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1.
Krešimir Živković Matija Prka Nikica Živković Ana Bucko Dubravko Habek 《Archives of gynecology and obstetrics》2014,290(5):855-858
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. 相似文献2.
《Journal of pediatric and adolescent gynecology》2020,33(6):748-751
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. 相似文献
3.
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. 相似文献
4.
5.
Jessica Y. Shim Frances W. Grimstad Marc R. Laufer 《Journal of pediatric and adolescent gynecology》2021,34(2):220-222
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. 相似文献
6.
《Journal of pediatric and adolescent gynecology》2014,27(2):104-106
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. 相似文献
7.
Kristl Tomlin Craig Barnes Kathy Van Leeuwen Amy Williamson 《Journal of pediatric and adolescent gynecology》2018,31(1):67-70
Background
Congenital atresia of the cervix in the setting of an obstructed hemivagina with ipsilateral renal anomaly (OHVIRA) is an unusual occurrence that is frequently missed using preoperative imaging modalities.Case
A 14-year-old female adolescent presented to a tertiary care facility with abdominal pain associated with a mass. Initial imaging with ultrasound and magnetic resonance suggested OHVIRA but 3-D magnetic resonance imaging (MRI) also correctly identified unilateral cervical atresia on the obstructed left side.Summary and Conclusion
Three-dimensional MRI in the setting of OHVIRA was used successfully in this case to preoperatively identify cervical atresia and to guide preoperative surgical counseling and planning. The use of 3-D MRI for this purpose has, to our knowledge, never been described in the literature. Three-dimensional printed models derived from 3-D MRI can play an evolving role in the management of Müllerian anomalies as preoperative planning and surgical counseling tools. 相似文献8.
《Journal of pediatric and adolescent gynecology》2022,35(1):94-97
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. 相似文献
9.
Sloane Berger-Chen Jessica M.B. Ritch Jin Hee Kim John Evanko Terry W. Hensle 《Journal of pediatric and adolescent gynecology》2012,25(6):e129-e131
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. 相似文献
10.
《Journal of pediatric and adolescent gynecology》2014,27(6):375-378
Study ObjectiveTo compare a case series of the obstructed hemivagina, ipsilateral renal anomaly and uterine didelphys triad with the literature, with a focus on a subset of patients with cervical aplasia.Design, Setting, and ParticipantsA retrospective case series was conducted of all patients with the triad managed between 2005-2013 at a tertiary center for adolescent gynecology.ResultsSimilarities in this cohort, compared to the literature, included heterogeneity of presentation, presence of endometriosis, and asymmetry of affected side. Notable differences included 1 patient with a 16p11.2 microdeletion and 2 patients with subsequent unilateral cervical aplasia. All patients underwent magnetic resonance imaging for diagnosis. Vaginal septum division was performed in 8 cases and excision in 1 case. Both cases with cervical aplasia ultimately underwent hemi-hysterectomy, and highlight the implications of this rare variant in regards to its existence, limitations of magnetic resonance imaging in this context, and suggestions for improvement in diagnosis and management.ConclusionThe complexity of these cases, especially the evolving manifestation of cervical aplasia postoperatively, illustrates the need to recognize limitations in imaging and divergence in definitive management. 相似文献
11.
《Journal of pediatric and adolescent gynecology》2016,29(3):e53-e55
BackgroundObstructed hemivagina and ipsilateral renal anomaly syndrome is a Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomalies.CaseA 12-year-old girl with a history of right renal agenesis presented to the emergency department with abdominal pain, dysuria, and urinary retention. Imaging identified a uterine didelphys with a large obstructed right hemivagina compressing the left ureter, causing hydronephrosis. She underwent vaginal septum resection for curative treatment.Summary and ConclusionIn female patients who present with abdominal pain and a history of renal abnormalities, obstructed hemivagina and ipsilateral renal anomaly syndrome must be considered in the differential diagnosis. This consideration is important in preventing complications such as hydronephrosis seen in this patient. 相似文献
12.
Mohamed A.F.M. Youssef 《Middle East Fertility Society Journal》2013,18(1):58-61
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. 相似文献
13.
《Taiwanese journal of obstetrics & gynecology》2020,59(6):948-951
ObjectiveHerlyn–Werner–Wünderlich (HWW) syndrome is a rare condition in which patients present with a palpable pelvic mass and pain caused by an obstructed hemivagina. Here we present a case of HWW syndrome characterized by prolonged menstrual bleeding.Case reportA 19-year-old nonsexually active unmarried women experienced irregular menstrual cycles and menorrhagia. The duration of menstrual bleeding was 10–14 days. She also suffered from mild dysmenorrhea since menarche at the age of 13. Transabdominal sonography revealed a double uterus and a heterogeneous myoma-mimicking mass over the left cervical region. The left kidney was absent. Magnetic resonance imaging revealed a double uterus, a double vagina with an unperforated left hemivagina, and ipsilateral renal agenesis. The patient underwent cervicovaginal orifice reconstruction surgery.ConclusionLeft hematocolpos compression, a partially obstructed right vaginal channel, and an orifice with local venous drainage abnormalities resulted in prolonged menstrual bleeding. In HWW syndrome, the occurrence of a pelvic mass and pain is common; however, prolonged menstrual bleeding is rare. 相似文献
14.
Valerie I. Shavell Samantha E. Montgomery Samuel C. Johnson Michael P. Diamond Jay M. Berman 《Archives of gynecology and obstetrics》2009,280(3):449-452
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. 相似文献
15.
《Journal of pediatric and adolescent gynecology》2022,35(3):387-390
BackgroundObstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported.CaseA 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair.ConclusionDelineation of reproductive anomalies in female patients with anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with a solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations, which, if not well managed in time, can be detrimental to fertility. 相似文献
16.
Elke Stevens Jan Baekelandt Lutgarde Lemmens Els Dufraimont André Valcke Marcel De Bruyn Jan Bosteels 《Gynecological surgery》2010,7(3):279-283
Didelphic uterus with blind hemivagina and ipsilateral renal anomaly is a rare congenital malformation. The pathogenesis of
this syndrome and its embryologic origin are still the subject of discussion. Due to the variable clinical picture and low
index of suspicion, diagnosis is often delayed or inaccurate. Conservative surgical treatment—excision of the obstructing
vaginal septum and marsupialization of the blind hemivagina—is considered the most appropriate treatment. This report describes
the case of a 16-year-old girl with didelphic uterus and double vagina, with obstruction of the left hemivagina. The patient
was also diagnosed with left renal dysplasia and ipsilateral ureter communication with the obstructed vagina. Following various
examinations and left uretero-nephrectomy, the patient was effectively treated with resection of the vaginal septum. 相似文献
17.
Ureteral obstruction caused by endometriosis is uncommon. It is, however, an important complication that imposes a 25% chance for permanent loss of renal function on the affected side. The standard management is surgical; however, three cases have been reported in which regression of obstruction followed medical therapy. This case report concerns a patient with long-standing partial ureteric obstruction due to endometriosis who was treated for 2 months with danazol. Clinical response of the endometriosis was excellent, but the obstruction persisted, a retroperitoneal ureteroneocystotomy was therefore performed. The ureter was found to be obstructed by dense fibrous tissue that contained endometrial glands. It seems therefore that a trial of danazol may be attempted in selected cases, but that the drug is unlikely to relieve endometriotic ureteric obstruction once dense fibrosis has occurred. 相似文献
18.
《Journal of pediatric and adolescent gynecology》2014,27(4):e79-e81
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. 相似文献
19.
Carmine Noviello Mercedes Romano Fabiano Nino Ascanio Martino Giovanni Cobellis 《Gynecological endocrinology》2018,34(1):56-58
Objective: The authors present their experience in the management of pediatric patients with Herlyn–Werner–Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation.Methods: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices).Results: Six patients were treated in the study period. The mean age was 9?years (2?months–15?years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18?months all patients were symptoms free.Conclusions: Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary. 相似文献
20.
Pentti K. Heinonen 《Archives of gynecology and obstetrics》2013,287(5):975-978