Adequate Hemostasis Research Articles

Predictive haemostatic biomarkers and transfusion efficacy, insights from fresh frozen plasma use in surgical patients, preliminary results

The aim of the study was to examine various haemostasis values to identify the most relevant biological indicators for detecting significant haemorrhage, to determine the effectiveness of fresh frozen plasma (FFP) transfusion. Our findings suggest that a low prothrombin time, elevated Von Willebrand Antigen, increased plasma fibrinogen, and reduced Ca2 + levels are associated with challenges in achieving proper haemostasis. However, measurements of factors II, V, VII, VIII, IX, X, XI, XIII, protein C, and protein S do not appear to be linked to difficulties in achieving adequate haemostasis. Additionally, the administration of FFP appears to impact factors V, VII, X, and II.Trial registration EudraCT number: 2019-002898-64.

Percutaneous decannulation of extracorporeal membrane oxygenation using MANTA device: A real-world single-center experience.

The MANTA vascular closure device (VCD) represents a novel approach to achieving hemostasis after large-bore femoral access procedures. Numerous clinical studies have evaluated the efficacy of the MANTA device across a range of patient populations undergoing different procedures. However, there is still a paucity of data available concerning the use of MANTA devices in aiding the decannulation of venoarterial extracorporeal membrane oxygenation (VA-ECMO). To present our single-center experience of utilizing the MANTA VCD in patients undergoing this procedure. This single-center study included all patients undergoing percutaneous decannulation of femoral VA-ECMO using the MANTA plug-based VCD between January 2021 and October 2023 at University Hospitals Cleveland Medical Center. Inclusion criteria were adult patients who required prolonged (> 24 hours) hemodynamic support with VA-ECMO. Outcomes included all-cause mortality, hemostasis, bleeding, limb ischemia, and site infection. This is a retrospective cohort study of 19 patients with a mean age of 56.8 years. Twelve of them were males with a mean body mass index of 29. The most common extracorporeal membrane oxygenation indication was acute coronary syndrome complicated by cardiogenic shock at 36.8%. The mean length of intensive care unit stay for these patients was 18.8 ± 8.42 days. Seventeen out of 19 patients survived to discharge. The MANTA device was successfully deployed in 19 patients, with 10 procedures conducted at the bedside and 9 in an operating room setting. Complete hemostasis was achieved within 5 minutes of MANTA deployment in 17 out of 19 patients. In 2 patients manual compression after Manta deployment was required to achieve adequate hemostasis. Additionally, acute lower extremity ischemia was noted in two patients, necessitating endovascular interventions. No infections were reported at the site of MANTA deployment. Overall, based on our experience and that of other centers, the MANTA VCD has proven to be a simple, safe, and effective percutaneous technique for facilitating in the OR, but most of all it opens the opportunity for bedside VA-ECMO decannulation. Post-decannulation ischemic complications are higher in this series of sick patients when compared with elective procedures like transcatheter aortic valve replacement and endovascular aneurysm repair. Additionally, operators should be mindful of the incidence of ischemic complications. Distal Doppler pulse signals should always be checked, to indicate bailout options when this occurs.

Traumatic Middle Meningeal Artery Aneurysm: A Rare Cause of Recurrent Acute Epidural Hematoma. A Case Report

AbstractTraumatic middle meningeal artery aneurysm (TMMA) is a rare condition and a known cause of several different bleeding patterns after head injury. Once detected, they need to be treated as an emergency due to their potential for morbidity and mortality. Generally, recurrence does not occur in surgery for acute epidural hematoma if adequate hemostasis is achieved. Here, we report a case of atypical postoperative recurrence of an acute epidural hematoma, possibly due to the development and rupture of a TMMA. A 41-year-old man with left acute epidural hematoma after a head injury was referred to our hospital. Emergency craniotomy was performed immediately, and the hematoma was removed. The source of the bleeding was near the fracture site in the middle cranial fossa, and sufficient hemostasis was confirmed. However, a head computed tomography (CT) scan the next day revealed a recurrence of the acute epidural hematoma. Magnetic resonance (MR) angiogram showed an aneurysm with a diameter of approximately 4 mm in the left middle meningeal artery. The recurrence of the acute epidural hematoma appeared to be related to the formation and a rupture of a middle meningeal artery aneurysm, and to prevent subsequent rebleeding, the patient underwent reoperation, and the hematoma and aneurysm were removed. In surgery for acute epidural hematoma, recurrence can be prevented by removing the hematoma and ensuring hemostasis. Although conventional surgery was performed in this case, a repeat of epidural hematoma occurred. A postoperative middle meningeal artery aneurysm had been thought to have developed, ruptured, and caused a repeat epidural hematoma.In treating acute epidural hematoma, a TMMA development should be considered when an atypical clinical course occurs, such as a recurrence of postoperative bleeding.

Hepatic and splenic venous access tract closure using the VASCADE vascular closure system following percutaneous intervention in patients with CHD.

Hepatic and splenic venous access are specialised techniques used to perform diagnostic and interventional procedures in the cardiac catheterisation laboratory. Bleeding events are the most commonly reported complication following hepatic or splenic venous access. The VASCADE Vascular Closure System (Cardiva Medical Inc. Santa Barbara, CA) is an approved device for closure of femoral vascular access tracts in patients ≥18 years of age. We report our experience using VASCADE to close the hepatic or splenic venous access site in the cardiac catheterisation laboratory. This is a single centre retrospective review of all patients who had percutaneous hepatic or splenic venous access obtained in the cardiac catheterisation laboratory from March 1, 2022 through October 30, 2023 and underwent tract closure with VASCADE. Ten patients (six male) underwent 16 procedures (median age and weight 3.5 years and 12.5 kg) with 15 hepatic and two splenic veins accessed. Successful closure of the access tracts with VASCADE was performed in all patients. There were no major adverse events related to closure of the access sites with VASCADE. VASCADE can be used following transhepatic and trans-splenic venous access in the cardiac catheterisation laboratory to safely close the access tract and potentially reduce the risk of post-procedural bleeding complications. Further evaluation in a larger cohort of patients is needed to ensure VASCADE is safe for use and provides adequate haemostasis following hepatic or splenic venous access, particularly in children.

Diagnosis and management of factor XI alloinhibitors in patients with congenital factor XI deficiency-A large single-centre experience.

Factor (F) XI deficiency is an inherited bleeding disorder with increased prevalence in Ashkenazi Jews where it is mainly caused by two variants, p.Glu135* (type II, leading to a null allele) and p.Phe301Leu (type III, missense variant). Inhibitor development is rare, and only seen in severe FXI deficiency (<20IU/dL) upon exposure to plasma-based products. We report our experience of a large cohort of patients with severe FXI deficiency, including seven patients who developed FXI alloinhibitors, their presentation, natural history and subsequent perioperative management. A single-centre retrospective database review of patients with FXI deficiency, including those who have subsequently developed inhibitors, and extraction of clinical, laboratory and genotype data, including operative management records. A total of 682 patients were identified with FXI deficiency, of whom 113 had FXI<20IU/dL and 42 had FXI≤1IU/dL. Factor XI inhibitors were seen in seven patients, six of whom were homozygous for the type II variant (prevalence of inhibitor with this genotype of 30%, risk of inhibitor upon plasma exposure 50%). FXI inhibitors were not seen, despite similar exposures, in patients with other genotypes. No alteration in bleeding phenotype occurred after inhibitor development and subsequent surgery was managed on 13 occasions with recombinant factor VIIa (rFVIIa), including low doses (15-30µg/kg), with good haemostasis. The inhibitor spontaneously disappeared in four of seven patients over 1-22 years. FXI inhibitors were only observed in severe FXI deficient patients homozygous for p.Glu135* (null allele) upon plasma or FXI concentrate exposure, with a 30% prevalence. The bleeding phenotype was not altered and inhibitors may disappear with time. Adequate haemostasis in the perioperative setting is achievable with low doses of rFVIIa.

Review of Inherited Coagulation Disorders.

Most invasive dental procedures elicit some degree of bleeding which ultimately leads to clotting and eventual hemostasis. However, patients with inherited coagulation disorders may exhibit prolonged or, in some cases, excessive bleeding requiring multiple perioperative interventions. Von Willebrand disease is the most common inherited coagulopathy and often manifests via easy bruising, epistaxis, or prolonged bleeding. Hemophilia A (factor VII) and B (factor IX) are factor deficiencies that are clinically indistinguishable and managed according to severity and the required dental treatment. Other coagulopathies are rare (ie, inheritance is autosomal recessive) and may only become evident in homozygotes or compound heterozygotes. Current lab values and medical consultation with the patient's hematologist are imperative prior to rendering invasive dental treatment. There are a myriad of sedation and general anesthesia considerations, including risks for epistaxis with nasal instrumentation and bruising with improper patient positioning. Preoperative treatment with desmopressin or factor replacement may be required and generally should facilitate normal hemostasis. Additional therapies should be considered to help ensure adequate postoperative hemostasis, including pressure dressings, resorbable clotting materials, laser therapy, and oral rinses.

Radial Artery Compression Comparison for Hemostasis with and without Steri-Strips Reinforcement: RACC Randomized Trial

Objectives: This study assessed the efficacy of using steri-strips in conjunction with conventional pneumatic trans-radial (TR) bands for achieving radial artery hemostasis after diagnostic coronary angiography (CA). With varying methods aimed at reducing compression duration post-CA via trans-radial access (TRA), our aim was to evaluate time dynamics. Methodology: We randomly assigned 209 patients in a 1:1 ratio to receive either the TR band with steri-strips (treatment) or conventional TR band alone (control) post-diagnostic CA, and followed them for one month. The primary endpoint was time to achieve adequate hemostasis, defined by the total TR band application duration. Secondary endpoints included radial artery occlusion (RAO) and major bleeding. The treatment group comprised 106 patients with steri-strips alongside the TR band, while the control group had 103 patients with the TR band alone. Median TR band application duration was 60 [IQR: 60-60] min in the treatment group and 250 [IQR: 240-360] min in the control group; p≤0.001. Results: The incidence of site bleed requiring TR band re-application was 9.4% vs. 4.9%, p=0.364, and hematoma (type I only) was 7.5% vs. 9.7%, p=0.578 in the treatment and control groups, respectively. Radial artery palpability after 24 hours was 95.3% vs. 85.4%; p=0.016, and at one month it was 96.2% vs. 88.3%; p=0.032 in the treatment and control arms, respectively. Conclusion: Steri-strips significantly reduce TR band compression duration and improve radial artery patency post-diagnostic angiography without causing hematoma or major bleeding complications.

Dexmedetomidine and surgical field visibility in nasal surgery: A systematic review and meta-analysis.

Nasal and sinus surgery, especially using endoscopy, relies upon adequate haemostasis to be safe and effective. Often other haemostatic methods, such as cautery are not viable, and other methods must be employed. This study examines the effectiveness of dexmedetomidine in controlled hypotension and for surgical field visibility in endoscopic sinus surgery and other nasal surgeries. A literature search was conducted in PubMed, Scopus, CINAHL and Central for randomised controlled trials using dexmedetomidine for controlled hypotension in adult patients undergoing endoscopic sinus surgery or other nasal surgery. Meta-analysis of mean differences and single means were performed. Of 935 identified studies, 31 met the inclusion criteria. A statistically significant difference in Fromme-Boezaart surgical field visibility scores was found comparing dexmedetomidine to placebo (p < 0.00001) and propofol (p < 0.0001), but not other agents. A significant difference in intraoperative blood loss volume was found compared with placebo (51.5mL, p < 0.00001) and propofol (13.6mL, p < 0.0001), but not other agents. Dexmedetomidine demonstrated significantly improved surgical field visibility and blood loss volume compared with placebo and propofol, but not other agents. Dexmedetomidine is viable and useful for controlled hypotension in nasal surgery. Choice of controlled hypotension agent should follow patient and procedure-specific considerations.

Modified Dunn Osteotomy for Moderate and Severe Slipped Capital Femoral Epiphysis

Background: Moderate to severe slipped capital femoral epiphysis is a condition that benefits from treatment with modified Dunn realignment osteotomy and fixation 1,2 . The results are acceptable when the procedure is performed by experienced surgeons 3,4 . Description: The patient is placed in the lateral decubitus position. Surgical hip dislocation is performed after a digastric trochanteric osteotomy. An extended retinacular flap is carefully prepared, and the epiphysis separated from the metaphysis, exposing the entirety of the femoral neck. Following this, an open reduction is performed by excising the posteromedial callus and trimming the femoral neck in order to allow capital realignment while avoiding tension on the retinacular vessels. Finally, the reduced slip is fixed with 6.5 or 7-mm cancellous screws and Kirschner wires. The femoral head is then repositioned in the acetabulum, and the trochanter is fixed distal to its original position with use of 3.5-mm self-tapping cortical screws. Closure is performed in layers after achieving adequate hemostasis. Alternatives: Operative alternatives to this technique include cuneiform osteotomy, compensatory osteotomy of the base of the neck or intertrochanteric osteotomy and controlled repositioning, and osteoplasty 5–7 . Osteotomies performed distally and away from the deformity can still lead to cam impingement, thereby failing to reduce the risk of future joint degeneration. In situ pinning has also been described for use in moderate to severe cases of slipped capital epiphysis, but the residual deformity leads to substantial femoroacetabular impingement and thus affects the prognosis of such a hip 8 . Rationale: This technique accounts for the complex anatomy of the hip joint, especially in terms of its precarious blood supply. Long-term studies have shown that corrective osteotomy performed near the physis can correct the alignment at the head-neck junction, thereby avoiding future femoroacetabular impingement. In the hands of experienced surgeons, results have been shown to be favorable 3,4 . Expected Outcomes: In a series of 30 consecutive hips, including 20 moderate and 10 severe slips, the slip angle improved significantly following the modified Dunn procedure (by 43.63° ± 8.42°; p &lt; 0.001). Osteonecrosis was observed in 2 hips (6.7%), and 1 hip had postoperative subluxation that required intervention 9 . In their series of 23 patients, Slongo et al. encountered 2 cases (9%) of osteoarthritis and osteonecrosis 10 . In their series of 40 patients from 2 different institutions, Ziebarth et al. reported no cases of chondrolysis or osteonecrosis 11 . Important Tips: The steps described need to be followed sequentially and meticulously in order to safely dislocate the hip while preserving the blood supply.The availability of an anterior pouch in draping, to deliver the leg during dislocation, will reduce the dependence on an assistant and maintain sterilityThe trochanteric osteotomy should be started with the help of a saw, completed with an osteotomy and must be about 1 to 1.5 cm thick in order to achieve good, stable fixation following relocation of the femoral head and to achieve good abductor function post-relocation.The use of curved, stout, thick scissors (“episiotomy scissors”) can help to ease the step of cutting the ligamentum teres during dislocation.The retinacular flap should be widely exposed along with removal of the posterior callus so that no tension remains on the blood supply. Acronyms and Abbreviations: SCFE = slipped capital femoral epiphysisFAI = femoroacetabular impingementMDP = modified Dunn procedureAVN = avascular necrosisTHR = total hip replacementFAER = flexion, abduction and internal rotationCT = computed tomographyMRI = magnetic resonance imagingAP = anteroposterior

First worldwide report on rectal resections with Hugo™ surgical system: description of docking angles and tips for an effective setup.

Rectal robotic surgery gained momentum in the last decade, but it is still associated with not-negligible costs. In order to reduce costs, recently different robotic systems have received approval for clinical use. This study aims to present the first case series of rectal resection with the novel cost-effective platform Robotic Assisted Surgery (RAS) Hugo™. Tips for effective set up of the system and detailed configuration of tilt and docking angles are also provided. Three cases of rectal resection with Hugo RAS™ system are reported. After the first two cases of resection with partial mesorectal excision in which surgeries were performed with the setup proposed by the vendor company, in the third case we tested a novel setup that allowed a full robotic low rectal resection performing vascular ligations, TME and colonic splenic flexure mobilization without the need of any de-docking. Our first three robotic rectal resections with the Hugo RAS™ system were completed without complications with a median docking time of 12 min (range 8-15) and a median console time of 345 minutes (range 271-475). In the first two cases, hybrid robotic and laparoscopic surgeries were performed to obtain an adequate haemostasis and traction during the pelvic phase. In the third case, a full robotic TME was successfully accomplished. Our experience demonstrates that a full robotic low rectal resection with TME with Hugo™ RAS system is feasible, safe and associated with satisfactory postoperative outcomes.

Unlocking the potential of fondaparinux: guideline for optimal usage and clinical suggestions (2023).

Background: Thromboembolic disease is associated with a high rate of disability or death and gravely jeopardizes people's health and places considerable financial pressure on society. The primary treatment for thromboembolic illness is anticoagulant medication. Fondaparinux, a parenteral anticoagulant medicine, is still used but is confusing due to its disparate domestic and international indications and lack of knowledge about its usage. Its off-label drug usage in therapeutic settings and irrational drug use are also common. Objective: The aim of this guideline is to enhance the judicious clinical application of fondaparinux by consolidating the findings of evidence-based research on the drug and offering superior clinical suggestions. Methods: Seventeen clinical questions were developed by 37 clinical pharmacy experts, and recommendations were formulated under the supervision of three methodologists. Through methodical literature searches and the use of recommendation, assessment, development and evaluation grading techniques, we gathered evidence. Results: This guideline culminated in 17 recommendations, including the use of fondaparinux for venous thromboembolism (VTE) prevention and treatment, perioperative surgical prophylaxis, specific diseases, special populations, bleeding and overdose management. For different types of VTE, we recommend first assessing thrombotic risk in hospitalized patients and then administering the drug according to the patient's body mass. In surgical patients in the perioperative period, fondaparinux may be used for VTE prophylaxis, but postoperative use usually requires confirmation that adequate hemostasis has been achieved. Fondaparinux may be used for anticoagulation prophylaxis in patients hospitalized for oncological purposes, in patients with atrial fibrillation (AF) after resuscitation, in patients with cirrhosis combined with portal vein thrombosis (PVT), in patients with antiphospholipid syndrome (APS), and in patients with inflammatory bowel disease (IBD). Fondaparinux should be used with caution in special populations, such as pregnant female patients with a history of heparin-induced thrombocytopenia (HIT) or platelet counts less than 50 × 109/L, pregnant patients with a prethrombotic state (PTS) combined with recurrent spontaneous abortion (RSA), and children. For bleeding caused by fondaparinux, dialysis may partially remove the drug. Conclusion: The purpose of this guideline is to provide all healthcare providers with high-quality recommendations for the clinical use of fondaparinux and to improve the rational use of the drug in clinical practice. Currently, there is a lack of a dedicated antidote for the management of fondaparinux. The clinical investigation of activated prothrombin complex concentrate (APCC) or recombinant activated factor VII (rFⅦa) as potential reversal agents is still pending. This critical gap necessitates heightened scrutiny and research emphasis, potentially constituting a novel avenue for future inquiries into fondaparinux sodium. A meticulous examination of adverse events and safety profiles associated with the utilization of fondaparinux sodium will contribute significantly to a more comprehensive understanding of its inherent risks and benefits within the clinical milieu.

Comparative study on the technique and efficacy of microscope-assisted MI-TLIF and naked-eye MI-TLIF in lumbar revision surgery

BackgroundLumbar revision surgery can be performed by simple lumbar nerve decompression or lumbar interbody fusion, including percutaneous endoscopic lumbar discectomy, transforaminal lumbar interbody fusion (TLIF), etc. However, lumbar revision surgery is very difficult in surgical operation. We sought to explore the technique safety and efficacy of microscope-assisted minimally invasive transforaminal lumbar interbody fusion (MI-TLIF) in lumbar revision surgery.MethodsCases of postoperative recurrence following lumbar spine surgery (n = 63) treated from December 2016 to July 2021 were retrospectively analyzed, including 24 cases of microscope-assisted MI-TLIF (microscopic group) and 39 cases of naked-eye MI-TLIF (naked-eye group). The operation time, intraoperative blood loss, incision length, postoperative drainage, length of hospital stay, initial operation, and visual analog score (VAS) of low back and leg pain before and at 7 days and 3 months after the operation and the last follow-up were compared between the two groups. The Oswestry Dysfunction Index (ODI) and the Japanese Orthopaedic Association (JOA) scores before and after the operation and the Bridwell interbody fusion grades at 1 year were compared. The independent t tests, Mann–Whitney U tests, and Chi-square tests were used for analysis.ResultsAll 63 patients were successfully treated by operation and were followed up for an average of 31.5 ± 8.6 months (range 12–48 months). The two groups had no significant difference in sex, age, incision length, initial operation, or operative segment (P > 0.05). There was no significance in operation time, VAS score, ODI score, and JOA score of low back pain or Bridwell interbody fusion grade between the two groups (P > 0.05). Significant differences in intraoperative blood loss, postoperative drainage, and the lengths of hospital stay were observed between the two groups (P < 0.05). Cerebrospinal fluid leakage (n = 2), edema of nerve roots (n = 2), and incision infection (n = 1) were observed in the naked-eye group. There were no complications in the microscopic group, such as cerebrospinal fluid leakage, edema of nerve roots, and incision infection.ConclusionAlthough microscope-assisted MI-TLIF and naked-eye MI-TLIF are both effective during lumbar revision surgery, microscope-assisted MI-TLIF brings less trauma, less bleeding, shorter postoperative hospital stay, and faster recovery. Unlike traditional surgery, microscope-assisted MI-TLIF provides a clear visual field, adequate hemostasis, and nerve decompression.

A Comparative Review of Two Major Topical Hemostasis Agents in Spine Surgery

Achieving adequate hemostasis in spine surgery is critical for both the patient and surgeon. Currently, there are two major topical hemostatic matrix agents used in spine surgery—Floseal (Baxter Healthcare, Deerfield, Illinois, USA) and Surgiflo (Ethicon Inc., Raritan, New Jersey, USA). While both products have been shown to be effective at achieving hemostasis, there is no consensus supporting the use of one agent over another in spine surgery. There are studies comparing outcomes and cost between Floseal and Surgiflo, but a significant portion of the studies have industry affiliations. Generally, existing literature reports low to moderate cost savings in favor of one agent or the other, with no clinically significant differences in other patient-based outcomes such as postoperative hematomas. Animal model studies also suggest unique complications, including epidural fibrosis and associated nerve root compression with use of a flowable topical hemostatic agent. Further non-industry-affiliated studies are warranted to compare efficacy, patient outcomes, cost analyses, and possible unique complications associated with the two hemostatic agents.

Novel amino-acid based metal–organic frameworks (Bio-MOFs) as hemostatic agents: Unveiling promising frontiers in hemostasis

Haemorrhage remains a significant challenge in many medical emergencies. The coagulation cascade involves a complex series of reactions in the body to form blood clots, preventing excessive bleeding. Adequate and rapid hemostasis is critical to avoid excessive blood loss and improve patient outcomes. Various hemostatic agents are used in clinics which are known to enhance the innate hemostatic capability of the body, however, the effort to find better agents continues. The main component of the coagulation cascade involves the participation of certain metals (Ca2+, Mg2+, Zn2+) and amino acids (Glycine, Lysine and L-glutamic acid), which play a significant role in tightly controlling it. In this hypothesis, we propose to prepare amino acid-based bio-compatible metal–organic frameworks (AA-Bio-MOFs) where we intend to combine the endogenous amino acid as organic linkers and the biocompatible metal as nodes creating a well-defined structure. We expect these BioMOFs to be better haemostatic agents than traditional haemostatic materials due to their synergistic effect on the coagulation cascade and the significant reduction in their toxicity. Therefore, integrating amino acids and metal ions within a bio-compatible metal–organic framework (BioMOFs) could potentially add a new paradigm to the field of hemostasis.

THE FEATURES OF ENDOVASCULAR SURGERY FOR DUODENAL ULCER BLEEDING

Aim of the research was to study improvement of the results of surgical treatment of duodenal ulcer bleeding based on the use of endovascular embolization. Gastrointestinal bleeding is a relatively common condition with a wide range of underlying causes. In most cases, this acute bleeding is effectively managed by conservative, medical or endoscopic procedures. However, the proportion of endoscopically unrecognized or controlled non-variceal gastrointestinal bleeding still requires alternative, sometimes surgical, treatment. The current S2k guideline Gastrointestinal Bleeding gives importance to interventional radiology in considering its minimally invasive endovascular interdisciplinary therapy options, guideline-oriented endovascular treatment of Gastrointestinal bleeding by embolization and implantation of covered stents is a treatment approach with good technical and clinical success rates and low complication rates. Gastrointestinal bleedings (GIBs) are pathological conditions associated with significant morbidity and mortality. Embolization without angiographic evidence of contrast media extravasation is proposed as an effective procedure in patients with clinical and/or laboratory signs of bleeding. The purpose of this systematic review is to define common clinical practice and clinical and technical outcomes of blind and preventive embolization for upper and lower gastrointestinal bleeding. Knowledge of vascular anatomy is essential to achieve adequate hemostasis. Endovascular embolization dramatically reduces the mortality rate in high-risk patients who require open surgery after failed endoscopy, further studies are needed to fully address these objectives.

Hemophilic Pseudotumor of Paranasal Sinuses in a Child with Severe Hemophilia a Receiving Emicizumab: A Case Report

Emicizumab, a bispecific humanized monoclonal antibody, bridges activated factor IX (FIX) and FX to restore the function of missing activated FVIII in individuals with hemophilia A (PwHA). Prophylaxis with subcutaneous emicizumab promotes adequate hemostasis in PwHA. Emicizumab prophylaxis in children with hemophilia A has been approved in the United States for use in PwHA ages newborn and older, with or without FVIII inhibitors. We report a 2-year-old boy with severe congenital hemophilia A who initially received prophylaxis with standard half-life factor VIII but later switched to subcutaneous emicizumab at 6 mg/kg every four weeks due to difficult IV access and parental preference. Shortly after starting emicizumab, the patient presented to the hospital with severe episodic epistaxis from the left nostril, requiring ICU admission and ENT consultation. Despite treatment, the condition did not improve, prompting the need for imaging studies. Subsequently, the patient developed ipsilateral eye proptosis and a mass protruding from the left nostril. CT and MRI imaging of the orbit revealed an expansible soft tissue mass measuring 2 x 2.5 x 4 cm occupying the right sphenoid sinus, extending to the nasal cavity. The mass had caused erosion of the clivus and the anterior cranial fossa floor, extending to the middle cranial fossa (Figures 1 &amp; 2). He underwent endoscopic nasal debulking of the left nostril mass, and the pathological examination revealed a cystic mass with a thick capsule and intra-cystic blood clots, consistent with the characteristics of a hemophilic pseudotumor (HP). The fibrous stroma contained hemosiderin-laden macrophages with no evidence of malignancy. Emicizumab was discontinued before the operation, and the patient received several doses of recombinant FVIII to maintain hemostasis. Despite the debulking procedure, the patient continued to experience episodic epistaxis and joint bleeding. An inhibitor assay was performed at that time and showed high levels, leading to a switch to on-demand FVIII treatment for hemophilia A with an inhibitor. Managing the bleeding episodes with bypassing agents proved challenging, and the family expressed dissatisfaction. A decision was made to resume emicizumab treatment, and the patient eventually stabilized, experiencing no bleeding for nearly six months. Additionally, he regularly follows up with ENT specialty, with no further intervention or progression of the tumor. Notably, he did not receive any bypassing agents concomitantly with emicizumab. HP is characterized by encapsulated blood clots accumulating in extravascular tissues, especially the musculoskeletal system, in patients with bleeding tendencies, particularly hemophiliacs. It is a benign, expansible, soft tissue-like mass that exerts pressure on surrounding structures, mimicking a locally invasive malignancy. HP is a hematoma that grows and causes bone erosion through repeated bleeding episodes. A literature review revealed that HP is a rare hemorrhagic complication that develops in 1-2% of patients with severe hemophilia (Magallón et al. Am J Hematol. 1994). Its occurrence in patients receiving emicizumab prophylaxis is extremely rare. The first reported case involved a Japanese child with hemophilia who presented with a pseudotumor occupying the left maxillary sinus after repeated episodes of epistaxis. The mass caused a downward deviation of the ipsilateral eye and bulged into the buccal cavity. The patient received monthly prophylaxis with emicizumab at 6 mg/kg (Kawahara et al. Int J Hematol. 2022), but unlike our patient, he received bypassing agents concomitantly. Our reported case is unique because HP developed independently of bypassing agent therapies, as the patient did not receive any bypassing agents before the tumor's development. The likely explanation is related to repeated bleeding insults to the same anatomical location, leading to the cumulative development of hematoma and HPs. Another possibility could be attributed to the known adverse thrombotic effects of emicizumab treatment. Physicians treating hemophilia should remain vigilant for abnormal clinical signs, such as new masses in patients receiving emicizumab, which could indicate HP.

Using of Emicizumab in Pediatric Hemophilia a Patients: KSA Single Center Experience

Introduction Emicizumab, a bispecific monoclonal antibody plays a crucial role in restoring adequate hemostasis by acting as a bridge between factors IX and X, effectively simulating the activity of factor VIII. Emicizumab is approved in several countries for bleeding episode prophylaxis in patients with HA with or without FVIII inhibitors regardless of severity. This study aimed to report results from an assessment of tolerability, efficacy, and safety of Emicizumab prophylaxis in pediatric patients with HA at a single center in Saudi Arabia. Methods This study was a retrospective study conducted between April 2019 and July 2023 at a Center for Blood Disorders for Pediatric Patients, Jeddah, Saudi Arabia. Eligible study participants were individuals &amp;lt; 16 years of age, weighing over 3 kg, presenting severe or moderate congenital hemophilia A with or without FVIII inhibitors and those who were treated with Emicizumab for at least 3months at our center. We decided to administer Emicizumab to patients with inhibitors, poor venous access, especially those of younger ages,and those with repeated breakthrough bleeds despite regular prophylaxis with FVIII concentrates. Parental drug preference was considered and discussed in all cases. The initial 2 loading injections of Emicizumab (3 mg/kg once weekly for four weeks) were administered at the center, and the patients were observed for 1 h for signs of any acute adverse events. Following discharge, the caregivers of the patients were educated about proper medication administration techniques and instructed to contact our center in case of any adverse events. Annual bleeding rate (ABR) were calculated at baseline pre and post-Emicizumab administration.Weight-based Emicizumab dosage adjustments were made at the end of each patient visit. Results 23 children with HA who started Emicizumab was initially included. A total of 6 patients were excluded as they didn't met the eligible criteria.10 patients with severe hemophilia (59%) and 7 patients with moderate hemophilia (41%). Patient were followed for median duration range of 217 weeks (15-232 weeks). All study patients were males and median (range) age at enrollment was 8.0 (11 months to 16 years).All of them were treated previously with factor either on demand or as prophylaxis. Two patients presented with inhibitors, while the rest were the non-inhibitor type. Among these patients, 2 received a maintenance dose of 3 mg/kg of Emicizumab every 2 weeks, while 16 patients received a monthly dose of 6 mg/kg. Selection of regimen was based guardian preference and also approximate the dose to available drug concentration to avoid drug wastage. Prior to enrollment, 9 patients had target joint involvement; among theses patients, 6 patients demonstrated improvement after Emicizumab initiation (66% reduction), while 3 patients still had persistent target joint involvement. The initial baseline ABRs median (Range) was 48 (Range 5-120). Compared to the ABRs after initiation of Emicizumab which showed median of 2 (Range 0-18), the ABR mean value decreased from 38.5 at baseline to 4.6 after Emicizumabtreatment. All bleeding events were traumatic and resolved with less than 48 h. one or two doses of Factor was/were required in four cases while bleeding was self-limited in others. 11 (64.7%) patients experienced zero bleeding. We observed unusual side effect of asymptomatic persistent neutropenia was observed in 10 (58.8%) out of 17 patients. The absolute neutrophil count (ANC) ranged from 0.6 to 1.45 cells/µL in these cases. There was no other adverse event was observed. Two patients underwent a tooth extraction without post-surgery bleeding, and neither required factor VIII treatment. Additionally, one patient underwent facial laceration repair and received a prophylaxis dose of factor VIII, leading to a successful outcome without any bleeding during and after the procedure. Conclusion Our study demonstrates the effectiveness of Emicizumab prophylaxis for treating patients with moderate to severe HA.The participants reported high adherence to the treatment, improved satisfaction rates and the concept of subcutaneous injections was well accepted. Although neutropenia is considered a unusual side effect of this treatment, the treatment progressed smoothly and favorable safety profile was observed.

The Narrative of an Aberrant Pericardiocentesis Catheter in the Heart: A Case Report on the Surgical Management of an Iatrogenic Left Ventricle Perforation

Pericardiocentesis catheter placement is a procedure for treating pericardial effusion and has a low complication rate. Inadvertent intraventricular perforation of a pericardiocentesis catheter is an uncommon yet a known complication of the procedure. Failure to establish adequate hemostasis during the removal of the foreign body could be catastrophic. Generically, puncture of a pericardiocentesis catheter into the ventricular space is tolerable, but the failure to achieve hemostasis in the ventricular wall amounts to injurious effects accounted to the size and caliber of the drain. Here we describe a case in which a pigtail catheter (8F) accidentally penetrated the left ventricle while attempting to drain pericardial effusion. The patient was brought to the surgical theater where the cardiac surgeon performed an off-pump left anterolateral thoracotomy and extracted the drain successfully. Pledgetted sutures (Prolene 3-0) were placed under tension and the knots were placed, thereby achieving excellent hemostasis of entry point of perforation. During the postoperative course, neither the x-ray of the chest nor an echocardiograph demonstrated hemothorax or pericardial tamponade. Five days later, the patient was discharged from our hospital.

Clip migration after minimally-invasive surgical procedures

Introduction. Today absorbable suture material as well as plastic nonabsorbable clips are widely used for surgical haemostasis and stabilisation of sutures during minimally invasive operations, however, they are capable of potential and spontaneous migration into the lower and upper urinary tract.Objective. In this paper, we present clinical cases of clip migration after radical prostatectomy and renal resection.Clinical cases. Clinical cases of spontaneous clip migration after the two most common minimally invasive interventions: kidney resection with subsequent removal of a foreign body after the use of lithokinetic therapy and a case of hemostatic material migration after robot-assisted radical prostatectomy. The literature review is also available.Conclusion. Excessive use of non-absorbable plastic clips around their close contact with the kidney cavity or the bladder wall should be avoided to achieve adequate hemostasis during minimally invasive methods of treating patients.

Erratum: Rat Model of Normothermic Ex-Situ Perfused Heterotopic Heart Transplantation.

Erratum: Rat Model of Normothermic Ex-Situ Perfused Heterotopic Heart Transplantation.