Uterus didelphys with blind hemivagina and ipsilateral renal agenesis complicated by pyocolpos and presenting as acute abdomen 11 years after menarche: presentation of a rare case with review of the literature

BACKGROUND: Congenital anomaly of the Müllerian duct system can result in various urogenital anomalies including uterus didelphys with blind hemivagina and ipsilateral renal agenesis. The diagnosis of this condition is usually made after menarche, but its rarity and variable clinical features may contribute to a diagnostic delay for years after menarche. CASE: A 24-year-old woman presented to the emergency room of the Department of Obstetrics and Gynecology complaining of severe worsening lower abdominal pain, vomiting and pus-like vaginal discharge. Physical examination revealed acute abdomen with diffuse lower abdominal tenderness, rebound and muscular resistance. Cervical and vaginal observation was impossible because of the patient's discomfort. Bimanual gynecological examination showed high tenderness cervical motion. Transabdominal ultrasound scan was performed and the radiologist interpreted the ultrasonographic findings as existence of a pelvic mass with mixed echogenicity. The preoperative diagnosis was ruptured tubo-ovarian abscess and emergency laparotomy was performed. Free pus in the pelvis was found. Also, a double uterus with an elongated and inflammatory right fallopian tube with pus passing out of its fimbrial end was observed. Vaginal examination under general anesthesia revealed an obstructed right hemivagina with vaginal pus-like discharge from a small fistula hole on the septate vagina. The final diagnosis was uterus didelphys with unilateral imperforate right hemivagina and pyocolpos. Transvaginal resection of the vaginal septum was performed and a large amount of pus and blood was spilled out. Postoperatively, intravenous pyelography demonstrated agenesis of the right kidney. CONCLUSION: We demonstrated the difficulty in making a correct diagnosis of this rare congenital anomaly of the female genital tract, especially after many years since menarche. This condition should be considered to prevent misdiagnosis or suboptimal treatment and decrease morbidity and unnecessary surgical procedures.     

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.     

Clinical characteristics and treatment of Herlyn–Werner–Wunderlich syndrome

Objective

To investigate the clinical characteristics and treatment of the Herlyn–Werner–Wunderlich syndrome (HWWS).

Methods

Sixty-one patients diagnosed with HWWS were retrospectively analyzed. HWWS is categorized into three types in China.

Results

Age at presentation of all the cases was after menarche. The most common clinical presentations were dysmenorrhea for type I and vaginal discharge for types II and III. Clinical presentations of types II and III may not occur until reproductive age. HWWS occurred on the right in 39/61. Excision of the obstructed vaginal septum was the treatment utilized in this cohort. After surgery, subsequent pregnancies were ipsilateral in 52.9 %.

Conclusions

Clinical presentation in untreated HWWS suggests the anatomic anomaly. Early recognition and treatment can reduce symptoms. Pregnancies occur in both the affected and unaffected uterus.     

Successful Pregnancy Following Surgery in the Obstructed Uterus in a Uterus Didelphys with Unilateral Distal Vaginal Agenesis and Ipsilateral Renal Agenesis: Case Report and Literature Review

SynopsisAfter surgical correction of unilateral distal vaginal agenesis, the obstructed didelphic uterus was able to harbor 2 subsequent pregnancies.BackgroundThere was a congenital anomaly syndrome of uterus didelphys, unilateral distal vaginal aplasia, and ipsilateral renal aplasia. Intuition suggests that later pregnancy would be in the unobstructed uterus.ObjectiveThe purpose is to report pregnancy occurring twice in a previously obstructed didelphic uterus after surgical correction.CaseA girl aged 13 years, 8 months presented with the syndrome of didelphic uterus, upper right hematocolpos, and right renal aplasia. Right vaginal marsupialization was done. Subsequently, she had 2 pregnancies in the right didelphic uterus.ConclusionPregnancy occurred in the obstructed uterus despite a large hematometra, extensive right pelvic peritoneal endometriosis, and hematocolpos. The surgeon should make every effort to preserve the obstructed uterus.     

Longitudinal Vaginal Septum Resection Using the Ligasure Device

BackgroundA longitudinal vaginal septum might present as a nonobstructive or obstructive type. Both can result in dyspareunia, difficult tampon insertion, persistent vaginal bleeding despite tampon placement, and dysmenorrhea. Surgical correction is warranted for symptomatic cases.CaseWe present a 16-year-old female adolescent with a complex cloacal anomaly, uterine didelphys, longitudinal vaginal septum, and other congenital anomalies. The patient was unable to use tampons during menses because of the small caliber on both vaginal canals. We performed a longitudinal vaginal septum resection using the Ligasure (Medtronic, Inc, Doral, FL) device. We were able to resect the vaginal septum without difficulty.Summary and ConclusionOur case introduces an innovative and safe alternative to the surgical management of a longitudinal vaginal septum, particularly useful in a confined surgical space.     

Resection of Longitudinal Vaginal Septum Using a Surgical Stapler

BackgroundA longitudinal vaginal septum is a rare congenital anomaly that can cause dyspareunia, difficulty with tampon insertion, persistent vaginal bleeding, and dysmenorrhea. Various surgical techniques have been described.CaseWe present the case of a 14-year-old girl with obesity and diabetes mellitus with uterine didelphys and double vagina with a longitudinal vaginal septum. The patient presented with dysmenorrhea. Resection of the longitudinal vaginal septum using a GIA and EndoGIA (Medtronic, Inc, Doral Fl) stapler device was performed.Summary and ConclusionWe introduce a safe and effective technique for resecting a longitudinal vaginal septum using stapler technology. This technique eliminates the potential risk of thermal injury to nearby structures from currently described methods.     

Longitudinal vaginal septum

A total of 83 women with longitudinal vaginal septum were encountered over a period of 19 yr. Of these, 47 patients had complete vaginal septum and 36 partial septum. Longitudinal vaginal septum was verified in 61 out of 168 women with uterine anomalies, mostly with uterus didelphys. Only 3 women with partial vaginal septum were found to have a normal uterine cavity. Dystocia and dyspareunia were the most common indications for excision of vaginal septa.     

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.     

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.     

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.     

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.     

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.

     

Didelphic uterus and obstructed hemivagina: recurrent hematometra in spite of appropriate classic surgical treatment

We report the case of a young girl with uterus didelphys, obstructed vagina and ipsilateral renal agenesis treated by diagnostic laparoscopy and resection of the vaginal septum by a vaginal approach in order to drain the distended uterus. Despite classic surgery, the myometrium on the treated side never recovered its normal function, with subsequent persistence of recurrent hematometra. Laparoscopic subtotal hemihysterectomy had to be performed to avoid retention of hematometra and secondary pelvic inflammatory disease.     

Premenarchal, recurrent vaginal discharge associated with an incomplete obstructing longitudinal vaginal septum

STUDY OBJECTIVE: To describe an unusual, premenarchal presentation of an obstructive vaginal anomaly. DESIGN: Case Report. SETTING: University Medical Center. PARTICIPANTS: Premenarchal subject INTERVENTIONS: Vaginogram, vaginal septum resection. RESULTS: Vaginal septum resection with resolution of vaginal discharge. CONCLUSIONS: This case demonstrates some of the typical features of uterus didelphys bicollis with incomplete obstructing hemivagina, but had a unique presentation with premenarchal, recurrent vaginal discharge. Typically, patients with an obstructing mullerian anomaly present after menarche with pelvic pain and a mass. The vaginogram assists in the preoperative definition of abnormal anatomy which allows the surgeon to develop the most appropriate surgical approach. Resection of this incompletely obstructing vaginal septum resulted in resolution of the recurrent vaginal discharge.     

Resectoscopic treatment of complete longitudinal vaginal septum.

Every gynecologist can easily recognize vaginal abnormalities and the subsequent disturbances of sexual function, such as dyspareunia and apareunia, arising from them. In the case of congenital vaginal septum, it is extremely important to completely assess the abnormality before planning for surgical treatment. Because normal vaginal development is subsequent to the canalization and fusion of the two Müllerian ducts once vacuolization has occurred, but not their union, a duplication of different degree will be the consequence. If the defect is complete, a didelphys uterus with a double vagina will result. The aim of our report is to describe the endoscopic technique used to treat three cases of complete longitudinal vaginal septum and to highlight the main differences between our technique and the standard surgical procedure.     

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.     

Uterus didelphys: a report of 26 cases

Twenty-six female patients with uterus didelphys were observed over a period of 21 yr. All the patients also had a longitudinal vaginal septum. Dysmenorrhea, dyspareunia and leukorrhea were the most common symptoms among the symptomatic cases. Eighteen women had a total of 40 pregnancies with a fetal survival rate of 67.5%, and perinatal mortality rate of 3.6%. Premature delivery occurred in 21% of the pregnancies. Breech presentation occurred in 43% and cesarean section was performed in 82% of the cases. Of the 17 intravenous pyelograms performed six (35%) showed abnormalities and four had the absence of a kidney. Women with uterus didelphys belong to a high-risk group, although pregnancy outcome is comparatively good.     

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.     

A Novel Approach for Resection of a Vaginal Septum

BackgroundThe resection of a vaginal septum using the Harmonic Scalpel in a patient on anticoagulation is described.CaseA 21-year-old female with bicollis didelphys uterus and a 4-cm vaginal septum underwent resection of the vaginal septum using the ultrasonic scalpel. Hemostasis was noted both during and after the procedure. The patient was given thombophylaxis for 2 weeks perioperatively secondary to her medical history of anti-thrombin III deficiency and deep venous thrombosis.Summary and ConclusionThe use of the Harmonic Scalpel appears to be a safe and effective method for resection of vaginal septa. Patient healing time may vary and may take longer than with traditional techniques.     

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.