OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation

BackgroundObstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly with uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with pelvic pain and/or a mass and rarely, in later years, with primary infertility. Strong suspicion and knowledge of this anomaly are essential for a precise diagnosis.CaseA 14-year-old female presented with acute retention of urine and was diagnosed as a case of OHVIRA syndrome with uterus didelphys. Acute retention of urine as the initial clinical presentation has been rarely reported in this syndrome. She was treated with hemivaginal septal resection.Summary and ConclusionOHVIRA syndrome should be considered among the differential diagnoses in young females with renal anomalies presenting with pelvic mass, symptoms of acute abdomen, and acute urinary retention.     

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

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis usually presents after menarche with progressive abdominal pain during menses secondary to hematocolpos. Initially, the anomaly remains unrecognized, while patients most frequently referred to surgeons for assistance. The method of choice for diagnosis is magnetic resonance imaging. A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical treatment as well as decreased long-term morbidity. Transvaginal excision of the septum is the appropriate mode of treatment.     

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

Study ObjectiveTo show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina.DesignStepwise demonstration of the technique with narrated video footage.SettingIn 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.InterventionsA 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.ConclusionHere, 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.     

OHVIRA with a Twist: Obstructed Hemivagina Ipsilateral Renal Anomaly with Urogenital Sinus in 2 Patients

BackgroundOHVIRA syndrome is a rare diagnosis involving the triad of obstructed hemivagina, uterine anomaly, and ipsilateral renal anomaly. OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system.CasesA 14-year-old female with known left renal agenesis, long-standing urinary incontinence, and history of recurrent urinary tract infections presented with abdominal distention.A 4-year-old female with known right renal agenesis and urinary incontinence was found to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left.Summary and ConclusionIt is important to identify the presence of a urogenital sinus in the OHVIRA setting, as surgical management for these patients may be affected. In both cases, the urogenital sinus was preserved as the vaginal opening.     

Unusual case of OHVIRA syndrome with a single uterus,unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina

Purpose

The acronym for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) was created to describe patients with an obstructed hemivagina and ipsilateral renal anomaly and enables inclusion of other uterine anomalies except uterus didelphys. The main goal of this article is to present a rare case of OHVIRA syndrome with intrapartal rupture of obstructed hemivagina.

Methods

We present an unusual case of OHVIRA syndrome with single uterus, uterine septum (previously resected by hysteroscopy) and renal agenesis, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina.

Results and conclusion

Various symptoms, included in OHVIRA acronym, are a result of different morphologic variants included in this syndrome. Most likely, in our case, the absence of communication between the cervical canal/patent vagina and the obstructed hemivagina prevented formation of mucocolpos or hematocolpos. This rare clinical variant made our MRI diagnosis of obstructed hemivagina, as well of the entire OHVIRA syndrome, ineffective. We did not find OHVIRA cases in the literature with a single septate uterus and single cervix, associated with obstructed hemivagina diagnosed during and after labor and presented with intrapartal rupture of hemivagina. Because of lack of guidelines, the final decision about management of the labor in such rare cases of OHVIRA syndrome is difficult.     

Síndrome de Herlyn-Werner-Wunderlich

Uterus didelphys with obstructed hemivagina frequently associated with ipsilateral renal agenesis is a rare congenital anomaly known as Herlyn-Werner-Wunderlich syndrome (HWW). We report the case of a 31-year-old woman with no relevant medical history, with scant, intermittent and ill-smelling metrorrhagia for the last 2-3 months. Intraoperative vaginal exploration revealed a hypotrophic cervix and the external orifice of a fistula communicating with the right uterine cavity and draining a dark hematic fluid. Intravenous urography showed right renal agenesis and confirmed the suspected diagnosis of HWW.     

Delayed Diagnosis of Herlyn-Werner-Wunderlich Syndrome due to Microperforation and Pyocolpos in Obstructed Vaginal Canal

BackgroundTo present a rare anomaly consisting of uterus didelphys, longitudinal vaginal septum, obstructed hemivagina with pyocolpos, fistula to the open vaginal canal, and ipsilateral renal agenesis, referred as Herlyn-Werner-Wunderlich syndrome (HWWS).CaseA 14-year-old girl with recurring purulent vaginal discharge lasting for a few months. Preoperative examination revealed one vaginal canal with one cervical opening on the right side. There was a fistula leading from the obstructed vaginal canal to the left vagina. Intravaginal ultrasound examination demonstrated a longitudinal vaginal septum and a closed pyocolpos on the right side. The longitudinal vaginal septum was excised by way of electrocauterization under direct vision.ConclusionHWWS should be considered in the differential diagnosis in patients with uterus didelphys and unusual symptoms such as pyocolpos and vaginal discharge.     

The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad

The triad of obstructed hemivagina, renal anomaly and uterus didelphys is an uncommon cause of abdominal pain and menstrual discomfort in adolescent girls. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Adolescent girls presenting with these symptoms should have a baseline pelvic ultrasound scan to establish uterine anatomy. In those with ultrasound findings of Müllerian anomalies appropriate follow up would include a magnetic resonance imaging, preferably at a tertiary centre with expertise in interpretation of Müllerian anatomy, as well as early consultation and referral to a centre with experience in the management of these rare conditions. Appropriate surgery would be a single stage procedure to either excise or completely divide the obstructing septum. There is currently no consensus on concurrent laparoscopy. We present a case series of four patients with the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis managed between 2005–2009 at a tertiary centre for paediatric and adolescent gynaecology.     

Hemivaginal Septum Resection in a Patient With a Rare Variant of Herlyn-Werner-Wunderlich Syndrome

Herlyn-Werner-Wunderlich syndrome is characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The syndrome should be suspected in any young woman with abdominal pain or cyclic dysmenorrhea, and radiologic evidence of müllerian duct and renal anomalies. Herein is presented the case of a 25-year-old woman with a rare variant of Herlyn-Werner-Wunderlich syndrome, characterized by an anomalous connection between the 2 endocervical canals, who underwent hemivaginal septum resection to relieve progressively worsening dysmenorrhea. The right hemivaginal septum was resected medially from the left cervix and laterally off the right vaginal wall. Hydrodissection between the hemivaginal septum and right cervix facilitated surgical resection. After resection of the hemivaginal septum there was complete resolution of dysmenorrhea and no recurrence of hematometra or hematocolpos.     

Complete septate uterus,obstructed hemivagina,and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly

Background  Abnormalities of the female reproductive tract are associated with a multitude of intrapartum complications. In the case that follows, we report the complicated pregnancy course of a woman diagnosed postpartum with the uncommon syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) associated with a complete septate uterus. Case  A 19-year-old nulliparous woman with a history of a right nephrectomy secondary to multicystic dysplastic kidney disease presented with premature rupture of membranes at 30 weeks’ gestation. After failed induction of labor at 34 weeks’ gestation, a uterine anomaly was discovered at the time of cesarean section. Magnetic resonance imaging performed postpartum revealed a complete septate uterus, longitudinal vaginal septum, and obstructed right hemivagina. Conclusion  The OHVIRA syndrome is classically associated with uterus didelphys, not complete septate uterus as in this woman. This case illustrates the potential for intrapartum complications in women with abnormalities of the urogenital tract.     

Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience.

OBJECTIVE: To present the experience of a large referral center with patients with the rare but specific syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. DESIGN, PATIENTS: Between 1953 and 1991, 15 patients with the syndrome were evaluated by the authors; each patient's records were carefully reviewed. MAIN OUTCOME MEASURES: For each patient, the clinical course, specific anatomic findings, treatment(s) offered, and obstetrical outcome are presented. RESULTS: The specificity of the association of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis was confirmed by our series, the largest in the literature to date. The findings suggest a specific development anomaly of the müllerian ductal system, probably secondary to a wolffian duct anomaly. The most common clinical presentation was that of the onset of pelvic pain and/or dysmenorrhea shortly after menarche, in association with the finding of a vaginal/pelvic mass. When a communication between the two sides existed, symptoms of abnormal vaginal discharge and bleeding were common. Optimal outcome appears to have occurred for patients initially managed using a conservative surgical approach, i.e., simple vaginal septum resection combined with conservative treatment of associated endometriosis. Obstetrical outcome in our patients was similar to that in patients with the more common combination of uterus didelphys and sagittal vaginal septum. CONCLUSION: A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity.     

Herlyn-Werner-Wunderlich Syndrome-Timely Diagnosis is Important to Preserve Fertility

BackgroundHerlyn-Werner-Wunderlich syndrome is an urogenital malformation with uterus didelphys and obstructed hemivagina with ipsilateral renal agenesis. Most of these patients present after the onset of menstruation. We describe two cases diagnosed too late to prevent the complications.CaseThe first patient presented with acute abdomen one year after the onset of menstruation and had salpingectomy due to pyosalpinx. The blind hemivagina was not recognized and she had severe endometriosis. She underwent hysterectomy 8 years later. The second patient presented with foul smelling vaginal discharge when she was 21 years old. She had a simple vaginal septum resection.Summary and ConclusionIn the presence of uterine cavities in a regularly menstruating girl with dysmenorrhea, the presence of both kidneys should be checked. When unilateral renal agenesis and uterus didelphys coexist the first thing that we should remember is to confirm or refute the presence of a blind vagina.     

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. A case report

BACKGROUND: Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis usually presents after menarche with progressive abdominal pain during menses secondary to hematocolpos. We describe a case with the unique presentation of rectal pain and constipation. CASE: A 13-year-old girl presented to the emergency department complaining of lower abdominal and rectal pain and constipation of two weeks' duration. Pelvic ultrasound, physical examination and laparoscopic findings established a diagnosis of hematometracolpos secondary to uterus didelphys with unilateral imperforate hemivagina. An incision in the vaginal septum allowed drainage of the hematocolpos, providing relief of the patient's symptoms. CONCLUSION: Uterus didelphys with unilateral imperforate hemivagina and ipsilateral renal agenesis may present with apparent gastrointestinal symptoms. With increased awareness of this problem, timely diagnosis may be achieved.     

Laparoscopic Strassman Metroplasty in a Postmenarcheal Adolescent Girl With Herlyn-Werner-Wunderlich Müllerian Anomaly Variant,Obstructed Noncommunicating Didelphic Uterus Without Gartner Duct Pseudocyst

Asymmetric obstructed uterus didelphys (Herlyn-Werner-Wunderlich syndrome), also known as obstructed hemivagina with ipsilateral renal agenesis syndrome, is a rare congenital müllerian duct anomaly. Herein we present a case report of incomplete Herlyn-Werner-Wunderlich syndrome, with absence of the hemivaginal septum, diagnosed in a 12-year-old girl. Treatment of the severe pain using an analgesic agent was ineffective. Therefore, laparoscopic metroplastic surgery via the modified Strassman procedure was performed. After surgery, the patient no longer reported dysmenorrhea.     

Spontaneous Cervicovaginal Fistula in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report

BackgroundObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina.CaseWe present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.     

Obstructed hemivagina and ipsilateral renal anomaly syndrome with uterus didelphys (OHVIRA)

To report a case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome) with uterus didelphys that has been diagnosed successfully with ultrasound and managed with a single stage vaginoplasty.     

Unique Diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) at the Time of First-Trimester Ultrasound Imaging

BackgroundObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is usually diagnose during adolescence due to pain-related symptoms; however, this syndrome can go unrecognized for several years, and unique presentations may occur later in life.CaseWe describe a 24-year-old-woman diagnosed with previously unknown OHVIRA syndrome and a 7-week intrauterine pregnancy in the obstructed side by the unique ultrasound image obtained during routine first-trimester ultrasonography. The patient was managed with single-stage vaginoplasty, and the rest of the pregnancy were uneventful.Summary and ConclusionThis report is unique in terms of showing that the pregnancy could develop in the uterine cavity on the side of the obstruction despite the blind hemivagina.     

An Unusual Presentation of Uterine Didelphys and Obstructed Hemivagina

BackgroundCongenital disorders of the genitourinary system can be unpredictable. There are both medical and surgical interventions that can help manage symptoms.CaseA 10-year-old girl was evaluated 6 months post-menarche with dysuria and pelvic pain. Her symptoms were not relieved with menstrual suppression. Evaluation revealed a probable ectopic ureter versus ureterocele and uterine didelphys with suspicion for obstructed hemivagina and hematocolpos. Surgical exploration revealed an obstructed hemivagina with a high vaginal septum. When resection failed, she ultimately underwent a robot-assisted hemihysterectomy, with resolution of her symptoms.Summary and ConclusionsIn this case presentation, a surgical approach was necessary to adequately make a diagnosis. When vaginal septum resection failed, robot-assisted laparoscopic hemi-hysterectomy and resection of a vaginal pouch led to symptom resolution in this pediatric patient with a complex anomaly.     

Diagnosis and gestational follow-up in a patient with Herlyn-Werner-Wünderlich syndrome,a case report

ObjectiveHerlyn-Werner-Wünderlich syndrome (HWW) is a rare congenital malformation of the urogenital tract due to a fusion failure in the Müllerian ducts. This anomaly consists of a didelphus uterus with obstructed hemivagina and sometimes associated with ipsilateral renal agenesis. The treatment of choice is surgical, it consists of a simple procedure of resection of the vaginal septum and drainage of the obstructed hemivagina and retained collections.Case reportWe report the case of a pregnancy in a 37-year-old woman with SHWW without resection of the vaginal septum.ConclusionsThe early detection is important due to the possible associated complications. Women with uterine defects are subject to an increased risk of complications in pregnancy and childbirth. Therefore, each case must be treated individually.