congenital uterine anomaly In the name of GOD congenital uterine anomaly

Congenital uterine anomalies E . Naghshineh M.D Infertility fellowship congenital uterine anomaly

congenital uterine anomaly Case presentation congenital uterine anomaly

Clinical fertility history A 31-year-old woman and her male partner aged 34 presented with secondary infertility of 1 year’s duration. She reported one previous pregnancy which had ended in a spontaneous miscarriage at 6 weeks of gestation 1 year previously. She had a regular menstrual cycle of 30 days and described a biphasic temperature curve indicative of ovulatory cycles. congenital uterine anomaly

General medical, family, and social history The couple was generally healthy and neither partner was taking any medication. They did not smoke and used alcohol only on social occasions. Their respective family histories were unremarkable. congenital uterine anomaly

congenital uterine anomaly Examination findings General examination of the woman was normal. She has a BMI of 22 kg/m2. Gynecological examination showed normal external genitalia, and a normal cervix and vagina at speculum examination. Bimanual examination revealed a mobile, normal-sized uterus and no palpable abnormalities. congenital uterine anomaly

Fertility investigations All investigations were performed on day 3 of the menstrual cycle. TVS showed a dense, irregular endometrium of 12 mm thickness. The uterine fundus and both ovaries appeared normal. Her laboratory results were normal, with a FSH of 8.1 IU/L and a negative Chlamydia antibody titer. Semen analysis is normal, with a total motile count of 126 million. congenital uterine anomaly

Other clinical investigations Due to the irregular endometrium on the TVS, saline infusion sonography was performed. This showed a thin septum extending half the length of the uterine cavity. No other abnormalities were visualized. congenital uterine anomaly

congenital uterine anomaly Diagnosis A secondary fertility disorder of 1 year of unexplained origin except for the possible impact of the septate uterus. congenital uterine anomaly

A uterine septum To treat or not to treat? congenital uterine anomaly

congenital uterine anomaly Action plan In the absence of a septate uterus, the prognosis to conceive within 1 year of unprotected regular intercourse in unexplained infertility of 1 year’s duration would be >50% and justify expectant management. Since no randomized controlled trials are available on the effect of metroplasty on infertility and miscarriage, surgical treatment was not advised. congenital uterine anomaly

congenital uterine anomaly Outcome Five months after the visit to the outpatient, the woman reported another spontaneous miscarriage at 5 weeks of gestation. No additional investigations were performed. After a further 4 months she again presented with a spontaneous ongoing pregnancy, which resulted in a vaginal delivery at 35 weeks of gestation after preterm rupture of membranes. congenital uterine anomaly

Congenital uterine anomalies congenital uterine anomaly

congenital uterine anomaly Prevalence 6.7% in the general population 7.3% in the infertile population 16.7% in the recurrent miscarriage population Arcuate uterus : commonest anomaly in the general and recurrent miscarriage population Septate uterus : commonest anomaly in the infertile population congenital uterine anomaly

congenital uterine anomaly Distribution Septate uterus- 35% Bicornuate-26% Arcuate uterus-18% Unicornuate uterus-10% Didelphys uterus-8% congenital uterine anomaly

Clinical Presentation Often an incidental diagnosis while seeing the patient for a different complaint. The Patient can present with: -Pelvic pain (cyclic or non-cyclic) -Dysmenorrhea -Abnormal vaginal bleeding -Vaginal pain -Uterine rupture during pregnancy -Recurrent pregnancy loss Patient may have a concurrent renal abnormalities congenital uterine anomaly

congenital uterine anomaly ARCUATE UTERUS congenital uterine anomaly

congenital uterine anomaly SUBSEPTATE UTERUS congenital uterine anomaly

congenital uterine anomaly SEPTATE UTERUS congenital uterine anomaly

congenital uterine anomaly UNICORNUATE UTERUS congenital uterine anomaly

congenital uterine anomaly BICORNUATE UTERUS congenital uterine anomaly

congenital uterine anomaly UTERUS DIDELPHYS congenital uterine anomaly

congenital uterine anomaly ASRM classification Class I: segmental agenesis and variable degrees of uterovaginal hypoplasia. Class II: unicornuate uteri (partial or complete unilateral hypoplasia ). Class III: uterus didelphys (duplication of the uterus results from complete non-fusion of the mullerian ducts. Class IV: bicornuate uteri with incomplete fusion of the superior segments of the uterovaginal canal. Class V: Septate uterus, the external shape of the uterus is a single unit. (distinct from the bicornuate uterus which can be seen branching into two distinct horns when viewed from the outside). Class VI: Arcuate uterus. The uterus is essentially normal in shape with a small, midline indentation in the fundus which results from failure to completely dissolve the median septum congenital uterine anomaly

congenital uterine anomaly

Which is the best diagnostic tool? HSG Vaginal US Accuracy: 90%–92% congenital uterine anomaly

hysteroscopy Diagnostic “office” Visual confirmation of US findings • Elective in case of uncertain ultrasound • High compliance of patients No need of anesthesia or analgesia congenital uterine anomaly

congenital uterine anomaly 3D ultrasound Sensitivity: 93% Specificity:100% congenital uterine anomaly

congenital uterine anomaly MRI accuracy :100% Complex anomalies & secondary diagnoses Such as endometriosis can Often be optimally characterized noninvasively. congenital uterine anomaly

Potential mechanisms for an effect on fertility Several theories explain the potential adverse effects of congenital uterine anomalies on fertility & reproductive outcome. Milder Müllerian defects (e.g., arcuate and subseptate uteri) can absolutely normal obstetric outcome The role of the mechanical factors in severe congenital uterine anomalies (e.g., Müllerian agenesis and cervical atresia) is evident ; may also play an important role in cases of less severe anomalies (e.g., bicornuate and septate uteri) that are likely to impact on uterine capacity and the arrangement of uterine musculature (and may consequently cause cervical incompetence). Such effects are likely to cause adverse pregnancy outcomes rather than impairment of fertility congenital uterine anomaly

congenital uterine anomaly Embryo implantation is affected by the morphology, thickness and vascularity of the endometrium, and by the shape and integrity of the uterine cavity; these factors are likely to be altered with congenital uterine anomalies. Therefore, these anomalies can affect endometrial receptivity, resulting in implantation failure that manifests as early pregnancy loss or infertility. Furthermore, congenital absence of the endometrium has been reported . congenital uterine anomaly

congenital uterine anomaly Uterine septa were found in 9.7% of women who had experienced preclinical miscarriage after IVF, and improved results with IVF after hysteroscopic metroplasty were reported. This suggests that a uterine septum may be an important factor predisposing to implantation failure and early pregnancy wastage. congenital uterine anomaly

congenital uterine anomaly The uterine septum is consist of fibroelastic tissue with inadequate vascularization and an altered relationship between the myometrial and endometrial vasculatures; the poor response of the endometrial mucosa covering the septum to estrogen, causing poor proliferation and estrogenic maturation. Removing the septum may eliminate an unsuitable site for implantation, improve endometrial function, expand uterine capacity and dramatically enhance the reproductive outcome in selected patients. congenital uterine anomaly

congenital uterine anomaly Several studies have suggested that the effects on fertility caused by congenital uterine anomalies could be mediated through other infertility factors. Infertility patients with mild Müllerian anomalies were 3 times more likely to also have oligo/amenorrhea ,that was almost 3 times more likely to be idiopathic. congenital uterine anomaly

congenital uterine anomaly A defect in steroid receptor proteins in the congenitally deformed uterus may be responsible for these patients’ eugonadotropic oligo/amenorrhea. In infertility patients, polycystic ovaries were more common among those with Müllerian anomalies ,Particularly in septate and bicornuate uteri. Endometriosis was found more than normal uterus in women with septate uteri, reflecting a possible role for the uterine septum in the pathogenesis of endometriosis . congenital uterine anomaly

congenital uterine anomaly Septate uterus A uterine septum: failure of resorption of the tissue connecting the two mullerian ducts prior to the 20th embryonic week Septate uterus configurations include partial septum, and complete septum in association with cervical septum or duplicated cervix. Most women with a septate uterus have efficient reproductive function. Arcuate uterus, although developmentally considered part of the spectrum of resorption failure, is considered a normal variant and should be differentiated from septate uterus for purposes of prognosis and surgical management congenital uterine anomaly

congenital uterine anomaly Prevalence difficult to ascertain many uterine septum defects are asymptomatic: 1 to 2 per 1,000 to as high as 15 per 1,000 congenital uterine anomaly

congenital uterine anomaly Structure Initially: uterine septa were believed to be predominantly fibrous tissue. Biopsy specimens and MRI primarily of muscle fibers and less connective tissue congenital uterine anomaly

congenital uterine anomaly Association Mullerian anomalies with renal anomalies in11% to 30% Uterine septum: No renal anomalies, not necessary to evaluate the renal system in all patients with a uterine septum congenital uterine anomaly

congenital uterine anomaly Normal/arcuate: Depth from the interstitial line to the apex of the indentation < 1cm. Angle of the indentation >90 degrees. does not cause adverse clinical outcomes. Septate: Depth from the interstitial line to the apex of the indentation >1.5cm. Angle of the indentation <90 degrees. Bicornuate: External fundal indentation >1 cm. .Internal endometrial cavity is similar to a partial septate uterus congenital uterine anomaly

Diagnosis of septate uterus There is fair evidence that 3-D ultrasound sono-hysterography, and MRI are good diagnostic tests for distinguishing a septate and bicornuate uterus when compared with laparoscopy/ hysteroscopy. congenital uterine anomaly

Does a septum impact fertility? There is insufficient evidence to conclude that a uterine septum is associated with infertility. congenital uterine anomaly

Does treating a septum improve fertility in infertile women? No RCTs with untreated controls assessing whether incision of uterine septum improves fertility. Several observational studies indicate that hysteroscopic septum incision is associated with improved clinical pregnancy rates in women with infertility. congenital uterine anomaly

congenital uterine anomaly Does a septum contribute to pregnancy loss or adverse pregnancy outcome? There is fair evidence that a uterine septum contributes to miscarriage and preterm birth. Some evidence suggests that a uterine septum may increase the risk of other adverse pregnancy outcomes such as malpresentation, IUGR, placental abruption, and perinatal mortality. congenital uterine anomaly

Does treating a septum improve Obstetrical outcomes? No RCTs with untreated controls assessing whether uterine septum incision improves pregnancy outcomes. Some limited studies indicate that hysteroscopic septum incision is associated with a reduction in subsequent miscarriage rates and improvement in live-birth rates in patients with a history of infertility or prior pregnancy loss. congenital uterine anomaly

congenital uterine anomaly Are septum characteristics associated With worse reproductive outcomes? There is insufficient evidence to conclude that obstetric outcomes are different when comparing the length or width of uterine septa. congenital uterine anomaly

Surgery to treat a uterine septum Commonly used techniques to resect uterine septum include incision or removal of the septum utilizing cold scissors, unipolar or bipolar cautery, or laser. Use of distending media for the uterus is dependent on the incision technique or energy source and includes CO2, saline, glycine, sorbitol, or mannitol. congenital uterine anomaly

Surgery to treat a uterine septum Laparoscopy and, more recently, transabdominal ultrasound have been used concurrently with hysteroscopic incision to confirm uterine contour, decrease the risk of uterine perforation, and assess complete removal of the septum and the presence of other anomalies. There is insufficient evidence to recommend a specific method for hysteroscopic septum incision. congenital uterine anomaly

congenital uterine anomaly How long after surgical treatment of a Uterine septum should a woman wait to Conceive? Although the available evidence suggests that the uterine cavity is healed by 2 months postoperatively, there is insufficient evidence to advocate a specific length of time before a woman should conceive. congenital uterine anomaly

Should preoperative management to thin the endometrium be used? There is insufficient evidence for or against recommending danazol or GnRH agonists to thin the endometrium prior to hysteroscopic septum incision. congenital uterine anomaly

Is adhesion prevention needed? There is insufficient evidence to recommend for or against adhesion prevention treatment, or any specific method. congenital uterine anomaly

congenital uterine anomaly Conclusion Uterine congenital anomalies seem to be an important factor of pregnancy failure and metroplasty seems to improve dramatically the outcome of pregnancies. In front of the benefit of favorable pregnancy outcome after metroplasty, at least vaginal ultrasound should be performed in all cases with history of spontaneous abortion. In case of positive US, office diagnostic hysteroscopy should be performed even after first abortion congenital uterine anomaly

congenital uterine anomaly The long duration of infertility before surgery, unexplained infertility, and short time interval subsequent to surgery in which conception occurs, suggests metroplasty has value in treatment of patients with septa and otherwise unexplained infertility. congenital uterine anomaly

congenital uterine anomaly