Complex genital malformation: ectopic ureter ending in a supposed mesonephric duct in a woman with renal agenesis and ipsilateral blind hemivagina

We present a clinical case of a patient with left renal agenesis and ipsilateral blind hemivagina who also had one or more atretic ectopic ureters opening into supposed mesonephric duct, which in turn opened into, or joined onto, the ipsilateral hemicervix, continuing with the blind hemivagina. The diagram of this complex genitourinary malformation would strongly support our hypothesis of the embryology of the human vagina as deriving from the Wolffian ducts and the Mullerian tubercle.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

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 uterus didelphys presenting in the retroperitoneum

We describe a case of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Magnetic resonance imaging (MRI) demonstrated uterus didelphys and laparoscopy revealed that an affected uterus was present in the retroperitoneum.     

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.     

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.     

The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis

Two patients are presented with a uterus didelphys with an obstructed hemivagina and an ipsilateral renal agenesis. Short remarks are made about the embryologic relationships between uterus and kidneys and the necessity therefore of examining the genital system when there is an anomaly in the renal system and vice versa. The therapy is excision and marsupialization of the vaginal septum.     

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.     

Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: report of three cases

Gynecologic and urinary congenital malformations are often associated and mostly diagnosed in teenagers. To update a 116-case international case registry, three cases of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis, detected in young girls, are reported. Diagnostic procedures and surgical treatment are discussed.     

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.     

Prenatal management of inherited urogenital malformation: case report

Documentation of unique kidney renal function early in pregnancy can be helpful in defining prenatal management and therefore in improving prognosis. Antenatal diagnosis of a solitary kidney was performed at 20 weeks' gestation in a foetus with a 1,7 chromosome translocation. Because of the decreasing renal function and the increasing pelvic dilatation, an early in utero stenting was placed at 23 weeks' gestation. Optimal outcome occurred and the baby was delivered at 32 weeks. Complete assessment of the malformation showed a left hydronephrosis due to a megaureter, right renal agenesis with ipsilateral cryprorchidism and agenesis of the right vas deferens. The chromosomic translocation was inherited from the mother who was affected by uterus didelphys, obstructed right hemivagina and right renal agenesis. Renal function of the unique kidney with hydronephrosis can be early diagnosed and promptly treated. This condition should also increase the index of suspicion of underlying genital and chromosomal anomalies.     

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.