Obstetrical and Peri-Operative Management of Patients with Factor XI Deficiency - a Retrospective Observational Study

Introduction Factor XI deficiency (FXI) is an autosomally inherited injury-related bleeding disorder. Although relatively rare worldwide, it is common amongst Ashkenazi and Iraqi Jewish ancestry with a heterozygosity rate as high as 8 to 9%. FXI deficiency does not provoke spontaneous bleeding; however, it predisposes to a potential risk of life-threatening bleeding at childbirth or surgery. Unfortunately, data regarding obstetrical and perioperative management of this condition is scarce, with less than 500 cases reviewed in the last 20 years. Therefore, this study aimed to expand this database and identify factors associated with increased bleeding risk. Methods We performed a retrospective chart review of patients (pts) with FXI deficiency who underwent childbirth or other surgical procedures between August 2011 to April 2021 within the Mount Sinai Health System in New York City. Data on age, sex, ethnicity, genotype, family or personal history of bleeding, type of anesthesia, estimated blood loss, peri-procedural bleeding complication, and type and timing of blood product or hemostatic agent administered in the peri-operative period were collected. Prior history of bleeding was defined as 1 or more of the following: easy bruisability, epistaxis, heavy menstrual bleeding, bleeding related to dental, surgical or obstetrical procedure. The paired t-test was used to compare the initial and subsequent FXI levels measured during pregnancy. We performed logistic regression to test the association between historical, laboratory, and procedural variables with the bleeding endpoint (defined as acute postpartum or postoperative hemorrhage or any bleeding warranting non-prophylactic administration of packed red blood cells, fresh frozen plasma [FFP], or tranexamic acid). Receiver operative characteristic (ROC) curve was plotted for FXI levels to identify the cutoff for optimal sensitivity and specificity. Analyses were performed using SPSS software. Results We identified 198 pts who underwent 252 procedures in total- including 143 vaginal deliveries, 64 C-sections and 45 other surgical procedures. Mean age was 36 years with 94% females, and ~70% were Ashkenazi Jews. c.403G>T p.E135X (42%) and c.901T>C p.F301L (44.8%) were the most common genotypes identified. 38 out of 252 procedures resulted in bleeding complications. In multivariable logistic regression, both prior history of bleeding (odds ratio (OR) 8.97, p=0.02) and lower FXI levels ( OR 1.03 per U/dL increase, p=0.05) were independently associated with the bleeding endpoint. Family history of bleeding, ethnicity, genotype, pre-procedural PTT and platelet levels were not associated with bleeding risk. There were no cases of epidural or spinal hematomas associated with neuraxial anesthesia in our cohort. Mean FXI level for pts receiving neuraxial anesthesia was 50 U/dl (3-118 U/dl). Five pts who had a negative bleeding history despite surgical challenges received neuraxial anesthesia at FXI level <10 U/dl without any complications (only 1/5 received prophylactic FFP). Mean FXI level for pts receiving prophylactic FFP was 25.6 U/dl (range 1-71 U/dl). 8 out of 21 (38%) pts suffered a bleeding complication despite prophylactic FFP use. ROC analyzing FXI levels as a risk factor for the bleeding endpoint resulted in an AUC of 0.605 with specificity of 96%, 94%, 91%, 83%, 49% and sensitivity of 11%, 12%, 19%, 35%, 65% respectively for cut-off values of 10, 20, 30, 40 and 50 U/dl. Of note, there was no significant variation in FXI levels during pregnancy [mean first measurement was 49.7 U/dl vs final measurement of 48.3 U/dl, p=0.3]. Conclusions Personal history of bleeding is the strongest predictor of perioperative or obstetrical bleeding in pts with FXI deficiency. Higher FXI levels correlate with a slightly lower but statistically significant odds of surgical bleeding. Although a FXI level cut-off of 40 U/dl may predict bleeding risk with reasonable specificity (83%), it lacks sensitivity and must be interpreted in the context of personal bleeding history. FXI levels remain stable during pregnancy and repeat measurements may not be necessary. Neuraxial anesthesia appears to be safe to use in this cohort. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Transhepatic tract hemostasis using thermal-ablation after percutaneous portal vein access

Objectives: Bleeding risk after percutaneous portal vein access procedures is not negligible. Various agents, coils and plug, have been used to minimize this risk, each with their own advantages and disadvantages. This study reports the results of coagulation using thermal-ablation (radiofrequency or microwave ablation) as an alternative to trans-hepatic puncture tract closure. Methods: Ten patients who benefited from portal vein recanalization or portal hypertension-relative bleeding complication embolization using percutaneous portal vein access and who underwent thermal-ablation of the puncture tract between December 30, 2019 and July 16, 2020 were included. Early efficiency and safety were evaluated using imaging (ultrasound and/or CT scan) and laboratory data (hemoglobin, hepatic function) at 24 h. Follow-up was performed until August 2020. Results: No bleeding from the puncture tract and no embolization-related complications were observed in all 10 patients at 24 h or during follow-up with median of 3 months (range 1–8 months), even in case of ascites or therapeutic coagulation. Conclusion: Thermal-ablation seems to be a safe, effective and rapid technique to avoid bleeding after percutaneous transhepatic direct portal vein access. Advances in knowledge: Thermal-ablation could be an alternative for transhepatic puncture tract closure especially for patients with high bleeding risk.

Catecholaminergic Crisis After a Bleeding Complication of COVID-19 Infection: A Case Report

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) presents in some cases with hemostatic and thrombotic complications. Pheochromocytomas are unusual, though potentially lethal tumors. Herein we describe the first case of hemorrhage in a pheochromocytoma related to SARS-CoV-2 infection. A 62-year-old man consulted for syncope, fever, and palpitations. He was diagnosed with SARS-CoV-2 pneumonia and presented with a hemorrhage in a previously unknown adrenal mass, which resulted in a catecholaminergic crisis. Medical treatment and surgery were required for symptom control and stabilization. We hereby alert clinicians to watch for additional/unreported clinical manifestations in COVID-19 infection.

To stop or to continue aspirin prior to manual small incision cataract surgery

To ascertain whether to stop or continue aspirin before cataract surgery. Prospective observational study from July 2018 to June 2019. Information on pre-existing medical conditions like cardiac disease/stroke & use of Aspirin was obtained from patients posted for cataract surgery. Physical examination was done by physician & decided whether aspirin has to be stopped before surgery. Intra-operative and post-operative outcomes were recorded. SPSS 20.0 by using descriptive statistics i.e. only frequency and percentage. Out of 64 patients, 38 discontinued aspirin and 26 continued. In the group of patients who discontinued aspirin, 2 had bleeding complication in the form of bleeding from cut ends of conjunctiva. Where as in the group who continued aspirin 4 patients had bleeding complication. No thromboembolic events were recorded in both the groups. The risk of medical & ophthalmic events surrounding cataract surgery were so low that absolute differences in risk associated with changes in Aspirin use were minimal.

A Case Series of Life-Threatening Hemorrhagic Events in Patients with COVID-19

Since venous microthrombotic and thromboembolic events in end organs have been pathophysiologically confirmed as a component of thrombo-inflammatory cascade in COVID-19 syndrome, anticoagulant prescription with prophylactic or therapeutic goal is recommended. Different guidelines for the above are introduced; however, there is no general consensus on any neither the type of anticoagulant nor for the dosage and duration of prescription. In our medical center, adopted internal guideline was considered for patients COVID-19. We consulted patients with COVID-19 who suffered from concurrent hematoma. Appropriate surgical approach was considered. Finally autopsy study was performed for patients. In this article, we presented a series of seven SARS-CoV-2 confirmed cases faced with bleeding complication following initiation of anticoagulation protocol. The rectus sheath hematoma with extension to pelvic and/or retroperitoneal space, even involving bowel mesentery was seen most commonly. Despite receiving appropriate surgical care, all seven cases died. Finally, in all cases, autopsy studies revealed no evidence for confirmation of DIC/SIC or organ failure as the reason of death although pulmonary involvement with SARS-CoV-2 and bleeding phenomena were approved. The nature of the COVID-19 syndrome makes patients vulnerable to hemorrhagic events following anticoagulant administration which relatively causes or accelerates patient’s expiration.

Iliac Conduits for Endovascular Treatment of Aortic Pathologies: A Systematic Review and Meta-analysis

Purpose: The treatment of thoracoabdominal aortic aneurysm has largely shifted to endovascular techniques. However, severe iliofemoral arterial disease often presents a challenge during these interventions. As a result, iliac conduits have been introduced to facilitate aortic endovascular therapy. The goal of the current study was to gauge utilization and to analyze iliac artery conduit outcomes to facilitate endovascular therapy to treat aortic pathologies. Materials and Methods: A meta-analysis of 14 studies was conducted with the use of random effects modeling. The incidence of periprocedural adverse events was gauged based on iliac conduit vs nonconduit cases and planned vs unplanned iliac conduit placement. Outcomes of interest included length of hospital stay, morbidity and mortality associated to conduits, and all-cause mortality. Results: Iliac conduits, either open or endo-conduits, were utilized in 17% (95% CI: 9%–27%) of 16,855 cases, with technical successful rate of 94% (95% CI: 80%–100%). Periprocedural complications occurred in 32% (95% CI: 22%–42%) of the cases, with overall bleeding complication rate being 10% (95% CI: 5%–16%). Female patients, positive history for smoking, pulmonary disease, and peripheral artery disease at baseline were associated with more frequent utilization of iliac conduits. Conduit use was associated with longer hospitalization, higher periprocedural all-cause mortality (OR: 2.85; 95% CI: 1.75–4.64; p<0.001), and bleeding complication rate (OR: 2.38; 95% CI: 1.58–3.58; p<0.001). Sensitivity analysis among conduit cases showed that planned conduits were associated with fewer periprocedural complications compared to unplanned conduits (OR: 0.38; 95% CI: 0.20–0.73; p=0.004). Conclusion: Iliac conduit placement is a feasible strategy, associated with high technical success to facilitate complex aortic endovascular repair. However, periprocedural adverse event rate, including bleeding complications is not negligible. All-cause mortality and morbidity rates among cases that require iliac conduits should be strongly considered during clinical decision making. High-quality comparative analyses between iliac conduit vs nonconduit cases and between several types of iliac conduit grafts aiming at facilitating endovascular aortic repair are still needed to determine the best strategy to address challenging iliac artery accesses.