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.     

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.     

Pregnancies in women with uterine malformation, treated obstruction of hemivagina and ipsilateral renal agenesis

Purpose

The aim of this study was to evaluate the outcome of pregnancies in women who had uterine malformation and surgically treated obstructed hemivagina with ipsilateral renal agenesis.

Methods

The study group comprised 21 women with malformed uterus (12 didelphic, 6 septate and 3 bicornuate uterus). All of them had a history of surgical excision of the longitudinal vaginal septum caused obstructed hemivagina and ipsilateral renal agenesis. All pregnancies and possible surgical interventions were evaluated during the follow-up period (median 13.2 years).

Results

Thirteen out of 21 women attempting pregnancy conceived. They produced 22 pregnancies, 17 (77 %) were contralateral to the treated obstructed hemivagina and unilateral renal agenesis. The median interval between surgical treatment of obstructed hemivagina and the first pregnancy was 10.5 years. Twenty (91 %) pregnancies ended in delivery of a living infant. Preeclampsia (14 %), preterm delivery (36 %), high frequency (38 %) of fetal breech presentation and the cesarean section rate (67 %) were found.

Conclusions

Accurate diagnosis and appropriate surgery to open an obstructed hemivagina in adolescence assure fertility. Preterm birth is associated with malformed uterus and unilateral renal agenesis may predispose to preeclampsia.     

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.     

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.     

Proposal of the 3O (Obstruction,Ureteric Orifice,and Outcome) Subclassification System Associated with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA)

Study ObjectiveTo propose a “3O” (obstruction, ureteric orifice, and outcome) subclassification system associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA).DesignRetrospective case series.SettingXiangya Hospital, Central South University, Changsha, Hunan, China.ParticipantsA total of 26 women with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) over a 9-year period.InterventionsNot applicable.Main Outcome MeasuresIn all cases, the obstruction, ureteric orifice, outcome and surgical strategy were reviewed.ResultsIn our study, the “obstruction” category included 14 cases of blind hemivagina, 8 cases of buttonhole septum, 3 cases of cervical fistula, and 1 case of cervical atresia. A total of 25 patients with vaginal obstruction underwent resection of the vaginal septum. The patient with cervical atresia underwent a failed cervicoplasty, followed by hemi-hysterectomy. The “ureteric orifice” category included 24 cases of absent ureter with no orifice, as well as 2 cases of ureteric orifice emptying into the obstructed hemivagina. The 2 patients were treated with laparoscopic extirpation of the ectopic ureter and renal moiety. Regarding the “outcome” category, 5 patients with severe recurrent hematometra, hematosalpinx, and ovarian endometrioma underwent hemi-hysterectomy, salpingectomy, and cystectomy of the ovarian endometrioma. Both patients (1 with a septate uterus and 1 with a bicornuate uterus) who experienced recurrent abortion accepted uterine correction.ConclusionWe provide new insights into the anatomical variants of this rare syndrome with the relevant surgical implications. Magnetic resonance imaging is the most useful tool in 3O diagnosis.     

Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report

Background

Herlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature.

Case presentation

A 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. She suffered from threats of abortion in the first trimester, recurrent urinary tract infections during all pregnancy. At 33 weeks + 5 days of gestational age, she was hospitalized for premature rupture of the membranes and uterine contractions and a caesarean section was performed because of breech presentation. Postpartum period was complicated by a pelvic abscess resolved with parental antibiotic therapies.

Conclusions

Our literature review shows an unusual aspect in our case: HWWS is not classically associated with skeletal anomalies. Moreover, the most frequent urogenital side affected is the right, not left side as in this woman. Preterm spontaneous rupture of membranes and fetal abnormal presentation represent frequent complications and probably post-caesarean infections are related to pregnancies in the context of this syndrome.

     

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.     

Hydronephrosis: A Rare Presentation of Uterine Didelphys with Obstructed Hemivagina and Ipsilateral Renal Anomaly

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.     

Three-Dimensional Technology to Diagnose Unilateral Cervical Atresia in Obstructive Hemivagina with Ipsilateral Renal Anomaly: A Case Report and Review of the Literature

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.

Diagnostic challenges of hemihematocolpos and dysmenorrhea in adolescents: obstructed hemivagina, didelphys or bicornuate uterus and renal aplasia is a rare female genital malformation

Objectives

To develop a clear diagnostic and therapeutic strategy for adolescents presenting with abdominal pain and vaginal tumor caused by congenital female genital anomalies, such as blind hemivagina and uterine anomalies, as the lack of the correct diagnosis of the underlying anatomical genitourinary malformation frequently leads to destructive surgical procedures.

Methods

Retrospective study, study group: patients with double/bicornuate uterus, blind hemivagina and hematocolpos (n?=?13), controls: patients with uterine malformation and complete vertical vaginal septum (n?=?11), analysis for: menarche, age at onset of symptoms, type of malformation, symptoms leading to admission and diagnostic/surgical techniques applied.

Results

Median age at diagnosis study group 19.85 (SD?±?6.23, range 13?C23?years) versus controls 26.09?years (SD?±?7.44, 16?C36?years); predominance of imperforated hemivagina: 69.2?% right-sided versus 30.8?% left-sided septum; renal agenesis ipsilateral to imperforate hemivagina 100?% study group versus 9.1?% controls; 84.6?% previous surgical interventions in the study group, such as partial removal of the septum and re-obliteration, unilateral salpingo-ovarectomy and vaginal drainage of pyometra. We used a single transvaginal surgical procedure, including removal of the obstructed vaginal septum and marsupialization of the blind hemivagina.

Conclusions

A diagnostic and therapeutic algorithm for young women presenting with progressive dysmenorrhea and abdominal pain and/or vaginal tumor reduces destructive interventions.     

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.     

Obstructive Müllerian Anomalies in Menstruating Adolescent Girls: A Report of 22 Cases

Study Objective

To assess the clinical course of obstructive Müllerian anomalies found in girls after menarche.

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.     

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.     

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.     

A case of didelphic uterus and blind hemivagina with renal dysplasia and ectopic ureter presenting with vulvodynia and recurrent fever

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.     

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.