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.     

Management of Lower Vaginal Agenesis in a Patient with Unicornuate Uterus

BackgroundAbsence of the lower vagina occurs from abnormal development of the sinovaginal bulbs and vaginal plate.CaseWe present a case of an adolescent girl, with a history of VACTERL and VATER status post flap vaginoplasty at 8 m of age and correction of vaginal-urethral fistula, with primary amenorrhea and cyclic pelvic pain. MRI showed a right hematocolpos. On exam the obstructed vagina was deviated to the right. Given prior urologic and rectal surgery, and laterality of unicornuate system, intra-operative ultrasonography (US) was performed. A needle was used to enter the inferior aspect of the obstructed vagina. A pull-through vaginoplasty was performed.Summary and ConclusionVaginal pull-through is the standard treatment for lower vaginal agenesis. In a unicornuate system, the use of intra-operative US is helpful to determine the appropriate angle of dissection.     

Uterus Didelphys Associated with Unilateral Cervical Atresia and Renal Agenesis

A case is reported of uterus didelphys associated with unilateral cervical atresia and renal agenesis on the same side. Two successful pregnancies occurred in the affected side after surgical correction.     

Surgical correction of vaginal anomalies

Summary of accurate diagnosis and surgical management of vaginal anomalies. Imperforate hymen, transverse septa, and distal vaginal agenesis present similarly with pain and hematocolpos. Likewise, imperforate hymen, transverse septa, distal vaginal agenesis, and Müllerian aplasia may appear similarly on examination. Imaging should be used to better differentiate the anomaly. Although surgical correction is the mainstay of hymenal anomalies, septa, obstructed hemivaginas, and distal vaginal agenesis, first-line therapy for Müllerian aplasia is progressive dilation. Many surgical techniques are also described but no consensus exists as to the best one. Clinicians should be aware of the appropriate evaluation, differential diagnosis, and management options available for vaginal anomalies.     

Clinical implications of the didelphic uterus: long-term follow-up of 49 cases

OBJECTIVE: The aim of the study was to evaluate reproductive performance of women with didelphic uterus and to consider possible long-term consequences associated with this uterine anomaly. STUDY DESIGN: Forty-nine women were diagnosed as having a didelphic uterus with a longitudinal vaginal septum at Tampere University Hospital, Finland between 1962 and 1998. The presence of other anomalies, gynecologic disorders, fertility and outcome of pregnancies were reviewed. The long-term clinical implications associated with a didelphic uterus were evaluated during the mean (S.D.) follow-up period of 9.1 (6.3) years. RESULTS: An obstructed hemivagina was found in nine (18%) out of forty-nine cases; eight of these had ipsilateral renal agenesis. A longitudinal vaginal septum was excised in twenty-six (53%) cases, but metroplasty in none. Five (13%) patients had primary infertility. Thirty-four (94%) out of thirty-six women who wanted to conceive had at least one pregnancy, and they produced seventy-one pregnancies; 21% miscarried, and ectopic pregnancy occurred in 2%. The fetal survival rate was 75%, prematurity 24%, fetal growth retardation 11%, perinatal mortality 5. 3%, and cesarean section rate 84%. Pregnancy located more commonly (76%) in the right uterus than in the left. During the follow-up period endometriosis was observed in seven (16%) out of forty-five cases. Ovarian neoplasm was found in four (9%) cases, one of them had ovarian cancer. CONCLUSIONS: Fertility in women with didelphic uterus is not notably impaired. The prognosis of pregnancy is comparatively good, while prematurity and fetal growth retardation indicate meticulous prenatal care. Long-term follow-up did not reveal that didelphic uterus is associated with increased frequency of endometriosis or genital neoplasm.     

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.     

Herlyn–Werner–Wünderlich syndrome: An unusual case with presentation of menorrhagia

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.     

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.     

Unilateral Non-communicating Cervical Atresia in a Patient with Uterus Didelphys and Unilateral Renal Agenesis

A 16-year-old adolescent girl presented with chronic pelvic pain. Pelvic ultrasound and MRI showing a uterus didelphys, normal left uterus and cervix, right pelvic fluid collection and right unilateral renal agenesis. After two unsuccessful vaginal surgeries for drainage of hematotrachelos and creation of an outflow tract, patient underwent unilateral total abdominal hysterectomy with final pathology confirming hematotrachelos and non-communicating cervical atresia on the right. Patient on postoperative follow-up doing well.     

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.     

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.     

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.     

Term delivery after in vitro fertilization in a patient with cloacal malformation

BackgroundCloacal malformation is extremely rare, occurring in approximately 1 in 25 000 births. It frequently has associated Müllerian anomalies that require surgical correction.CaseWe describe here a patient with cloacal malformation, solitary kidney, bilateral fallopian tube obstruction, and didelphic uterus who required in vitro fertilization to conceive.ConclusionCareful surveillance resulted in an excellent pregnancy outcome with term delivery.     

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.     

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.     

Hemivaginal Septum Resection in a Patient With a Rare Variant of Herlyn-Werner-Wunderlich Syndrome

Herlyn-Werner-Wunderlich syndrome is characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The syndrome should be suspected in any young woman with abdominal pain or cyclic dysmenorrhea, and radiologic evidence of müllerian duct and renal anomalies. Herein is presented the case of a 25-year-old woman with a rare variant of Herlyn-Werner-Wunderlich syndrome, characterized by an anomalous connection between the 2 endocervical canals, who underwent hemivaginal septum resection to relieve progressively worsening dysmenorrhea. The right hemivaginal septum was resected medially from the left cervix and laterally off the right vaginal wall. Hydrodissection between the hemivaginal septum and right cervix facilitated surgical resection. After resection of the hemivaginal septum there was complete resolution of dysmenorrhea and no recurrence of hematometra or hematocolpos.     

An Unusual Presentation of Uterine Didelphys and Obstructed Hemivagina

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.     

Resectoscopic treatment of uterus didelphys with unilateral imperforate vagina complicated by hematocolpos and hematometra: case report.

OBJECTIVE: To describe a technique for treating hematocolpos and hematometra in patients with uterus didelphys and unilateral imperforate vagina involving the use of resectoscopy under ultrasonographic control. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A 13-year-old girl with uterus didelphys with unilateral hematometra, hematocolpos, and ipsilateral renal agenesis. The girl complained of severe abdominal pain, which appeared with each of her menses. INTERVENTION(S): The intervention was performed by a vaginoscopic approach to preserve the integrity of the hymen. The first incision on the vaginal wall was performed in correspondence with the hematocolpos under continuous ultrasonographic guidance with the use of a straight resectoscopic loop. Resection of the vaginal septum was continued with the use of an angled resectoscopic loop until almost complete excision of the septum was achieved. MAIN OUTCOME MEASURE(S): Clinical, echographic, and vaginoscopic findings before the operation and 2 and 6 months after the operation. RESULT(S): The surgical procedure was easy to perform. Almost complete excision of the septum was achieved with just a few passages of the resectoscope. Complete drainage of both the hematocolpos and the hematometra was confirmed by ultrasonography. The postoperative period was completely uneventful. Clinical and vaginoscopic evaluations 6 months after the operation confirmed the integrity of the hymen, the complete resolution of clinical symptoms, and the persistence of a large communication between the two vaginas. CONCLUSION(S): Resectoscopic excision under ultrasonographic guidance of the vaginal septum in a girl with uterus didelphys with unilateral hematometra and hematocolpos was effective and easy to perform, and it fully respected the integrity of the reproductive system.