immediately resolved. The patient was followed up for 1 month postoperatively with ultrasonography at 2-week intervals, which revealed the progression of mild fluid retention in the right hemivagina. However, no additional urogenital findings were identified, and the patient continued to exhibit no overt clinical symptoms. This case report describes the diagnosis and surgical treatment of the first documented case of OHVIRA syndrome in an animal. Unlike in human medicine, where vaginal septectomy is performed to prevent dilatation of the obstructed hemivagina and thereby resolve clinical symptoms, performing ovariohysterectomy combined with fluid aspiration from the obstructed hemivagina showed a favorable postoperative prognosis in the dog. [ABSTRACT FROM AUTHOR]

Background/Objectives: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. Methods: A literature search was conducted in PubMed and Web of Science, using the following search terms: "endometriosis and uterine malformation, endometriosis and Herlyn–Werner–Wunderlich syndrome", "endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome" and "uterus didelphys, renal agenesis and endometriosis". Results: We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA. The most prevalent symptoms were dysmenorrhea and lower abdominal pain. Renal agenesis occurred more frequently on the right side. In the majority of cases, vaginal septum resection was performed to alleviate hematometrocolpos. Among the 97 cases necessitating abdominal exploration, endometriosis was identified in 61 patients (62.9%), although this figure is most likely an overestimation. However, a significantly heightened risk of endometriosis was evident. Conclusions: This literature review highlights the importance of considering the potential for urogenital malformation and endometriosis in cases of dysmenorrhea during adolescence. Ultrasound examination has proven to be a valuable diagnostic tool for identifying uterine abnormalities and guiding subsequent diagnostic and, if necessary, surgical interventions. Thorough assessment and appropriate management are imperative to mitigating the long-term consequences associated with deep infiltrating endometriosis. [ABSTRACT FROM AUTHOR]

Background: Mullerian duct anomalies include a broad spectrum of genital tract defects that arise from developmental abnormalities of the genital tract. Herlyn–Werner–Wunderlich syndrome (HWWS) refers to the combination of uterus didelphys, blind hemivagina and ipsilateral renal agenesis. In the literature, the syndrome often appears as a few sporadic case reports. Case: We report a case of symptomatic Herlyn–Werner–Wunderlich syndrome diagnosed by transavaginal scan and MRI and successfully treated with a laparoscopic-assisted hysteroscopic vaginal septum resection. The postoperative course was without complications, and clinical symptoms completely resolved the surgery. Summary and Conclusions: Combination of laparoscopy and hysteroscopy was found to be an easy, safe, effective and appropriate approach for patients with HWWS. [ABSTRACT FROM AUTHOR]

The Herlyn-Werner-Wunderlich syndrome is a rare congenital disorder with uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, more common on right side. HWW syndrome usually presents at puberty with symptoms like pelvic pain, dysmenorrhea and palpable mass due to the associated hematocolpos or hematometra. Delayed presentation in adulthood as infertility can occur in case of incomplete vaginal septum. Short term complications like pyohematocolpos, pyosalpinx, or pelviperitonitis, and long-term complications, such as endometriosis, increased risk of abortion and infertility can occur. Ultrasound is the initial investigation which demonstrates uterine anomaly, renal agenesis and hematocolpos.Magnetic resonance imaging (MRI) is the imaging modality of choice which helps in confirmation of diagnosis, delineation of vaginal septum, communication between 2 cavities and associated pathologies like adenomyosis better.Vaginal septum resection is the treatment of choice for obstructed hemivagina with hematocolpos.We present you a case of 43 years female with cyclical dysmenorrhea starting few years after menarche with uterine didelphys, left sided vaginal septum with hematocolpos and left renal agenesis. Along with this, diffuse bilateral adenomyosis was present which is uncommonly associated with this anomaly as obstruction is at the level of vagina. Patient was diagnosed late due to misdiagnosis and later treated with septal resection for hematocolpos and with hormonal medications for adenomyosis.

Background: Mullerian duct anomalies include a broad spectrum of genital tract defects that arise from developmental abnormalities of the genital tract. Herlyn–Werner–Wunderlich syndrome (HWWS) refers to the combination of uterus didelphys, blind hemivagina and ipsilateral renal agenesis. In the literature, the syndrome often appears as a few sporadic case reports. Case: We report a case of symptomatic Herlyn–Werner–Wunderlich syndrome diagnosed by transavaginal scan and MRI and successfully treated with a laparoscopic-assisted hysteroscopic vaginal septum resection. The postoperative course was without complications, and clinical symptoms completely resolved the surgery. Summary and Conclusions: Combination of laparoscopy and hysteroscopy was found to be an easy, safe, effective and appropriate approach for patients with HWWS.

Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA) also known as Herlyn-Werner-Wunderlich syndrome is a rare type of Müllerian duct anomaly with concomitant mesonephric duct anomalies. It is characterized by uterus didelphys, obstructed hemi-vagina, and commonly renal agenesis however; other types of mesonephric duct anomalies such as duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia have also been reported. We present a case report of a 21-year-old female patient experiencing new-onset vaginal bleeding and pelvic pain over the past month. Subsequent multi-sequence MRI revealed characteristic features indicative of OHVIRA syndrome. Clinical manifestations typically encompass pelvic pain and menstrual abnormalities but mainly depend upon the presence of communication between the hemi-uterus. This article aims to discuss the clinical presentation, types, and diagnostic utility of ultrasound and MRI in OHVIRA syndrome. Additionally, we discuss the possible management plans.

Abstract Background Herlyn–Werner–Wunderlich syndrome also known as OHVIRA syndrome is a rare complex congenital developmental anomaly characterized by the triad of uterine didelphys, obstructed hemivagina causing hematometrocolpos and ipsilateral renal anomaly. Case presentation Here we report a case of a 14-year-old girl who presented with acute onset lower abdominal pain. Ultrasound and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina and right renal agenesis. Patient underwent hematocolpos drainage. Conclusions OHVIRA syndrome is an uncommon congenital anomaly. Imaging plays a major role in diagnosis. Surgery is the treatment of choice to resect the septum and relieve the obstruction. An early correct diagnosis is the goal to relieve the symptoms and prevent complications, caused by retrograde menstruation which may result in endometriosis and, also, preserve sexual and conception abilities.

A 27‐year‐old nulliparous woman presented with a feeling of fullness in the lower abdomen and abdominal pain. A left ovarian tumor, uterus didelphys, left renal agenesis, and left vaginal atresia were observed on imaging. The ovarian tumor was presumed to have caused the abdominal pain, and an abdominal left adnexectomy was performed. After 3 months, she reported severe lower abdominal pain during menstruation. Transvaginal ultrasonography revealed uterine enlargement. After 17 days, the patient presented with abdominal pain and fever. She was diagnosed with peritonitis due to infection and left uterine hematometra. Because she did not improve with antibiotic treatment, left laparoscopic hysterectomy was performed. Subsequently, she did not experience the lower abdominal pain. Appropriate diagnosis and treatment based on the morphology of the reproductive tract and symptoms must be considered in patients with Herlyn–Werner–Wunderlich syndrome. Treatment must permit the outflow of menstrual blood. [ABSTRACT FROM AUTHOR]

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), also called Herlyn–Werner–Wunderlich syndrome, is an extremely rare Müllerian duct anomaly accompanied by Wolffian duct anomalies. A 10-year-old intact female Yorkshire Terrier weighing 3.35 kg was presented with anorexia, depression, vomiting, and abdominal pain. Radiography, ultrasonography, and computed tomography revealed uterine didelphys, obstructed hemivagina, a cystic structure around the right uterus, and right renal agenesis, leading to the diagnosis of OHVIRA syndrome. An ovariohysterectomy and decompression of the obstructed right hemivagina were performed, and the cystic structure near the right uterine horn was removed by en bloc resection, along with the right uterus and ovary. After the surgical intervention, the patient’s symptoms including abdominal pain, anorexia, and depression were immediately resolved. The patient was followed up for 1 month postoperatively with ultrasonography at 2-week intervals, which revealed the progression of mild fluid retention in the right hemivagina. However, no additional urogenital findings were identified, and the patient continued to exhibit no overt clinical symptoms. This case report describes the diagnosis and surgical treatment of the first documented case of OHVIRA syndrome in an animal. Unlike in human medicine, where vaginal septectomy is performed to prevent dilatation of the obstructed hemivagina and thereby resolve clinical symptoms, performing ovariohysterectomy combined with fluid aspiration from the obstructed hemivagina showed a favorable postoperative prognosis in the dog.

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wünderlich syndrome is a rare gynecologic malformation, which consists of the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. This syndrome usually presents during puberty with unspecific symptoms such as pelvic pain, dysmenorrhea and palpable mass due to hematocolpos. It can also manifest during adulthood with primary infertility. A timely diagnosis is valuable in order to prevent long term complications such as endometriosis and infertility. We present the case of an 11-year-old preadolescent with the characteristic imaging findings of this pathology [ABSTRACT FROM AUTHOR]

Twelve-years-old girl, with prenatal diagnosis of right renal agenesis, menarche at eleven-years-old, presenting with dysmenorrhea, metrorrhagia and menorrhagia. On objective examination, she presented diffuse pain on deep abdominal palpation, without masses. Laboratory tests were unremarkable. A supra-pubic pelvic ultrasound revealed a heterogeneous nodular structure with benign characteristics measuring 4x3,5cm, which seemed to correspond to a teratoma. The patient was referred to pediatric oncology. The analytical study was extended, including tumor markers, without alterations. Abdominal-pelvic CT and MR scan revealed uterine malformation and distension of the vaginal canal by hematic content. Diagnosis of Herlyn-Werner-Wunderlich Syndrome (HWWS) was made. Oriented to gynecology and submitted to drainage of the hematocolpos. Currently, she is on oral iron, due to anemia, contraceptives and analgesia with progressive improvement. HWWS is a rare congenital anomaly of the female urogenital tract, characterized by bicorporeal uterus, obstructed hemivagina and ipsilateral renal agenesis. Symptoms are usually after menarche and nonspecific. [ABSTRACT FROM AUTHOR]

Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by obstructed hemivagina and ipsilateral renal anomaly, a rare congenital anomaly of the genitourinary tract, resulting from malformations of the renal tract associated with Müllerian duct anomalies. The initial symptoms of HWW frequently present after menarche and may be nonspecific, leading to a delayed diagnosis. We presented a 19-year-old female with 3-year hematuria and abdominal pain. The final diagnosis of HWW syndrome with a rare vesicovaginal fistula was made. The treatment of HWW syndrome typically involves surgical intervention. The primary treatment is resection or removal of the obstructed vaginal septum. The patient underwent excision of vaginal septum and vaginal reconstruction via hysteroscopy, as well as repair of the vesicovaginal fistula. The patient improved well after surgery and fully recovered without sequelae after 3 months. In addition, unilateral renal agenesis is one of congenital abnormalities of the kidney and urinary tract, which are the most frequent cause of chronic kidney disease (CKD) in children. This report describes a patient of HWW syndrome with rarely combined vesicovaginal fistula, and highlights the importance of early recognition and management to prevent associated complications. [ABSTRACT FROM AUTHOR]

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13‐year‐old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi‐uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post‐procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases. Synopsis: A case report of a patient with obstructed hemivagina with ipsilateral renal agenesis syndrome supports hemihysterectomy as a potential therapeutic approach in refractory cases. [ABSTRACT FROM AUTHOR]

Zinner Syndrome (ZS) and Herlyn-Werner-Wunderlich Syndrome (HWWS) are congenital genitourinary anomalies accompanied by ipsilateral renal agenesis. These syndromes, which can cause symptoms such as infertility, pelvic pain, and bladder irritation, can be diagnosed by digital rectal examination, transrectal and abdominal ultrasonography (US), Computer Tomography (CT) and more ideally Magnetic resonance imaging (MRI). Similar physiopathological processes are observed in both syndromes and urogenital malformations accompany renal agenesis. We aimed to discuss the three cases of ZS in men and two HWWS cases in women, which are rarely observed in the literature. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital condition characterized by renal agenesis, uterine didelphys, and obstructed hemivagina. This report presents the case of a 19-year-old woman who reported lower abdominal pain and offensive vaginal discharge. Imaging revealed a didelphys uterus, two vaginas, two cervixes, hematocolpos, and an absent right kidney. Surgical intervention involved draining the hematocolpos and excising the uterine septum. After surgery, the patient successfully conceived and had a full-term pregnancy, delivering via cesarean section without complications. This case highlights the importance of early diagnosis and surgical management in preventing complications such as endometriosis and infertility. Prompt recognition and treatment are crucial for preserving fertility in patients with HWW syndrome.

Key Clinical Message: OHVIRA syndrome can be misdiagnosed due to its rarity, resulting in the need for more invasive interventions than vaginoscopy. Also, delayed diagnosis of OHVIRA syndrome can affect patient's quality of life by leading to chronic gynecological diseases such as endometriosis and pelvic inflammatory disease. Obstructive hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is one of the infrequent congenital Mullerian duct anomalies characterized by obstructed hemivagina and ipsilateral renal agenesis. This study presents a 20‐year‐old virgin female who was diagnosed with OHVIRA syndrome and treated by vaginoscopy using the hymen preservation technique. Also, she was misdiagnosed with non‐communicating rudimentary uterine horn 4 years ago. Late or misdiagnosis of OHVIRA syndrome can affect fertility and pregnancy outcomes. Therefore, early diagnosis and management are crucial. OHVIRA syndrome's misdiagnosis is possible with other Mullerian duct anomalies, such as a rudimentary uterine horn. Also, patients with misdiagnosis undergo unnecessary interventions. [ABSTRACT FROM AUTHOR]

It is unclear whether clinical background differs between endometriosis in adolescent patients with obstructive Müllerian anomalies and those without anomalies. The aim of the study is to identify the difference in clinical characteristics of endometriosis in patients with or without obstructive Müllerian anomalies. The study involved 12 patients aged under 24 years old who underwent primary surgery for obstructive Müllerian anomalies and 31 patients aged under 24 years old who underwent surgery for ovarian endometrioma. A total of 6 out of 12 cases with obstructive Müllerian anomalies developed endometriosis (4 Herlyn–Werner–Wunderlich syndrome, 2 non-communicating functional uterine horn, 2 cervical aplasia). The age at surgery was significantly younger in endometriosis with obstructive Müllerian anomalies, compared with those without obstructive Müllerian anomalies (17.8 ± 4.4 vs. 23.1 ± 1.3, p = 0.0007). The rate of endometrioma was 50.0% and the rate of hydrosalpinx was significantly higher (66.7% vs. 0%, p = 0.0002) in the group of obstructive Müllerian anomalies. The recurrence rate of endometriosis was 20.0% in the group of anomalies and 25.9% in the group of those without anomalies. Adolescent patients with obstructive Müllerian anomalies more easily developed endometriosis and co-occurred with higher rate of hematosalipinx. [ABSTRACT FROM AUTHOR]

Background: Herlyn-Werner-Wunderlich Syndrome or OHVIRA syndrome is uterine didelphys with obstructed hemi-vagina and ipsilateral renal agenesis syndrome is a very rare congenital urogenital anomaly. Case: A 21-year-old female presented with acute abdomen and severe dysmenorrhea. Ultrasonography performed in obstetric emergency room showed a cystic lesion in right pelvic area, free fluid in Douglas’s pouch and right renal agenesis. Diagnostic laparoscopy revealed uterine didelphys with right ovarian hemitorsion. A unilateral hysterectomy was conducted. Conclusion: HWW syndrome could manifest as acute abdominal symptoms and typically requires treatment involving resection of the vaginal septum and drainage of hematometrocolpos. [ABSTRACT FROM AUTHOR]

A quiz is presented related to a case where a 14 year old girl who was suffering from chronic, severe abdominal pain with a renal anomaly.

Introduction: Herlyn-Werner-Wunderlich (HWW)/obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Müllerian duct anomaly (MDA). Usually presenting after menarche classically with dysmenorrhea. Here we present a challenging case, where the diagnosis was missed initially. Case presentation: A 10-year-old girl presenting with dysmenorrhea and constipation, treated repeatedly with analgesics, laxatives and enema. A pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of OHVIRA syndrome, which requires surgical intervention. We describe a "no -touch- technique" for resecting the septum. The patient had immediate relief of symptoms and a smooth post-operative recovery. Conclusions: Early diagnosis and surgical management are crucial not only to relieve symptoms but to prevent long term complications that may affect the patients' reproductive potential. Despite pelvic ultrasound being an affordable and a useful diagnostic tool, the diagnosis can be missed by inexperienced operators. Therefore, MRI is recommended to confirm and classify MDAs. The hysteroscopic "no-touch-technique" to resect the septum is an effective, safe and a convenient approach to treat the condition while preserving hymen integrity. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital malformation of the Mullerian duct. We report on a 34-year-old female who presented to the emergency department for cramping lower abdominal pain and pelvic pressure with vaginal spotting. Physical exam showed marked swelling in the right adnexa, and laboratory exams were within normal limits except for positive Coronavirus disease 2019 (COVID-19) status. Transvaginal ultrasound revealed 3 well circumscribed, round hypoechoic complex cystic lesions with arterial doppler detected within the peripheral walls. Magnetic resonance imaging of the abdomen and pelvis showed a right hemivagina, right hematosalpinx, right hematometra and right renal agenesis, compatible with OHVIRA syndrome. The patient was informed of elective surgical procedure but was unable to undergo surgery at this time secondary to COVID status. The patient was therefore recommended oral contraceptive therapy for suppression of menses and protection of endometrial lining.

Herlyn–Werner–Wunderlich (HWW) syndrome is characterized by obstructed hemivagina and ipsilateral renal anomaly, a rare congenital anomaly of the genitourinary tract, resulting from malformations of the renal tract associated with Müllerian duct anomalies. The initial symptoms of HWW frequently present after menarche and may be nonspecific, leading to a delayed diagnosis. We presented a 19-year-old female with 3-year hematuria and abdominal pain. The final diagnosis of HWW syndrome with a rare vesicovaginal fistula was made. The treatment of HWW syndrome typically involves surgical intervention. The primary treatment is resection or removal of the obstructed vaginal septum. The patient underwent excision of vaginal septum and vaginal reconstruction via hysteroscopy, as well as repair of the vesicovaginal fistula. The patient improved well after surgery and fully recovered without sequelae after 3 months. In addition, unilateral renal agenesis is one of congenital abnormalities of the kidney and urinary tract, which are the most frequent cause of chronic kidney disease (CKD) in children. This report describes a patient of HWW syndrome with rarely combined vesicovaginal fistula, and highlights the importance of early recognition and management to prevent associated complications.

Purpose: This study aimed to perform a systematic review of patients with Herlyn–Werner–Wunderlich syndrome (HWWS) and present the prevalence of symptoms, anatomical variants, endometriosis, surgical interventions, and short- and long-term outcomes. Methods: A structured search was performed in PubMed, Scopus, Embase, and China National Knowledge Infrastructure, and studies published between 1 January, 2000 and 19 April, 2022 were included. The following data on HWWS were recorded: symptoms, anatomical variations, surgical interventions and short- and long-term outcomes. Results: A total of 1673 patients were included in our analysis. The main symptoms were dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%). The principal anomalies were right-obstructed hemivagina (57.3%), haematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%). A majority of patients belonged to classification 1.1 (46.7%), with a blind hemivagina, and classification 2.1 (39.2%), with a small communication between two vaginas. The mainstay of treatment was vaginal septum excision (91.8%). Minimally invasive surgery (48.5%) was performed only after vaginal surgery (61.9%), and only a few patients required a second surgery (2.2%). Endometriosis was found in 9.6% of the patients. Fifty-two percent of them had ipsilateral ovarian endometriosis cysts. Pregnancy rate of these patients was 72.1%. The rate of adverse pregnancy outcomes was 22.4%. The caesarean section rate was 61.2%. Conclusions: Patients with HWWS presented with nonspecific symptoms and demonstrated various combinations of anomalies. The most common anatomical variants are classifications 1.1 and 2.1. Vaginal septum excision is effective in relieving symptoms and preventing complications, with hysteroscopic surgery as an option where there is concern about protecting the hymen from minor injury. The pregnancy rate for these patients after surgery was satisfactory, and the rate of adverse pregnancy outcomes after surgery was acceptable. We advise females with urological anomalies to be screened for Müllerian anomalies because of the close association between these two types of anomalies. Thus, HWWS contributes to the occurrence of endometriosis; however, more research is required to investigate the relationship between pelvic endometriosis and HWWS. [ABSTRACT FROM AUTHOR]

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a very rare type of Müllerian duct anomaly. Cases of OHVIRA syndrome have varied clinical manifestations. We report the case of a 15-year-old virgin with endometrial cyst and OHVIRA syndrome. Magnetic resonance imaging confirmed the diagnosis of OHVIRA syndrome. A salpingo-oophorectomy laparotomy was performed, but the patient refused to perform a septotomy or vaginal septal resection. We compared the cases of OHVIRA syndrome that we found with several other reports. The complaints of patients with OHVIRA syndrome may vary from one another, with different histories. Imaging investigations can confirm the diagnosis. The main treatment in OHVIRA syndrome is intended to alleviate symptoms that appear.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare variant of mullerian duct anomalies characterized by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. We report two cases of HWW syndrome in young women with abdominal pain. This diagnosis can easily be missed if a clinician is not aware of the syndrome. In one case, the computed tomography image of obstructed hemivagina with hematocolpos was initially mistaken for a cystic tumor of adnexa by the radiologist; in the other case, correct diagnosis was missed for years despite numerous visits to different hospitals due to lack of awareness of the syndrome.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a very rare type of Müllerian duct anomaly. Cases of OHVIRA syndrome have varied clinical manifestations. We report the case of a 15-year-old virgin with endometrial cyst and OHVIRA syndrome. Magnetic resonance imaging confirmed the diagnosis of OHVIRA syndrome. A salpingo-oophorectomy laparotomy was performed, but the patient refused to perform a septotomy or vaginal septal resection. We compared the cases of OHVIRA syndrome that we found with several other reports. The complaints of patients with OHVIRA syndrome may vary from one another, with different histories. Imaging investigations can confirm the diagnosis. The main treatment in OHVIRA syndrome is intended to alleviate symptoms that appear. [ABSTRACT FROM AUTHOR]

Purpose: Endometrial carcinoma (EC) is a common gynecological malignancy. Vaginal cuff brachytherapy (VBT) is an adjuvant treatment for EC. Since a single-channel cylinder sometimes delivers inadequate dose coverage to the vaginal apex, three-dimensional (3D) printing technology can be used to achieve satisfactory dose distribution. Here, we report the first case of an EC patient with Herlyn-Werner-Wunderlich syndrome (HWWS) treated with VBT using 3D-printed applicators. Case Presentation: Here, we present a case study of an endometrial cancer patient with HWWS who underwent surgery. During adjuvant radiotherapy, 3D-printed applicators were used in VBT. To accomplish the reconstruction of the source pathways on magnetic resonance imaging, catheters with copper sulfate were placed in two 3D-printed applicators. The early tolerance of this treatment was positive. During the 6-month follow-up, locoregional recurrence was not detected. Conclusion: Our findings strongly indicate that VBT with 3D-printed applicators may be a reasonable treatment option for EC with HWWS. [ABSTRACT FROM AUTHOR]

It is unclear whether clinical background differs between endometriosis in adolescent patients with obstructive Müllerian anomalies and those without anomalies. The aim of the study is to identify the difference in clinical characteristics of endometriosis in patients with or without obstructive Müllerian anomalies. The study involved 12 patients aged under 24 years old who underwent primary surgery for obstructive Müllerian anomalies and 31 patients aged under 24 years old who underwent surgery for ovarian endometrioma. A total of 6 out of 12 cases with obstructive Müllerian anomalies developed endometriosis (4 Herlyn–Werner–Wunderlich syndrome, 2 non-communicating functional uterine horn, 2 cervical aplasia). The age at surgery was significantly younger in endometriosis with obstructive Müllerian anomalies, compared with those without obstructive Müllerian anomalies (17.8 ± 4.4 vs. 23.1 ± 1.3, p = 0.0007). The rate of endometrioma was 50.0% and the rate of hydrosalpinx was significantly higher (66.7% vs. 0%, p = 0.0002) in the group of obstructive Müllerian anomalies. The recurrence rate of endometriosis was 20.0% in the group of anomalies and 25.9% in the group of those without anomalies. Adolescent patients with obstructive Müllerian anomalies more easily developed endometriosis and co-occurred with higher rate of hematosalipinx.

Introduction: OHVIRA syndrome is a rare congenital anomaly of Müllerian duct development characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The primary treatment is surgical excision of the obstructed hemivaginal septum and hematometrial drainage. In recent years, minimally invasive approaches such as hysteroscopic or vaginoscopic septum resection have been reported. Furthermore, we originally developed some novel pneumovaginoscopic gynecologic surgeries for years using a device that consists of a cylinder that fits into the vagina and a lid that mounts multiple ports, allowing the vagina to be dilated with carbon dioxide gas, similar to a single‐port laparoscope. Materials and Surgical Technique: We report a successful pneumovaginoscopic surgery for a complicated recurrent abscess in a patient with OHVIRA syndrome. Conventional surgery was performed with a single forceps in a liquid, as in cystoscopy or hysteroscopy. However, this new surgery allowed multiple forceps in a gas, as in laparoscopy. So pus and blood were aspirated and washed away without leaking into the abdominal cavity via fallopian tubes. The surgical smoke generated by thermal coagulation also aspirated to clean the field of vision immediately. And thick, complicated abscesses were drained successfully. The patient conceived through IVF with ICSI and delivered safely at full term. Discussion: Pneumovaginoscopy could benefit complex vaginal surgery cases, such as abscess formation in patients with OHVIRA syndrome. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich (HWW) syndrome OHVIRA syndrome (Obstructed HemiVagina Ipsilateral Renal Agenesis) is rare type of Mullerian duct anomaly, with uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Usually presenting in post-pubertal adolescent girls presenting as pain abdomen or pelvic masses on the side of the obstructed Hemivagina. Here we represent a case report of a 14-year-old girl who presented to our outpatient department complaining lower abdominal pain for 2 months with previous normal menstrual cycles. On General Physical examination well-developed breasts and normal axillary and pubic hair distribution were noted. The Baseline laboratory analysis and hormonal profile were reported normal. Magnetic Resonance Imaging (MRI) provided a fruitful help in correct diagnosis of this rare anomaly. The treatment modality being the surgery to relieve obstruction and its associated complications. [ABSTRACT FROM AUTHOR]

Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Most cases of OHVIRA have been reported in adolescents or adults. Gartner duct cysts, including those manifesting as vaginal wall cysts, are also rare. Fetal OHVIRA syndrome and Gartner duct cysts are difficult to diagnose. Case Presentation: Here, the authors report a case of combined OHVIRA and Gartner duct cyst diagnosed prenatally by ultrasonography, along with a brief review of the relevant published reports. A 30-year-old nulliparous female was referred to our institution at 32 weeks' gestation for fetal right kidney agenesis. Detailed ultrasonographic examinations using 2D, 3D, and Doppler ultrasounds revealed hydrocolpometra, and uterus didelphys, with a normal anus and right kidney agenesis. Conclusions: When encountering female fetuses with ipsilateral renal agenesis or vaginal cysts, clinicians should be aware of OHVIRA syndrome and Gartner duct cysts and perform systematic ultrasonographic examinations for other genitourinary anomalies. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich syndrome is a rare complex congenital disorder, with combined Müllerian and mesonephric duct anomalies, presenting with uterus didelphys, unilateral blind hemivagina and ipsilateral renal agenesis. Hemivaginal obstruction usually leads to impairment of normal menstrual flow, resulting in symptoms after menarche, namely dysmenorrhea, pelvic pain or infertility. Age of presentation depends on the anatomical features of this anomaly. We report a case of a 21-year-old female presenting with few symptoms and incidental findings on transvaginal ultrasound, with typical findings of this disorder on magnetic resonance imaging, which remains the gold standard imaging technique for thorough assessment of Herlyn-Werner-Wunderlich syndrome, allowing for a correct diagnosis and adequate surgical management. Our case also highlights some unusual features, such as the presence of a blind ectopic ureter, with hematic content, and an incomplete septum within the obstructed hemivagina.

We provide a systematic review of articles related to OHVIRA syndrome (Herlyn-Werner-Wunderlich syndrome), found in PubMed/MedLine, Web of Science, Scopus, Scirus, The Cochrane Library and eLIBRARy to show how the urological component of this congenital malformation, previously considered as a concomitant anomaly, has become an important diagnostic marker. Several attempts were made to classify this condition, and they got more complex to include these variants of abnormalities of the kidney and ureter that were found due to advances in imaging technology. As a result, the understanding of the medical community about the OHVIRA syndrome has gradually changed over the past hundred years, and the growing interest of pediatric urologists and surgeons, in turn, has contributed to the early diagnosis of the syndrome and the improvement of examination methods.

Introduction: The o bstructed h emi v agina and i psilateral r enal a nomaly (OHVIRA) syndrome is characterized by the triad uterus didelphys, obstructed hemivagina, and ipsilateral renal dysplasia. To make a radiological diagnosis, knowledge of this syndrome is of paramount importance. Early recognition may prevent complications such as unnecessary surgical procedures, endometriosis, and infections, which could adversely affect fertility. Case report: A 1-day-old female newborn in whom a right-sided cystic kidney abnormality was seen on antenatal ultrasound was admitted with anuria and intralabial mass. Besides the multicystic dysplastic right kidney, ultrasound revealed a uterus didelphys with right-sided uterus dysplasia, an obstructed right hemivagina, and an ectopic ureteric insertion. The diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome with hydrocolpos was made and the hymen was incised. Later, ultrasound helped in diagnosing a pyelonephritis in the afunctional right kidney that was not draining into the bladder (hence no culture could be obtained), requiring intravenous antibiotics and a nephrectomy. Discussion: Obstructed hemivagina and ipsilateral renal anomaly syndrome is an anomaly of the Müllerian and Wolffian ducts of unknown cause. Patients typically present after menarche with (progressive) abdominal pain, dysmenorrhea, or urogenital malformations. In contrast, prepubertal patients can present with urinary incontinence or an (external) vaginal mass. The diagnosis is confirmed by an ultrasound or magnetic resonance imaging. Follow-up includes repeated ultrasounds and monitoring of kidney function. Treatment consists of drainage of the hydrocolpos/hematocolpos; in some cases, further surgery is indicated. Conclusion: Consider obstructed hemivagina and ipsilateral renal anomaly syndrome in girls with genitourinary abnormalities: early recognition prevents complications later in life. [ABSTRACT FROM AUTHOR]

Introduction: We analyzed the frequency, presentation and pitfalls in the diagnosis and surgical management of a large group of normomenstruating adolescents with obstructive reproductive tract anomalies. Material and methods: Retrospective analysis of prospectively collected data. Of the 143 outpatients referred for severe dysmenorrhea and persistent pelvic pain, 42 (29.3%) young women with obstructive Müllerian anomalies and regular menstrual flow were identified. These patients were divided into four groups: (1) patients with duplicate uterine cavities, obstructed hemivagina and ipsilateral renal agenesis (n = 34); (2) patients with unicornuate uterus and noncommunicating cavitated rudimentary horn (n = 5); (3) patients with accessory cavitated uterine mass (n = 2); (4) patients with partially obstructed transverse vaginal septum (n = 1). All 42 patients were conservatively treated via laparoscopy and 35/42 patients had also vaginal surgery. Results: Of the four groups, patients in groups 2 and 3 (n = 7) were conservatively managed by laparoscopy alone; for patients in groups 1 and 4 (n = 35), laparoscopy and the vaginal approach were used. Patients of group 1 were treated by resecting the obstructed vaginal septum with drainage of retained collections. In patients in group 2, surgery consisted of the removal of the rudimentary horn. Patients of group 3 were treated by the removal of myometrial neoformations. In the patient in group 4, treatment consisted of removal of the septum. All surgical procedures were successful and no major complications were recorded. Follow‐up reports highlighted the disappearance of obstruction and clear improvement in pain symptoms. Conclusions: Unilateral obstructive anomalies of the female genital tract are difficult to identify. Early diagnosis allows the preservation of reproductive activity and avoids potential complications. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the Mullerian and Wolffian ductal system, manifesting as a triad of dipelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Patients usually remain asymptomatic till menarche and experience progressive dysmenorrhea, suprapubic lump, and/or features of infection (pyometra, pelvic collection, etc.) afterward. We hereby present a case of a young lady with Herlyn-Werner-Wunderlich syndrome with a large endometriotic cyst, likely arising from the right hemiuterus. She presented with dysmenorrhea and progressive abdominal distention for seven years. She was treated by laparoscopic ovarian cyst excision and right hemihysterectomy that relieved her symptoms. [ABSTRACT FROM AUTHOR]

Rationale: Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemi-vagina with ipsilateral renal agenesis (OHVIRA) syndrome, is a rare congenital anomaly of the Müllerian and Wolffian ducts. It is often asymptomatic until puberty and is usually misdiagnosed as infertility. Patient concerns: A 39-year-old woman desirous of pregnancy was referred to our radiodiagnosis unit for imaging evaluation on account of secondary infertility. Diagnosis: Incidental discovery of uterine bicornis bicollis and blind-ending hemivagina was noted at hysterosalpingography. Interventions: This necessitated the search for other genitourinary tract anomalies, and ipsilateral renal agenesis was also discovered. Outcomes: A diagnosis of Herlyn-Werner-Wunderlich syndrome was made. Lessons: Magnetic resonance imaging (MRI) is considered the gold standard imaging modality for the assessment of uterine malformations. However, in resource-poor centers where MRI is unavailable, a heightened clinical suspicion with the combination of available imaging modalities is important and reliable in making an accurate diagnosis, hence, successful management.

Herlyn–Werner–Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment. [ABSTRACT FROM AUTHOR]

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital anomaly characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Autopsy performed on a third trimester stillborn fetus unexpectedly revealed uterine didelphys, an obstructed left hemivagina, and a left pelvic, atrophic, duplex kidney, with both left ureters entering the obstructed left hemivagina. Furthermore, the fetus had an imperforate anus, a single right umbilical artery, and spina bifida occulta. Although rare, OHVIRA is a well-documented congenital anomaly. However, prior to this case, there had been no description of OHVIRA in an autopsy or in a fetus. Furthermore, the association of OHVIRA, anorectal malformation, and spinal bifida has never been reported. By sharing this case, we hope to increase the awareness of this entity among perinatal healthcare providers and to help further elucidate genitourinary embryology, which is still not fully understood. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital malformation of the urogenital tract involving both the müllerian and the wolffian ducts, and it consists of the association of didelphys uterus, ipsilateral obstructed hemivagina, and ipsilateral kidney agenesis. Its etiology is related to the abnormal development of two embryonic structures—mesonephros and paramesonephros—although its precise mechanism is not known, but theories involving signaling molecules and gene expression are studied for potential explanations. Because of its rarity, there is limited literature on this subject. We present a case diagnosed in our department and elaborate on management. In HWW syndrome, symptoms appear after menarche and include pelvic pain, with progressive intensity due to the developing of hematocolpos. Menstruation may be present or absent depending on the type of anomaly. The use of magnetic resonance imaging (MRI) is the most recommended method of investigation since, in most cases, at this age, sexual life has not started yet and transvaginal ultrasound cannot be used. Surgical treatment in our case consisted of an exploratory laparoscopy followed by a vaginal surgical approach to resect the vaginal septum of the obstructed hemivagina. [ABSTRACT FROM AUTHOR]

We report three cases with Herlyn-Werner-Wunderlich syndrome in adolescents and young female. The objective of this report was to describe the clinical presentation, ultrasound (US) and magnetic resonance imaging (MRI) findings of Herlyn-Werner-Wunderlich syndrome. The three patients were 12, 13 and 34 years old, respectively. The reason for admission and clinical symptoms varied between the 3 patients, including menstrual cramps, vaginal bleeding, and dull pain in the hypogastric region. Nevertheless, the sonographic and MRI findings of all three cases were typical for HWWS, including didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital urogenital anomaly characterized by uterine didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. We present a very rare case of HWWS-associated cervical cancer in which the presence of a genital anomaly was not noticed until the patient experienced postmenopausal vaginal bleeding. A 74-year-old nulliparous Japanese woman presented with vaginal bleeding. Pre-treatment workup revealed uterine didelphys, obstructed hemivagina/hemicervix, renal agenesis, and cancer development from the remnant-obstructed hemivagina/hemicervix. The patient was diagnosed with HWWS and HWWS-associated vaginal or cervical cancer, treated with radical surgery, and a diagnosis of clear cell carcinoma (CCC) of the uterine cervix was histopathologically confirmed. A literature review revealed an increased incidence of CCC in women with HWWS.

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital malformation characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. The obstructed vagina affects menstrual flow, leading to related clinical symptoms after menarche. However, the age of onset, initial symptoms, and clinical complications differ among patients owing to the different types of vaginal septum. Herein, we report 2 cases. The first case is of a 20-year-old woman who presented with fever; she was diagnosed with vaginitis and pelvic inflammation due to the vaginal septum with ostiole. The second case is of a 12-year-old girl who complained of abdominal pain; she was diagnosed as having pelvic inflammation, omentitis, and suppurative appendicitis due to the atretic vaginal septum.