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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. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder of the blood vessels that leads to the formation of telangiectasias and arteriovenous malformations (AVMs). HHT affects ∼1/5,000 people, but this varies significantly by geography and ancestry. The Curaçao criteria for HHT consist of four diagnostic criteria: spontaneous epistaxis, first-degree relative with HHT, AVMs in characteristic location (liver, lung, brain), and telangiectasias. Sequelae and major symptomology include recurrent epistaxis, dyspnea, heart failure, and stroke from paradoxical emboli among others. HHT patients are best cared for by a multidisciplinary team, ideally all with HHT-specific experience, but in this review, we will discuss the major aspects of the disease including etiology, diagnosis, and treatment recommendations. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Rendu–Weber syndrome, is a rare autosomal dominant disorder characterized by vascular malformations. This comprehensive review aimed to provide an overview and summarize various aspects of HHT, including the genetic abnormalities, complications associated with visceral arteriovenous malformations (AVMs), prognosis of HHT, quality of life (QOL), and treatment of epistaxis. In addition, this review highlights the challenges in diagnosing HHT and emphasizes the critical role of otolaryngologists in the early detection of HHT. Otolaryngologists can refer patients with refractory epistaxis for AVM screening to expedite intervention. Mutation of the genes involved in the transforming growth factor-β signaling pathway leads to the incidence of HHT, resulting in the formation of abnormal blood vessel formation. These vascular malformations commonly manifest as telangiectasia on the skin and mucous membranes; however, epistaxis remains the hallmark symptom of HHT. The impact of HHT goes beyond the visible symptoms and often includes the formation of life-threatening visceral AVMs in the lungs, liver, and brain. The prognosis of patients with HHT is closely related to the development of these complications, necessitating timely diagnosis and intervention. Refractory epistaxis diminishes the QOL of patients with HHT. The management of epistaxis ranges from conservative measures to advanced interventions such as prevention, conservative treatments, ablation, surgical procedures, and the administration of anti-angiogenic agents. However, effective management requires a multidisciplinary approach. The diagnosis of HHT remains challenging due to its variable presentation and lack of awareness among physicians. This review highlights the importance of reducing the duration between symptom onset and diagnosis. Otolaryngologists who are experienced in the management of refractory epistaxis can aid in identifying potential cases of HHT. They can facilitate the initiation of screening for visceral AVMs via prompt recognition of the signs and symptoms of HHT, contributing to improved patient outcomes. Early detection and intervention through screening can extend the life expectancy of patients with HHT to levels comparable with that of the general population. In conclusion, this review provides insight into various aspects of HHT and emphasizes the importance of timely diagnosis and intervention in the mitigation of the potentially life-threatening complications associated with this disorder. Otolaryngologists play a critical role in this process, serving as gatekeepers to the identification of cases of HHT and implementation of appropriate screening and management pathways, thereby improving the life expectancy and QOL of patients. [ABSTRACT FROM AUTHOR]

Introduction: Epistaxis is vascular bleeding in the area of the nasal mucosa. Anticoagulation therapy increases the incidence of epistaxis. Anticoagulants are divided into direct and indirect depending on their effects. Direct anticoagulants are primarily novel oral anticoagulants (NOACs). Warfarin belongs to the group of indirect anticoagulants. Methodology: The retrospective study evaluates the benefit of the introduction of NOACs for otorhinolaryngology in connection with the occurrence of severe epistaxis. A comparison was done of the number of hospitalized adult patients with epistaxis over the course of 6 years during warfarin and NOAC treatment who were hospitalized with epistaxis at the ENT department of KNTB in Zlín between 2017–2022. The patient's age and gender were also monitored. The severity of epistaxis was evaluated in relation to the used nasal packing and general anesthesia. The relationship with SÚKL data on the number of issued anticoagulant drugs is described. Results: During 6 years, a total of 221 patients were hospitalized for epistaxis, 32 were taking warfarin, and 20 were taking NOACs. The average age of patients with anticoagulant treatment was 78 years, represented by 61.5% men and 38.5% women. Over the years, we see a decrease in the number of epistaxis cases in warfarin patients; with NOACs the number is stationary. According to SÚKL statistics on preparations delivered to pharmacies in 2017 and 2022, there was a decrease in the prescription of warfarin by 44%; on the contrary, an increase of NOAC was up to 170%. Conclusion: With the decrease in the prescription of warfarin, we observe a reduced number of hospitalizations with severe epistaxis with this type of treatment. On the contrary, the increase in anticoagulation therapy with preparations from the NOAC group does not lead to an increase in the incidence of epistaxis, which also benefits the ENT field. [ABSTRACT FROM AUTHOR]

Background: Epistaxis, particularly in Hereditary Hemorrhagic Telangiectasia (HHT) patients, is a common otolaryngological emergency, often requiring complex management. A hierarchy of increasingly invasive interventions, from external compression of the nasal pyramid to nostril closure, is typically proposed and applied. Methods: We conducted a retrospective study on HHT patients to assess the effectiveness and longevity of invasive procedures postoperatively. Data were collected using the Epistaxis Severity Score (ESS) questionnaire. The primary focus was on changes in the frequency and intensity of epistaxis, while the secondary focus was on the overall quality of life. Results: This study found that invasive procedures initially improved the frequency and intensity of epistaxis in HHT patients. However, within 1 to 9 months postoperatively, these benefits often diminished, with hemorrhagic symptoms recurring at similar or worsened levels. Conclusions: The findings suggest a need for a cautious and restrained approach to using invasive treatments in managing epistaxis in HHT patients. Highly invasive procedures should be reserved for cases where less invasive methods fail, due to their temporary effectiveness and the risk of causing anatomical–functional changes in the rhino-sinus area, complicating future management of severe epistaxis. [ABSTRACT FROM AUTHOR]

Epistaxis is one of the common causes of emergency department (ED) visits. However, most cases are not severe and undergo an unnecessary medical evaluation, especially in non-traumatic epistaxis. This study investigated how many patients require a definite observational unit (DOU) and what factors are associated with DOU among nontraumatic epistaxis patients. This retrospective observational study included 1197 nontraumatic epistaxis patients who visited the ED from January 2016 to December 2020. Multiple logistic regression analysis was performed to evaluate the association between risk factors and DOU care. In addition, the receiver operating characteristic (ROC) curve for predicting DOU care was analyzed to estimate the diagnostic ability of risk factors. A total number of 1122 patients with non-traumatic epistaxis were included in the final analysis. Among them, 41 (3.65%) patients needed DOU care. Male sex (odds ratio (OR) = 3.606, p = 0.003), hypertension (OR = 2.362, p = 0.020), inter-hospital transfer (OR = 2.358, p = 0.039), verbal mental status (OR = 29.436, p = 0.035), hemoglobin (Hb) level (OR = 0.724, p < 0.001), revisit after initial discharge for epistaxis (OR = 8.813, p < 0.001), and delayed ED arrival (≥180 min) (OR = 2.451, p = 0.030) were significant factors for DOU care. In addition, the area under the curve of the multiple logistic regression model for predicting DOU care was 0.870. Among the patients who visited ED due to non-traumatic epistaxis, only 3.65% of patients required DOU care. Male sex, mental status, Hb level, ED revisit after initial discharge, inter-hospital transfer and delayed ED arrival (>180 min) were associated with DOU care. These findings will help to triage epistaxis patients before they visit the ED. [ABSTRACT FROM AUTHOR]

Most cases of pediatric epistaxis are spontaneous and self-resolve. However, a subset of children may experience significant bleeding and require procedural or medical intervention. We aim to identify risk factors associated with moderate and severe epistaxis in the emergency department (ED) and explore management outcomes. We retrospectively reviewed all patients under 22 years old with epistaxis who presented to our ED between 2013 and 2022. Epistaxis severity was defined as mild (required nasal compression or intranasal medications), moderate (required cautery or packing), or severe (required factor replacement, transfusion, hospital admission, or surgery). We performed univariable and multinomial regression analyses, with risk factors and outcomes analyzed according to severity. Of 858 visits, 41 (5%) patients had moderate and 67 (8%) had severe epistaxis. Patients with moderate epistaxis were older than those with mild and severe epistaxis (median 15.6 vs. 8.3 vs. 10.7 years, p < 0.001). In regression analysis, moderate epistaxis was associated with older age, prior ED visit within 72 h, and antiplatelet medication use (p < 0.01). Severe epistaxis was associated with bleeding disorders, nasal procedures within 30 days, and anticoagulation medication use (p ≤ 0.001). Bleeding over 30 min prior to arrival was associated with both moderate and severe epistaxis (p < 0.05). Of the 67 patients with severe epistaxis, 10 (15%) required factor replacement, 28 (42%) required transfusion, 52 (77%) required hospital admission, and 5 (7%) underwent surgery. Epistaxis severity is associated with certain risk factors. However, most cases of pediatric epistaxis are mild and do not require intervention or ED evaluation. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by vascular malformations, which cause frequent bleeding events including epistaxis and gastrointestinal bleeding.1 We describe the management of an 84-year-old woman with HHT who received end-of-life care on a palliative care ward. When the patient was unable to swallow her tranexamic acid tablets, this was converted to a continuous subcutaneous infusion, which was continued for a week until death. She had no significant bleeding events or site reactions. To our knowledge, this case report is the first to describe the palliative management of a patient with HHT at the end of life. This also adds to the current evidence base that subcutaneous tranexamic acid may be a safe and effective alternative to oral administration. Further research is needed to improve understanding of the appropriate patient selection, safety, and efficacy. [ABSTRACT FROM AUTHOR]

Pyogenic granuloma is a benign proliferative fibrovascular lesion commonly arising from the skin and mucous membranes of the head and neck region. Histologically, this tumor is characterized by vascular proliferation and a circumscribed group of capillaries organized in lobules. It is usually located in the oral cavity and nasal location is less frequent. When it occurs in pregnant women, it is usually referred as pyogenic granuloma gravidarum. In this article we present the case of a pyogenic granuloma gravidarum in a young woman with intermittent epistaxis during the last trimester of pregnancy that did not resolve after childbirth and was treated with transnasal endoscopic resection and cautery at the base of the lesion for hemostasis under local anesthesia. [ABSTRACT FROM AUTHOR]

Background: The Epistaxis Severity Score (ESS) is the gold-standard patient-reported outcome measure for evaluating nosebleed severity in patients with hereditary hemorrhagic telangiectasia (HHT). To date, the ESS has been assessed only for content validity and concurrent validity. Objective: We evaluate the internal consistency and test–retest reliability of the ESS. Materials and Methods: After receiving institutional review board approval, we sent an online survey battery, including the ESS survey, to 305 (39% male) English-speaking HHT patients ≥18 years old at a single center. Of those, 140 (46%) patients completed the battery, and 110/140 (79%) reported epistaxis. Cronbach's alpha and correlation analyses were used to evaluate internal consistency. For the test–retest reliability evaluation, we recruited 69 HHT patients during HHT clinic to complete 2 self-administered ESS surveys 2 weeks apart. Participants also completed a modified Clinical Global Impression-Improvement scale with readministration of the ESS survey. We calculated the intraclass correlation coefficient in a 2-way mixed model with absolute agreement. Results: The ESS survey demonstrated low internal consistency (Cronbach's alpha = 0.495), suggesting that it measured multiple unrelated concepts. Factor analysis revealed 3 latent factors with moderate intercorrelation, suggesting the presence of 3 related but distinct constructs underlying the ESS. However, the ESS demonstrated excellent test–retest reliability (intraclass correlation coefficient = 0.955; 95% CI, 0.91-0.98). Conclusion: Although the ESS demonstrates high test–retest reliability, it may not adequately assess different dimensions of nosebleed severity. Additional correlated survey questions and sub-scores may be needed to increase internal consistency to accurately measure each component of epistaxis severity. It is necessary to acknowledge epistaxis severity from different dimensions and to consider evaluating individual ESS items separately for a comprehensive understanding. [ABSTRACT FROM AUTHOR]

The most common presenting symptoms for acute sinusitis include fever and toxicity; chronic sinusitis symptoms include facial pressure, headache, nasal obstruction, postnasal drip, and fatigue. All the patients who satisfied the inclusion criteria of the study were subjected to history taking and physical examination to identify clinical signs at presentation. Then the patients were subjected to CT scan of nose and paranasal sinus region and the anatomy of the sinonasal region was thoroughly assessed after a probable diagnosis was made. After completing all investigations, definitive management was done and the radiological features were correlated with the clinical and endoscopic diagnosis. In the present Study, 18.0% had Nasal Discharge, 42.0% had Headache, 16.0% had Nasal Obstruction, 10.0% had Loss of Smell, 12.0% had Post Nasal Drip, 4.0% had Epistaxis, 10.0% had Nasal Mass, 6.0% had Giddiness and 8.0% had Fatigue. The most common symptom in the present study was headache (42%) followed by nasal discharge (18%). The least common symptom was epistaxis (4%). [ABSTRACT FROM AUTHOR]

Background: Hereditary Hemorrhagic Telangiectasia (HHT) is a vascular autosomically inherited rare disease. Epistaxis (nose bleeds) is the most common symptom in HHT, leading to anemia and affecting the patient's quality of life. In addition to epistaxis, gastrointestinal bleeding (GI), more often at older ages, may lead to severe anemia and the need for blood transfusions. Thus, finding drugs to control both types of bleeding is a primary necessity in HHT. Methods: A cross-sectional observational study was conducted in a series of 11 HHT patients treated with low tacrolimus doses (0.5–2 mg/day) on an off-label prescription basis. Patients showed refractory bleeding to previous treatments. The epistaxis severity score (ESS) and hemoglobin levels were the parameters used to evaluate the impact of tacrolimus. The occurrence of side effects was also recorded. Results: Tacrolimus was well tolerated in all of the patients except 2 (who stopped the treatment). The remaining patients tolerated the treatment, with a general improvement in their health condition. Epistaxis was significantly reduced when comparing the ESS before and after the treatment. Hemoglobin levels significantly increased, overcoming the anemia, during the course of the treatment. Conclusion: Tacrolimus at low doses should be considered as a promising treatment for epistaxis and gastrointestinal bleeding in HHT. [ABSTRACT FROM AUTHOR]

Background: Congenital erythropoietic porphyria (CEP), also known as pink tooth or Gunther disease, is a rare hereditary disorder caused by an enzyme mutation in the heme biosynthesis pathway, which leads to the accumulation of immature and non-physiological protoporphyrin rings in various tissues. CEP is characterized by sun-exposed bullous skin lesions, hemolytic anemia, red/brown urine, and teeth staining. Case presentation: We present a unique case of a 10-year-old Asian boy with CEP who presented with recurrent epistaxis, an unusual presentation for this condition. Based on clinical presentation and laboratory findings, including elevated urine uroporphyrin and coproporphyrin I and III levels, microcytic anemia, a higher red cell distribution width (RDW), and a lower platelet count, a thorough assessment and detailed workup resulted in a diagnosis of CEP. The patient underwent a successful splenectomy and recovered without any complications. Conclusion: This case report aims to raise awareness among healthcare professionals about the uncommon and atypical presentation of CEP and its management options. [ABSTRACT FROM AUTHOR]

Background: Due to a myriad of risk factors, epistaxis is a very frequent presentation to the emergency room (ER). This study aims to ascertain the prevalence of epistaxis in our population, risk factors, effectiveness of ER treatment, complications, and ER outcome.Materials and Methods: This was a prospective observational study performed in the ER of a referral tertiary care center in south India. Data were categorized, coded, and analyzed to determine the objective of the study. Results: During the study's six-month duration, 188 (0.6%) patients presented with epistaxis. The mean age was 42.9 (SD: 16.49) years, with a male preponderance of 143 (76.1%). A majority of these patients (n: 156; 82.9%) were triaged as priority II, with hypertension (n: 53, 28.2%) as the commonest comorbidities. Trauma-related epistaxis (n: 107, 56.9%) was the most frequent cause. Anterior nasal packing was carried out for 85 (45.2%) patients, posterior nasal packing was carried out for one (0.5%) patient, and bleeding had spontaneously resolved in the majority (n: 102; 54.3%) patients. Seven (3.7%; p-value: 0.001) patients had recurrent epistaxis, and of those, three (1.6%) required urgent resuscitation with crystalloid fluid and blood products. Two of these patients had bleeding dyscrasias, four had history of trauma, and one patient presented with uncontrolled hypertension. Two (1.1%) patients came back to us with recurrent bleeding within 12 h of discharge. Majority (69.2%; 130) were discharged stable, (23.9%; 45) were admitted for observation and (6.9%; 13) were discharged against medical advice. There was no mortality among these study populations. Conclusion: Middle-young, aged males most commonly presented with epistaxis. Most of them were secondary to trauma. Anterior nasal bleeding was the most common source and hemostasis could be obtained by anterior nasal packing. Majority could be discharged stable from the ER. However, this cohort had seen patients in life-threatening conditions, so the severity cannot be overlooked. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article provides insights into the death of the Roman Emperor Hadrian, an emperor with a history of epistaxis who developed progressive epistaxis, cachexia, and edema. Topics discussed include conditions to which the emperor's acute deterioration could be attributable, his diagnosis to explain heart failure and epistaxis, and indication of his rare autosomal dominant syndrome.

Epistaxis, commonly encountered in the Otolaryngology practice, refers to bleeding that occurs as a result of vascular pathologies and coagulation disorders. It can develop due to traumatic, iatrogenic, or spontaneous etiologies. In our study, we aim to retrospectively examine the epistaxis patients treated in our clinic to investigate the etiology causing epistaxis, associated diseases, and the use of anticoagulants-antiplatelets. Additionally, we plan to evaluate hemogram and coagulation parameters. The study included patients diagnosed with epistaxis and admitted for treatment at Samsun Education and Research Hospital ENT Clinic between 2019 and 2022. A retrospective analysis of the patients' medical records was conducted to gather data on demographic characteristics, comorbidities, medications used regularly, laboratory values, and clinical findings. A total of 130 patients were included in the study. No statistically significant difference was observed between the location of the bleeding focus and gender, presence of hypertension, use of antihypertensive medication, or anticoagulant use. When comparing patients with and without antiplatelet-anticoagulant use, a significantly longer nasal packing duration was found in patients who used these medications. In terms of the need for electrocauterization, statistically significant higher intervention rates were observed in patients who did not use anticoagulant medication. Our study identified a higher rate of surgical intervention in hospitalized epistaxis patients, potentially due to the inclusion of more severe cases requiring specialized care. Further research is needed to investigate the factors influencing the need for surgical management and to develop appropriate guidelines for the management of different severities of epistaxis. [ABSTRACT FROM AUTHOR]

Epistaxis is a common presenting symptom either emergency condition or ambulatory in Otorhinolaryngology, affecting people of all ages. A multicentric retrospective descriptive study of 380 patients who were hospitalized with epistaxis over a 3.8 year period was carried out. Data collected was analyzed using IBM SPSS Statistics 25 and illustrated using Microsoft Office/Word 2016. The current study showed that male adults after 60 years old are most affected by nasal bleeding. More than half of hospitalized patients have severe epistaxis at the time of hospitalization. However, further studies are necessary to be done in order to fully elucidate the epidemiology of nosebleeds requiring hospitalization. [ABSTRACT FROM AUTHOR]

This article discusses a case of an elderly patient with recurrent epistaxis (nosebleeds) and purple vascular stains on the skin. The patient was diagnosed with hereditary hemorrhagic telangiectasia (HHT), a rare genetic disorder characterized by abnormal blood vessels and arteriovenous malformations. The patient had a late onset of the disease and no family history, which can be attributed to sporadic mutations or non-recognition of the disease in affected parents. The article provides information on the diagnostic criteria, genetic mutations, and management options for HHT. [Extracted from the article]

Hemophilic pseudotumor is a rare, yet dangerous complication of hemophilia. It has been reported previously at sites prone to recurrent trauma like long bones and pelvis. However, in the field of otorhinolaryngology, few cases are reported and therefore there is no established protocol for management. We hereby report a case of a 2-year-old boy, a known case of hemophilia A (factor VIII deficiency), who presented with recurrent epistaxis not responding to medical management. Imaging was done and revealed a heterogenous nasal mass compressing the left orbital wall, extending to the sphenoid sinus, and causing skull base erosion. The patient was successfully managed by evacuation and drainage of the pseudotumor via endoscopic endonasal approach and replacement of factor VII pre-and post-operatively. To our knowledge, this is the first case of nasal hemophilic pseudotumor managed by evacuation and drainage through an endoscopic endonasal approach, which was deemed successful. [ABSTRACT FROM AUTHOR]

Recurrent epistaxis represents an alarming sign that may suggest a sinister aetiology, especially in patients with previous head and neck malignancy. The recognition of certain potentially life-threatening conditions, namely pseudoaneurysm or tumour recurrence, remains prudence to avoid disastrous repercussion. Nasal endoscopy has become an essential tool in otolaryngology. It can aid identify the underlying cause of epistasis and facilitate therapeutic management. On the other hand, radio imaging is highly sensitive in detecting vascular lesions, besides providing a pre-operative mapping if surgical intervention is planned. This paper reported a patient with sphenoidal sinus squamous cell carcinoma in remission presented with torrential epistaxis not relieved with nasal packing. Despite a repeated angiogram and magnetic resonance image, the identification of the source of bleeding remained futile, culminating in an examination under general anaesthesia. The diagnosis of carotid blowout syndrome was made intraoperatively, and the bleeding was temporarily secured with a muscular patch, preceded by the insertion of a vascular stent. The authors wish to highlight the importance of examination under general anaesthesia if radio imaging does not correlate to the clinical findings. Management options for carotid blowout should be tailored to the patients' medical conditions. [ABSTRACT FROM AUTHOR]

Objective: We report four cases of migraine induced epistaxis and reviewed the published literature of migraine with epistaxis to establish demographic profiles, types of migraine, severity, family history of headache and other associated disorders in adults. Methods: A panoramic search of the Medline database through Pubmed was done in May 2022 using search terms: {Migraine with Epistaxis}, case reports. All articles/case reports in English from January 2001 to April 2022 where age of patients was > 18 years were included in our review. Results: Our search recognized total of three cases and with inclusion of the four cases who reported to us, we reviewed these seven cases and studied demographic profile, clinical features, association of epistaxis with types and severity of migraine and relationship with other medical disorders. Mean age of presentation was 28.7 years (range 18–49), with five female and two male patients. The intensity of headache was severe in three of seven cases and there was one case each of moderate and mild category. Five of seven (71%) patients had reduction in intensity of headache with onset of bleeding and various types of migraine (Migraine with and without Aura, vestibular migraine, sporadic familial hemiplegic migraine) according to ICHD classification were associated with epistaxis. Four of seven had positive family history of migraine. There was no diagnostic finding in any patient and all patients responded to migraine preventive medication. Conclusion: Recurrent epistaxis is not so uncommon manifestation of various types of migraine and specialist should keep this clinical diagnosis in mind to avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

Hereditary haemorrhagic telangiectasia, also known as Rendu–Osler–Weber disease, is an autosomal dominant disorder of the fibrovascular tissue common in Western countries. It is characterized by the classical triad of mucocutaneous telangiectasia, arteriovenous malformations with recurrent epistaxis. Here we report a rare case of Hereditary haemorrhagic telangiectasia in a 66-year-old Indian male who presented with a history of recurrent epistaxis of forty years duration. The nasal telangiectasias were ablated under narrow band imaging guidance. The rarity of diagnosis was aided by clinical exome sequencing to confirm the disease. [ABSTRACT FROM AUTHOR]

Background: Endovascular embolization (EE) is a treatment option for epistaxis refractory to first-line interventions. Data regarding embolization is limited to small case series and a meta-analysis has not been performed. Methods: PubMed, Scopus, and EMBASE were used to identify studies that reported outcomes for at least 10 patients undergoing EE for epistaxis. Outcomes included procedural success, rebleeding, and complications. Pooled rates for each outcome were obtained with random effects models. Results: A total of 44 studies comprising 1664 patients met the inclusion criteria. The mean age ranged from 28.1 to 67 years and there were 28.4% females. The pooled procedural success rate was 87% (95% CI 83.9–89.6, I 2 = 53%). Age (OR 0.95, 95% CI 0.91–1) and hereditary hemorrhagic telangiectasia ([HHT], OR 0.97, 95% CI 0.96–0.99) were associated with decreased odds of success. The pooled rebleeding rate was 16.4% (95% CI 13.6–19.6, I 2 = 48%), and HHT was associated with greater odds of rebleeding (OR 1.02, 95% CI 1–1.03). The pooled overall complication rate was 14.4% (95% CI 9.8–20.6, I 2 = 85.8%). The pooled rates of stroke and vision loss were 2.1% (95% CI 1.5–3.1, I 2 = 1.5%) and 1.8% (95% CI 1.2–2.6, I 2 = 0%), respectively. Conclusion: EE for epistaxis has a high rate of procedural success. Interventionalists should be aware of the risk for rebleeding, especially among patients with HHT. [ABSTRACT FROM AUTHOR]

Background and Objectives: Epistaxis is one of the most common emergencies in otolaryngology, and the recently developed Rapid Rhino nasal pack, a balloon-type nasal packing device, is widely used in emergency departments. Rebleeding after initial treatment increases patients' discomfort and medical costs. The aim of this study was to investigate risk factors for rebleeding in patients treated with Rapid Rhino packing. Methods: In this retrospective study, 93 patients with epistaxis treated with Rapid Rhino from January 2020 to November 2022 were divided into the well-controlled group (39 patients) and the rebleeding group (54 patients), and the baseline characteristics, management methods, and complications were compared between these groups. The rebleeding group was divided according to whether patients experienced a single episode of rebleeding (38 patients) or multiple rebleeding episodes (16 patients), and the differences between these two groups were compared. Results: Oral anticoagulation therapy was associated with a higher risk of rebleeding after Rapid Rhino packing (odds ratio [OR]= 8.41, p=0.047). A history of nasal surgery was associated with multiple rebleeding (OR=22.55, p=0.009). Age, sex, the management method, complications, and the site of bleeding were not found to be related to rebleeding. Conclusion: Patients with rebleeding after Rapid Rhino nasal packing had a higher rate of concurrent oral anticoagulation therapy. A history of nasal surgery was strongly associated with multiple episodes of rebleeding. A detailed medical history can be important for assessing the risk of rebleeding in epistaxis patients treated with Rapid Rhino packing. [ABSTRACT FROM AUTHOR]

Background: Risk factors and outcomes associated with severe epistaxis are not well understood. This study explores the associations between epistaxis severity, comorbidities, use of antiplatelets or anticoagulants and management outcomes. Methods: This is a retrospective cross‐sectional study of all epistaxis cases presenting to the emergency department at a tertiary academic hospital from January 2016 to December 2019. Epistaxis severity was defined as mild (no intervention), moderate (required cautery and/or packing) and severe (clinical instability with reversal products, surgical or radiological intervention). Univariable and multivariable regression analyses were undertaken, with risk factors and management outcomes analysed according to severity. Results: A total of 543 patients with epistaxis (54.2% male, mean age 74.4 ± 15.7 years) were included in this study, with 14.7% (80) having severe epistaxis. Of these presentations 216 (39.8%) were on antiplatelets, while 207 (38.1%) were on anticoagulants. In univariate analyses, clopidogrel use, hereditary haemorrhagic telangiectasia (HHT), haematological malignancy, bleeding disorders and chronic liver disease (CLD) were associated with moderate to severe epistaxis (P < 0.05), while the use of rivaroxaban was inversely associated severity (P = 0.002). Only HHT, haematological malignancy and CLD remained significant in multivariate models. Cautery as first‐line management was infrequently utilized while anticoagulation was frequently withheld. A longer length of stay (1.1 days vs. 4.3 days; P < 0.001) and higher 2‐week readmission rates (2.2% vs. 12.5%; P < 0.001) were noted with severe epistaxis compared with mild presentations. Conclusion: Epistaxis severity is associated with certain clinical conditions and poor outcomes. Despite recommended guidelines, variations in first‐line management were evident. [ABSTRACT FROM AUTHOR]

Study Design: Cross sectional. Background: Epistaxis is one of the most common otorhinolaryngological emergencies affecting the majority of the population in their lifetime, with some of population requiring serious medical attention. This study aimed to assess the prevalence and associated factors of epistaxis among random people lives in Asser region in Saudi Arabia. Method: A cross-sectional study of Saudi community in Asser region. This study intended to assess the prevalence of Epistaxis among male and female. Other objectives, included exploring any associated risk factors of Epistaxis among Saudi community in Asser region. Data were collected through pre-tested interviewee administered questionnaire divided into several sections. Result: 400 participants, 80.3% of them were female and 19.7% of them were male. The overall epistaxis prevalence was 22.9%. This study found a link between epistaxis and duration, frequency of epistaxis, seasons, psychological stress, a family history of epistaxis, high blood pressure, cardiac disease, hematological disease, smoking, allergy, use of nasal spray, and head trauma. and duration of epistaxis. Conclusion: This study revealed that the level of epistaxis among Asser region population is significantly associated with summer season, hypertension, cardiac diseases, hematological diseases, face trauma, allergy, stress and smoking. It is better to work on increase the awareness about the meaning of epistaxis and identify the main and important causes and factors may lead to it. [ABSTRACT FROM AUTHOR]

Objectives: Hereditary hemorrhagic telangiectasia (HHT) is a common inherited condition characterized by mucosal telangiectasias, recurrent epistaxis, and arteriovenous malformations. HHT results in detriment to quality of life. Morbidity and mortality result from severe anemia. Conventional interventions for HHT-related epistaxis include nasal packing, diathermy, lasers, coblation, microdebridement, bevacizumab (topical and systemic), as well as septodermoplasty and nasal closure. Sclerotherapy has been recently described in the literature as a novel approach to HHT-related epistaxis. We hypothesize that sclerotherapy is an effective treatment for HHT-related epistaxis and improves upon the current standard of care for this disease. Methods: A systematic review was conducted to study sclerotherapy for treating HHT-related epistaxis. Ovid MEDLINE, Ovid EMBASE, Scopus, and Web of Science were searched. Articles were evaluated and excluded according to PRISMA guidelines and reviewed by 2 authors. Reported variables included number of injections, months of follow up, changes in Epistaxis Severity Score, previous treatments used to control epistaxis, and post-injection side effects. Results: Seven studies with a total of 196 patients met inclusion criteria. Three studies reported significant improvement as measured by the Epistaxis Severity Score scale. One reported improvement through subjective patient surveys and others used the Bergler-Sadick scale to measure frequency and intensity of epistaxis. All studies reported improvement in HHT-related epistaxis. The lack of uniform reporting measures however precluded formal meta-analysis. Conclusions: Based on limited data, sclerotherapy appears to be effective for treating HHT-related epistaxis and offers promise for treating this recalcitrant condition. However, larger, prospective, multi-centered studies using universally validated instruments for epistaxis are needed to definitively evaluate outcomes from sclerotherapy. [ABSTRACT FROM AUTHOR]

To date, there is no approved local therapeutic agent for the treatment of epistaxis due to hereditary hemorrhagic telangiectasia (HHT). Several case reports suggest the topical use of timolol. This monocentric, prospective, randomized, placebo-controlled, double-blinded, cross-over study investigated whether the effectiveness of the standard treatment with a pulsed diode laser can be increased by also using timolol nasal spray. The primary outcome was severity of epistaxis after three months, while the main secondary outcome was severity of epistaxis and subjective satisfaction after one month. Twenty patients were allocated and treated, of which 18 patients completed both 3-month treatment sequences. Timolol was well tolerated by all patients. Epistaxis Severity Score after three months, the primary outcome measure, showed a beneficial, but statistically nonsignificant (p = 0.084), effect of additional timolol application. Epistaxis Severity Score (p = 0.010) and patients' satisfaction with their nosebleeds after one month (p = 0.050) showed statistically significant benefits. This placebo-controlled, randomized trial provides some evidence that timolol nasal spray positively impacts epistaxis severity and subjective satisfaction in HHT patients when additively applied to standard laser therapy after one month. However, the effect of timolol was observed to diminish over time. Trials with larger sample sizes are warranted to confirm these findings. [ABSTRACT FROM AUTHOR]

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease affecting 1 in 5000 individuals. Epistaxis is seen in more than 90% of patients with HHT. Severe recurrent epistaxis can significantly decrease quality of life and may be resistant to standard treatment measures. Dysregulation of angiogenesis has been shown to cause the proliferation of abnormal blood vessels. As such, antiangiogenic treatments have been investigated including beta-blockers. Objective: A systematic review of the efficacy of beta-blockers in topical treatment of epistaxis in patients with HHT based on epistaxis duration, frequency, and severity. Methods: A systematic search was performed using the PubMed, Embase via Ovid, and Cochrane databases. The Preferred Items for Systematic Reviews and Meta-Analyses guidelines were followed. Studies that measured the efficacy of beta-blocker treatment of epistaxis in patients with HHT were included for qualitative analysis. Results: Five studies (3 randomized controlled trials and 2 case series) with a total of 132 patients were included. Administration (systemically or topically via a spray or gel) of timolol and propranolol showed mixed evidence of improvement in epistaxis frequency, severity, and duration when compared with control groups. The evidence for propranolol appears more promising than timolol. Conclusion: There are significant limitations in the included studies, and further investigation with larger longitudinal or randomized prospective trials is recommended. The available evidence suggests that beta-blocker treatment may have a positive effect on HHT-related epistaxis. [ABSTRACT FROM AUTHOR]

Epistaxis is one of the most common emergencies. Topical agents such as silver nitrate, is known to be a strong oxidizing agent and have been widely used, especially in recurrent and refractory cases. The extent of depth of coagulative necrosis is dependent on time of exposure and concentration. 2 mm wide bar of nasal septum from 30 patients was taken and AgNO3 was applied for 10, 20, 40 s. H&E staining was then performed to see depth of penetration and intensity of stain deposits. It was observed that with increase time of application, depth of penetration and density of deposits were increased for both 75% and 100% concentration of solution. Distribution in depth of penetration was significant when 75% concentration was applied for more than 20 s. But when 100% concentration was used, similar findings were found even after 10 s in young age group. Our study shows that when silver nitrate is used for chemical cauterization in different concentrations and for different times, their penetration is different. Moreover, the depth of penetration depends more on the time of contact than concentration of silver nitrate. We would recommend using 75% solution for use with a contact time not more than 20 s in adults and 10 s in children. [ABSTRACT FROM AUTHOR]

Background: Given its role in the disease pathophysiology, inhibition of vascular endothelial growth factor (VEGF)‐mediated angiogenesis has received attention as a potential strategy to reduce epistaxis associated with hereditary hemorrhagic telangiectasia (HHT). In this study we evaluated the efficacy of a submucosal injection of bevacizumab, a VEGF inhibitor, in reducing the severity of epistaxis and improving quality of life when given at the time of operative electrocautery. Methods: This randomized, double‐blinded, placebo‐controlled trial was conducted at a single institution from 2014 to 2019. Patients scheduled to undergo operative bipolar electrocautery of nasal telangiectasias were randomized to receive a submucosal injection of saline or bevacizumab at time of surgery. Surveys to assess epistaxis severity and quality of life (QOL), including the Epistaxis Severity Score (ESS) and the 12‐item Short Form (SF‐12), were administered preoperatively and at 1, 2, 4, and 6 months postoperatively. The minimal clinically important difference (MCID) of the ESS instrument is reported to be 0.71. Results: Of 39 patients enrolled, 37 (94.9%) completed the study. The saline group demonstrated a reduced ESS vs baseline at 1 (−1.2; p = 0.01) and 4 (−1.2; p = 0.05) months postprocedure. The bevacizumab group demonstrated a reduced ESS score vs baseline at 1 (−2.3; p < 0.001), 2 (−2.3; p < 0.001), 4 (−2.0; p = 0.003), and 6 (−1.3; p = 0.05) months postprocedure. The additive benefit of bevacizumab over saline exceeded the MCID at 1, 2, and 4 months, but the difference was not statistically significant. Conclusion: The addition of a single treatment of submucosal bevacizumab may be associated with additional clinically meaningful benefit for up to 4 months when compared with electrocautery alone. [ABSTRACT FROM AUTHOR]

A boy, agcd 2 years and 5 months, had recurrent cpistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombincmia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin Kt. The boy has been followed up for 6 months, and no cpistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis. [ABSTRACT FROM AUTHOR]

Copyright of Ginecología y Obstetricia de México is the property of Federacion Mexicana de Ginecologia y Obstetricia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article highlights that epistaxis is defined as bleeding from the nasal tissue and is one of the most common presentations seen in ENT and primary care practice. It also discusses about the plexus which is located in the anterior nasal septum and can be readily visualised via anterior rhinoscopy.

Objective: This study aimed to assess the health-related QoL (HR-QoL) of patients with hereditary hemorrhagic telangiectasia (HHT), with emphasis on the role/social aspects, and validate the Japanese version of the epistaxis severity score (ESS) in these patients.Methods: The Japanese version of the ESS was created through forward and reverse translation, and consultation with the original author. A validation analysis was performed by comparing ESS severity with the invasiveness of previous treatments for epistaxis and assessing the correlation between the ESS and HR-QoL. Medical history forms, ESS questionnaires, and the Medical Outcomes Study Short Form 36 (SF-36) were distributed to participants with HHT in August 2020. The relation between the ESS and summary scores of SF-36 was assessed by performing analysis of variance and Spearman's correlation.Results: In total, 73 participants were included in this study. The average ESS was 5.02; there were mild (32.9%), moderate (45.2%), and severe (21.9%) epistaxis groups. Patients with higher ESS received a significantly more invasive treatment (Fisher's exact test, p < 0.05). The ESS was also negatively correlated with the physical component score (PCS) (r = -0.489, p < 0.001). Comorbid liver and gastrointestinal arteriovenous malformations significantly reduced the PCS (p < 0.05). Multiple regression analysis revealed that the ESS was a significant variable (p < 0.01). The role/social component score was significantly lower in the severe ESS group than in the mild or moderate group.Conclusion: The Japanese version of the ESS was considered valid and may be useful as an outcome measure of future HHT-associated epistaxis trials in Japan. [ABSTRACT FROM AUTHOR]

Copyright of QhaliKay: Revista de Ciencias de la Salud is the property of QhaliKay Revista de Ciencias de la Salud and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease with manifestations of epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations of multiple organs, including the lungs, liver, and brain. It can also lead to fatal events such as high-output cardiac failure, respiratory failure, stroke, sepsis, and other hemodynamic complications. Therefore, early awareness of HHT is essential. We describe a 55-year-old man highly suspected of HHT with a rare initial presentation of atrial fibrillation (AF) and remarkable varicose veins at an unusually young age. AF occurring at a young age and refractory varicose veins caused by high-output heart failure might be a whistleblower for HHT, and early awareness may help diagnosis and appropriate management. [ABSTRACT FROM AUTHOR]

Appropriate management of hereditary hemorrhagic telangiectasia (HHT) is of particular importance in females, as HHT-mediated modifications of the vascular bed and circulation are known to increase the risk of complications during pregnancy and delivery. This study was undertaken to evaluate female HHT patients' awareness of and experience with HHT during pregnancy and delivery, with a focus on epistaxis. In this retrospective study, 46 females (median age: 60 years) with confirmed HHT completed a 17-item questionnaire assessing knowledge of HHT and its pregnancy-associated complications, the severity of epistaxis during past pregnancies and deliveries, and the desire for better education and counselling regarding HHT and pregnancy. Results revealed that 85% of participants were unaware of their disease status prior to the completion of all pregnancies. Further, 91% reported no knowledge of increased pregnancy-related risk due to HHT. In regard to epistaxis, 61% of respondents reported experiencing nosebleeds during pregnancy. Finally, approximately a third of respondents suggested that receiving counseling on the risks of HHT in pregnancy could have been helpful. Findings suggest that awareness of HHT and its potential for increasing pregnancy-related risk is poor. Best practices in HHT management should be followed to minimize negative effects of the disorder. [ABSTRACT FROM AUTHOR]

Introduction Epistaxis is a common presentation among children. Objective To investigate the suitability of a simple tool of assessment for patients with epistaxis that could guide in subgrouping those with possible bleeding tendencies who may need further assessment. Methods Children who presented to a tertiary outpatient clinic with epistaxis of an unknown cause were recruited. They underwent thorough clinical assessment and answered the pediatric bleeding questionnaire and the epistaxis severity score. All patients underwent complete blood count as well as coagulation profile, and confirmatory diagnostic tests were performed as needed. Results Among the 30,043 patients who presented to the outpatient clinic over a year, 100 children had epistaxis, with an estimated annual frequency of 1 in 300. A total of 84% of the patients were younger than 12, and nearly half of these were younger than 6 years. Seventy-six patients had recurrent epistaxis, and 12 had systemic comorbidities. A significant higher percentage of patients presented with epistaxis in the hot months of the year. A total of 90% of the patients presented anterior bleeding, and the majority were treated with nasal compression only. Forty-three patients presented with epistaxis only; 37 of them were diagnosed as idiopathic epistaxis, and 6 had local causes. Fifty-seven patients presented with other bleedingmanifestations, 47 of whomhad a definite bleeding disorder and the other 10 had undiagnosed bleeding tendency. Those with other bleeding manifestations showed a higher frequency of positive family history of epistaxis; of being referred from a primary care physician; of having alarming low platelet count, and of presenting less seasonal variability. A bleeding score - 2 showed significant value in suspecting an underlying systemic pathology as a cause of epistaxis. Conclusion The pediatric bleeding questionnaire is a useful and simple tool in the identification of pediatric patients who need further diagnostic testing to detect any underlying bleeding tendency. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia, is a rare genetic disease that causes recurrent epistaxis and anemia. Numerous bleeding vascular malformations can be found throughout the body. CASE PRESENTATION: A 75-year-old woman presented to her hematologist with recurrent epistaxis, iron deficiency anemia, menorrhagia, and hypothyroidism. Her mother had similar nosebleeds, and physical examination revealed small vascular malformations on the conjunctiva, oropharynx, tongue, lip, and palate. Heavy epistaxis occurred several times per week. Multiple nasal and gastrointestinal endoscopic procedures were performed. She received over 100 iron infusions and multiple blood transfusions. Overall treatment involved integrated care with multiple medical specialties. CONCLUSION: Hereditary hemorrhagic telangiectasia and other complex diseases are best treated with a multidisciplinary approach within an integrated health care setting. [ABSTRACT FROM AUTHOR]

Background: Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic vascular disorder which is characterised by the development of arteriovenous malformations and telangiectasias. A key clinical manifestation is recurrent epistaxis. This study examined the impact of a dedicated HHT clinic in a major Australian tertiary hospital on epistaxis symptoms and subjective quality of life. Methods: A multidisciplinary HHT clinic was established in 2015. All patients satisfied either genetic diagnosis or fulfilled Curacao criteria. A protocol based clinical assessment was performed and a standardised treatment regimen was implemented. Patients completed quality of life and epistaxis severity score (ESS) questionnaires at each review. Results: Participants of the dedicated clinic included 21 females (58.3%) and 15 males (41.7%), with a mean age of 49.0 ± 24.0 years. The ACVRL1 variant was the most common (n = 20, 55.6%). A statistically significant reduction in epistaxis severity was noted between the baseline and second review (P = 0.02) and was maintained to the third visit (P = 0.015). Patients older than 50 years demonstrate a consistently higher ESS than those less than 50 years old (P = 0.03). This trend is noted throughout the follow up period with the dedicated clinic. Conclusion: The introduction of a multidisciplinary, dedicated HHT clinic to provide enhanced assessment, monitored treatment regimen and greater access to care, resulted in improvement in the management of epistaxis and quality of life in these patients. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is a rare angiogenic disorder causing chronic gastrointestinal bleeding, epistaxis, and severe anemia. Pazopanib is an oral multi-kinase angiogenesis inhibitor with promise to treat bleeding in HHT. We analyzed outcomes of HHT patients with the most severe bleeding causing RBC transfusion dependence treated on a predefined institutional pazopanib treatment pathway (with data collected retrospectively). The primary endpoint was achievement of transfusion independence. Secondary endpoints included hemoglobin, epistaxis severity score, RBC transfusion and iron infusion requirements, number of local hemostatic procedures, ferritin and transferrin saturation, compared using paired and repeated measures mean tests. Thirteen transfusion-dependent HHT patients received pazopanib [median (range) dose 150 (25–300) mg daily)] for a median of 22 months. All patients achieved transfusion independence. Compared with pretreatment, pazopanib increased mean hemoglobin by 4.8 (95% CI, 3.6–5.9) g/dL (7.8 vs. 12.7 g/dL, P < 0.0001) and decreased mean epistaxis severity score by 4.77 (3.11–6.44) points (7.20 vs. 2.43 points, P < 0.0001) after 12 months of treatment. Compared with 3 months of pretreatment, RBC transfusions decreased by 93% (median of 16.0 vs. 0.0 units, P < 0.0001) and elemental iron infusion decreased by 92% (median of 4500 vs. 0 mg, P = 0.005) during the first 3 months of treatment; improvements were maintained over time. Pazopanib was well-tolerated: hypertension, lymphocytopenia, and fatigue were the most common TEAEs. In conclusion, pazopanib was safe and effective to manage severe bleeding in HHT, liberating all patients from transfusion dependence and normalizing hematologic parameters at doses lower than used to treat malignancies. These findings require confirmation in a randomized trial. [ABSTRACT FROM AUTHOR]

The article presents a case study of vasospastic angina (VSA) in a 13 year old boy with hereditary hemorrhagic telangiectasia (HHT). It is reported that HHT is characterized by telangiectasis and arteriovenous malformations (AVMs). It is further reported that the boy experienced intense nocturnal chest pain, ST-segment elevation on the electrocardiogram, and elevated cardiac troponin I levels.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an inherited orphan disease, in which the absence of capillary beds between arterioles and venules lead to arteriovenous shunts. Epistaxis is the core symptom. Several case reports have described the nonselective beta-adrenergic receptor antagonist timolol as a successful treatment method of nosebleeds due in HHT patients. OBJECTIVE: TIM-HHT is a single-site, prospective, randomized, placebo-controlled, double-blind, cross-over study to investigate whether the efficacy of standard laser treatment of epistaxis in HHT patients can be increased by the additional use of timolol nasal spray (1 mg/d). METHODS: Twenty patients will be randomly allocated to one of two treatment sequences. Primary outcome is the severity of epistaxis determined by the Epistaxis Severity Score (ESS). Secondary outcomes are subjective satisfaction, quality of life, as well as the hemoglobin, ferritin, and transferrin levels of the participating patients. Safety outcome is assessed by means of pulse, blood pressure, and adverse events. CONCLUSION: TIM-HHT will evaluate the efficacy and safety of timolol as an additional treatment of epistaxis in HHT patients in a three-month trial period. Benzalkonium chloride is used as a placebo, which has no documented positive effect on the nasal mucosa and hence on epistaxis in HHT patients (in contrast to saline). TRIAL REGISTRATION: German Clinical Trials Register (DRKS), DRKS00020994. Registered on 10 March 2020 [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder. [ABSTRACT FROM AUTHOR]

Background: Transnasal endoscopy has recently become common in Japan. Although transnasal endoscopy has many advantages, nasal pain and epistaxis are common complaints. To reduce nasal pain and epistaxis, we developed a new tube sheath system for transnasal endoscopy. This new tube sheath system (outer sheath and inner tube), called the Nasal Slider, is produced by TOP Corporation, Japan. Methods: A tube sheath longer than the nasal concha is inserted to reduce pain along the nasal turbinate. Because the sheath is left in place, tubes can be passed through the nose multiple times without causing additional pain. A total of 34 consecutive patients (mean age 68.1 years; 22 men and 12 females) who had undergone transnasal endoscopy in the past were selected for transnasal endoscopy with the Nasal Slider. After the transnasal endoscopy was completed, patients who gave consent for use of the Nasal Slider were interviewed by using 3 questionnaires on nasal discomfort, nasal pain, and epistaxis. Results: Because the transnasal endoscope passes inside the sheath, epistaxis can be prevented. Thirty of 34 selected patients underwent transnasal endoscopy using the Nasal Slider. Twenty-seven and 28 patients reported feeling less nasal discomfort and pain, respectively, with the Nasal Slider than during examinations without the Nasal Slider. No epistaxis developed in any patient examined with the Nasal Slider. Conclusions: The Nasal Slider appears to reduce nasal pain and epistaxis during transnasal endoscopy and is currently used in many hospitals in Japan. [ABSTRACT FROM AUTHOR]

Purpose: Hereditary hemorrhagic telangiectasia (HHT) is a vascular disorder that presents with recurrent, intractable epistaxis. The aim of this study was to retrospectively analyze the efficacy of various treatment options for epistaxis in patients with HHT, over a period of 18 years, and to correlate these findings with available evidence in the literature. Methods: Records of patients with HHT, treated for epistaxis between 2000 and 2018 were analyzed. Treatment procedures carried out and their efficacy were extracted and analyzed. Results: Forty-three records were evaluated. All patients were given nasal humidifying ointments, 93% required acute treatment with bipolar electrocautery, and 60% underwent atraumatic nasal packing. Recurrent cases were treated medically with tranexamic acid (26%), oestrogen (19%), and bevacizumab (2%). Laser photocoagulation was done in selected cases (40%) and if unsuccessful, septal dermoplasty was performed (2.3%). Endovascular embolization was reserved for life-threatening emergencies (7%). Conclusion: Epistaxis in HHT is not curable, but can be managed by employing a comprehensive stepwise approach. An algorithm for effective and comprehensive management has been presented. [ABSTRACT FROM AUTHOR]