Diagnosis approach, treatment, evaluation and fertility preservation in patient with Herlyn-Werner-Wunderlich syndrome: a case report

Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare variant of Mullerian duct anomalies. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis, recently known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome. The most common presentation is abdominal and pelvic pain, dysmenorrhea, and abdominal mass secondary to hematocolpos, hematosalping or hematometra.Objective: to determine the diagnosis and operative management of HWW syndrome case with pelvic pain as the chief complaint. Method: case examination and tracing medical records at Dr Sardjito Hospital Yogyakarta.Results and Discussion: Fourteen-year-old female, presented with pelvic pain. Physical examination revealed a cystic mass in the abdomen inferior, palpable up to 3 cm inferior to the umbilicus. From the Intravenous Pyelography (IVP) examination, it was found normal anatomy and function of the right kidney, but there was no left kidney. On a contrast-enhanced abdominal CT scan, magnetic resonance imaging (MRI) and laparoscopy showing a complete duplication of the uterus from the horn to the cervix with no connection between the two uterine cavities, both ovaries were normal, the right fallopian tube was normal, the left tube was enlarged, attached to the uterus and the left ovary, no left kidney was found. Operative management of the vaginal septectomy procedure was performed.Conclusion: MRI is most accurate for providing details regarding the altered anatomy and for identifying associated hematocolpos,hematosalping or hematometra for HWW syndrome cases. Surgical intervention by vaginal septectomy is performed to relieve symptoms, provide better reproductive and sexual functions.Keywords: Herlyn-Werner-Wunderlich syndrome; Mullerian disgenesis; pelvic pain.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single septate uterus: first case report

Herlyn-Werner-Wunderlich (HWW) syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare Müllerian duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Early diagnosis is essential to prevent complications and preserve reproductive potential.We report the case of a 14-year-old female patient with significant dysmenorrhea and menstrual irregularities since menarche. The initial pelvic ultrasound was unremarkable. Nine months later, she presented repeatedly to the emergency department with left flank pain and vomiting. Further evaluation revealed the absence of the left kidney, uterus didelphys with an obstructed left hemi-uterus, hematocolpos, hematosalpinx, left ovarian endometrioma, and left renal agenesis on magnetic resonance imaging (MRI). Surgical management included laparoscopic hemihysterectomy of the left uterus and ipsilateral salpingectomy. Postoperatively, the patient was started on continuous progestogen-only therapy, with marked improvement in symptoms and quality of life.HWWsyndrome typically manifests soon after menarche with cyclic pelvic pain, dysmenorrhea refractory to treatment, or pelvic mass. MRI is the gold standard for diagnosis, allowing precise anatomical characterization and guiding surgical planning. Without a functional or communicating obstructed hemicavity, hemihysterectomy with salpingectomy is an effective approach, preventing recurrence and reducing the risk of complications such as infection and endometriosis.This case highlights the importance of maintaining a high index of suspicion for HWW syndrome in adolescents with refractory dysmenorrhea and associated renal anomalies. Early diagnosis and individualized surgical management can relieve symptoms, improve quality of life, and preserve reproductive potential.

Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome – one clinical center case series and the systematic review of 734 cases

The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital anomaly of the Mullerian (paramesonephric) and Wolffian (mesonephric) ducts. It was initially reported in 1922 by Purslow and also referred to as the HerlynWerner-Wunderlich syndrome. It typically occurs concurrently with uterine didelphys and ipsilateral renal agenesis (1-5). Ipsilateral renal agenesis can be accompanied with other urologic anomalies simultaneously. A blind ectopic ureter combined with ipsilateral agenesis and Mullerian duct anomalies is extremely rare (6, 7). To our knowledge, a blind ectopic ureter associated with OHVIRA syndrome has not been reported in English literature. The diagnosis of ureteral anomalies can be challenging due to their rare incidence and cystic features that can be mistaken for an adnexal lesion in female patients. Regarding this matter, MRI is considerably helpful not only for diagnosing Mullerian duct anomalies, but also for detecting and characterizing associated non-uterine anomalies (1-5). In this case report, we present an unusual case of a 13-year-old girl with OHVIRA syndrome and a blind ectopic megaureter, which was This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Received: July 19, 2015 Revised: August 19, 2015 Accepted: August 27, 2015

Introduction: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare Mullerian duct anomaly with uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. In most cases, Patients who rarely present this structural anomaly, start to experience sympotms such as chronic pelvic pain and mass sensation that usually swap that star wirh menarche and variate with the menstrual cycle. Objective: this is an original article which aims to describe the wide range of symptoms presented by patients with (OHVIRA) syndrome, how their quality of life is affected due to underdiagnosis, and how 2D and 3D ultrasound is an important tool for the diagnosis of an adequate surgical technique. Matherials and methods: narrative describes the case of 2 adolescent patients who were incidentally diagnosed with the syndrome in the city of Cali Colombia at the imbanaco Clinic. information was obtained from their respective clinical histories, and ultrasound images and the respective surgical techniques used for the management of the symptoms were also extracted. the informed consent of both patients was obtained. Results: The diagnosis is usually late or incidental because the clinical presentation only becomes evident once menarche occurs. As the clinical and structural presentation of the pathology comprises a wide spectrum, therefore the diagnosis today is made by means of 2D and 3D ultrasonography, diagnostic imaging such as computed axial tomography and magnetic resonance imaging. the management will depend on the structural anomaly and the intensity of the symptoms. Today surgical method are usually the treatment of choice. Conclusion: OHVIRA syndrome is a rare congenital anomaly that represents a diagnostic challenge because of the regular menstruation and nonspecific clinical presentation . Health care providers should be aware of the different extending symptoms in order to employ different diagnose images such as 2D and 3D ultrasonography operated by highly trained staff (sensibility >90 % y specificity 100% to facilitate early and accurate management and improve patients quality life.

Congenital Adrenal Hyperplasia and Ohvira Syndrome: First Report of Unique Combination in a Child

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital Müllerian anomaly typically characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. However, variants involving atypical uterine configurations and vaginal septa may occur, complicating diagnosis and management. We report the case of a 14-year-old adolescent girl who presented with recurrent lower abdominal pain a few months after menarche. Clinical evaluation revealed no palpable mass, but pelvic ultrasound showed the absence of the right kidney and two separate uterine cavities. MRI confirmed the presence of a bicorporeal uterus with two endometrial cavities and two cervical canals, a longitudinal non-obstructing vaginal septum, and a transverse obstructing vaginal septum. The obstruction resulted in hemato-trechalos and bilateral hematometra. The anomaly was classified as U3C2V1 under the ESHRE/ESGE classification. The patient underwent exploratory laparotomy with resection of the transverse vaginal septum and drainage of retained blood. Postoperative recovery was uneventful, and the patient was counselled regarding reproductive implications. This case represents a rare and complex variant of OHVIRA syndrome. Dual vaginal septa are uncommon, and their presence, along with bicorporeal uterus, underscores the diagnostic complexity. MRI proved invaluable in delineating anatomy and guiding management. Literature supports early diagnosis and timely surgical correction to prevent complications like endometriosis and infertility. This case highlights the importance of considering Müllerian anomalies in adolescents with post-menarche cyclical pain. MRI facilitates accurate diagnosis, and early surgical intervention can optimize clinical and reproductive outcomes.

The obstructed hemivagina and ipsilateral renal anomaly (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. 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. 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. Consider obstructed hemivagina and ipsilateral renal anomaly syndrome in girls with genitourinary abnormalities: early recognition prevents complications later in life.

Adolescent abdominal pain due to rare mullerian duct anomaly

The authors report the first case of prenatal fetal MRI diagnosis of unilateral hydrocolpos, uterus didelphys, and ipsilateral renal agenesis in a case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. A 33-year-old woman was referred to the present institution because of a fetal pelvic cyst. Detailed ultrasound revealed a female fetus with a retrovesical cyst and right renal agenesis. Fetal MRI confirmed unilateral hydrocolpos, uterus didelphys, and normal intestinal tract of the fetus. Fetal MRI is a useful modality for prenatal diagnosis of OHVIRA as a method of evaluating the pelvic structures and excluding other cloacal anomalies.

Clinical characteristics of 415 patients with obstructed hemivagina and ipsilateral renal anomaly syndrome: A nationwide multicenter retrospective study.

PII: S0015-0282(07)01456-2

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a complex Mullerian and Wolffian duct anomaly, which is difficult to diagnose before puberty. We present a rare case of a congenital syndrome known as OHVIRA in a 21-year-old female who came with complaints of intermittent type of lower abdominal pain, dysmenorrhea, and oligomenorrhea with frequent visits to different hospitals without any radiological investigations done. Early magnetic resonance imaging (MRI) investigations helped her in diagnosing and managing this syndrome.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.