Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management.

Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions.

Pregnancy outcomes in systemic lupus erythematosus (SLE) women.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition with multi-organ involvement predominantly affecting young women. There are very limited studies in pregnancy in Asian SLE patients and therefore we embarked on this study to identify pregnancy outcomes of Malaysian women with SLE. Materials and methods We performed a retrospective study of pregnancy outcomes in SLE patients in our institution from January 2007 to December 2014. A total of 71 pregnancies from 44 women were analysed. Results The mean age of our cohort was 30.5 ± 3.9 years. The rate of active disease at conception, antiphospholipid syndrome and lupus nephritis were 22.5%, 32.4% and 57.7% respectively. SLE flare occurred in 33 out of 71 pregnancies whereas 19 pregnancies were complicated with preeclampsia. The livebirth rate for our cohort was 78.9%, whilst preterm delivery was 42.9%. On univariate analysis, active disease and flare in pregnancy were both strongly associated with foetal loss and preterm delivery. Lupus nephritis (p = 0.011), SLE flare (p = 0.008) and antiphospholipid syndrome (p = 0.032) significantly increased the risk of preeclampsia. Aspirin and hydroxychloroquine were protective against foetal loss [odds ratio (OR) 0.12] and preeclampsia (OR 0.25), respectively. On multivariate analysis, active disease was a predictor of SLE flare (p = 0.002) and foetal loss (p = 0.018) and SLE flare was the main predictor of preterm delivery (p = 0.006). Conclusions Pregnancies in women with SLE should be planned and aspirin and HCQ use were beneficial in reducing adverse pregnancy outcomes.

Sodium pentosan polysulfate efficacy as thromboprophylaxis agent in high-risk women following gynecological surgery.

AIM: Sodium pentosan polysulfate (Na-PPS) is a plant-based agent that has similar action with low-molecular-weight heparin. It inhibits factor Xa, preventing blood clot formation. To date, its use in clinical practice as thromboprophylaxis agent is still limited. In addition, the efficacy and safety profile of this agent was not robustly reported globally, especially for countries with major Muslim population. We hypothesized that Na-PPS was equally effective as the standard thromboprophylaxis. We aim to compare the efficacy and safety of Na-PPS against standard agent (fondaparinux or enoxaparin). METHODS: This was a randomized control, open-label trial. Women underwent major gynecological surgery were randomized to receive either subcutaneous 50 mg of Na-PPS twice daily or subcutaneous enoxaparin 40 mg once daily. Fondaparinux 2.5 mg once daily was given to Muslim women as an alternative to enoxaparin. The treatment was started 6 h postoperatively, for at least 3 days. All the patients received thromboembolic deterrent stockings. The primary efficacy outcome was venous thromboembolism up to 3 days postsurgery. The main safety outcomes were minor and major bleeding. RESULTS: Among 109 participants, there was no incidence of venous thromboembolism. None of the women developed major bleeding. Minor bleeding was observed in 28.3% (15/53) and 5.4% (3/56) of Na-PPS and standard thromboprophylaxis group, respectively (P = 0.001). CONCLUSION: Na-PPS was associated with increased risk of minor bleeding. There was insufficient data to conclude its efficacy as thromboprophylaxis. Further research is needed to evaluate Na-PPS safety as a standard thromboprophylactic agent.