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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant vascular disorder characterized by intractable epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations (AVMs) in multiple organs, including the lungs, liver, gastrointestinal tract, brain, and spinal cord. We herein report a 50-year-old Japanese man with HHT who experienced recurrent epistaxis, telangiectasia in the cornea, apex of the tongue and fingers; hepatic AVM; and a poorly developed main arterial trunk in the right middle cerebral artery. A genetic analysis revealed a novel heterozygous mutation in the activin A receptor-like type 1 gene, with a frameshift mutation in NM_000020.3:c.826_836del (p.Ile276ProfsTer112).

Background: Hereditary hemorrhagic telangiectasia (HHT) is a bleeding disorder characterized by arteriovenous malformations, commonly presenting with epistaxis and gastrointestinal (GI) bleeding. Bleeding symptoms may be difficult to manage and may become life-threatening, with many patients developing dependence on parenteral iron and/or blood transfusion. There is a growing body of evidence that antiangiogenic therapies may be effective in management of bleeding symptoms, presumably targeting pathogenic HHT pathways such as vascular endothelial growth factor receptor., Objectives: To report single-center, retrospective real-world use of pazopanib, an orally administered tyrosine kinase inhibitor that blocks vascular endothelial growth factor receptors, in 6 patients with HHT-associated epistaxis and/or GI bleeding., Methods: A retrospective observational analysis was performed to assess the safety/efficacy of pazopanib use in patients with confirmed HHT-associated epistaxis and/or GI bleeding between January 1, 2019, and June 14, 2023. The Indiana Hemophilia and Thrombosis institutional electronic medical record was queried for HHT patients who were treated with pazopanib for ≥3 months. Patient data were obtained from patient documentation, physician/nursing notes, and on-call documentation. Institutional review board approval was obtained for data pull as an exempt study., Results: Our observations on the real-world use of pazopanib in 6 HHT patients with moderate-to-severe bleeding showed improvement in hemoglobin levels, with reduction in iron infusions and red blood cell transfusion requirement., Conclusion: Pazopanib may be a reasonable option for patients with HHT with epistaxis or gastrointestinal bleeding that are refractory to standard treatment., Competing Interests: Declaration of Competing Interest M.L. has received consultant’s fee from Alnylam, Agios, and Genentech/Roche. She serves on the advisory board for Hema biologics. She serves as a member of the Medical and Scientific Advisory Council (MASAC) of the National Bleeding Disorder Foundation (NBDF). S.G. and A.B. have no conflict of interest to disclose. A.S. serves as the medical director at the National Hemophilia Program Coordinating Center and as a board member for the Novo Nordisk Haemophilia Foundation. She has received consultant’s fee from Novo Nordisk and Kedrion Biopharma, speakers bureau from Genentech/Roche, Kedrion Biopharma, and Sanofi-Genzyme/Bioverativ. She serves on the advisory board for Novo Nordisk, Pfizer, Genentech/Roche, Sanofi-Genzyme/Bioverativ, Hema Biologics, Be Biopharma, and BioMarin., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Epistaxis is common with antithrombotic therapy and is often troublesome to patients, yet its frequency, severity, and outcomes are poorly characterized., Methods and Results: Effective Anticoagulation with Factor Xa Next Generation in Atrial Fibrillation-Thrombolysis in Myocardial Infarction 48 (ENGAGE AF-TIMI 48) randomized 21 105 patients with atrial fibrillation and CHADS2 risk score ≥2 to higher-dose edoxaban regimen (60 mg daily, dose-reduced to 30 mg), lower-dose edoxaban regimen (30 mg, dose reduced to 15 mg, daily), or warfarin. Bleeds were adjudicated using International Society on Thrombosis and Haemostasis criteria. Patients with intracranial hemorrhage during follow-up were excluded; those with >1 bleeding event were categorized according to their most severe event. The safety cohort with interval censoring during drug interruption was analyzed. Proportions were compared using Pearson's chi-square test and treatment arms were compared using a Cox proportional hazards model. Among 5247 patients with a bleeding event, 1008 (19.2%) had epistaxis and 4239 (80.8%) had nonepistaxis bleeding. Epistaxis events were less severe than nonepistaxis bleeds (International Society on Thrombosis and Haemostasis major: 3.2% versus 20.7%; clinically relevant nonmajor: 64.7% versus 60.1%; minor: 32.1% versus 19.2%; P <0.001). Permanent drug discontinuation was similar following epistaxis versus nonepistaxis bleeding in patients with major (59.4% versus 53.6%; P =0.52) or clinically relevant nonmajor (32.5% versus 33.3%; P =0.70) bleeding but was significantly higher in patients with minor epistaxis versus other minor bleeds (33.3% versus 23.9%; P =0.001). Compared with warfarin, higher-dose edoxaban regimen had similar risk of epistaxis (hazard ratio [HR], 1.09 [95% CI, 0.95-1.26]), whereas lower-dose edoxaban regimen conferred reduced risk (HR, 0.73 [95% CI, 0.62-0.86])., Conclusions: Epistaxis was frequent, and despite being overall less severe than nonepistaxis bleeding, was associated with similar rates of anticoagulant discontinuation. Compared with warfarin, lower-dose edoxaban regimen reduced the risk of epistaxis by 27% whereas higher-dose edoxaban regimen had no effect., Registration: URL: https://clinicaltrials.gov; Unique Identifier: NCT00781391.

Hereditary haemorrhagic telangiectasia (HHT) is a vascular dysplasia inherited as an autosomal dominant trait and caused by loss-of-function pathogenic variants in genes encoding proteins of the BMP signalling pathway. Up to 90% of disease-causal variants are observed in ENG and ACVRL1, with SMAD4 and GDF2 less frequently responsible for HHT. In adults, the most frequent HHT manifestations relate to iron deficiency and anaemia owing to recurrent epistaxis (nosebleeds) or bleeding from gastrointestinal telangiectases. Arteriovenous malformations (AVMs) in the lungs, liver and the central nervous system cause additional major complications and often complex symptoms, primarily due to vascular shunting, which is right-to-left through pulmonary AVMs (causing ischaemic stroke or cerebral abscess) and left-to-right through systemic AVMs (causing high cardiac output). Children usually experience isolated epistaxis; in rare cases, childhood complications occur from large AVMs in the lungs or central nervous system. Management goals encompass control of epistaxis and intestinal bleeding from telangiectases, screening for and treatment of iron deficiency (with or without anaemia) and AVMs, genetic counselling and evaluation of at-risk family members. Novel therapeutics, such as systemic antiangiogenic therapies, are actively being investigated. Although HHT is associated with increased morbidity, the appropriate screening and treatment of visceral AVMs, and the effective management of bleeding and anaemia, improves quality of life and overall survival., Competing Interests: Competing interests: C.L.S. is listed as the inventor in the patent application filed by Imperial College London for the use of MEK1 inhibitors to treat telangiectasia in HHT (European Patent Application 23705641.1). O.F.E. is a consultant for Microvention, CERENOVUS and Balt, and is also a member of DSMB and on the advisory board for STREAM Study. All other authors declare no competing interests., (© 2025. Springer Nature Limited.)

A 2-year-old female entire Golden Retriever with a history of being subdued was seen. Her physical and neurological examinations were initially unremarkable, but she acutely progressed to non-ambulatory paraparesis, with absent cervical or thoracolumbar hyperaesthesia. Magnetic resonance imaging of the vertebral column was performed, showing a well-defined, intradural-extramedullary mass at the level of the caudal aspect of L2 causing right-sided ventrolateral marked cord compression. The lesion was T2W hyperintense and mildly hyperintense on T1W images. The signal intensity pattern of this lesion suggested the presence of an early stage hyperacute hemorrhagic process. Cerebrospinal fluid (CSF) was collected from the cisterna magna. CSF analysis exhibited a marked mixed pleocytosis with a slight neutrophilic predominance and elevated protein content. Due to the dog going from being ambulatory to non-ambulatory paraparetic with absent postural reactions in her pelvic limbs, surgery was performed to allow decompression of the spinal cord. A hemilaminectomy with durotomy was performed. Over the course of the dog's hospitalisation, she had two episodes of left-sided epistaxis which resolved with local application of adrenaline. Seven days post-operatively, the dog was discharged being non-ambulatory paraparetic with voluntary movement in both pelvic limbs. Three months after discharge, the dog was ambulatory with no ataxia noted in the pelvic limbs. As there is no current definitive diagnostic test for steroid responsive meningitis-arteritis (SRMA) available, the diagnosis of it in this case is supported by the dog's signalment, bloodwork, CSF analysis and response to corticosteroid therapy. This report details a rare clinical presentation, including epistaxis, intradural-extramedullary haemorrhage and absence of cervical or thoracolumbar hyperaesthesia, in a dog diagnosed with suspected SRMA., (© 2025 The Author(s). Veterinary Medicine and Science published by John Wiley & Sons Ltd.)

Introduction: Nasal packing is commonly employed in the emergency department (ED) to manage epistaxis that does not respond to conservative treatments. However, this intervention may increase the risk of nasal septal perforation (NSP) due to reduced blood flow to the nasal septum. No prior study has explored the relationship between nasal packing and risk of NSP., Methods: We examined all patients who received non-absorbable nasal packing in the ED at our institution, identifying 19 patients diagnosed with NSP post-treatment and 50 randomly selected patients without NSP for comparison. t-Tests and Chi square tests were utilized to compare numeric and categoric variables respectively and a multivariable logistic regression model was developed to assess risk factors., Results: Baseline characteristics of individuals who developed NSP were similar to those of the comparison group, with the exception of tobacco use. Interestingly, individuals with NSP had a lower rate of tobacco use (21 % vs 64 % current/former users, p = 0.004). Univariable analysis revealed that patients with NSP had a longer mean duration of nasal packing (5 vs 3 days, p = 0.001). Multivariable analysis showed that each additional day of packing increased odds of NSP by 77 %, and bilateral packing, compared to unilateral, was associated with four times the odds of NSP., Conclusions: Our findings indicate that prolonged nasal packing increases the risk of NSP. Prospective, large-scale studies are needed to identify patients at risk for NSP after nasal packing in the ED and to inform guidelines on the removal of nasal packing., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier Inc.)

Epistaxis greatly affects patients with hereditary hemorrhagic telangiectasia (HHT). Although few systemic treatment exist, nintedanib, is a good candidate thanks to its anti-angiogenic activity. Our main objective was to evaluate the efficacy of oral nintedanib on epistaxis duration in HHT patients with moderate to severe epistaxis. This multicenter phase 2 randomized, placebo-controlled, double-blind trial was conducted between June 2020 and February 2023. Inclusion criteria were being over 18 years old and having a confirmed HHT diagnosis with an epistaxis severity score greater than 4. Sixty patients were randomized to receive either nintedanib or placebo for 12 weeks with a 12 week follow-up. The primary endpoint was the proportion of patients achieving a reduction of at least 50% in mean monthly epistaxis duration comparing the 8 weeks before treatment to the last 8 weeks of treatment. Main secondary outcomes included monthly duration and frequency of epistaxis and hemoglobin levels. Of the 60 randomized patients, 56 completed the trial. Thirteen patients (43%) in the nintedanib group vs 8 (27%) in the placebo group met the primary endpoint (p = 0.28). We observed a significant decrease in median epistaxis (57% vs 27%, p = 0.013) and a significant increase in median hemoglobin levels (+ 18 vs - 1 g/L, p = 0.02) in the nintedanib vs the placebo group. Although we did not achieve our primary outcome, we observed a significant reduction in epistaxis duration and a significant increase in hemoglobin levels in patients treated with nintedanib. This supports the efficacy of nintedanib, and further studies are needed., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s).)

Background: This study describes the management of patients on oral anticoagulation (OAC) undergoing endoscopic endonasal transsphenoidal surgery (EETS) and analyzes the risk of postoperative hematoma and epistaxis following treatment of pituitary adenoma (PA)., Methods: Patients with OAC prior to EETS for PA were analyzed in a single center retrospective case series of consecutive patients with PA, who were treated between December 2008 and July 2022. Patient data (age, sex, clinical, endocrinology, tumor histology) were entered into a SPSS ® database. The rate of postoperative hemorrhage (intracranial and epistaxis) and other perioperative complications were assessed., Results: Of 305 patients, 20 patients were on OAC prior to EETS for PA. Indications included non-valvular atrial fibrillation (AF) in 10 patients and previous venous thromboembolic event (VTE) in 8 patients, in 2 patients had overlapping indications. Twelve patients on direct oral anticoagulants (DOAC) paused medication 1-3 days (43.6 ± 23.6 h) before surgery, while phenprocoumon was paused 234 ± 123.55 h (min 6, max 22 days) before surgery. Baseline characteristics such as age, sex, tumor growth direction, tumor volume, and largest diameter showed no significant differences. No significant increase in postoperative hemorrhage was observed in patients with OAC compared to those without. One patient on apixaban paused 48 h before surgery experienced postoperative epistaxis. Among patients without OAC, one experienced intracranial hemorrhage and seven experienced epistaxis., Conclusion: Patients with OAC prior to EETS for PA have no increased risk for postoperative hematoma when OAC is paused based on individual risk assessment and recent general recommendations., Competing Interests: Declarations. Ethical approval: The study was approved by the local ethics committee (Reference number 22748/2016/32, Landesärztekammer Thüringen). Patients gave informed consent for surgery, data collection and further analysis. The data were processed pseudonymously and therefore consent for publication was not required. Informed consent: Was obtained from all individual participants included in the study. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

A 64-year-old woman with hereditary hemorrhagic telangiectasia (HHT) characterized by a pathological variant in ACVRL1 presents to the clinic for follow-up. Manifestations of HHT include frequent epistaxis and gastrointestinal bleeding, leading to iron-deficiency anemia. Bevacizumab is initiated, with resolution of the anemia. While maintained on a regimen of bevacizumab every 6 weeks, she continues to report frequent epistaxis and has ongoing iron-deficiency requiring periodic iron infusions. She also finds the bevacizumab infusions inconvenient. She is interested in discussing other options for managing her disease., (Copyright © 2024 by The American Society of Hematology.)

This case report presents a ten-year-old boy with recurrent nosebleeds (epistaxis) over an extended period. In addition, he showed signs of slowed weight gain and developed symptoms such as snoring and sweating during sleep. Rhinoscopy revealed a mass in the nasopharynx, and further tests confirmed the presence of an Epstein-Barr Virus-positive nasopharyngeal carcinoma. After several rounds of chemo-, proton-, and immunomodulating therapy, the boy has recovered and is now in good health. This case highlights the importance of a thorough examination when epistaxis is accompanied by other risk factors., (Published under Open Access CC-BY-NC-BD 4.0. https://creativecommons.org/licenses/by-nc-nd/4.0/.)

This study investigated the frequency of idiopathic epistaxis onset and its severity relative to the time of day. Idiopathic epistaxis was defined as epistaxis in the emergency department, with no epistaxis diagnosis in any hospital 12 months before incident epistaxis. The timing of epistaxis onset was divided into four categories: morning (6:01-12:00), afternoon (12:01-18:00), evening (18:01-24:00), and overnight (00:01-6:00). The chi-square test was used to analyse the time distribution of epistaxis (p ≤ .05).. During the study period, a total of 1684 patients with a median age of 56 years developed epistaxis. Epistaxis incidence was highest in December (n = 213), lowest in July (n = 95), and highest in winter, followed by fall, spring, and summer. Epistaxis occurred most frequently overnight (n = 823, 48.8%), followed by evening (n = 410, 24.3%), morning (n = 254, 15.1%), and afternoon (n = 197, 11.7%; p < .001). Afternoon was selected as the reference. After adjustment for covariates, odds ratios (ORs) and 95% confidence intervals (CIs) of the risk of epistaxis were 1.34 (95% CI, 1.26-1.43), 1.47 (1.38-1.56), and 3.52 (3.14-3.91) in the evening, morning, and overnight, respectively. Epistaxis rates overnight were significantly strongest and positively proportional to blood pressure levels between 0:01 am and 6:00 am (r = 18.1, p < .001), followed by overnight rates (r = 11.5, p = .017). Patients who developed epistaxis overnight were more likely to develop posterior epistaxis and to receive endoscopic electrocoagulation haemostasis (p < .001). In this cohort, these results suggest that adult idiopathic epistaxis occurred most frequently at overnight, especially in the winter, and more need management to immediately control the bleeding in ENT emergency departments. In addition, this study found that high frequent of epistaxis at overnight associated with serious blood pressure between 0:01 am and 6:00 am. This analysis supports that health care professionals and caregiver should be aware of individuals with hypertension, and the status of blood pressure at overnight should be considered in preventing nasal bleeding's risk., Competing Interests: Declarations. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Background: The incidence of epistaxis during nasotracheal intubation via the left nostril is more frequent than that during intubation via the right nostril. This study evaluated the effect of the reverse bevel and tip direction of the nasotracheal tube on the incidence of epistaxis during nasotracheal intubation via the left nostril., Methods: Patients undergoing right-sided maxillofacial surgery requiring left nasotracheal intubation were randomly allocated to the control (tracheal tube in the conventional direction) or reverse (a 180˚ reverse direction, with the tube bevel facing the nasal septum and the leading edge (i.e., the tip) of the bevel pointing away from the nasal septum) groups (n = 37 for both). The primary outcome was the incidence of epistaxis evaluated using videolaryngoscopy., Results: The incidence of epistaxis in the reverse group was significantly lower than that in the control group (9 [24.3%] vs. 20 [54.1%], P = 0.009; relative risk: 0.45, 95% CI [0.24, 0.85], absolute risk reduction: 29.8%, number needed to treat: 3). The severity of epistaxis was significantly lower in the reverse group (P = 0.002). The first attempt nasal passage (P = 0.027) was significantly higher in the reverse group. Postoperative nasal pain was lower (P < 0.001), and patient satisfaction was higher (P < 0.001) in the reverse group. Nasotracheal tube-related complications did not occur in either group., Conclusions: The reverse bevel and tip direction of the nasotracheal tube reduced the incidence and severity of epistaxis and increased patient satisfaction among patients undergoing left nasotracheal intubation.

Objectives: To analyze the risk factors and explore effective treatments for epistaxis in nasopharyngeal carcinoma (NPC) patients., Methods: From March 2006 to February 2020, 351 epistaxis patients visited our center and 195 patients meeting the inclusion criteria were enrolled in the study. Characteristics and treatments, including step-up hemostatic treatment (including medication, anterior ± posterior nostril packing, or further surgical hemostasis) and the CTPI emergency hemostasis method (including common carotid artery compression, tracheotomy / intubation, packing of nasal and nasopharynx, and interventional treatment), were analyzed., Results: The median total bleeding volume was 100.0 ml (range 20-4430 ml). 126 (64.6 %) and 69 (35.4 %) patients suffered from non-massive epistaxis and massive epistaxis. The 1-year overall survival (OS) rate was 60.1 % for patients with massive epistaxis and 97.3 % for those with non-massive epistaxis treated with step-up hemostatic treatment. Among patients with massive epistaxis, the 1-year OS rate was 80.0 % for those who received CTPI and 13.3 % for those who received step-up hemostatic treatment., Conclusion: ICA exposure and hemostasis failure was adverse prognostic factors for OS in NPC patients with epistaxis. The step-up hemostatic treatment is effective for controlling non-massive epistaxis. The CTPI emergency method might be an effective hemostasis treatment for NPC patients with massive epistaxis, especially those with PRNN and ICA exposure., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Purpose of Review: Hereditary hemorrhagic telangiectasia (HHT) diagnostic and management approach for pediatrics underwent significant advances over the last couple of years., Recent Findings: In 2020, new guidelines for HHT were published that included a pediatric section thus attracting special focus into the childhood presentation., Summary: Curacao criteria are specific, but not sensitive enough in children. Genetic testing is encouraged for all family members even if asymptomatic. Standardized scoring for epistaxis is strongly encouraged, as it allows monitoring and can stratify therapeutic approaches. Early screening for pulmonary and brain visceral arteriovenous malformations (AVMs) in pediatric patients with confirmed genetic alterations of HHT should be instituted. Graded trans-esophageal echocardiogram with agitated saline contrast can be used as screening method for pulmonary AVMs. As pulmonary AVMs can develop throughout lifetime, guidelines recommend repeated screening even in asymptomatic patients at least every 5 years. Signs of stroke in childhood are more subtle than in adults. Cerebral imaging in early childhood can identify brain AVMs that may benefit from early intervention. Embolization of high-risk pulmonary and cerebral AVMs should be performed at specialized centers even at pediatric age. One or two classic HHT telangiectasia can be considered diagnostic in children. Antibiotic prophylaxis with dental procedures continues to be recommended., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Key Points: In patients with severe refractory epistaxis due to hereditary hemorrhagic telangiectasia, the most effective results are achieved by surgical closure of the nares to permanently prevent nasal airflow. Flap failure and dehiscence can occur and result in fistula formation and clinical relapse. We describe the utility of a V-Y advancement flap in repairing localized cutaneous defects in patients who have previously undergone nasal closure for severe HHT., (© 2024 ARS‐AAOA, LLC.)

Background: Epistaxis is one of the most common and debilitating symptoms of hereditary hemorrhagic telangiectasia (HHT), significantly impacting patients' quality of life. While various medical and surgical interventions exist for managing epistaxis in patients with HHT, patients with moderate to severe epistaxis are high health-care utilizers who frequently need surgical treatment., Objective: To compare the efficacy, complications, and patient-reported outcomes for common surgical interventions utilized in treating epistaxis in patients with HHT., Methods: Studies were identified in PubMed, Embase, Scopus, Redalyc, and LILACS databases and uploaded to Covidence. A systematic review following PRISMA guidelines was conducted on studies evaluating outcomes in adults with HHT with moderate to severe epistaxis who had undergone surgical interventions. We compared the respective outcomes for pre-operative and post-operative epistaxis severity/intensity, need for further interventions or transfusions, estimated blood loss, length of surgery, complications, and patient satisfaction., Results: Twenty studies with a total of 546 patients were included. The most common surgeries studied were nasal closure and laser photocoagulation. Seven studies recorded the change in epistaxis severity score and observed a significant reduction postoperatively (3.91, [95% CI 2.73-5.09]). Eleven studies found a decrease in the number of transfusions and a rise in hemoglobin levels post-operatively. Common complications reported were partial dehiscence of a nasal closure, septal perforation, and continued bleeding requiring re-operation. Most patients report improved quality of life and satisfaction with surgical intervention., Conclusion: Surgery can significantly reduce the severity of epistaxis in patients with HHT and improve quality of life. Further studies should focus on head-to-head comparisons of procedures and standardization of outcome measures., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: It is often recommended that, in children with bilateral epistaxis, only one side of the nasal septum should be cauterised at a time in order to reduce the risk of septal perforation. This advice may have been reasonable when hot wire electrocautery was in common use. The risk of septal perforation after silver nitrate cautery is unknown but probably low., Methods: Retrospective case note review of children attending the nurse-led epistaxis clinic between 2019 and 2022., Results: Nine hundred and twenty children were seen in the nurse-led clinic between January 2019 and December 2022. Six hundred and one children (79%) underwent nasal cautery. Simultaneous bilateral nasal cautery was carried out in 176 (29%) children. Our follow up period ranged between 303 and 1744 days, with a median of 809.5 days. No child presented to emergency or ENT services with septal perforation or any other complication of simultaneous bilateral nasal cautery., Conclusion: To the author's knowledge, this is the largest study assessing the safety of simultaneous bilateral nasal cautery with silver nitrate in children. No adverse results have been found after cauterising both sides of the septum at the same sitting, and doing so potentially saves time and money for patients and the health service., (© 2024 John Wiley & Sons Ltd.)

Background: We present this case to highlight the importance of considering seromucinous hamartoma in the differential diagnosis of nasal cavity lesions, particularly due to its rarity and potential for being mistaken for more aggressive pathologies. Seromucinous hamartoma, although benign, can exhibit clinical and histological features that overlap with those of malignant tumors, posing a diagnostic challenge. This case is especially noteworthy due to the unusual presentation of sebaceous differentiation within seromucinous hamartoma, a feature not previously documented in the literature. Recognizing such rare entities is crucial for ensuring appropriate patient management and avoiding unnecessary interventions., Case Presentation: We report a unique case of a 41-year-old Caucasian man with seromucinous hamartoma, presenting with chronic left nasal obstruction and recurrent mild epistaxis. Nasal endoscopy revealed a well-defined, multilobulated lesion in the left nasal cavity. Computed tomography scans confirmed an anteromedial polypoid lesion, 31 mm × 15 mm. The lesion was endoscopically resected without complications, with no recurrence at 6-month follow-up. Pathological examination showed a filiform polypoid lesion with clusters of seromucinous glands, ducts, and tubules, and no invasive growth patterns. Immunohistochemical studies revealed distinct epithelial membrane antigen (EMA) and p63 staining patterns. Notably, mature sebaceous gland formation interspersed with seromucinous glands was observed, a novel finding in seromucinous hamartoma. Recognizing seromucinous hamartoma is crucial to avoid unnecessary treatments, and it should be included in differential diagnoses of nasal cavity lesions., Conclusion: Seromucinous hamartoma is a rare and benign nasal cavity lesion that presents significant diagnostic challenges due to its potential to mimic more aggressive pathologies. This case highlights the importance of including seromucinous hamartoma in the differential diagnosis of sinonasal lesions, particularly when unusual histological features, such as sebaceous differentiation, are present., Competing Interests: Declarations. Ethics approval and consent to participate: This study has obtained IRB approval from the hospital ethical committee. Informed consent was obtained from the patient. Consent for publications: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare that they have no conflict of interest., (© 2024. The Author(s).)

Introduction: Epistaxis in children is a very common reason for consultation in ENT and pediatric emergencies. Most of these epistaxis are benign, but it is their recurrence that motivates anxious parents, to consult. Allergic rhinitis is a common pathology among children in Tunisia, it seemed interesting to us to study the association between allergic rhinitis and epistaxis., Aim: Identify the clinical features and the risk factors for epistaxis in children with allergic rhinitis., Methods: Retrospective descriptive cohort study over a period of 4 years, including children, who consulted for epistaxis at the ENT and CCF department of FSI Hospital, La Marsa Tunis. All cases were divided into 2 groups: ERA+ Group: epistaxis with allergic rhinitis versus ERA- Group: epistaxis without allergic rhinitis., Results: 79 children were included, including 21 (26.6%) meeting the diagnostic criteria for allergic rhinitis. A female predominance was noted in the ERA+ group. In the ERA+ group, epistaxis mainly occurred in summer and autumn (38% and 28%) while it predominated in winter and summer in the ERA- group. Rhinorrhea and nasal pruritus were factors significantly associated with epistaxis in children with allergic rhinitis., Conclusion: the ENT doctor must think about allergic rhinitis in case of Repetitive epistaxis in a child, and look for suggestive symptoms, particularly rhinorrhea and nasal prurit.

Background: A growing body of literature explores environmental risk factors for pediatric epistaxis, yielding variable results. We aim to clarify these associations through a systematic review and meta-analysis., Methods: PubMed, Scopus, Cochrane Central Register of Control Trials, Web of Science, Medline, Google Scholars, and Embase were systematically searched up to April 2024. Eligible articles were reviewed, and the quality was assessed. A systematic review and meta-analysis was conducted to clarify correlations between the incidence of epistaxis and multiple environmental factors according to Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines., Results: A total of 8 studies, comprising 55,176 participants, met the inclusion criteria. The incidence of epistaxis peaked during the summer months (Proportion = 12.73 %, CI: 9.629 %-16.201 %). Significant risk factors included environmental variables elevated in the summer, including higher monthly mean temperatures, increased sunlight exposure, elevated O3 levels, and lower atmospheric pressure. In contrast, factors like mean monthly humidity, wind speed, SO2, CO, NO2, and PM-10 levels were not associated with an increased risk of epistaxis., Conclusions: This meta-analysis underscores the significant impact of multiple environmental factors, particularly those more pronounced during the summer months, on the incidence of pediatric epistaxis., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Purpose: Epistaxis in critically ill patients may prevent the use of non-invasive ventilation and impair nasal oxygen delivery. Since the onset of COVID-19, high-flow nasal oxygen has dramatically increased. There is a paucity of literature on characteristics of epistaxis in critically ill, COVID-19 positive patients. We aimed to establish the incidence of epistaxis and identify risk factors., Materials and Methods: This was a retrospective observational study conducted at a large academic medical center. Chart review was performed on patients with an intensive care admission and COVID-19 diagnosis between January 2020 and May 2022. Data included epistaxis events, supplemental oxygen delivery and duration, anticoagulation, and antiplatelet therapies., Results: 932 patients met study criteria. Epistaxis incidence was 7.4 %. Of those with epistaxis, 78 % were administered supplemental oxygen. For each additional day on nasal oxygen, patients were at a 7.1 % higher risk for epistaxis (p < .001). Most antiplatelet agents and therapeutic anticoagulation were not found to increase risk., Conclusions: Nasal oxygen was a major risk factor for epistaxis in this population. Nasal hygiene is a standard regimen recommended by otolaryngologists for epistaxis. Protocolizing the inclusion of nasal hygiene measures may be an easy, inexpensive way to prevent epistaxis in this already unstable patient population., Competing Interests: Declaration of competing interest All authors declare no personal or financial conflicts of interest as related to the submitted work., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

A 10-year-old female neutered Labrador Retriever presented with epistaxis, discoloration and crusting of the nose and a necrotic lesion on the lip. Bloodwork revealed pancytopenia, azotemia, hypoalbuminemia and hyperglobulinemia. Aggregates of amorphous basophilic material were seen in a room-temperature blood smear which were not present in the sample after warming to 37°C, and grossly a cryoprecipitate was noted in the patient's serum at 4°C. This was interpreted as cryoglobulin. Computed tomography showed multiple heterogeneous lesions in the spleen. Cytology of the splenic lesions revealed marked plasma cell infiltration, consistent with neoplasia. Bone marrow aspiration revealed an increased proportion of plasma cells (approximately 38% of the total cells). Serum protein electrophoresis showed a monoclonal spike in the gamma globulin region. A diagnosis of multiple myeloma associated with cryoglobulinemia was made. The patient received palliative care with prednisolone while the owner was considering chemotherapy. However, she rapidly deteriorated and was euthanized. The combination of cryoglobulin precipitation and hyperviscosity syndrome was considered responsible for the patient's original symptoms. Cryoglobulinemia is an extremely rare phenomenon that is often associated with lymphoproliferative disorders. This report describes its association with multiple myeloma in a dog presenting with atypical initial signs., (© 2024 The Author(s). Veterinary Medicine and Science published by John Wiley & Sons Ltd.)

Background: Endoscopic sphenopalatine artery cauterization (ESPAC) has become an important method to manage posterior epistaxis., Aims/objectives: To investigate the application of plasma knife and lateral nasal wall incision in ESPAC in the treatment of posterior epistaxis., Material and Methods: A retrospective study of 32 cases who underwent ESPAC for epistaxis was conducted. A vertical incision was made on the lateral nasal well to expose the sphenopalatine artery (SPA). The main branches of SPA were cauterized with a plasma knife or bipolar coagulation forceps. Cases were divided into plasma knife group (group PK) and bipolar group (group BP). The re-bleeding rates, operation time and the incidence of serious complication were compared between the two groups., Results: ESPAC was successfully completed via a lateral nasal wall incision without maxillary antrostomy in all cases. All the patients were followed-up for 3 months, no serious complication was reported. There was no significant difference in re-bleeding rates and incidence of serious complication between the two groups. The operation time of group PK was shorter than group BP., Conclusions and Significance: Lateral nasal well incision without maxillary antrostomy is feasible for ESPAC. The application of a plasma knife may help to shorten the operation time.

Primary extracranial sinonasal meningiomas are one of the rarest tumours involving the head and neck region. Very few reports exist with additional involvement of the palato-antral region. A 45-year-old male presented with frequent episodes of epistaxis, complaints of nasal blockage and nasal regurgitation for 8 months in 2021 at our tertiary care government hospital in East Godavari district, India. The patient was diagnosed with a primary extracranial sinonasal meningioma with palatal involvement and surgical excision via intra-oral and endoscopic approaches were performed. Postoperative healing was uneventful with no recurrence noted over 2 years. A palatal obturator was used for rehabilitation. The diagnosis of this pathology requires additional immunohistochemistry testing for confirmation and treatment entails complete surgical excision which assures no recurrence or delayed presentation of residual disease in follow-up., (© Copyright 2024, Sultan Qaboos University Medical Journal, All Rights Reserved.)

Hamartomas, as non-true tumors, are commonly found in the lungs, digestive tract, and kidneys, and there are few clinical reports on nasal cases. Nasal histiocytosis accompanied by lymphatic lesions is a common feature of nasal hamartoma. Three patients with nasal cavity hamartoma were treated in our department, including 1 male and 2 females, with nasal obstruction or epistaxis. The imaging (computed tomography, magnetic resonance imaging) of 2 patients showed a soft tissue mass in the nasal cavity and no erosion of bone, while in 1 patient, the imaging was inconclusive. The excised material in all patients was found to be a soft mass. The 3 cases showed the same pattern under histological examination, and all 3 patients were diagnosed with nasal cavity respiratory epithelial adenomatoid hamartoma (REAH). Nasal hamartoma is a rare disease with nonspecific clinical manifestations and imaging features and is often misdiagnosed. Only by a better understanding of this disease can the rate of correct clinical diagnosis be improved., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Epistaxis is a common condition that affects about 60% of the population in their lifetime, with 6% needing medical attention. Little is known about the epidemiology and risk factors of epistaxis outside the health care system. This study aimed to investigate the prevalence and risk factors of epistaxis in a rural Danish population using data from the Lolland-Falster Health Study (LOFUS)., Methods: We conducted a cross-sectional survey based on data from LOFUS, a household-based, prospective cohort study in the rural provincial area of Lolland-Falster, Denmark. We enrolled 10,065 participants (≥ 50 years) and collected data on demographics, comorbidities, medication, lifestyle factors, and laboratory parameters. Logistic regressions were used to test for correlations between epistaxis and different risk factors., Results: In total 5.3% of the participants had experienced epistaxis within the past 30 days, and 7.9% had sought medical attention for epistaxis at some point in their lives. We identified several factors that were significantly correlated with increased odds of epistaxis, such as male gender, age group 50-59 years, high BMI (> 25), allergy, diabetes, hypertension, atherosclerosis, angina, and anticoagulant treatment. Excellent or good self-reported health was correlated to significantly lower odds of epistaxis., Conclusion: This study provides a comprehensive overview of the prevalence and risk factors of epistaxis outside the health care system. Our study suggests that preventive measures targeting these risk factors may reduce the incidence and severity of epistaxis in this population., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Nasotracheal intubation is associated with a risk of epistaxis. Several drugs, including cocaine and xylometazoline may be used as decongestants prior to nasotracheal intubation to prevent this. We hypothesized that xylometazoline would prevent epistaxis more effectively than cocaine, demonstrated by a lower proportion of patients with bleeding after nasotracheal intubation., Methods: We conducted a single-center, outcome assessor and analyst-blinded, clinical randomized controlled trial following approval from the local research ethics committee and the national medicine agency. Written informed consent was obtained from all patients. Patients scheduled for surgery under general anesthesia with nasotracheal intubation were randomized to receive either 2 mL 4% cocaine or 2 mL 0.05% xylometazoline prior to nasotracheal intubation. Immediately following intubation, epistaxis was evaluated by the blinded intubating anesthetist on a four-point scale. We measured heart rate and blood pressure the first 5 min after drug administration. Adverse events were followed up after 24 h., Results: A total of 53 patients received cocaine and 49 patients received xylometazoline. Bleeding occurred in 32 patients receiving cocaine (60.4%) and in 34 patients receiving xylometazoline (69.4%) (p = .41, Fisher's exact test) with a difference of 9.0% (95% CI: -9.4% to 27%). There was no statistically significant difference between groups regarding the heart rate or blood pressure. No adverse cardiac events were recorded in either group., Conclusion: We found no statistically significant difference between cocaine and xylometazoline in preventing epistaxis after nasotracheal intubation, and the choice of vasoconstrictor should be based on other considerations, such as pricing, availability and medicolegal issues., (© 2024 The Author(s). Acta Anaesthesiologica Scandinavica published by John Wiley & Sons Ltd on behalf of Acta Anaesthesiologica Scandinavica Foundation.)

India contributes to 26% of the global tuberculosis (TB) burden, with very high mortality and morbidity. The exact incidence of disseminated TB cannot be established among the general population but accounts for <2% of cases among immunocompromised hosts and constitutes 20% of all extrapulmonary TB cases. Disseminated TB has a very high mortality rate of around 25-30%. Miliary TB, a disseminated form, is another entity of TB that poses a health burden due to its difficulty in diagnosis. This entity usually presents with subclinical symptoms and poses a diagnostic challenge in the absence of specific diagnostic tests. We present a case of a young female with epistaxis and thrombocytopenia who was diagnosed with disseminated TB [miliary, bone cyst, bone marrow (BM) involvement]., (© Journal of the Association of Physicians of India 2024.)

Abstract: Olfactory neuroblastoma (ONB) is an uncommon malignant tumor typically located in the upper nasal cavity. Olfactory neuroblastoma originating in the nasopharynx is extremely rare and tends to be misdiagnosed. The authors describe a rare case of ONB arising ectopically in the nasopharynx. The patient was a 65-year-old woman with recurrent epistaxis and a feeling of fullness in the right ear. After evaluation, endoscopic surgery was performed. The pathological result proved to be ONB. Postoperative magnetic resonance imaging showed that the tumor was completely resected. The patient proceeded to have 66 Gy of postoperative intensity-modulated radiotherapy and was followed for 36 months without tumor recurrence. Olfactory neuroblastoma originating from the nasopharynx is more rare condition compared with ONB located in other areas in the literature. The symptoms of ONB ectopic to the nasopharynx are similar to those of other nasopharyngeal tumors, which were likely to be misdiagnosed. The treatment principle is the same as that of nonectopic ONB, which is surgery combined with radiotherapy. Surgery can be performed using an endoscopic transnasal approach., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic disease with idiopathic etiology and unique pathology. Extra-nodal RDD that occurs in the nasal cavity is extremely unusual and the characteristic clinical features are unknown. Herein, we report a case of nasal septum RDD, with intermittent epistaxis from the left nasal cavity, which led to collapse of the nasal bridge. The patient underwent surgical biopsy, and a diagnosis of nasal septum RDD was established. No further treatment was performed. An enlarged mass was found in the second postoperative year which was treated by surgical excision in the third postoperative year. To improve the current diagnostic and therapeutic approach of extra-nodal RDD, we incorporate previous reports from the literature to discuss the pathological characteristics, pathogenesis, clinical manifestations, diagnosis, and therapy for this rare disease., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Hereditary hemorrhagic telangiectasia (HHT) is characterized by extensive telangiectasias and arteriovenous malformations. The primary clinical manifestation is epistaxis that results in iron-deficiency anemia and reduced health-related quality of life., Methods: We conducted a randomized, placebo-controlled trial to evaluate the safety and efficacy of pomalidomide for the treatment of HHT. We randomly assigned patients, in a 2:1 ratio, to receive pomalidomide at a dose of 4 mg daily or matching placebo for 24 weeks. The primary outcome was the change from baseline through week 24 in the Epistaxis Severity Score (a validated bleeding score in HHT; range, 0 to 10, with higher scores indicating worse bleeding). A reduction of 0.71 points or more is considered clinically significant. A key secondary outcome was the HHT-specific quality-of-life score (range, 0 to 16, with higher scores indicating more limitations)., Results: The trial was closed to enrollment in June 2023 after a planned interim analysis met a prespecified threshold for efficacy. A total of 144 patients underwent randomization; 95 patients were assigned to receive pomalidomide and 49 to receive placebo. The baseline mean (±SD) Epistaxis Severity Score was 5.0±1.5, a finding consistent with moderate-to-severe epistaxis. At 24 weeks, the mean difference between the pomalidomide group and the placebo group in the change from baseline in the Epistaxis Severity Score was -0.94 points (95% confidence interval [CI], -1.57 to -0.31; P = 0.004). The mean difference in the changes in the HHT-specific quality-of-life score between the groups was -1.4 points (95% CI, -2.6 to -0.3). Adverse events that were more common in the pomalidomide group than in the placebo group included neutropenia, constipation, and rash., Conclusions: Among patients with HHT, pomalidomide treatment resulted in a significant, clinically relevant reduction in epistaxis severity. No unexpected safety signals were identified. (Funded by the National Heart, Lung, and Blood Institute; PATH-HHT Clinicaltrials.gov number, NCT03910244)., (Copyright © 2024 Massachusetts Medical Society.)

Background: Hereditary hemorrhagic telangiectasia (HHT, Rendu- Osler-Weber disease, or Osler's disease for short) is a systemic disease that can severely impair the quality of life and that requires interdisciplinary treatment. Among rare diseases, it is relatively common, with a prevalence of approximately 1/5000., Methods: This review is based on publications retrieved by a selective literature search, including the two international guidelines on clinically relevant aspects of HHT., Results: On average, about two decades elapse between the initial symptoms and the diagnosis of HHT. 95% of patients have nosebleeds; these usually begin before age 20 but can occur at any time, from infancy to old age. The diagnosis is usually made on clinical grounds on the basis of the characteristic telangiectases, a positive family history, and possible involvement of the gastrointestinal tract, lungs, liver, and brain. Nosebleeds can sometimes be reduced by outpatient measures including counseling on keeping the nose moist (expert consensus), self-application of a nasal packing (which improves the quality of life, according to an online survey), and the prescription of tranexamic acid (reduction of nosebleeds from 17.3% [5.5; 27.6] to 54%). In particular, screening (expert consensus) for pulmonary vascular malformations (frequency 10-50%) can prevent many adverse outcomes. If pulmonary vascular malformations cannot be ruled out, antibiotic prophylaxis is recommended before medical procedures that can cause bacteremia (expert consensus)., Conclusion: Broad awareness of the condition, early diagnosis, and interdisciplinary treatment improve the quality of life and ultimate outcome of persons with HHT. Nevertheless, there are few options supported by good evidence for the appropriate treatment of this rare, often serious disease..

Purpose: This study aims to investigate the bleeding sites and their relationship with clinical characteristics in hospitalized epistaxis patients., Methods: We retrospectively reviewed the data of 646 hospitalized epistaxis patients., Results: The bleeding sites were identified in 395 (61.1%) patients and unidentified in 251 (38.9%). We found that age > 50 years (P = 0.030) and the history of cardiovascular diseases (P = 0.027) were more frequent in patients with unidentified bleeding sites. Among patients with identified sites, inferior meatus (n = 130, 32.9%) was the most common site, followed by the septal surface of the olfactory region (n = 102, 25.8%), nasal septum (n = 80, 20.3%), middle meatus (n = 60, 15.2%), and others (n = 23, 5.8%). After dividing patients into five groups by the area of the bleeding sites, we found significant differences in age (P = 0.026), history of hypertension (P = 0.001), cardiovascular diseases (P = 0.032), and nasal packing (P = 0.011). The logistic regression also revealed that these four factors were predictors for different bleeding sites., Conclusion: The bleeding sites can be identified in most epistaxis patients. Age > 50 years and the history of cardiovascular diseases are more frequent in patients with unidentified bleeding sites. In our patients, the most common bleeding site is inferior meatus, followed by the septal surface of the olfactory region, nasal septum, and middle meatus. Age, histories of hypertension, cardiovascular diseases, and nasal packing are factors associated with the bleeding risks of different bleeding sites. According to the different clinical characteristics of patients, the order of the nasal endoscopic examination should be adjusted to develop their treatment plans., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To analyze the related influencing factors of epistaxis in extremely high altitude area, and to provide evidence for the prevention and treatment of epistaxis in extremely high altitude area. Methods: From January 2021 to December 2022, 206 outpatients with epistaxis, 54 inpatients with epistaxis and 69 inpatients withoutepistaxis in theDepartment of Otorhinolarygology, Naqu People's Hospital were collected. The previous history, drinking history, smoking history, serum homocysteine（Hcy）, white blood cell count（WBC）, red blood cell count（RBC）, hematocrit（HCT）, hemoglobin（HGB） and mean hemoglobin concentration（MCHC） were compared between inpatients with or without epistaxis. The factors with significant differences were analyzed by binary Logistic regression. The monthly average temperature,air pressure, humidity and 2-minute wind speed were collected from January 2021 to December 2022 in Naqu City to analyze the correlation between epistaxis and climate factors. Results: The number of patients with hypertension in the case group was more than that in the control group, and the difference was significant（ P =0.013）. Serum Hcy level in the case group was higher than that in the control group（ P <0.001）. RBC, HCT, HGB and MCHC were lower than that in the control group（ P =0.001, 0.001, 0.001, 0.039）, and the difference was significant. History of hypertension and Hcy were risk factors for epistaxis. Patients with a history of hypertension were 3.713 times more likely to suffer from epistaxis than those without a history of hypertension（ P =0.022）. Each 1 increase in Hcy concentration increased the risk of epistaxis by 13.1%（ P =0.001）. Conclusion: Patients with epistaxis in Naqu area had higher serum Hcy level and lower RBC, HCT, HGB and MCHC. History of hypertension and Hcy were risk factors for epistaxis. Patients with a history of hypertension were 3.713 times more likely to suffer from epistaxis than those without a history of hypertension. Every 1 increase in Hcy concentration increased the risk of epistaxis by 13.1%. Active intervention of hypertension and serum Hcy can effectively prevent the incidence of epistaxis., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a recently described neoplasm entity that presents only in the sinonasal tract. Histologically, it displays features of both a surface-derived carcinoma and a salivary gland carcinoma, and is associated with high-risk HPV, specifically HPV type 33. Whereas majority of the cases display high-grade histologic features, HMSC paradoxically behaves in a relatively indolent fashion. It is important and meaningful to distinguish HMSC from other histopathologic mimickers as the clinical features and management are distinctive. A 64-year-old woman presented having intermittent left-side epistaxis and progressive nasal obstruction. A well-defined, solitary, friable mass with an irregular surface that easily bled upon contact was found in the posterior part of the left nasal cavity. Endoscopic excision of the tumor which was originated from left nasal septum was done and the tumor was confirmed as HMSC. After surgery, definitive radiotherapy was performed in 28 fractions. HMSC is a histopathological type that has been rarely reported so that we report this case with literature review., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We present a rare case of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia (LPL/WM) diagnosed in a 65-year-old female initially presenting with recurrent bilateral epistaxis. Despite multiple cauterizations and a history of ineffective conventional treatments, comprehensive evaluations led to the diagnosis, underscoring the critical need for thorough investigation in persistent epistaxis cases, particularly when standard approaches fail. This case emphasizes the importance of considering indolent lymphomas in the differential diagnosis of recurrent epistaxis and showcases the diagnostic pathway leading to successful identification and treatment of a rare etiology. Laryngoscope, 134:3974-3976, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Abstract: Hereditary hemorrhagic telangiectasia (HHT; Osler-Weber-Rendu disease) affects 1 in 5000 persons, making it the second most common inherited bleeding disorder worldwide. Telangiectatic bleeding, primarily causing recurrent epistaxis and chronic gastrointestinal bleeding, is the most common and most important manifestation of this multisystem vascular disorder. HHT-associated bleeding results in substantial psychosocial morbidity and iron deficiency anemia that may be severe. Although there remain no regulatory agency-approved therapies for HHT, multiple large studies, including randomized controlled trials, have demonstrated the safety and efficacy of antifibrinolytics for mild-to-moderate bleeding manifestations and systemic antiangiogenic drugs including pomalidomide and bevacizumab for moderate-to-severe bleeding. This has led to a recent paradigm shift away from repetitive temporizing procedural management toward effective systemic medical therapeutics to treat bleeding in HHT. In this article, 4 patient cases are used to illustrate the most common and most challenging presentations of HHT-associated bleeding that hematologists are likely to encounter in daily practice. Built on a framework of published data and supported by extensive clinical experience, guidance is given for modern evidence-based approaches to antifibrinolytic therapy, antiangiogenic therapy, and iron deficiency anemia management across the HHT disease severity spectrum., (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)

Purpose: Maxillary sinus carcinomas usually present as a locally advanced disease at the time of diagnosis and it is extremely unusual to have a second primary maxillary carcinoma on the contralateral side after many years of completion of treatment of the first malignancy. We present here a case report of a sphenopalatine artery (SPA) pseudoaneurysm mimicking the second primary maxillary carcinoma., Methods: We reviewed the literature for SPA pseudoaneurysm., Results/case Report: This report describes the case of a 90-year-old man with a background of adenoid cystic carcinoma of the right maxillary sinus, diagnosed and treated with surgery and radiotherapy 14 years ago, who presented with a history of multiple episodes of epistaxis. The radiological evaluation showed a heterogeneously enhancing mass with a central hemorrhagic component and surrounding bony erosions in the left maxillary sinus and the patient was planned for biopsy from the suspicious mass along with SPA ligation. However, on opening the maxillary antrum there was excessive bleeding and it was determined unsafe to proceed further. The patient was subsequently taken to interventional radiology for diagnostic angiography which revealed an SPA pseudoaneurysm that was subsequently embolized successfully., Conclusions: Sphenopalatine artery pseudoaneurysms should be considered as a differential for recurrent epistaxis in patients with a history of sinonasal malignancy. In such cases, endovascular embolization is a viable management option., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: We sought to compare three intubation methods using different intubation techniques/tube materials for tube advancement from the nasal cavity into the oral cavity during nasotracheal intubation., Methods: We conducted a randomized clinical trial with adult patients scheduled to undergo elective surgery requiring nasotracheal intubation for general anesthesia. Participants were randomly allocated to a polyvinyl chloride (PVC) tube group (group P), PVC tube attached to a rubber catheter group (group PR), or velvet-soft PVC tube group (group V). Tracheal intubation was then performed based on group allocation. The primary outcome was the first-attempt success rate of tube advancement into the oral cavity; secondary outcomes included the time required for tube advancement into the oral cavity, total intubation time, and the incidence of epistaxis., Results: A total of 149 patients were included in the study. The first-attempt success rate in group V (90%) was significantly higher than that in group P (58%) (odds ratio, 6.5; 95% confidence interval [CI], 2.2 to 19.2), but similar to that in group PR (100%). The mean (standard deviation) time required for tube advancement into the oral cavity was significantly shorter in group V (16 [13] sec) than in group PR [40 (10) sec; 95% CI of mean difference, 17 to 30] and group P (26 [16] sec; 95% CI of mean difference, 3 to 16). Total intubation time was longest in group PR. Epistaxis occurred the least in group V., Conclusions: Among the three intubation techniques/tube materials for nasotracheal intubation, the velvet-soft PVC tube provided the highest first-attempt success rate, most expeditious advancement into the oral cavity, and lowest incidence of epistaxis., Study Registration: ClinicalTrials.gov (NCT04695444); first submitted 30 December 2020., (© 2024. Canadian Anesthesiologists' Society.)

Key Points: Severe epistaxis occurs in 2% of PNN ablation cases, independent of method or device type. Major epistaxis requiring intervention after PNN ablation can occur on average 20 days post-procedure., (© 2024 ARS‐AAOA, LLC.)

Background: The endoscopic modified medial maxillectomy (MMM) and prelacrimal approach (PLA) are two routinely performed endoscopic approaches to the maxillary sinus when access via a middle meatal antrostomy is insufficient. However, there is no data in the literature that has compared outcomes and complication profile between the two procedures to determine which approach is superior., Objective: To compare the approach related morbidity of PLA and MMM., Methods: A retrospective cohort study of all consecutive adult patients undergoing either MMM or PLA from 2009 to 2023 were identified. The primary outcome was development of epistaxis, paraesthesia, lacrimal injury, iatrogenic sinus dysfunction within a minimum of 3 months post-operative follow up., Results: 39 patients (44 sides) underwent PLA and 96 (96 sides) underwent MMM. There were no statistically significant differences between the rates of paraesthesia (9.1 % vs 14.6 %, p = 0.367) or prolonged paraesthesia (2.3 % vs 5.2 %, p = 0.426), iatrogenic maxillary sinus dysfunction (2.3 % vs 5.2 %, p = 0.426) or adhesions requiring removal (4.5 % vs 4.2 %, p = 0.918). No cases of epiphora or nasal cavity stenosis occurred in either arm in our study., Conclusions: According to our data, the endoscopic modified medial maxillectomy and prelacrimal approach are both equally safe approaches with their own benefits to access., Competing Interests: Declaration of competing interest Richard J Harvey is a consultant/advisory board with Medtronic, Novartis, Glaxo-Smith-Kline and Meda pharmaceuticals. He has been on the speakers' bureau for Glaxo-Smith-Kline, Astra-zeneca, Meda Pharmaceuticals and Seqirus. Larry Kalish is on the speakers' bureau for Care Pharmaceuticals, Mylan, and Seqirus Pharmaceuticals. All other authors have no personal, financial, or institutional interest in any drugs, materials, or devices described in this article. This is an unfunded project., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Epistaxis is an emergency medical condition that sometimes requires admission to the emergency department. Pediatric epistaxis differs from epistaxis in the older population in terms of etiology, severity, and management. Our objective was to identify the distinctive features of pediatric epistaxis and determine the appropriate management., Methods: This was a retrospective study of 231 medical records of children (<18 years old) with epistaxis of a total of 1171 cases in the general population who presented to our medical center's emergency department between 2013 and 2018., Results: Among 231 admissions, 10 children (4.3%) presented more than once. Male patients accounted for the majority of cases (64.5%), and the average age was 9.4 years. Two children were treated with aspirin because of cardiac valve disease. Anterior bleeding was detected in 101 cases (43.7%), whereas posterior origin was observed in 8 cases (3.5%). In 122 cases (52.8%), there was no active bleeding observed. Nose injury was the cause of epistaxis in 24 cases (10.4%), and 16 admissions (6.9%) followed nasal surgical interventions. Nineteen children (8%) had abnormal coagulation tests, and 7 patients (3%) received blood transfusions. Chemical cauterization was performed in 89 cases (39.3%), and anterior packing was needed in only 9 cases (3.9%). Nine children required hospitalization (3.9%), and 2 needed surgical intervention to control bleeding. Compared with the adult population, there were significantly fewer cases of active bleeding, recurrent epistaxis, anterior packing, or need for hospitalization in the pediatric population., Conclusions: Epistaxis is significantly less severe in the pediatric population, with only a few cases requiring major intervention. Endoscopic examination of the entire nasal cavity and routine coagulation tests are not mandatory unless there is a history of recurrent epistaxis, known coagulopathy, antiplatelet/anticoagulation therapy, or a suspicion of juvenile idiopathic angiofibroma. We suggest using absorbable packs, which offer advantages over cauterization or nonabsorbable packs., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Abstract: Hereditary hemorrhagic telangiectasia (HHT) is the second-most common inherited bleeding disorder (BD) worldwide and remains without approved therapies. HHT causes serious mucosal bleeding resulting in severe iron-deficiency anemia, major psychosocial complications, and visceral arteriovenous malformations in the brain, lung, and liver, which can cause life-threatening hemorrhagic complications. No study has examined the relative morbidity of HHT and von Willebrand disease (VWD), which is the most common inherited BD in women. We performed an observational cohort study of women with HHT or VWD, comparing a representative sample of 100 randomly selected women with HHT to 100 randomly selected age-matched women with VWD. In HHT vs VWD, recurrent epistaxis and gastrointestinal bleeding were more likely (odds ratio [OR], 32.73 [95% confidence interval, 13.81-71.80]; P < .0001 and 5.69 [2.59-12.89]; P < .0001) and heavy menstrual bleeding was less likely (OR, 0.32 [0.18-0.57]; P < .0001). Iron-deficiency anemia was significantly more likely, and the lowest hemoglobin was significantly lower in HHT than in VWD. The odds of iron infusion dependence, requirement for red cell transfusion, and hemostatic surgical procedures were significantly higher-17-fold, threefold, and eightfold higher, respectively-and hospital admissions to manage disease complications were both ∼14 times more frequent in women with HHT vs those with VWD. In conclusion, much higher disease-related morbidity, mortality, and health care use were observed in women with HHT vs VWD, providing evidence that HHT may be the most clinically significant inherited BD in women. Given the vast gap in research funding for HHT compared with both hemophilia (a disease primarily of men) and VWD, these findings have significant implications for gender equity in hematology., (© 2024 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Epistaxis is a common problem in pediatric patients. Most cases are benign in nature and resolve with minimal intervention. However, there are many unusual conditions that present with epistaxis that can be very serious and should be promptly investigated and treated. This review aids in the distinction between the benign causes of epistaxis and those that are more serious and require further evaluation. The various etiologies of epistaxis are discussed, and recommendations for general and scenario-specific management of pediatric patients with epistaxis are provided.

Key Points: The septal branch of the anterior ethmoid artery (sbAEA) is an underrecognized source of severe refractory epistaxis. Herein, we describe the presentation, predisposing factors, treatment strategies, and outcomes of a series of patients with this condition., (© 2024 ARS‐AAOA, LLC.)

Background: Delayed epistaxis after endoscopic transnasal pituitary tumor resection (ETPTR) is a critical complication, tending to cause aspiration or hemorrhagic shock. This study assessed clinical characteristics, risk factors, and provide treatment and prevention advice of this complication., Methods: This was a retrospective monocentric analysis of 862 patients who underwent ETPTR. Statistical analyses of clinical data revealed the incidence, sources and onset time of delayed epistaxis. Univariate analysis and binary logistic regression were used to identify risk factors., Results: The incidence of delayed epistaxis was 2.78% (24/862), with an average onset time of 20.71 ± 7.39 days. The bleeding sources were: posterior nasal septal artery branch of sphenopalatine artery (12/24), multiple inflammatory mucosae (8/24), sphenopalatine artery trunk (3/24) and sphenoid sinus bone (1/24). Univariate analysis and binary logistic regression analysis confirmed that hypertension, nasal septum deviation, chronic rhinosinusitis and growth hormone pituitary tumor subtype were independent risk factors for delayed epistaxis. Sex, age, history of diabetes, tumor size, tumor invasion and operation time were not associated with delayed epistaxis. All patients with delayed epistaxis were successfully managed through endoscopic transnasal hemostasis without recurrence., Conclusions: Delayed epistaxis after ETPTR tends to have specific onset periods and risk factors. Prevention of these characteristics may reduce the occurrence of delayed epistaxis. Endoscopic transnasal hemostasis is recommended as the preferred treatment for delayed epistaxis., (© 2024. The Author(s).)