Herlyn-Werner-Wunderlich Syndrome: A Rare Congenital Uterine Anomaly

Introduction: Herlyn-Werner-Wunderlich Syndrome is a rare anomaly and exact incidence is not known. It is usually diagnosed in young girls who present with cyclical abdominal pain along with cyclical menstruation. Case Report: We present case of two girls who had this syndrome. An ultrasound was performed in both patients. In first patient, it revealed uterus didelphys with hematocolpos on right side. In the second patient, a large hematometra of size 9.8×12.2 cm, along with hematocolpos was present on the left side. Both the patients underwent examination under anaesthesia and excision of septum. They remained asymptomatic during one year follow-up. Conclusion: Treatment of such cases is excision of septum and to maintain the patency of the outflow tract and prevent stricture formation later. A good follow-up in immediate post-operative period is necessary.

Unicornuate Uterus with Rudimentary Horn as a Rare Etiology of Secondary Dysmenorrhea: A Case Report

Congenital uterine anomalies are an uncommon type of female genital malformations caused by abnormal development of müllerian ducts during embryogenesis. Patients with an obstructive uterine anomaly have a higher risk of developing gynecological and obstetric complications that may present at menarche or later in life. We present a case of severe dysmenorrhea in a young teenager caused by obstructive hematometra in a noncommunicating horn of the unicornuate uterus. A differential diagnosis of a possible anomaly was made using 2-dimensional pelvic ultrasonography, which was later confirmed using MRI that revealed an anomalous uterine cavity with a single left-sided cornua communicating with the cervix and a distended right-sided rudimentary horn. She underwent a right salpingectomy with rudimentary horn excision, which was successfully managed laparoscopically. This case emphasizes the importance of physicians being cognizant in identifying patients with uterine anomaly to provide appropriate treatment and prevent adverse reproductive outcomes.

P-377 Association between antinuclear antibodies and pregnancy prognosis in recurrent pregnancy loss patients

Study question Can antinuclear antibody (ANA) affect the subsequent live birth rate in patients with recurrent pregnancy loss (RPL) who have no antiphospholipid antibodies (aPLs)? Summary answer ANA did not affect the pregnancy prognosis of RPL women. What is known already The prevalence of ANA is well-known to be higher in RPL patients. Our previous study found no difference in the live birth rates of ANA-positive and -negative patients who had no aPLs. Higher miscarriage rates were also reported in ANA-positive patients compared to ANA-negative patients with RPL. The RPL guidelines of the ESHRE state that “ANA testing can be considered for explanatory purposes.” However, there have been a limited number of studies on this issue and sample sizes have been small, and the impact of ANA on the pregnancy prognosis is unclear. Study design, size, duration An observational cohort study was conducted at Nagoya City University Hospital between 2006 and 2019. The study included 1,108 patients with a history of 2 or more pregnancy losses. Participants/materials, setting, methods 4D-Ultrasound, hysterosalpingography, chromosome analysis for both partners, aPLs and blood tests for ANA and diabetes mellitus were performed before a subsequent pregnancy. ANAs were measured by indirect immunofluorescence. The cutoff dilution used was 1:40. In addition, patients were classified according to the ANA pattern on immunofluorescence staining. Live birth rates were compared between ANA-positive and ANA-negative patients after excluding patients with antiphospholipid syndrome, an abnormal chromosome in either partner and a uterine anomaly. Main results and the role of chance The 994 patients were analyzed after excluding 40 with a uterine anomaly, 43 with a chromosome abnormality in either partner and 32 with APS. The rate of ANA-positive patients was 39.2 % (390/994) when the 1: 40 dilution result was positive. With a 1:160 dilution, the rate of ANA-positive patients was 3.62 % (36/994). The live birth rate was calculated for 798 patients, excluding 196 patients with unexplained RPL who had been treated with any medication. With the use of the 1 40 dilution, the subsequent live birth rates were 71.34 % (219/307) for the ANA-positive group and 70.67 % (347/491) for the ANA-negative group (OR, 95%CI; 0.968, 0.707-1.326). After excluding miscarriages with embryonic aneuploidy, chemical pregnancies and ectopic pregnancies, live birth rates were 92.41 % (219/237) for the ANA-positive group and 92.04 % (347/377) for the ANA-negative group (0.951, 0.517-1.747). Using the 1:160 dilution, the subsequent live birth rates were 84.62 % (22/26) for the ANA-positive group, and 70.47 % (544/772) for the ANA-negative group (0.434, 0.148-1.273). Subgroup analyses were performed for each pattern on immunofluorescence staining, but there was no significant difference in the live birth rate between the two groups. Limitations, reasons for caution The effectiveness of immunotherapies could not be evaluated. However, the results of this study suggest that it is not necessary. Wider implications of the findings The measurement of ANA might not be necessary for the screening of patients with RPL who have no features of collagen disease. Trial registration number not applicable

P–377 Association between antinuclear antibodies and pregnancy prognosis in recurrent pregnancy loss patients

Study question Can antinuclear antibody (ANA) affect the subsequent live birth rate in patients with recurrent pregnancy loss (RPL) who have no antiphospholipid antibodies (aPLs)? Summary answer ANA did not affect the pregnancy prognosis of RPL women. What is known already The prevalence of ANA is well-known to be higher in RPL patients. Our previous study found no difference in the live birth rates of ANA-positive and -negative patients who had no aPLs. Higher miscarriage rates were also reported in ANA-positive patients compared to ANA-negative patients with RPL. The RPL guidelines of the ESHRE state that “ANA testing can be considered for explanatory purposes.” However, there have been a limited number of studies on this issue and sample sizes have been small, and the impact of ANA on the pregnancy prognosis is unclear. Study design, size, duration An observational cohort study was conducted at Nagoya City University Hospital between 2006 and 2019. The study included 1,108 patients with a history of 2 or more pregnancy losses. Participants/materials, setting, methods 4D-Ultrasound, hysterosalpingography, chromosome analysis for both partners, aPLs and blood tests for ANA and diabetes mellitus were performed before a subsequent pregnancy. ANAs were measured by indirect immunofluorescence. The cutoff dilution used was 1:40. In addition, patients were classified according to the ANA pattern on immunofluorescence staining. Live birth rates were compared between ANA-positive and ANA-negative patients after excluding patients with antiphospholipid syndrome, an abnormal chromosome in either partner and a uterine anomaly. Main results and the role of chance The 994 patients were analyzed after excluding 40 with a uterine anomaly, 43 with a chromosome abnormality in either partner and 32 with APS. The rate of ANA-positive patients was 39.2% (390/994) when the 1: 40 dilution result was positive. With a 1:160 dilution, the rate of ANA-positive patients was 3.62% (36/994). The live birth rate was calculated for 798 patients, excluding 196 patients with unexplained RPL who had been treated with any medication. With the use of the 1: 40 dilution, the subsequent live birth rates were 71.34% (219/307) for the ANA-positive group and 70.67% (347/491) for the ANA-negative group (OR, 95%CI; 0.968, 0.707–1.326). After excluding miscarriages with embryonic aneuploidy, chemical pregnancies and ectopic pregnancies, live birth rates were 92.41% (219/237) for the ANA-positive group and 92.04% (347/377) for the ANA-negative group (0.951, 0.517–1.747). Using the 1:160 dilution, the subsequent live birth rates were 84.62% (22/26) for the ANA-positive group, and 70.47% (544/772) for the ANA-negative group (0.434, 0.148–1.273). Subgroup analyses were performed for each pattern on immunofluorescence staining, but there was no significant difference in the live birth rate between the two groups. Limitations, reasons for caution The effectiveness of immunotherapies could not be evaluated. However, the results of this study suggest that it is not necessary. Wider implications of the findings: The measurement of ANA might not be necessary for the screening of patients with RPL who have no features of collagen disease. Trial registration number Not applicable

Clinical and anamnestic data and morphofunctional characteristics of the endometrium in women with uterine developmental anomalies

BACKGROUND: Uterine developmental anomalies have a negative impact on the implementation of female reproductive function, leading to a high risk of reproductive failures, premature birth, placental insufficiency, intrauterine growth retardation syndrome, labor anomalies and postpartum bleeding. The presence of both structural anomalies themselves and the high frequency of reproductive failures leads to repeated intrauterine interventions, which are considered as a premorbid background for the endometrial pathology development. Despite the combined causes of reproductive failure, morphological studies of the endometrium in women with various uterine anomalies are scarce. AIM: The aim of this study was to evaluate the clinical and anamnestic data and morphological characteristics of the endometrium in women with uterine anomalies and reproductive failures in the anamnesis. MATERIALS AND METHODS: We examined 123 women with uterine developmental anomalies (49 patients with an arcuate uterus, 38 patients with a uterine septum, 16 patients with a bicornuate uterus, 10 patients with an unicornuate uterus, 10 patients with dimetria). Standard clinical and laboratory work-up, hysteroscopy with endometrial biopsy and laparoscopy were performed in all patients. Histological and immunohistochemical examination of the endometrium was carried out according to the standard technique with assessment of the relevant receptor profile (estrogen and progesterone receptors) and pro-inflammatory markers (CD8+, CD20+, CD4+, and CD138+). RESULTS: Clinical and anamnestic data evaluation in patients with uterine anomalies revealed menstrual abnormalities, commonly, dysmenorrhea. Regardless of the type of uterine anomaly, a high incidence of pelvic inflammatory disease, endometriosis and a high frequency of reproductive failures were found. The morphological structure of the endometrium with uterine anomalies was characterized by a higher frequency of endometrial hyperplasia, impaired secretory transformation and the presence of chronic endometritis. CONCLUSIONS: Patients with a variety of uterine developmental anomalies are characterized by menstrual irregularities, a high incidence of gynecological pathology and reproductive failures. There is no association between pathognomonic signs of endometrial morphofunctional abnormalities and the type of uterine anomaly; however, they are similar to those seen in recurrent miscarriages and infertility of various origins.

Herlyn-Werner-Wunderlich Syndrome: A Case Report on a Congenital Uterine Anomaly With Literature Review

During fetal development, abnormalities during Mullerian duct formation can lead to varied types of uterine, cervical, or vaginal anomalies. Herlyn-Werner-Wunderlich Syndrome (HWWS) is a rare, congenital Mullerian duct anomaly characterized by the triad of uterine didelphys, obstructed hemi-vagina, and ipsilateral renal agenesis. If the diagnosis is delayed or missed, permanent sequalae such as chronic pain, infection, and infertility can result. This is a case of a 16-year-old woman with heavy vaginal bleeding and menstrual cramping in which sonography was used in the diagnosis of HWWS. Sonography plays a vital role in this diagnosis, as it is the most common initial imaging examination. For this reason, it is important that the characteristics of uterine anomalies, like HWWS, be recognized with sonography. It is important that this diagnosis is made early and treatment can be initiated to prevent irreversible complications.

CASE REPORT ON FINDING OF ABNORMAL PRESENTATION OF FETUS IN A PARTIAL SEPTATE UTERUS DURING CESAREAN SECTION WITH PARTIAL PLACENTAL SEPTAL IMPLANTATION

Congenital uterine anomalies are seen 1-3% of women, usually asymptomatic and therefore unrecognized until desire of childbearing.[1] Uterine septum is the most frequent (35-48%) structural uterine anomaly and associated with the poorest reproductive outcome. Even if association of septum with infertility is not certain, it is well recognized that it worsens obstetric outcomes with high abortion (44%) and preterm delivery rate (22%).[1] In this case report, we aim to present a term pregnancy with successful outcome with placental implantation on uterine septum.