Mullerian Anomalies Research Articles

Laparoscopic approach to adnexal mass in adolescents: A retrospective analysis

Background: Adnexal masses result from benign or malignant lesions of ovarian, tubal and paratubal origin as well as pregnancy related causes such as mullerian anomalies, infectious causes and ectopic pregnancy. The current study aimed to evaluate the preoperative diagnosis, surgical procedure and histopathological findings of adolescent girls who underwent laparoscopic surgery for adnexal mass in our hospital. Materials and Methods: A retrospective chart review of patients with adnexal mass was conducted over a period of one year from Jan 2014-2015. Overall 96 patients were included in the study after reviewing clinical records. In all the patients' malignancy was excluded by preoperative imaging with sonography, CT or MRI and tumor marker study. Results: Laparoscopy was the operative approach for all 96 patients. None resulted in malignant histology. Majority of the adnexal masses were simple cyst (28%), 11.4% were dermoid cyst, 2% were endometriotic cyst. One patient had tubal ectopic, 3 patients had Koch's abdomen and pelvis. Among them one 13 year old girl underwent rudimentary horn excision who presented as a para tubal mass with acute abdomen. Almost half of the patients (68.7%) presented with acute abdomen of which 41.6% were adnexal torsion. All adnexal masses were resected laparoscopically and the outcome was uneventful in all the patients. Conclusion: Laparoscopy is regarded as an appropriate surgical method for benign adnexal masses which was safe and effective even for giant ovarian cyst in well selected cases as most of the pelvic adnexal masses seen in adolescents and young girls are of benign nature.

Non-communicating Rudimentary Horn Ectopic Pregnancy in a Patient with a Known Urinary Tract Anomaly

Ectopic pregnancy in the context of undiagnosed Mullerian anomalies can present diagnostic and therapeutic challenges to the general Obstetrician/Gynecologist. We present a case of a noncommunicating rudimentary horn ectopic pregnancy discovered at the time of laparoscopy after ultrasound findings were suggestive of, but not diagnostic for, Mullerian anomaly.

Three-Dimensional (3D) Sonography in Gynecology

In Chang-Gung Memorial Hospital, Lin-Kou, three-dimensional (3D) sonography with power Doppler angiography had been applied in clinical evaluation since 2002. The technique can be utilized in many complicated conditions, and we had cumulative experiences in mainly three circumstances. The first one was to evaluate Mullerian duct anomaly such as HWW syndrome, uterine septum, and vaginal agenesis. The second was to assist differenciating benign from malignant tumors in uterus and ovaries. Finally, abnormal placentation such as placenta accreta was evaluated to predict the obstetric outcome. Currently, over five hundred cases of uterine anomaly and over one thousand cases of uterine tumor were collected. With all these experience, we summarized several diagnostic criterias for vessel characteristics in uterine and ovarian malignancy. Marked increased sensitivity and specificity were obtained in comparison with traditional two-dimensional sonography. Furthermore, we could provide precise and real-time images of congenital Mullerian duct anomaly and adjustable to fit individual angle of uterus with 3D sonography. MRI is also utilized for diagnosis of Mullerian duct anomaly. However, MRI has angle limitation that may affect the accurate diagnosis. It is also more expensive then 3D sonography. In our experiences, 3D sonography is a useful modality in diagnosis of Mullerian anomaly and can provide more details for treatment strategy.

Unicornuate Uterus with Hematometra

Introduction : Unicornuate uterus is one less common mullerian anomalies .unicornate uterus with non-communicating rudimentary horn may associated with gynecologic complication such endometrosis,infertility,hematometra,hematosalpix ,chronic pelvic pain and urinary tract abnormality. It has an affect quality of life a patient and morbidity. Case presentation: we report case of 45-year old black woman presenting with unicornate uterus with non-communicating rudimentary horn. The diagnosis was made by two dimensional ultrasound and later confirmed with laparotomy. The removal of rudimentary horn and uterus was removed, upon surgery it found that left kidney was absent .the post-surgery course was smooth and discharged on six post-operative day of surgery. Conclusion: although diagnosis of unicornate uterus difficult clinical ,it possible pick using two dimensional ultrasound with an experienced hand .it should not be forgotten that the possibility associated anomaly of urinary tract anomaly and can present with chronic pelvic pain and abdominal mass.

Uterus Didelphys with an Obstructed Unilateral Vagina by a Transverse Vaginal Septum Associated with Ipsilateral Renal Agenesis: OHVIRA Syndrome − An Unusual Case Report and Review of Literature

The triad of uterine didelphys, obstructed hemivagina, and unilateral renal agenesis, called as OHVIRA syndrome is the least common of all Mullerian malformations. We report a case of a 16-year-old girl who presented with lower abdominal pain and amenorrhea. Radiological investigations led us to the diagnosis of OHVIRA syndrome. She was hospitalized and operated upon. OHVIRA syndrome, though a rare Mullerian anomaly, should be kept as a differential diagnosis in a young girl presenting with cyclical pain and amenorrhea with renal agenesis so that surgical management can be performed before long-term complications set in.

Endometriosis in the Adolescent Patient.

The recognition and management of endometriosis in the adolescent patient is challenging. A strong clinical suspicion for endometriosis should be maintained in the adolescent who suffers from acyclic pelvic pain as well as absenteeism from school and lack of participation in daily activities. Risk factors include the presence of an obstructive Mullerian anomaly, a family history of endometriosis, and conditions that prolong exposure to endogenous and exogenous estrogens. Empiric medical therapy with nonsteroidal anti-inflammatory drugs and combined oral contraceptive pills may be considered in most adolescents with endometriosis. Failure of empiric therapy may warrant diagnostic laparoscopy, which affords a concomitant opportunity for treatment via excision of endometriosis. Endometriotic implants in the adolescent tend to be more atypical, appearing red/flame-like, clear/polypoid, or vesicular. Endometriosis tends to recur more often in adolescents when compared with adults, and the role of postoperative medical therapy for the suppression of disease progression is not entirely clear. Current knowledge on the impact of adolescent endometriosis on future fertility is limited but overall reassuring.

Surgical Management of Mullerian Anomalies

Surgical Management of Mullerian Anomalies

Oblique Vaginal Septum Syndrome (OVSS), an Unclassified Mullerian Anomaly in a 22-year old Nulligravid Presenting with Severe Dysmenorrhea and a Paravaginal Bulge: A Case Report

Oblique Vaginal Septum Syndrome (OVSS), an Unclassified Mullerian Anomaly in a 22-year old Nulligravid Presenting with Severe Dysmenorrhea and a Paravaginal Bulge: A Case Report

Genetic association between PAX2 and mullerian duct anomalies in Han Chinese females.

The study aims to investigate the genetic association between paired box gene 2 (PAX2) and mullerian duct anomalies (MDA) in Chinese Han females. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) was used to identify the genotypes of three tag single nucleotide polymorphisms (SNPs) in PAX2 in 362 MDA cases and 406 controls. We found that one tag SNP (rs12266644) of PAX2 was associated with susceptibility to MDA. The genotype distributions of the SNP rs12266644 have a statistically significant difference in the MDA patients and controls with a p value = 0.008. In the dominant model, we also observed that the GT + TT genotype increased the risk for MDA (p = 0.015, OR = 1.637, 95% CI = 1.096-2.443). The polymorphism rs12266644 of PAX2 might be a risk factor for MDA in Chinese Han females.

Role of 2d Ultrasound, 3d Ultrasound And Mri in The Diagnosis of Mullerian Duct Anomalies

Objective : To evaluate and compare the diagnostic accuracies of 2D,3D Ultrasonography and Magnetic Resonance Imaging in the diagnosis of Mullerian Duct Anomalies. Setting: Tertiary care centre Design: analytical study Method: 50 outpatient women presenting with the complaints of primary amenorrhea, primary infertility, pregnancy wastage and menstrual abnormalities & suspicious of mullerian duct anomalies were enrolled in the study and subjected to clinical examination, 2D & 3D Ultrasonography and Magnetic Resonance Imaging. Results: MRI was considered as the gold standard and sensitivity and specificity of each diagnostic modality is calculated. 3D ultrasound diagnosed 11 true negative cases, 33 cases as true positive and 6 as false negative cases. MRI diagnosed 11 cases as true negative and 39 cases as true positive. Thus 3D ultrasound showed 85% sensitivity and 100% specificity in diagnosing Mullerian Duct Anomalies; and MRI has 100% sensitivity and specificity in diagnosing Mullerian Duct Anomalies. Conclusion: the sensitivity and specificity of 3D ultrasonography in diagnosing Mullerian Duct Anomalies is better than2D Ultrasonography and comparable to that of MRI.Hence it is a better alternative, cost effective, acceptable and convenient to the patients.

Asymptomatic uretheral intercourse associated with Mayer Rokitansky Küster Hauser syndrome type II: A case with crossed fused renal ectopia and scoliosis

IntroductionUrethral coitus is very rare with significant consequences and less than 30 cases were reported in the literature. It is most commonly associated with Mullerian anomalies. ObservationWe report a case of 28 years married woman with Mullerian agenesis and associated anomalies who was engaged in urethral coitus and urethral dilation was detected on examination under anesthesia. The patient did not accept vaginoplasty after she learned that it would not be a cure for her infertility problem. ConclusionsUrethral coitus is very rare with few cases reported. Woman diagnosed with MRKHS II should be assessed for associated abnormalities especially skeletal and renal ones. Urinary incontinence associated with coitus and dyspareunia with Mullerian anomalies should raise the suspicion of urethral coitus. Follow-up is important to avoid urinary tract infections if the female rejects surgery and is satisfied with intra-urethral coitus. Mullerian anomaly should be corrected to allow vaginal intercourse if possible.

Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome with imperforate anus

Abstract OHVIRA syndrome (Obstructed hemivagina with ipsilateral renal agenesis) is a rare Mullerian duct anomaly that results in uterine didelphys, obstructed vaginal vault, and unilateral renal agenesis. We report an 11 year old girl with a history of imperforate anus presenting with progressive abdominal pain. This represents the first documented case of OHVIRA syndrome in association with an ano-rectal malformation.

Reconstructive And Conservative Surgery by Tubal Implantation in Mullerian Anomalies – A Case Report

Reconstructive And Conservative Surgery by Tubal Implantation in Mullerian Anomalies – A Case Report

A Case of Successful Pregnancy in a Complete Bicornuate Uterus

Background & aim: Complete bicornuate uterus is a type of Mullerian duct deformity, resulting from abnormal duct fusion. Similar to other Mullerian anomalies, bicornuate uterus is associated with specific complications during possible pregnancies. While various studied have reported successful deliveries in a bicornuate uterus, it might be accompanied with various complications, ranging from preterm labor to more catastrophic outcomes such as uterine rupture. Case report: One of the current approaches to diagnosis this abnormality is ultrasound monitoring. In the present report, we presented a 25-years-old multiparous women with a history of abortion. The patient was not diagnosed with a complete bicornuate uterus in her first unsuccessful pregnancy. However, she was suspected with a complete bicornuate uterus based on the findings of ultrasound in the present pregnancy. A successful cesarean section was performed on the subject in the 39th week of gestation. Conclusion: According to the results, successful delivery could be achieved in patients with bicornuate uterous. Considering factors such as recurrent miscarriage, suspicious findings, and unreliable results of ultrasound, other diagnostic evaluations, including magnetic resonance imaging and hysterosalpingography, are recommended.

Obstetric outcome of a primagravida with a uterine didelphys, a 20 cm fibroid, and a vaginal septum

Background: Mullerian anomalies, like a uterine didelphys (UD) and vaginal septae, are at increased risk for adverse pregnancy outcomes. Some obstetrical complications include spontaneous abortion, bleeding, fetal malpresentation, and cesarean delivery. Surgical treatment is rarely performed in UD.Uterine fibroids in pregnancy are frequent and pain is the most common symptom. Fibroids in pregnancy are associated with an increased risk of spontaneous abortions, preterm labor and delivery, malpresentation, and placental abruption. Myomectomy is avoided during pregnancy and at delivery, due to the risk of hemorrhage. Acetaminophen is the treatment of choice.Case: A 31-year-old female, gravida 1 para 0 with an intrauterine pregnancy (IUP), a 20 cm fibroid, a uterine didelphys (UD), and a longitudinal vaginal septum, was followed for pain caused by degeneration of the 20 cm fibroid. At term, she underwent labor induction due to oligohydramnios and delivered a healthy infant via vaginal delivery. A vaginal septoplasty was performed due to a partial tear of the septum. Estimated blood loss was 800 ml, and she received 2 units of PRBCs. She was discharged home on postpartum day 2. Her postpartum recovery was complicated with endometritis which was treated with methergine and antibiotics.Conclusion: Few pregnancies with both UD and fibroids have been described. Many result in a cesarean delivery due to the inability for fetal head descent. The literature search did not find another successful vaginal delivery of an IUP with a uterine didelphys, a large fibroid, and a vaginal septum.

Utility of MRI in uterine and adnexal lesions

Objective:To ascertain the role of MRI as a problem solving modality in characterising indeterminate uterine and adnexal masses from USG.Methods:A prospective study was conducted with100 female patients with pelvic pathologies who underwent an ultrasound examination and proved to be diagnostic dilemmas.These patients were further investigated with an MRI of the pelvis without contrast. Routine sequences were performed in all patients including T1Wi ,T2Wi and GRE.Results:There were 70 uterine lesions (fibroids -39, adenomyosis -14,Mullerian anomalies – 4,bulkiness of uterus -8,low lying placenta antenatal scan -2) and 30 adnexal lesions(malignant ovarian cysts -10,dermoid cyst – 6,chocolate cyst -8,tubovarian masses – 6,chronic torsion -1,chronic ectopic – 1,peritoneal inclusion cyst -1).The diagnosis made after the MRI scan was compared to the working diagnosis made from USG and these were separately compared with the HPR diagnoses in 53 patients who underwent surgery. The remaining 43 patients who were treated conservatively the MRI diagnoses were compared with the working USG diagnoses.The statistical data was analysed bycontingency coefficient, CC, method (variant of Chi square method).The CC for USG diagnoses and postoperative diagnoses was statistically significant.The CC for MRI diagnoses and postoperative diagnoses was 0.684 at a p value of 0.013.Conclusions:The analysis of data from the study indicates a strong association between the MRI diagnoses and the postoperative diagnoses, more so than the USG diagnoses and the postoperative diagnoses. There is more variability between the USG diagnoses and the postoperative diagnoses by comparison.

Misdiagnosed Case of a Rudimentary Horn Pregnancy

Objective: To emphasize the importance of knowledge of mullerian anomalies while evaluating a suspected case of pregnancy in a bicornuate uterus. Design: Case report Setting : Obstetric unit in a training and research hospital. Patient : A 25 year old primigravida woman with 26 weeks pregnancy in a non communicating rudimentary horn. Intervention: The exact diagnosis of ruptured non communicating rudimentary horn pregnancy was diagnosed after explorative laparotomy. Conclusion : Pregnancy in a non communicating rudimentary horn should always be considered as a differential diagnosis of a pregnancy in a bicornuate uterus. Lack of experience and knowledge may lead to incorrect diagnosis in such cases, and hence compromising maternal and fetal health.

Incidence of Septate Uterus in Reproductive-Aged Women With and Without Endometriosis

Study ObjectiveTo compare the incidence of a uterine septum in women with and without endometriosis and if such incidence correlates with the stage of endometriosis Although a correlation between obstructive Mullerian anomalies and endometriosis has been well established, its link with non-obstructive anomalies remains controversial. To elucidate whether there is a correlation between endometriosis and non-obstructive Mullerian anomalies, we conducted this prospective study on all patients admitted to our Reproductive Endocrinology and Infertility surgical service from February 1, 2010 through June 30, 2012. All patients underwent both hysteroscopy and laparoscopy. Surgical indications included: infertility, pain, and/or menorrhagia. The presence or absence of endometriosis and uterine anomalies were recorded immediately after each surgery and subsequently analyzed. Endometriosis was staged according to the r-ASRM Classification and treated by resection and ablation of deep and superficial lesions, respectively. Since uterine septum is the most common Mullerian anomaly, we considered only this anomaly to test the hypothesis that uterine septum may be associated with an increased incidence of endometriosis. DesignProspective Study. Evidence from a well-designed case-control study (Canadian Task Force classification II-2). SettingUniversity-affiliated tertiary care center. PatientsReproductive aged women admitted to our service for treatment of pelvic pain, abnormal uterine bleeding, and/or infertility. InterventionAll patients underwent both hysteroscopy and laparoscopy as part of their evaluation and treatment of pelvic pain, abnormal uterine bleeding, and/or infertility. Measurements and Main Results343 patients were included in the study. The diagnosis of each patient included infertility - 52, pain - 215, both - 30 and other - 46. The diagnosis of septate uterus was made at hysteroscopy when the endometrial cavity was separated by an avascular septum that obscured visualization of both cornua when the hysteroscope was advanced to the mid-uterine segment. The septum was lysed sharply from cornua to cornua restoring normal fundal configuration. In all cases, the septolysis was bloodless, confirming its avascular nature. The overall incidence of uterine septum was 33% in our patient population. In patients with a histologically confirmed diagnosis of endometriosis, the incidence of septum was 37% versus 27% in patients without endometriosis (P = .046). In patients with advanced endometriosis, Stage IV disease, the incidence of septate uterus was 41% (P = .022). The odds ratio of Stage IV endometriosis with a uterine septum was 1.94 (CI 1.09-3.44). ConclusionThe incidence of septate uterus in our population of women with infertility and/or pelvic pain ranges from 27% to 37%, being significantly higher in women with endometriosis and mores so with Stage IV disease. Our data suggests that the presence of a uterine septum may predispose to more advanced disease.

Evidence for No Significant Impact of Müllerian Anomalies on Reproductive Outcomes of Twin Pregnancy in Korean Women.

The present article aimed to evaluate the impact of congenital Müllerian anomalies (MA) on twin pregnancy after 24 gestational weeks in Korean women. All records of twin pregnancies in a large maternity hospital in Korea between January 2005 and July 2013 were analyzed. Patients with monochorionic monoamniotic (MCMA) twins, non-Korean patients, patients with twins delivered prior to 24 gestational weeks, and patients with miscarriage of one fetus or intrauterine fetal death (IUFD) before 24 gestational weeks were excluded from data analysis. In total, 1,422 women with twin pregnancy were eligible for data analysis, including 17 (1.2%) who had a known congenital MA (septate uterus, bicornuate uterus, arcuate uterus, and unicornuate uterus). Except for the mode of conception, baseline demographics were similar between women with MA and those without MA. No significant differences were found in pregnancy outcomes of gestational age at delivery (p = .86), birth weight of smaller and larger twins (p = .54 and p = .65), and number of twins with birth weight <5th percentile for gestational age (p = .43).The rates of obstetrical complications such as pre-eclampsia, gestational diabetes mellitus (GDM), placenta previa, cerclage, IUFD, and postpartum hemorrhage were not significantly different between the two groups either. We concluded that the presence of congenital MA may not increase obstetrical risks in outcomes of pregnancy of twins delivered after 24 gestational weeks.

Herlyn–Werner–Wunderlich Syndrome: Clinical Presentation and Outcome After Repeat Surgery

Abstract Background: Herlyn–Werner–Wunderlich syndrome (HWW syndrome), is a rare congenital anomaly consisting of the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The diagnosis of HWW syndrome is often delayed because of less understanding of Mullerian anomalies by practitioners. Case: A 13-year-old female presented with severe dysmenorrhea. A diagnosis of HWW syndrome was confirmed by magnetic resonance imaging. Drainage of her hematocolpos was performed by resection of the vaginal septum. Laparoscopic fulguration of endometriosis lesions was performed; the lesions had been formed as a result of retrograde menstrual blood flow. Results: The patient's postoperative period was uneventful, and she was relieved of dysmenorrhea completely. After 2 years, she developed dysmenorrhea again as a result of restenosis of the septum and development of hematocolpos. Her vaginal septum was excised with suturing of the edges, and she was advised to retain her vaginal stent for 1 mon...