A rare case of OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome: Case report.

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.

Clinical and Imaging Characteristics of Herlyn-Werner-Wunderlich Syndrome: a Comprehensive Analysis.

PURPOSE: To describe the clinical and imaging characteristics of Herlyn-Werner-Wunderlich syndrome (HWWS). METHODS: This study presented an observational case series involving consecutive patients diagnosed with HWWS, whose medical records were retrospectively reviewed. From June 2012 to December 2022, there were a total of 85 patients with HWWS enrolled in our study. We obtained the medical history, including demographic characteristics, clinical presentation, treatment, complications, and radiologic examinations performed. Patients > 18 years of age (n = 58) were recontacted. RESULT: In our analysis, 27 patients were categorised as having complete obstruction, and 58 were categorised as having incomplete obstruction. The mean age at the onset of symptoms and diagnosis of complete obstruction was significantly younger than incomplete obstruction (P < 0.05). For complete obstruction, the median time between menarche and the onset of symptoms was 2.1 years, while for incomplete obstruction, it was 5.3 years. There was a significantly lower incidence of intermittent mucopurulent discharge, irregular vaginal haemorrhage, and occasional examination findings of complete obstruction than incomplete obstruction (P < 0.05). Complete obstruction was significantly associated with dysmenorrhea and pelvic endometriosis compared with incomplete obstruction (P < 0.05). CONCLUSIONS: There are distinct clinical differences between patients with complete obstruction of the hemivagina and those with incomplete obstruction. HWWS can manifest as various combinations of uterine anomalies, communications anomalies, and renal anomalies. Early recognition and treatment can avoid complications and preserve fertility. KEYSWORDS: Herlyn-Werner-Wunderlich syndrome (HWWS); complete obstruction; incomplete obstruction; obstructed hemivagina; congenital malformation.

Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.

Successful Laparoscopic Management of Zinner Syndrome in a Pediatric Patient.

Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.

Surgical Management of OHVIRA and Outcomes.

STUDY OBJECTIVE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) includes uterine didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Surgical management of this condition relies on accurate diagnosis to excise the obstructed longitudinal vaginal septum (OLVS). Vital considerations involve identifying the side affected, ipsilateral renal anomaly (IRA), thickness of the septum (TS), septal axis (SA), and distance of the septum to perineum (DSP). The study aimed to evaluate the preoperative characteristics, imaging findings, and surgical outcomes of OHVIRA. METHODS: Institutional review board approval was obtained for this retrospective chart review. ICD-10 codes identified OHVIRA cases between 2012 and 2019 at a single children's hospital. Patient demographic characteristics, magnetic resonance imaging findings, surgical management, outcomes, and complications were reviewed. Descriptive statistics were utilized. RESULTS: Twenty-six patients met inclusion criteria. Most were diagnosed at puberty (92%). Abdominal pain (50%) was the most common presenting complaint. The mean age of diagnosis was 13.2 years overall and 11.2 years for those with regular cycles vs 13.4 years for those with irregular cycles. Preoperative imaging showed predominant right-sided OLVS (50%), IRA (77%), and oblique SA (65%). All patients underwent vaginoscopy, septum resection, and vaginoplasty, except 1 who was managed with an abdominal drain as a neonate. Four required postoperative vaginal stent or Foley, with DSP greater than 5 cm in all cases. One intraabdominal abscess complication occurred. No instances of hematocolpos re-accumulation or reoperation were observed during the 3-year follow-up period. CONCLUSION: This study demonstrates that detailed preoperative planning and a systematic surgical approach lead to favorable outcomes in OHVIRA irrespective of the OLVS laterality, TS, SA, or DSP.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Obstructed Hemivagina and Renal Anomalies in Patients with and without Anorectal Malformations.

STUDY OBJECTIVE: To compare the anatomic variation between patients with a diagnosis of an obstructed hemivagina with an anorectal malformation (ARM) and those without an ARM. METHODS: This was a retrospective chart review conducted at a single tertiary children's hospital. Patients with an obstructed hemivagina seen from 2004 to 2019 were included. RESULTS: We identified a total of 9 patients diagnosed with an obstructed hemivagina: 4 patients with a history of ARM and 5 patients without an ARM. Patients presented with obstructive symptoms between the ages of 11 and 20. Two-thirds of patients had a left-sided obstruction. All patients without an ARM had ipsilateral congenital anomalies of the kidney and urinary tract. Half the patients with a history of ARM had an ipsilateral renal anomaly, and the other half had a contralateral renal anomaly. CONCLUSION: Obstructed hemivagina occurs in patients with a history of ARM. However, unlike patients with isolated obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), patients with an ARM and an obstructed hemivagina can present with associated renal anomalies on either the ipsilateral or contralateral side. In our small case series, patients with a history of ARM had high septa and required more complex surgical management due to the inability to access the septum vaginally. Knowledge of renal anatomy and ureteral path is important because a hysterectomy may be needed to relieve the obstruction in patients with ARMs. A larger case series is needed to better characterize the spectrum of complex anomalies in patients with ARMs.

Challenges in Diagnosing Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: Case Report of a Rare Condition.

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.

Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Systematic Review about Diagnosis and Surgical Management.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital defect of the Müllerian ducts characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. The aim of this systematic review is to summarize the main symptoms and presentation of the OHVIRA syndrome, as well as the different types of management, fertility, and obstetrical outcomes. A comprehensive search was performed in PubMed, EMBASE, SCOPUS, and Web of Science databases since inception to May 1, 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. After duplicate records removed, the search strategy retrieved 103 articles. The full texts of 73 articles further were assessed for eligibility, and 44 studies were finally included in the systematic review. The mainstay surgical treatment of OHVIRA syndrome is usually a minimally invasive vaginal approach to remove the septum. Ultrasound-guided hysteroscopic resection and laparoscopic resection of the septum have been described as alternatives. Considering the feasibility of minimally invasive approach for the management of the syndrome, laparotomy should be avoided as much as possible and considered only in selected cases.

Obstructed Hemivagina Ipsilateral Renal Agenesis (OHVIRA) Syndrome-A Retrospective Cohort Analysis of the Sabah Population.

OBJECTIVES: The objectives of the study were to compare the demographic characteristics, presenting complaints, timeliness of diagnosis, and treatments given to all patients diagnosed with obstructive hemivagina ipsilateral renal agenesis (OHVIRA) between the adolescent group (age 10-19) and the adult group (age 20 and above) and to propose a feasible screening test consisting of routine bedside ultrasound to detect renal anomalies in addition to pelvic ultrasound during the assessment of females with gynecologic complications in resource-limited settings. METHOD AND ANALYSIS: We conducted a retrospective cohort analysis of all patients with OHVIRA at our institution, Sabah Women and Children's Hospital, Malaysia, between the 2013 and 2022. Data were collected by reviewing patients' medical notes. RESULTS: There were a total of 18 patients diagnosed with OHVIRA from 2013 to 2022, aged 10-41 years old. Nine patients (50%) presented during adolescence. Most (88.9%) came with symptoms such as abdominal pain, urinary symptoms, abnormal uterine bleeding, foul-smelling vaginal discharge, and primary infertility, whereas only 2 patients (11.1%) were asymptomatic and diagnosed incidentally. Acute abdomen was more common in the adolescent group (P = .015). No significant difference was found on the side of the renal anomaly. CONCLUSION: Presenting symptoms vary and are often mimicked other gynecologic or surgical conditions, making the diagnosis difficult and delayed. Some patients were incidentally diagnosed while being managed for other problems, and it was not uncommon to have unnecessary surgery before the correct diagnosis was made. We suggest that all female patients with abdominal and pelvic complaints should be screened for renal anomaly during pelvic scan to improve diagnostic rates.

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature.

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.

Prenatal Diagnosis of Fetal Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome.

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.

Successful management of Herlyn-Werner-Wunderlich syndrome in a 17-year-old virgin girl in the challenging socio-cultural-logistic setting of Syria: a case report.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation: A 17-year-old girl presented to the authors' department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion: The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion: Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.

Vaginoscopic Resection of Oblique Vaginal Septum in OHVIRA Syndrome Before Menarche.

OBJECTIVE: To demonstrate vaginoscopic resection of the oblique vaginal septum in a girl with Obstructed Hemi Vagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome before menarche. DESIGN: Stepwise demonstration of surgical technique with narrated video footage. SETTING: OHVIRA syndrome is a rare urogenital anomaly in which patients present after menarche, with progressive dysmenorrhea and a palpable pelvic mass due to hematocolpos and hematometra on the obstructed side. Delay in diagnosis may cause endometriosis, pelvic adhesions, and infertility [1,2]. A 12-year-old, premenarchal girl with complaints of pelvic pain and an ultrasound report of right renal agenesis was referred to the urology department of our hospital. She was also found to have uterus didelphys and a loculated fluid collection behind the urinary bladder on ultrasonography and on magnetic resonance imaging (Figs. 1 and 2). A probable diagnosis of OHVIRA syndrome with mucocolpos was made [3]. INTERVENTION: Vaginoscopy showed an obstructing bulging vaginal septum on the right side. The left uterine horn was inspected by hysteroscopy. The vaginal septum was incised vaginoscopically with monopolar cautery using Collins knife (Video still 1) [4-6]. The collected mucus was drained. The right cervix and the right uterine horn were visualized by hysteroscopy. Edges of the septum were resected with a resectoscope loop (Video still 2). The hymen was not injured during the surgery. Relook vaginoscopy done after 2 months showed a normal and healed vagina. CONCLUSION: Vaginoscopic resection of the obstructing oblique vaginal septum in OHVIRA syndrome is a very simple, minimally invasive, and virginity conserving surgery. Early diagnosis and treatment can prevent future complications due to cryptomenorrhea.

Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases.

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.

Successful vaginoscopic excision of the vaginal septum in a virgin girl of obstructed hemivagina and ipsilateral renal anomaly: Case report and review of literature.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare malformation that not only causes severe menstrual cramps shortly after menarche but can also lead to endometriosis and infection in the future. We report a case of OHVIRA successfully managed by vaginoscopic excision of the vaginal septum. A 12-year-old virgin girl presented to our hospital with dysmenorrhea and lower abdominal pain. OHVIRA was diagnosed using magnetic resonance imaging. Vaginoscopic surgery was performed for drainage of hematocolpos and excision of the vaginal septum. Vaginoscopic excision of the vaginal septum was performed using a resectoscope, without a vaginal speculum. The procedure was completed safely without injuring the hymen. This is the first case report of successful excision of the vaginal septum by vaginoscopic surgery for OHVIRA in Japan. Vaginoscopic excision may be one of the effective options for the treatment of vaginal obstruction.

Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management.

Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions.

Adolescent abdominal pain due to rare mullerian duct anomaly.

Abdominal pain is a common presenting complaint to the Emergency Department (ED). Often, rare etiologies can be discovered in the work up of this common complaint. Here we present the case of an adolescent female who presented with abdominal pain and was found to have obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) or Herlyn-Werner-Wunderlich Syndrome. A 12 year old female with known renal agenesis presented with 5 days of left sided abdominal pain that then developed into right lower quadrant pain. She had regular menses for the last 2 years. Ultrasound (US) showed a fluid collection in the lower uterine segment and a complex cystic structure anterior to the uterus. Magnetic resonance imaging (MRI) showed the patient to have didelphys uterus with "severe dilatation of the cervix/vaginal canal… extending from the right uterine horn" and left-sided ovarian and Fallopian tube torsion. She was taken to the operating room where she underwent vaginal septum excision and a left salpingo-oopherectomy. OHVIRA includes the triad of obstructed hemivagina, uterine didelphys, and ipsilateral renal agenesis. This occurs due to embryologic arrest of the mullerian and mesonephric ducts at 8 weeks of gestation. Most abnormalities are right sided which leads to right lower abdominal and pelvic pain approximately 4 months post-menarche. Diagnosis of OHVIRA is made utilizing US and CT scans. MRI can also be useful to further delineate specific anatomy. It is important for the emergency physician to be aware of this entity as most patients don't present to care until acute, severe symptoms develop. This makes it more likely for them to seek care in the ED as opposed to the outpatient setting.

Conservative Treatment of Didelphyc Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis.

STUDY OBJECTIVE: To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina. DESIGN: Stepwise demonstration of the technique with narrated video footage. SETTING: In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis. INTERVENTIONS: A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen. CONCLUSION: Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction.