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.     

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.     

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.     

La endometriosis profunda: un desafío diagnóstico y terapéutico

We report a case that illustrates the challenges of deep infiltrating endometriosis of the rectovaginal septum. The patient underwent a total hysterectomy in our department in May, 2011 due to symptomatic uterine leiomyomas, with no clinical or histopathologic findings of endometriosis. She continued to experience incapacitating pelvic pain, dyschezia and urinary symptoms until February 2012, when she developed ureteral obstruction, bilateral hydronephrosis and intestinal pseudo-obstruction that required radical resection of a fibrous pelvic mass, adhering to the rectum and compressing both ureters. The histological diagnosis was endometriosis. We emphasize the diagnostic difficulties of this entity and the aggressive treatment required.     

Diagnosis of endometriosis

Endometriosis is a common disorder of women of reproductive age, yet diagnosis of this condition is often problematic. The most frequent clinical presentations of endometriosis include dysmenorrhea, pelvic pain, dyspareunia, infertility, and pelvic mass. However, the correlation between these symptoms and the stage of endometriosis is poor. Currently available laboratory markers are of limited value. At present, the best marker, serum CA-125, is usually elevated only in advanced stages and therefore not suitable for routine screening. Transvaginal ultrasound and magnetic resonance imaging are often helpful, particularly in detection of endometriotic cysts. Recently, transrectal ultrasound and magnetic resonance imaging were shown to be valuable in detection of deep infiltrating lesions, especially in the rectovaginal septum. Although direct assessment of endometriotic foci at laparoscopy may be viewed as a "gold standard" for identifying endometriosis, the correlation of laparoscopic observations with histological findings is often low. Ultimately, diagnosis of endometriosis requires a careful clinical evaluation in combination with judicious use and critical interpretation of laboratory tests, imaging techniques, and, in most instances, surgical staging combined with histological examination of excised lesions.     

Laparoscopic en bloc resection of a para-cervical cancer with OHVIRA syndrome

Objective

There are some reports of cervical cancer with uterus didelphys but a case of clear cell carcinoma (CCCC) with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome is extremely rare. The aim of this paper was to report a case of CCCC with OHVIRA syndrome and the difficulty in making a preoperative diagnosis.

Image-Guided Transvaginal and Transabdominal Drain Placement for Surgical Navigation in a Patient with Tubo-Ovarian Abscess and Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome

BackgroundWe describe a minimally invasive, image-guided technique for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome complicated by tubo-ovarian abscess (TOA).CaseA 15-year-old female adolescent with OHVIRA syndrome presented with TOA. Magnetic resonance imaging of the abdomen/pelvis showed a loculated pelvic mass and fluid collection near the obstructed left hemivagina. Tissue quality and ill-defined surgical planes prevented surgical excision. A transabdominal drain was placed via laparoscopic port by Interventional Radiology. She later underwent transvaginal and transabdominal drain placement into the hemivaginal collection using computed tomography and ultrasound guidance, allowing for surgical excision of the vaginal septum, drainage and excision of the TOA, and neosalpingostomy.Summary and ConclusionBecause of the anatomic complexity in OHVIRA syndrome, preoperative minimally invasive techniques with Interventional Radiology collaboration can assist intraoperative anatomic navigation for successful surgical treatment.     

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.     

Ovarian remnant syndrome

Ovarian remnant syndrome (ORS) refers to a condition occurring in women who have had a bilateral salpingo-oophorectomy (BSO), with or without a hysterectomy, that leaves behind ovarian tissue. This residual ovarian tissue then results in pelvic pain or a pelvic mass. Risk factors associated with incomplete removal of an ovary and subsequent development of ORS include a history of endometriosis, pelvic inflammatory disease, multiple previous surgeries, and pelvic adhesive disease. Patients most frequently present with chronic pelvic pain, pelvic pain associated with a pelvic mass, or an asymptomatic pelvic mass. Definitive criteria for diagnosis of ORS include a history of BSO with histologic documentation of ovarian tissue obtained during subsequent surgical excision. The recommended treatment for ORS is surgical excision by laparotomy or, more recently, laparoscopy. We present the presentation and management of patients with ORS and a review of the published literature.     

Rectovesical ligament and fusion defect of the uterus with or without obstructed hemivagina and ipsilateral renal agenesis

Objective

The rectovesical ligament is a peritoneal band in women with failure of fusion of the two Müllerian ducts. The aim was to evaluate existence of this abnormal structure in women with dysfused uterus and its possible relations to concomitant vaginal and renal anomalies.

Study design

The study group comprised 47 women with uterine fusion defect (37 didelphic and 10 bicornuate uterus). They had undergone laparoscopy or laparotomy to visualize the pelvic cavity, and imaging for renal evaluation. The rectovesical ligament was present if inspection of the pelvic cavity revealed a broad peritoneal band between the two hemiuteri, attached anteriorly to the bladder and posteriorly to the sigmoid. Presence or absence of the ligament was reported, and concomitant renal and vaginal anomalies were evaluated.

Results

The rectovesical ligament was not visualized in 14 patients with didelphic or complete bicornuate uterus associated with unilateral renal agenesis: of these 13 had a previously treated obstructive longitudinal vaginal septum. A peritoneal band was found in 27 women with didelphic uterus with longitudinal vaginal septum with no obstruction and normal bilateral kidneys. Six women with bicornuate uterus had normal kidneys and an identified rectovesical ligament between the uterine hemicorpora, except for one with partial bicornuate uterus.

Conclusion

The rectovesical ligament is not merely a consequence of the failed fusion of two Müllerian ducts, but its relation to uterine malformation with or without vaginal and renal anomalies indicates some share of this structure in the early development of the urogenital system.     

Síndrome OHVIRA (hemivagina obstruida y anomalía renal ipsilateral) asociado a útero didelfo

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity that usually presents after menarche with dysmenorrhea and cyclic pelvic pain due to hemihematocolpos. Although ultrasound scanning allows correct diagnosis, magnetic resonance imaging plays a decisive role in characterizing the malformation. Early diagnosis is important so that prompt excision of the vaginal septum can relive pain and prevent further complications.     

Urinary Incontinence in Puberty: A Rare Clinical Presentation of the Herlyn-Werner-Wunderlich Syndrome

BackgroundWe report on an unusual presentation of Herlyn-Werner-Wunderlich syndrome in two 11-year-old girls within a year of menarche. The setting was a training and research hospital.CaseWe present two patients in the pubertal period with cyclic abdominal pain and urinary incontinence who received hysteroscopic septal resection. Menstrual flow was resumed and the complaints of incontinence were eliminated after the hysteroscopic resection of the vaginal septum.Summary and ConclusionOverflow incontinence was completely resolved after septum resection in two patients. The risk of stricture is high in Herlyn-Werner-Wunderlich syndrome if the septum is partially excised to open the obstruction, whereas the risk of stricture is low if a complete or wide excision is performed. In the presence of abdominal pain and urinary incontinence in puberty, Herlyn-Werner-Wunderlich syndrome must be considered in the differential diagnosis, and a detailed evaluation of the urinary system and pelvic anatomy must be performed.     

Anterior abdominal wall parasitic leiomyoma: case report

The leiomyomas are a common gynecologic entity that may present unusual growth patterns or unusual locations. Its atypical presentations creates a diagnostic challenge. This is a case report of a parasitic leiomyoma located in the anterior abdominal wall in a 53?years old woman with pelvic compressive and urinary symptoms, with no history of any gynecological surgery. This case illustrates the diagnostic difficulties and describes the complementary images used in the preoperative evaluation.     

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.     

Primary Amenorrhea with an Abdominal Mass at the Umbilicus

BackgroundTransverse vaginal septum is a rare cause of primary amenorrhea. It has a reported incidence of 1:2,100–1:72,000 and a variety of clinical presentations.CaseA 16-year-old patient presented with primary amenorrhea and a large abdominal mass that was palpated on clinical exam and confirmed on MRI imaging. A diagnosis of transverse vaginal septum was ultimately made and the patient underwent a successful surgical excision of the septum and vaginal reconstruction.ConclusionThe presentation, etiology, diagnosis, and surgical treatment of a transverse vaginal septum are discussed.     

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.     

Isolated tubal torsion at menarche- a case report

Background: Adnexal torsion is a well-recognized cause of acute pelvic pain. Isolated tubal torsion with ovarian sparing has certainly been documented, but is uncommon. Although risk factors for the latter include a menstrual period, menarche in particular is not known to predispose a patient to this event. Severe unilateral pelvic pain with first menses is more likely to herald a congenital mullerian anomaly and cryptomenorrhea, particularly when accompanied by a pelvic mass. We present a case of tubal torsion where a coincidental, yet misleading temporal relation to menarche led to a delay in laparoscopy and ultimate diagnosis.Case: KG, an eleven-year-old female, experienced severe right-sided dysmenorrhea with her first and second menses in August and September 1999 respectively. Between episodes, pain, although still present, was more tolerable and the patient never required hospitalization. Ultrasound revealed a lobulated inhomogeneous mass posterior to the uterus and extending from one normal ovary to the other (Figures). MRI further described the mass as pseudoencapsulated with inhomogeneous areas of high attenuation on T1 and T2 images (Figures). Findings were consistent with an endometrioma, but admittedly could have represented a hemorrhagic cystic mass. No definite mullerian anomaly was seen to explain advanced endometriosis, but two focal areas within the endometrial canal raised the possibility of a uterine septum. Examination of the patient (one week after presentation) was not very helpful although she was pubertal, did have a hymenal septum and was mildly tender on bimanual examination in the Pouch of Douglas. The patient had been started on continuous oral contraceptives while undergoing investigations. Pain only recurred during an episode of break-through bleeding. Ultimately she came to laparoscopy and hysteroscopy where chronic right tubal torsion and necrosis was identified with an inflammatory/hemorrhagic reaction in the pelvis (Photos). There were no identifiable fimbria of the right tube which was densely adherent distally to perirectal fat (Photo). No obvious precipitant was found. Laparoscopic lysis of adhesions and right distal salpingectomy was performed (Photo). Her uterine cavity was in fact normal (Photo)Conclusion: Whether or not this patient's right tube was originally normal will never be known. Congenital abnormalities of fallopian tubes do occur and can predispose to torsion. Nonetheless, adnexal torsion must always be kept in mind whenever a woman presents with unilateral pelvic pain. Early diagnosis is paramount in children and women of reproductive age in order to improve the likelihood of adnexal salvage and future fertility. A "gold-standard" radiological investigative tool continues to elude us. Laparoscopy, albeit more invasive, remains an invaluable procedure in this context with relatively low morbidity as compared to the consequences of delayed diagnosis.     

Acute Abdominal Pain as a Result of a Ruptured Hematosalpinx: A Rare Complication of an Unusual Müllerian Anomaly

Case ReportA 15-year-old virgin Caucasian female presented to the emergency room with a 40-hour history of acute left lower quadrant abdominal pain and nausea. Evaluation suggested a left pelvic kidney with obstructed ureter being the etiology. Her pain continued to escalate so further workup with laparoscopy was performed. This demonstrated a left pelvic sidewall hemi uterus with ruptured hematosalpinx. This is an unusual clinical presentation of a müllerian anomaly not previously documented.DiscussionThe differential diagnosis of acute unilateral abdominal pain in adolescent females should include müllerian anomalies. The incidence of this diagnosis is low but the evaluation and treatment can be performed in an expeditious manner if the diagnosis is considered. The laparoscopic excision of a unilateral noncommunicating uterine horn is a valid and recommended treatment approach of this rare malformation.     

Torsion of the fallopian tube and acute pelvic pain: an unusual presentation of genital tuberculosis

A case of a genital tuberculosis found during laparoscopic evaluation for recurrent acute pelvic pain is presented. The symptoms of genital tuberculosis can vary greatly, and a laparoscopic approach is effective for making the diagnosis. Clinicians should consider tuberculosis as a differential diagnosis when encountering clinical presentations of pelvic pain and ascites.