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.     

Conservative treatment of a Herlyn-Werner-Wunderlich müllerian anomaly variant, noncommunicating hemiuterus with Gartner duct pseudocyst

Asymmetric obstructed uterus didelphys (Herlyn-Werner-Wunderlich syndrome) is a rare congenital müllerian anomaly consisting of uterus didelphys, hemivaginal septum, and ipsilateral renal agenesis. Herein is reported a case of incomplete Herlyn-Werner-Wunderlich syndrome diagnosed using 3-dimensional transvaginal ultrasound in a 14-year-old patient with absence of the hemivaginal septum. The most contributive diagnostic factors and appropriate therapeutic management in such cases are discussed.     

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.     

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.     

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.     

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.     

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.     

Laparoscopic management of pregnancy in a patient with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis

Pregnancy in a rudimentary uterine horn is a rare and potentially lethal condition. The highest risk of rupture is reported to be during the late first and second trimester. The risk of rupture correlates with the thickness of the myometrium surrounding the fetal pole. In 2005, a 20-year-old woman was incompletely diagnosed by imaging studies and laparoscopy to have an absent right kidney, a bicornate uterus with a right rudimentary uterine horn and a single cervix, a transverse vaginal septum with hematocolpos, and endometriosis caused by reflux menstruation. The transverse vaginal septum was excised, and the surgeon observed a single cervix. Oral contraceptives were prescribed as complementary treatment for the endometriosis and associated dysmenorrhea. In 2009, magnetic resonance imaging confirmed resolution of hematocolpos and revealed a right cervix connected to the right horn of a uterus didelphys and covered by a partial longitudinal vaginal septum. The patient had a contraception failure and presented in 2010 at 9(6/7) weeks' gestation. By ultrasonography and subsequent magnetic resonance imaging, the pregnancy was in the right uterus and the corpus luteum was on the left ovary. The myometrium was thinned to 2 to 3 mm atop the gestational sac. Using the Harmonic ACE, laparoscopic excision of the right fallopian tube and a supracervical right hysterectomy with an intact pregnancy was performed. This case supports the Acién hypothesis that the vagina forms from both Müllerian and Wolffian duct elements, and it illustrates the risk for uterine rupture when pregnancy forms in a rudimentary structure; presumed transperitoneal migration of an ovum that was captured by the opposite fallopian tube; and?surgical management of the in situ pregnancy by laparoscopic supracervical excision of the rudimentary uterine body.     

Herlyn-Werner-Wunderlich syndrome: 3D ultrasonographic diagnosis in premenarche

IntroductionUterine malformations are a heterogeneous group of congenital anomalies resulting from abnormal development of Mullerian ducts. Herlyn-Werner-Wunderlich syndrome is a rare condition that includes the triad of uterus didelfus, blind hemivagina and ipsilateral renal agenesis. We report a case with premenarche diagnosis and treatment and make a review of the illness.Case reportA premenarche 11 year old female was referred for pediatric nephrology and neurodevelopment consultation due to prenatal diagnosis of left renal agenesis. A pelvic ultrasound was performed and an anomalous uterus and a cystic pelvic mass was incidentally discovered. A 3D trans-rectal ultrasound identified a bicolis didelfus uterus and mucocolpos. A unidigital vaginal exam revealed a single cervix with soft bulging of the left apical vaginal wall. Using monopolar knife, an incision was done allowing drainage of the cavity. A part of the apical vaginal septum was removed and a normal cervix was seen.CommentsA high degree of clinical suspicion is important for the diagnoses. 3D ultrasound or MRI are the imaging diagnostic exams of choice, helped by a careful bimanual pelvic examination. An early diagnosis is important to prevent chronic pelvic pain and perhaps severe endometriosis.     

Rectosigmoid deep infiltrating endometriosis and ureteral involvement with loss of renal function

Endometriosis is a complex disease with unclear pathogenesis, defined as the presence of endometrial tissue (glands and stroma) outside its usual location in the uterine cavity. Ureteral involvement is rare, with an estimated frequency of 10-14% in cases of deep endometriosis with nodules of 3 cm or larger. An important complication of ureteral involvement is asymptomatic loss of renal function. In a patient with asymptomatic renal failure the relevance of extrinsic ureteral involvement by deep endometriosis has been taken to account. CASE REPORT: A 32-year-old nulliparous woman presented with chronic pelvic pain associated with severe dysmenorrhea, dyspareunia and digestive problems including diarrhea, occasional constipation and rectal bleeding. She reported no urological symptoms. Magnetic resonance imaging (MRI) identified a 4 cm nodule in the recto-vaginal septum, compressing and infiltrating the rectal wall, and chronic left hydronephrosis. Isotope renogram revealed 91% function in the right kidney and 9% in the left kidney. A multidisciplinary surgical team including consultants from the departments of digestive surgery and urology assessed the patient. The treatment recommended was a joint approach of laparoscopic surgery to perform adhesiolysis, ureterolysis, freeing of the uterus and appendages, resection of the rectovaginal septum nodule, and left nephrectomy. COMMENT: Diagnosis and treatment of deep endometriosis should be performed in specialized centers and in the context of multidisciplinary collaboration. We must be aware of the potential risk of ureteral involvement and the asymptomatic loss of renal function in any patient with endometriosis nodules of 3 cm or larger.     

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.     

Double uterus with an obstructed hemivagina and ipsilateral renal agenesis: report of 5 cases and a review of the literature.

Maldevelopment of the Müllerian duct system can result in various uterine, vaginal, and renal abnormalities. Complete or partial absence of Müllerian duct fusion and/or canalization results in a septate, bicornuate or didelphys uterus with various types of hemivaginal obstruction. In addition, unilateral renal agenesis is a common association. Five patients with uterine anomalies and an obstructed hemivagina are described according to the complete or incomplete obstruction between the double vagina and uterus. Early and accurate diagnosis is important, but difficult, due to the variable clinical pictures. Sonographic evaluation can provide valuable diagnostic information. Early diagnosis and excision of the obstructed vaginal septum can completely relieve the symptoms and prevent further sequelae.     

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.     

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.     

Duplicated uterus and hemivaginal or hemicervical atresia with ipsilateral renal agenesis: an institutional clinical series of 52 cases

Objectives

To retrospectively review cases of unilateral vaginal or cervical atresia with ipsilateral renal agenesis at our institution and to analyze the clinical presentation, diagnostic pitfalls, management, and embryological implications for the vaginal origin that arise from this syndrome.

Study design

A retrospective observational study that included 52 patients diagnosed with this syndrome between 1998 and 2008 at Peking Union Medical College Hospital.

Results

The median age at diagnosis was 21.5 years, and the median time between the first onset of symptoms and diagnosis was 12 months. The most common presenting complaints were dysmenorrhea, purulent discharge and irregular spotting, despite the wide spectrum of symptoms at referral. Patients with and without a communication between the two hemivaginas or hemiuteri had different clinical characteristics. Of the patients, 59.6% had an obstruction on the right side. Of patients who had received a check-up prior to referral, 92.9% (n = 28) had been misdiagnosed, and 53.9% had received inappropriate surgery as therapy. The pathology of the resected septum showed squamous epithelium in 13 samples, while 5 samples had epithelium with paramesonephric characteristics.

Conclusion

Knowledge of the origins and clinical presentation of this syndrome is the foundation for correct and timely diagnosis and treatment. Moreover, this unique anomaly may offer essential clues for determining the embryological origins of the vagina and cervix.     

In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series

Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare congenital malformation of the female urogenital track characterized by a triad consisting of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. We report 5 consecutive cases of patients diagnosed with HWWs treated in our center. Imaging studies with 2-dimensional/3-dimensional ultrasound and abdominopelvic magnetic resonance imaging were obtained to confirm the diagnosis. The treatment consisted of a 1-step surgical in-office hysteroscopic incision of the vaginal septum using 5 Fr hysteroscopic bipolar electrodes. At the follow-up visit, between 1 and 2 months after the initial procedure, the patients underwent a diagnostic vaginoscopy with excision of exceeding vaginal tissue if needed, performed with a vaginal hysteroscopic approach. In-office hysteroscopic treatment of patients diagnosed with HWWs is a safe and effective, minimally invasive treatment modality that provides symptomatic relief and preserves fertility, avoiding the cost and risks of the use of general anesthesia in an operating room setting. We recommend shifting the management of this challenging condition to the office setting.     

Uterus Didelphys, a Rare Cause for Tubal Sterilization Failure

EDITORIAL COMMENT: : We accepted this case report for publication because uterus didelphys and its often-associated longitudinal vaginal septum can cause a variety of complications in the practice of obstetrics and gynaecology. When performing tubal ligation the surgeon would search for the second Fallopian tube and presumably could, as suggested in this paper, misdiagnose one half of the didelphys as a unicomuate uterus. The fold of peritoneum between bladder and sigmoid colon could obscure the other uterine horn. When the editor was a resident with Lance Townsend at The Royal Women's Hospital, Melbourne, in the 1950's, when abortion by the medical profession was not readily available, we had to cope with a patient with an unwanted pregnancy in one horn of her uterus didelphys, a condition unknown to her doctors when they had inserted a Grafenberg ring into the other horn for contraception. The vaginal septum can lead to diagnosis by causing dyspareunia and obstructed labour. The editor has also diagnosed the condition when performing a Manchester repair in a postmenopausal woman. In this case it was not until the cervix was amputated that the laterally-displaced vaginal septum and the second cervix it had hidden from view, was first recognized. This woman had used one half of her vaginal and uterine complement for coitus, conception and delivery of her children without symptoms due to extreme lateral displacement of the vaginal septum. See also Editorial Comment. Uterus didelphys with unilateral imperforate vagina and ipsilateral renal agensis. Aust NZ J Obstet Gynaecol 1991; 31: 288.
Summary: Uterus didelphys is a rare congenital anomaly. It can result in a variety of misadventures even in the hands of an expert. The present case reports uterus didelphys as the cause for tubal sterilization failure. This is the first case of this type seen in our institution in 35 years.     

阴道斜隔综合征的诊断与治疗

阴道斜隔综合征是双宫体、双宫颈、双阴道,一侧阴道完全或不完全闭锁的先天性畸形,多伴有闭锁阴道侧的泌尿系统畸形,以肾脏缺如多见。超声检查简便、无创是诊断阴道斜隔综合征的首选检查。磁共振成像(MRI)具有良好的组织分辨力及多参数、多方位成像功能,影像学表现更具特征性。手术是治疗阴道斜隔综合征唯一有效的方法。目前最根本的手术方式是阴道斜隔切除术。手术要点在于确定阴道斜隔部位和斜隔孔。手术时机以月经期为宜。阴道斜隔要切除充分,并保证引流通畅,防止阴道切口粘连、狭窄。宫腹腔镜联合手术不仅可以明确阴道斜隔综合征的诊断与分型,也可同时准确的治疗。阴道斜隔侧子宫尚有妊娠可能,应尽量避免切除,以提高患者的受孕能力。     

Septate uterus with a double cervix and longitudinal vaginal septum

OBJECTIVE: To report the clinical features of 9 cases and review the previously reported 8 cases of an uncommon müllerian anomaly, characterized by the presence of a complete uterine septum with cervical duplication and represent a a longitudinal vaginal septum. STUDY DESIGN: Retrospective clinical study. RESULTS: Nine patients aged 21-32 presented to our clinic with complaints of dyspareunia, dysmenorrhea, and primary or secondary infertility. All 9 patients displayed a single uterus with smooth fundal contour, double cervix and longitudinal vaginal septum. Some additional abnormal findings were associated with these anomalies in some patients: polycystic ovaries in 3 cases and different degrees of endometriosis in 2 cases. Most of the patients had normal tubal patency. CONCLUSION: According to a MEDLINE search, only 8 cases of septate uterus with cervical duplication and a longitudinal vaginal septum have been reported to date. We report 9 more cases, which suggest that this atypical septate uterus type is probably more frequent than reported. These cases support the bidirectional müllerian theory and may imply the presence of another type of mullerian anomaly.     

Obstructed Hemivagina with Ipsilateral Renal Anomaly

The association of obstructed hemivagina with ipisilateral renal anomaly (OHVIRA) is a well-described entity. While there has been an increased familiarity with this disorder, the exact incidence of OHVIRA is unknown. Our aim was to review the available literature on this topic, look at common presentations, and uncommon presentations. This condition is a well-described entity but requires careful evaluation, because unique presentations do occur. Diagnostic challenges include time of presentation and symptoms associated with presentation. Surgical challenges include microperforation of the hemivaginal septum, pelvic inflammatory disease, thick septum, or high septum. Each of these must be managed carefully. Although a solitary kidney is frequently thought to be associated with OHVIRA, dysplastic kidneys, pelvic kidneys, or ectopic ureters can occur. Prompt and accurate diagnosis is essential for relief of symptoms and prevention of complications. To minimize problems associated with delayed diagnosis, magnetic resonance imaging evaluation is recommended along with referral to a center with expertise in these conditions.