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Background: Studies evaluating health-related quality of life (HRQOL) in patients with hereditary hemorrhagic telangiectasia (HHT) have expanded rapidly in the past decade. These studies have evaluated QOL aspects ranging from the general QOL for patients living with HHT to intervention-specific outcomes. However, few tools have been fully validated across the spectrum of disease manifestations and interventions in HHT., Objective: In this scoping review, we aim to map the literature on HHT-QOL metrics, identify gaps, inform future QOL research, and facilitate future metric development., Methods: We analyzed articles in English that assessed at least 1 measure of general HRQOL, including physical health, mental health, social health, or intervention-specific QOL in patients with HHT. Searches across 2 bibliographic databases (PubMed and Scopus) yielded 186 articles after duplicates were removed. Sixty-three studies met eligibility criteria: 22 prospective studies (34.9%), 20 retrospective studies (31.7%), 12 cross-sectional studies (17.5%), 6 randomized controlled trials or secondary analyses of a randomized controlled trials (9.5%), 2 qualitative studies (3.2%), and 1 case-control study (1.6%). Two additional studies-1 prospective and 1 cross-sectional study-were identified at the October 2022 14th International HHT Conference and included, making a total of 65 studies., Results: The 65 eligible studies used 30 QOL instruments. Twenty studies characterized baseline HRQOL, and 45 studies evaluated QOL before and after treatment. Of those 45 studies, 37 evaluated HRQOL before and after therapies targeting epistaxis and nasal symptoms, 4 targeted therapies for liver arteriovenous malformations and high-output heart failure, 3 evaluated therapies for both epistaxis and gastrointestinal bleeding, and 1 evaluated treatment targeting gastrointestinal bleeding alone., Conclusions: Comparison of results across studies remains challenging given the heterogeneity in outcomes measures. Further development of HHT-specific patient-reported outcomes instruments that capture the global illness experience of HHT is needed., Competing Interests: Declaration of Conflicting InterestsThe authors would like to declare the following conflict of interest with respect to the research, authorship, and/or publication of this article: Unrelated funding from Cure HHT.

Objectives/hypothesis: Surgical interventions for epistaxis management in hereditary hemorrhagic telangiectasia (HHT) demonstrate short-term success and require repeated procedures for disease control. Although electrocautery and/or laser photocoagulation (C ± L) are most frequently performed, sodium tetradecyl sclerotherapy (STS) is emerging as a promising newer treatment. We hypothesized that in a 24-month time period, STS would require fewer treatments than C ± L to maintain epistaxis severity within the mild range., Study Design: Retrospective study., Methods: We retrospectively assessed 67 patients with HHT with moderate and severe epistaxis that were treated periodically with C ± L (34 patients) versus STS (33 patients). The primary outcome was the number of procedures needed to maintain the epistaxis severity score (ESS) as mild. Secondary outcomes assessed for differences in postoperative complications, hemoglobin levels, iron stores, hematologic support, and quality-of-life (QoL) scores., Results: To maintain ESS in the mild range, 1.6 STS procedures (range, 1-4) were performed versus 3.6 C ± L procedures (range, 1-8) (P = .003). Significant postoperative differences included reduction in nasal crusting (3% vs. 32%, P = .001), foul odor (3% vs. 35%, P < .001), and septal perforation (3% vs. 29%, P = .006) after STS. There were no significant differences between the two treatments in hemoglobin levels, iron stores, hematologic support, or QoL scores., Conclusion: STS is able to attain satisfactory epistaxis control with significantly fewer procedures and lower postoperative complications than C ± L. STS should be considered as the initial surgical intervention for epistaxis in patients with HHT., Level of Evidence: 4 Laryngoscope, 132:920-925, 2022., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc.)

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., (© 2021. The Author(s).)

Background and Objectives: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, causes recurrent mucous membrane hemorrhage, especially epistaxis. In this systematic review, we discuss the efficacies of the three most common laser photocoagulation treatments for HHT-related epistaxis., Study Design/materials and Methods: A systematic literature search was conducted in PubMed and MEDLINE from database inception to March 2019. Studies reporting epistaxis outcomes following argon, neodymium-doped yttrium aluminum garnet (Nd:YAG), and diode laser photocoagulation for HHT were included. χ 2 and Barnard's exact tests were utilized to detect differences in reduced epistaxis frequency and intensity rates., Results: Fifteen out of 157 published studies met our eligibility criteria, spanning a collective 362 patients. Argon, Nd:YAG, and diode laser therapy reduced epistaxis frequency in 90.4%, 88.9%, and 71.1% of patients, respectively, and reduced epistaxis intensity in 87.8%, 87.2%, and 71.1% of patients, respectively. Diode laser photocoagulation significantly underperformed in both outcome measurements when compared with argon (frequency: P = 0.005; intensity: P = 0.034) and Nd:YAG (frequency: P = 0.012; intensity: P = 0.041). There was no significant difference between argon and Nd:YAG in reducing HHT epistaxis frequency (P = 0.434) or intensity (P = 0.969). Categorizing HHT patients by clinical severity demonstrated a higher rate of improvement in the mild-moderate group compared with the severe group in both argon (P < 0.001) and Nd:YAG (P < 0.001) therapeutic methods. While no significant differences were found in rates of improved epistaxis outcomes between argon and Nd:YAG in mild-moderate HHT patients (frequency: P = 0.061; intensity: P = 0.061), Nd:YAG demonstrated greater rates of reduction in epistaxis frequency (P = 0.040) and intensity (P = 0.028) than argon among severe HHT patients., Conclusions: HHT is a lifelong disease, plaguing patients with debilitating epistaxis. Intranasal laser photocoagulation of telangiectasias using argon or Nd:YAG laser therapy can yield improved epistaxis outcomes compared with diode laser photocoagulation. In severe cases of HHT, Nd:YAG laser therapy provides greater improvements in epistaxis outcomes than argon photocoagulation. Lasers Surg. Med. © 2019 Wiley Periodicals, Inc., (© 2019 Wiley Periodicals, Inc.)

Objectives: Epistaxis is the most frequent clinical manifestation of hereditary hemorrhagic telangiectasia (HHT). Several topical, systemic, and surgical treatments have been tried, but none have been completely effective. The aim of the present study is to evaluate whether a combined treatment sclerotherapy and topical therapy with propranolol 0.5% nasal formulation would reduce the epistaxis due to HHT and improve patient's quality of life., Methods: An observational cross-sectional study was carried out. The primary outcome measure was frequency and severity of epistaxis as measured by the epistaxis severity score (ESS) at baseline (4 weeks before therapy) and at least 4 weeks after the treatment was implemented. Quality of life was analyzed using EuroQol-5D (EQ-5D) scale and visual analogue (VAS) scale before and after treatment., Results: A total of 38 consecutive patients subjected to the combined treatment were evaluated (mean age: 57.2 years, standard deviation [SD] = 13.9; 60.5% women). The mean time of treatment was 37.1 weeks (SD = 14.9). Combined therapy significantly reduces frequency and severity of epistaxis, with an ESS improvement of 5 points from 6.9 ± 2.6 to 1.9 ± 1.3 (P < 0.05); however, the EQ-5D scale increased from 0.66 ± 0.27 to 0.93 ± 0.12 (P < 0.05). The difference in VAS means showed an increase from 44.6 ± 28.3 to 82.5 ± 12.5 (P < 0.05). The increases in quality of life are in line with the drop in ESS., Conclusion: The study demonstrated that combined therapy (sclerotherapy and topical nasal propranolol) significantly reduced the epistaxis due to HHT and increased patients' quality of life., Level of Evidence: 2c Laryngoscope, 129:2216-2223, 2019., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Hereditary hemorrhagic telangiectasia is a rare vascular genetic disease. Epistaxis is the most frequent and disabling manifestation, and timolol appears to be a new therapeutic option as non-selective beta-blockers have in vitro and in vivo anti-angiogenic properties. Our main objective was to evaluate the efficacy of TIMOLOL nasal spray as a treatment for epistaxis in hereditary hemorrhagic telangiectasia. This study is a single-center, randomized, phase 2, double-blind placebo-controlled study with an allocation ratio of 1:1. It was proposed to patients with hereditary hemorrhagic telangiectasia monitored at the French Reference Center, and we included patients aged over 18 years, diagnosed with hereditary hemorrhagic telangiectasia and epistaxis. The treatment was self-administered by the patient with a posology of one spray (50 µL) of timolol 0.5% or placebo in each nostril twice a day for 28 consecutive days. The primary efficacy endpoint was mean monthly epistaxis duration, assessed by monitoring epistaxis grids. A total of 58 patients were randomized and treated. The baseline characteristics were similar in the 2 groups. Mean monthly epistaxis duration measured at 3 months was not significantly different in the 26 patients receiving the drug in comparison with the placebo group (p = 0.54). Toxicity was low and no severe adverse events were reported. One limitation is that we included all HHT patients with nosebleeds and did not take into account history of nasal surgery or nasal crusts. Timolol, administered by nasal spray at a dose of 0.25 mg in each nostril twice a day for 28 consecutive days, did not improve epistaxis in patients with hereditary hemorrhagic telangiectasia at 4 months after the beginning of the treatment.

Objective: The aim of this study is to investigate the gene diagnosis and the treatment of hereditary hemorrhagic telangiectasia (HHT) with epistaxis as the main symptom, and to analyze the mutation of ACVRL1 gene in the family. Method: Detailed pedigree investigation was carried out on the proband.Sixtynine genes related to coagulation disorder were sequenced and analyzed by high throughput sequencing for the father and son of the proband. Result: Four generations of the family consisted of 30 probands, including 11 patients. A pathogenic mutation ACVRL1&#95;ex9 c.1313T > C (p.M438T) was detected in both patient and his father. Conclusion: Recurrent epistaxis is the main manifestation of hereditary hemorrhagic telangiectasia in this family. The mutation of ACVRL1 gene is the pathogenic gene of this family, which can be treated by surgery and medicine., 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.)

Objective Epistaxis is a primary complaint in 90% to 96% of patients with hereditary hemorrhagic telangiectasia (HHT). Numerous surgical and medical treatments aim to decrease the frequency and severity of epistaxis in this patient population. Bevacizumab is a recombinant, humanized monoclonal antibody to vascular endothelial growth factor, an angiogenic factor elevated in HHT. It has been used in several forms to treat epistaxis in HHT but thus far, evidence-based recommendations are limited. Study Design Systematic review with evidence-based recommendations. Methods A systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed using Embase, MEDLINE, MEDLINE In-Process/Epub, and Cochrane databases. English language abstracts were reviewed for relevance. Results Eleven manuscripts met inclusion criteria and were analyzed. Submucosal injection, submucosal injection plus laser coagulation, intravenous (IV), and topical formulations of bevacizumab were evaluated for their therapeutic impact on epistaxis in patients with HHT. Three randomized controlled trials failed to show topical bevacizumab to be more effective in controlling epistaxis than saline or other moisturizers. Conclusions The use of submucosal and IV bevacizumab shows promise, but further study is necessary to determine the true efficacy in the treatment of epistaxis as only grade C level exists currently. Based on the available literature, the use of topical bevacizumab is not recommended (grade B).

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

The objective of the study was to evaluate the influence of temporary nasal occlusion (tNO) with hypoallergenic tape on the frequency and severity of epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). This prospective observational study included 20 HHT patients who were undergoing Nd:YAG laser therapy at regular intervals. Over a 3-month period, laser therapy was supplemented by tNO with hypoallergenic tape for 5 h/day on average. On a 0-10 numeric rating scale, the patients reported significantly greater satisfaction in epistaxis terms after tNO treatment, with mean scores of 5 before and 7 after 3-month tNO (p = 0.05). The Epistaxis Severity Score also fell significantly from a median of 3.59-2.43 after 3-month tNO compared with laser therapy alone (p = 0.01). The patients' hemoglobin levels remained stable during the study (median: 12.2 g/dL before tNO; median: 11.7 g/dL after tNO; p = 0.387). Overall, the present study confirms the positive influence of tNO on epistaxis in HHT patients and on subjective satisfaction. This simple and inexpensive strategy is therefore a helpful option, especially in addition to regular Nd:YAG laser therapy, and is recommended by the authors.

Objectives/hypothesis: To examine whether there is a rationale for iron treatments precipitating nosebleeds (epistaxis) in a subgroup of patients with hereditary hemorrhagic telangiectasia (HHT)., Study Design: Survey evaluation of HHT patients, and a randomized control trial in healthy volunteers., Methods: Nosebleed severity in response to iron treatments and standard investigations were evaluated by unbiased surveys in patients with HHT. Serial blood samples from a randomized controlled trial of 18 healthy volunteers were used to examine responses to a single iron tablet (ferrous sulfate, 200 mg)., Results: Iron tablet users were more likely to have daily nosebleeds than non-iron-users as adults, but there was no difference in the proportions reporting childhood or trauma-induced nosebleeds. Although iron and blood transfusions were commonly reported to improve nosebleeds, 35 of 732 (4.8%) iron tablet users, in addition to 17 of 261 (6.5%) iron infusion users, reported that their nosebleeds were exacerbated by the respective treatments. These rates were significantly higher than those reported for control investigations. Serum iron rose sharply in four of the volunteers ingesting ferrous sulfate (by 19.3-33.1 μmol/L in 2 hours), but not in 12 dietary controls (2-hour iron increment ranged from -2.2 to +5.0 μmol/L). High iron absorbers demonstrated greater increments in serum ferritin at 48 hours, but transient rises in circulating endothelial cells, an accepted marker of endothelial damage., Conclusions: Iron supplementation is essential to treat or prevent iron deficiency, particularly in patients with pathological hemorrhagic iron losses. However, in a small subgroup of individuals, rapid changes in serum iron may provoke endothelial changes and hemorrhage., Level of Evidence: 4. Laryngoscope, 126:2468-2474, 2016., (© 2016 The Authors. The Laryngoscope published by Wiley Periodicals, Inc. on behalf of American Laryngological, Rhinological and Otological Society Inc, “The Triological Society” and American Laryngological Association (ALA).)

Unlabelled: PROBLEM/OBJECTIVES: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT); it is present in >90% of HHT patients > 45 years old. Depending on severity, treatment consists of managing bleeding via medical and surgical methods. The aim of our study was to evaluate the efficacy and safety of endonasal surgical arterial cauterization to treat chronic epistaxis in patients with HHT who are no longer responding to first-line therapies., Methodology: Five patients were included in our study. The day before surgery, all patients underwent devascularization embolization of the external carotid arterial branches involved in nasal bleeding. The primary efficacy endpoint of treatment was assessed by the Epistaxis Severity Score (ESS), which was systematically evaluated preoperatively in our department, as well as every 3 months postoperatively. Data were collected retrospectively from the medical records of patients., Results: Endonasal surgical arterial cauterization was associated with a ≥ 50% reduction in the ESS up to 9 months postoperatively. In one of our patients, cerebrospinal fluid (CSF) leakage occurred intraoperatively during cauterization of the posterior ethmoid artery. Closure of the dural skull base defect was successfully performed intraoperatively., Discussion: Although our results are preliminary and included few patients, they support a role for endonasal surgical arterial cauterization as a second-line treatment method for chronic epistaxis in patients with HHT.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder in which epistaxis is the most frequent manifestation, responsible for high morbidity. Management of this symptom has no standard, and local treatments are often aggressive. Their efficacy is variable and has not been proven. Anti-angiogenic drugs, such as bevacizumab, are a new treatment strategy. Its systemic administration in patients with HHT improves liver damage-related symptoms and epistaxis. To limit the systemic adverse effects of bevacizumab and to ease administration, a local administration seems suitable., Primary Objective: To evaluate the tolerance of increasing doses of bevacizumab administered as a nasal spray in patients with HHT-related epistaxis. Secondary objectives were to study the bioavailability and efficacy of bevacizumab against epistaxis when given as a nasal spray., Methodology: Phase 1, randomized, double-blind, placebo-controlled, monocentric study performed sequentially (dose escalation) on 5 groups of 8 patients. Each group was made up of 6 verum and 2 placebos. Five increasing doses of bevacizumab nasal spray (25 mg/mL) were evaluated: 12.5, 25, 50, 75 and 100 mg., Results: A total of 40 patients were included between October 2011 and October 2012. Bevacizumab nasal spray was well tolerated in all patients and the drug was not detected in their serum. No dose limiting toxicity was observed. No efficacy was observed at any dose in this study., Conclusion: Based on these results, bevacizumab nasal spray is a safe treatment of epistaxis in HHT. However, a randomized Phase 2 study is needed to determine its efficacy., Trial Registration: ClinicalTrials.gov Identifier #NCT01507480.

Background: The aim of this study was to identify and evaluate adverse clinical outcomes following office-based sclerotherapy using sodium tetradecyl sulfate (STS) for epistaxis due to hereditary hemorrhagic telangiectasias (HHT or Osler-Weber-Rendu)., Methods: A retrospective chart review of 36 adult patients treated with STS sclerotherapy for severe and/or recurrent epistaxis due to HHT was performed., Results: A total of 153 separate treatment sessions were analyzed. Each patient underwent an average of 4.3 sessions with an average of 7 intralesional injections per session. Bleeding during the procedure was experienced by 8 patients with a maximum reported blood loss of 200 mL in 1 patient, but less than 50 mL in all others. Seven patients reported some postinjection pain, which included nasal, cheek, and eye pain. Nasal congestion, sneezing, and vasovagal responses were each noted to occur 2 times. No complications of postprocedural visual loss, deep venous thrombosis/pulmonary embolus, transient ischemic attack (TIA)/stroke, or anaphylaxis were encountered., Conclusion: Conventional therapies used in the management of HHT-related epistaxis, such as laser coagulation, septodermoplasty, selective arterial embolization, and Young's occlusion each have specific associated complications, including worsened epistaxis, septal perforation, foul odor, nasal crusting, and compromised nasal breathing. STS is a safe office-based treatment option for HHT-mediated epistaxis that is associated with exceedingly few of the aforementioned serious sequelae., (© 2014 ARS-AAOA, LLC.)

Objective: To assess the safety and efficacy of submucosal radiofrequency (RF) treatment for hereditary hemorrhagic telangiectasia (HHT) with mild or moderate epistaxis., Methodology: We carried out a prospective pilot study of 16 consecutive patients with HHT-related epistaxis from June 2010 to April 2012. Under local anesthesia, RF was applied to one or both sides of the nose from the columella beneath the septal mucosal (50 joules per puncture). Patients were sent a questionnaire at least six months after the procedure., Results: RF under local anesthesia was well tolerated, according to visual analog scale scores. Neither crusting nor pain was reported one week after the intervention. The frequency of epistaxis per day and per month was significantly lower after RF. The duration of bleeding also decreased from more than 10 minutes to less than 5 minutes in two thirds of patients. Thirteen of the 16 patients were satisfied with the technique and would request it for subsequent procedures to treat repeated bleeding., Conclusion: Submucosal RF treatment for HHT is a safe, well tolerated procedure with significant efficacy in the short term. It should be considered as an alternative technique for managing HHT-related epistaxis, although long-term results remain to be evaluated.

Objectives/hypothesis: Topical Sesame/Rose geranium oil compound is an effective therapy for hereditary hemorrhagic telangiectasia (HHT) associated epistaxis., Study Design: Cohort Study., Methods: Twenty patients with HHT confirmed by the Curaçao criteria were treated with a sesame/rose geranium oil topical compound between January 2010 and June 2011. A treatment evaluation survey was conducted at least 3 months after treatment initiation. Changes in epistaxis severity scores (ESS), patient satisfaction, and any adverse effects were assessed., Results: A total of 20 patients completed the study. The average (SD) age was 54.4 (14.6), and 14 (70%) were female. The median time on rose geranium oil was 183 days (IQR: 114-311). At the conclusion of the study, 18 (90%) were still using rose geranium oil. The majority (75%) of patients subjectively felt improvement with the treatment. The improvement was felt to be gradual in 25% and immediate in 50% of patients. Mean (SD) overall satisfaction using a 10-point Likert scale was 7.8 (3.1), with 50% of the patients reporting a satisfaction rating of 10. Mean (SD) epistaxis severity score (ESS) prior to treatment was 5.3 (1.7). After treatment with sesame/rose geranium oil, mean (SD) ESS was found to be 3.5 (1.8). Treatment with sesame/rose geranium oil was associated with a statistically significant improvement in ESS by 1.81 (P <0.0001). There were no adverse side-effects from the treatment., Conclusion: A sesame/rose geranium oil compound can significantly reduce the epistaxis severity scores of patients with hereditary hemorrhagic telangiectasia-related epistaxis., (Copyright © 2013 The American Laryngological, Rhinological, and Otological Society, Inc.)

Background: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90% of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities., Methods: A systematic review of the literature for HHT-related epistaxis was performed using the search terms "hereditary hemorrhagic telangiectasia" and "epistaxis." Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed., Results: A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years., Conclusion: HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient., (Copyright © 2012 American Rhinologic Society-American Academy of Otolaryngic Allergy, LLC.)

Hereditary hemorrhagic telangiectasia (HHT), known as well as Osler-Weber-Rendu syndrome (ORW disease) is autosomal dominant inheritance disease with the worldwide prevalence of 1 case per 5000-10000 population. The pathophysiology of the disease consists of disorders in the growth and migration of endothelial cells, which leads to telangiectasias and arterio-venous malformations (AVM) development. Vascular abnormalities can form in various organs. The most frequent locations are nose and mouth mucous membranes, as well as the rest of GI tract, skin, lungs, urinary system and central nervous system. The most common symptom is reccurent epistaxis (80-90% of patients). Advanced stage disease can result in extensive bleeding with dicrease in hemoglobin levels. Unfortunately, the only available treatment options for Osler-Weber-Rendu syndrom fight the symptoms, not the essential cause, and because of the rarity of the disease there are no guidelines for effective therapy. We are presenting a case of a patient suffering from recurrent episodes of nose bleeding due to hereditary hemorrhagic telangiectasia, who was successfully treated using low-invasive, intravascular arterial embolisation in interventional radiology department.

Objective: Epistaxis is a common clinical problem, and the majority of bleedings can be managed conservatively. However, due to extensive and sometimes life-threatening bleeding, further treatment, such as superselective embolization, may be required. We report our experience with endovascular treatment of life-threatening epistaxis., Methods: All patients presenting with excessive epistaxis, which received endovascular treatment at a German tertiary care facility between January 2001 and December 2009, were retrospectively identified. Demographic data, etiology, origin and clinical relevance of bleeding, interventional approach, therapy-associated complications, and outcome were assessed., Results: A total of 48 patients required 53 embolizations. Depending on the etiology of bleeding, patients were assigned to three groups: 1) idiopathic epistaxis (31/48), 2) traumatic or iatrogenic epistaxis (12/48), and 3) hereditary hemorrhagic telangiectasia (HHT) (5/48). Eleven of 48 patients required blood transfusions, and 9 of these 11 patients (82%) were termed clinically unstable. The sphenopalatine artery was embolized unilaterally in 10 of 53 (18.9%) and bilaterally in 41 of 53 (77.4%) procedures. During the same procedure, additional vessels were embolized in three patients (3/53; 5.7%). In 2 of 53(3.8%) cases, the internal carotid artery (ICA) was occluded. Long-term success rates of embolization were 29 of 31 (93.5%) for group 1 and 11 of 12 (91.7%) for group 2 patients. Embolization of patients with HHT offered at least a temporary relief in three of five (60%) cases. Two major complications (necrosis of nasal tip and transient hemiparesis) occurred after embolization., Conclusions: Endovascular treatment proves to be effective for prolonged and life-threatening epistaxis. It is easily repeatable if the first procedure is not successful and offers a good risk-benefit profile.

Introduction: Osler-Rendu-Weber syndrome is an inherited disorder characterized by mucocutaneous and visceral telangiectasia. It is often revealed by recurrent epistaxis. It is sometimes profuse and intractable. Hemostasis may be problematic., Case Study: A 61-year-old male patient presented with severe epistaxis due to Osler-Rendu-Weber syndrome. Embolization of the external carotid branches was needed for hemostasis., Discussion: Embolization of external carotid artery branches can be an effective therapeutic alternative when usual treatments fail., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare systemic fibrovascular dysplasia also known as Rendu-Osler-Weber disease. Epistaxis is often the first and foremost symptom of the disease and recurrent epistaxis is the main complaint., Purpose: Evaluation of effectiveness and safety of endovascular treatment of epistaxis in HHT patients., Material and Methods: Between June 2004 and January 2008, 14 patients with HHT underwent embolization of external carotid artery (ECA) branches due to severe epistaxis. There were eight men and six women aged 27-42 years. Patients were referred for endovascular treatment when primary management with electrocautery and nasal packing was ineffective. Arteries suspected of bleeding were catheterized super-selectively with micro-catheters to perform embolization. Polyvinyl alcohol particles (PVA) or embospheres in diameter of 500-700-µm were used as an embolic material., Results: Embolization was technically successful in 11 (78.5%) patients. Immediate, complete control of bleeding was achieved in 12 (85%) patients. During long-term follow-up recurrent nasal bleeding occurred in six (43%) patients 6-24 months after treatment. There were no severe complications after embolization. Nine patients experienced mild facial pain which resolved within three days after the treatment. Three patients in whom arterial spasm was observed, suffered from facial paraesthesia lasting for 3-5 days after embolization. Out of 14 patients, 12 were available for 24-month follow-up evaluation. All except one claimed reduction in frequency and severity of epistaxis which had a positive impact on their quality of life., Conclusion: Endovascular embolization of epistaxis in patients with HHT is an effective and safe procedure although it cannot provide a long-term cure in most cases. Embolization reduces the frequency, severity and duration of nasal bleeding in patients with HHT, improving patients' quality of life.

Background: Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT). In severely affected cases, cessation of the nasal airflow seems to be the only long-term effective treatment. Such procedure deeply affects patient's quality of life (QoL) and is sometimes refused., Methods: This study investigated the effectiveness of a tailored silicone nasal swab on 2 patients affected by HHT and severe epistaxis., Results: In both cases, we observed a good clinical effect, with significant reduction of epistaxis and improved QoL., Conclusions: Our preliminary results indicate that the silicone nasal swab can be considered an effective way of treating severe epistaxis in patients with HHT. Its advantages, in terms of maintained airflow patency, reversibility, and improved QoL, are highlighted., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: Most patients with hereditary haemorrhagic telangiectasia suffer with frequent episodes of epistaxis. The aim of this case report is to highlight the effect on epistaxis, occurring in hereditary haemorrhagic telangiectasia, when nasal airflow ceases., Case Report: We present the interesting case of a patient with hereditary haemorrhagic telangiectasia who experienced cessation of her recurrent, refractory epistaxis through the development of coexisting polyp disease. The patient's enlarged, grade three nasal polyps were behaving as physiological obturators, limiting airflow through her nose. This reduced the intranasal trauma and subsequent frequency of her nosebleeds., Conclusion: Epistaxis is a debilitating part of hereditary haemorrhagic telangiectasia, and poses a frequent management challenge. Our patient was more tolerant of her grade three nasal polyps than of her recurrent epistaxis. This case highlights the importance of reducing nasal airflow when treating patients with hereditary haemorrhagic telangiectasia.

Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease is a rare fibrovascular dysplasia that makes vascular walls vulnerable to trauma and rupture, causing skin and mucosa bleeding. It is of dominant autosomal inheritance, characterized by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations and positive family history. Epistaxis is often the first and foremost manifestation. It's associated to arteriovenous malformations in several organs. There are possible hematologic, neurologic, pulmonary, dermatologic and gastrointestinal complications. Treatment is supportive and helps prevent complications. This study is a case report of a patient with this syndrome who came to the ENT Outpatient Ward of the Faculdade de Medicina de Marília; and we have done a bibliographic review of the disease's etiopathogenesis, clinical manifestations and clinical-surgical treatment options.

Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disorder characterized by widespread mucosal, dermal, and visceral telangiectasias, which can appear as red spots on the lips, tongue, fingers, nose, or intestines. Recurrent epistaxis is the universal presentation, but almost any organ can be affected. Gastrointestinal (usually stomach and small bowel), oral, and urogenital sites are the most common areas affected, whereas pulmonary arteriovenous fistulae can be the most catastrophic. This disorder can present at age 10, and if present, appears in most people by age 21. Gastrointestinal manifestations can cause minor-to-life-threatening bleeding and can lead to the need for liver transplantation. Therapy for HHT includes laser and Argon treatments, embolization, hormones, and surgery. Family members should be screened to provide early recognition and treatment. Gastroenterology nurses should be aware of this rare but potentially fatal disorder as it can be diagnosed or ruled out in the endoscopy suite.

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by abnormal angiogenesis with resultant telangiectasia formation in mucocutaneous tissues, visceral organs, and the central nervous system. The most common manifestation of HHT is epistaxis resulting from trauma to thin-walled telangiectasias. Many patients with HHT experience worsened epistaxis due to the presence of a septal perforation. Septal perforation in HHT patients results from aggressive noncartilage sparing treatments such as monopolar cauterization. Although the mainstay of treatment for patients with severe transfusion-dependent HHT remains to be septal dermoplasty (SD), patients with a septal perforation are less likely to have a successful outcome. In this small subset of patients, septectomy (ST) combined with SD is proposed to eliminate this variable to improve skin graft uptake and therefore outcome. This study reviews the indications, procedure, and outcome of nine patients with severe transfusion-dependent HHT and septal perforation who underwent the combined procedure of SD/ST., Methods: Nine HHT patients with severe transfusion-dependent epistaxis and septal perforation underwent SD/ST at our institution over a 5-year period. Quality of life, including number of daily events of epistaxis, and transfusion requirements were determined before and after surgery. Technical aspects of the procedure as well as complications were reviewed., Results: The combined procedure of SD/ST resulted in a long-lasting subjective improvement in quality of life for all patients. Similarly, transfusion requirements were reduced from 22.61 to 9.57 (p < 0.05). There were no complications or increased morbidity from the procedure., Conclusion: Combined SD/ST is a safe and effective treatment for HHT patients with transfusion-dependent epistaxis and septal perforation.

The authors have described case of patient suffering from Rendu-Osler-Weber Syndrome for many years, after many unsuccessful attempts to treat massive hemorrhages from the nose. This was what made them use maxillary artery embolisation method. It was done using Seldinger technique. Radiologic visualisation of blood vessels was obtained by use of C-arm. The use of this method in that case has been successful so far. According to authors opinion embolisation of maxillary arteries is an effective alternative in treating recurring hemorrhages in patients with Rendu-Osler-Weber Syndrome.

Conclusions: In skilled hands, selective embolization is a safe procedure and represents an effective treatment for prolonged epistaxis. Embolization therapy can be repeated if necessary., Objective: Severe posterior epistaxis is a common clinical problem in an ENT department and controlling the bleeding may present difficulties. Several methods are used to control posterior epistaxis, one of the latest treatment strategies being selective embolization of the nasal arteries. The aim of this study was to describe the effect of selective embolization in 22 patients treated with a total of 30 procedures at the ENT Department of Odense University Hospital between January 1995 and March 2004. To our knowledge this is the first Nordic work in which selective embolization has been used as a treatment strategy for patients with hereditary hemorrhagic telangiectasia (HHT)., Material and Methods: This was a retrospective review. Post-treatment effects and complications were evaluated by means of a questionnaire and a telephone interview. Owing to the different treatment strategies used, the results were evaluated for 2 groups of patients: Group A, 9 patients with HHT; and Group B, 13 patients with causes of epistaxis other than HHT., Results: In Group A, 15 procedures were performed, 12 of which were beneficial as the duration and number of episodes of epistaxis were reduced. In Group B, 15 procedures were performed and the success rate was 87%. One patient suffered from skin necrosis at the tip of the nose. No other serious side-effects of the treatment were observed.

Unlabelled: Hereditary Hemorrhagic Telangiectasia is a systemic autosomal dominant disorder involving blood vessels. The most common symptom is recurrent epistaxis. The treatments of these epistaxis are numerous but such treatments are often symptomatic and their effects are often not sustained. Some of these treatments may be complicated by visceral vascular malformations. The aim of this study is to propose a treatment plan for these patients with hierarchical organisation of therapeutic options taking into account of their previous therapy., Method: H. Plauchu organized in Paris, december 2002 a meeting with any medical specialists of this disease. They have analysed variety of therapies that have been proposed for epistaxis control in Hereditary Haemorrhagic Télangiectasia., Results: Most common use packing of nasal fossa and then hyperselective embolization of the internal maxillary and facial arteries for severe epistaxis. For chronic epistaxis, best treatment use sclerotics products (Ethibloc) and laser. After discussion, primary embolization could be useful to reduce vascularization of nasal fossa., Conclusion: Treatment of epistaxis in Hereditary Haemorrhagic Telangiectasia could increase in few years. Use of an index card of for epistaxis in the disease of Rendu-Osler could help to find treatment of choice.

Rendu-Osler-Weber syndrome is a rare genetically determined disorder that affects blood vessels throughout the body and results in a tendency for bleeding. Authors describe the case of superselective embolization of the left maxillary artery with polyvinyl alcohol particles in a patient with the Rendu-Osler-Weber syndrome hospitalized and treated in the Department of Otolaryngology and the Department of Radiology of the Military Institute in Warszawa, Poland due to persistent, severe and difficult to manage nasal bleeding. After the procedure had been performed patient condition improved and frequency and severity of nasal bleeding significantly diminished. Authors conclude that superselective embolization of the maxillary artery in a patient with Rendu-Osler-Weber syndrome is safe and effective and can be a valuable alternative to the maxillary artery or the carotis externa artery ligation. Authors also describe other methods of nasal bleeding management: laser photocoagulation, argon plasma coagulation, nasal dermoplasty and pharmacological treatment. Authors indicate that treating patients with Rendu-Osler-Weber syndrome is a diagnostic and therapeutic challenge for a physician and surgeon that require special approach to a patient due to difficult to manage symptoms. Patients with Rendu-Osler-Weber syndrome should be treated in a hospital setting due to access to diagnostic imaging techniques that can be helpful in revealing possible life threatening conditions.

The purpose of the present study was to evaluate the haemostatic efficacy of fibrin sealant in patients with hereditary haemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease suffering epistaxis. A retrospective observational study of patients with HHT who were admitted to an emergency room for anterior or posterior epistaxis during May 2000-March 2003. A total of 24 patients were evaluated, of whom 15 were managed with foam nasal packing during May 2000-March 2002 and another nine were treated during March 2002-March 2003 with 0.3 ml fibrin sealant spray (Quixil; Omrix, Belgium). The immediate and the distant results were compared. Immediate haemostasis was achieved in all seven patients treated with fibrin glue, with good healing of bleeding sites, no secondary bleeding, no inflammation, and no plaque or crists. Twelve months of follow-up monitoring (until October 2003) of atrophic changes of nasal mucosa, bleeding frequency and intensity proved absence of atrophy of nasal mucosa and decreased bleeding frequency. In this group, the bleeding episode duration averaged 2 min 35 s since the moment of admittance. In the nasal packing group, we found local swelling, pain, and slow healing of the bleeding site with accidental atrophy of nasal mucosa and no effect on further bleeding frequency and intensity. Removal of nasal packing frequently initiates secondary bleeding. The rates of these side effects were higher in comparison with the fibrin glue group. The bleeding episode duration was also longer. In patients with HHT suffering profuse epistaxis, fibrin glue is more effective and convenient for the patients as compared with foam nasal packing. It is also safer, since it lacks the complications that usually accompany packing as swelling, atrophy of the nasal mucosa, and secondary bleeding provoked by the removal of the pack.

Background and Objective: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant fibrovascular dysplasia with the main symptom of recurrent epistaxis. At present, only limited data are available on long-term results in the treatment of epistaxis. A part from the surgical treatment of the bleeding telangiectasia with argon plasma, an additional postoperative long-term treatment with estriol nose-ointment was performed and the results were analysed., Patients and Methods: 69 patients with HHT were treated with APC and estriol nose ointment. 43 patients could be examined over a follow-up period of more than 20 months. The bleeding frequency and intensity as well as the patient's satisfaction with the treatment result were evaluated and the serum estriol level determined., Results: After more than 20 months, the bleeding frequency and intensity were reduced in 95% of the patients compared to their preoperative findings. Under the influence of estriol, former berry-like telangiectasias became flatter and more even. Neither systemic side effects under topically applied estriol nor a relevant increase of serum estriol levels were observed., Conclusion: The combined treatment regimen with APC and topically applied estriol provides a new treatment concept of epistaxis in HHT.

Patients with Rendu-Osler-Weber disease can present severe nose bleeding. The treatment of 6 patients with this condition is reported. Supraselective embolization was the treatment of choice. One patient showed no improvement with embolization and suffered massive nosebleed requiring blood transfusion. After a review of the literature, the nasal cavity was irradiated.s.

Objectives: Patients with (HHT) often suffer from recurrent epistaxis, which poses considerable therapeutic problems. Theoretical considerations render argon plasma coagulation (APC) a promising new therapeutic approach., Study Design: In a prospective study 12 patients (aged 8-68 y) who presented with a long history of treatment for epistaxis were treated with telangiectasia in the nasal mucosa. After 2 weeks and again after 4 months the treatment results were evaluated by questionnaire., Methods: APC is based on high-frequency electric energy transmitted through ionized argon gas to the tissue in a noncontact mode. Coagulation and desiccation of tissue are limited to 1 to 2 mm of penetration and therefore risk of tissue damage is low. Coagulative effects are best in tissue with high electrical conductivity, especially blood vessels., Results: All patients were satisfied with the postoperative results. Frequency and intensity of bleeding were significantly reduced. All patients reported better postoperative results than with any other treatment they had received previously., Conclusion: First clinical experience shows that APC is a useful alternative for the treatment of telangiectasia in the nasal mucosa and should be a therapeutic option for this disease.

Unlabelled: The records of 24 patients with severe intractable non-traumatic epistaxis were reviewed to evaluate the efficacy of embolism therapy., Material and Method: The histories of 17 patients with idiopathic epistaxis, 6 patients with Osler-Rendu-Weber disease and 1 patient with a high-flow angioma were analyzed. All patients were treated by means of percutaneous embolization of the maxillary artery and its branches., Results: In 5/17 patients with idiopathic epistaxis repeat embolization was necessary in 5 cases, while permanent hemostasis was achieved in 16/17 patients with embolism alone. Five of the 6 patients with Osler-Rendu-Weber disease labelled stage III before treatment were managed successfully with repeated embolizations, while all cases were reduced to stage I disease. The high-flow angioma was successfully ablated after embolization combined with direct injection of thrombosing material and laser resection.

Background and Aims: The paper reports the use of Nd-Yag laser treatment for some major otolaryngoiatric symptoms in hereditary hemorrhagic teleangectasia (Rendu-Osler-Weber disease), focusing on the importance of epistaxis and oral hemorrhage as a cause of continuous hemoreintegrative treatment, without forgetting the constant psychophysical stress undergone by these patients., Materials and Methods: Photocoagulation of teleangectasia in the ENT district has been used by the authors since January 1994. A total of 8 patients, 5 males and 3 females, have been treated aged between 31 and 65 years old. During the first year specialist outpatient controls were performed every two months, after which the follow-up became four-monthly., Results: The authors provide a detailed illustration of the methods used, the choice of power, and the advantages and results obtained, emphasising the outpatient nature of treatment, excellent patient tolerability and the low cost to the health service., Conclusions: In the light of these results, the Authors underline the unquestionable usefulness of Nd-Yag laser in the treatment of a few of the undoubtedly more stressing symptoms of Rendu-Osler-Weber disease.

Background: As causal therapy of Rendu-Osler-Weber syndrome is not yet possible, there are many different therapeutical approaches. It seemed expedient to investigate the efficacy of super-selective angiography in treating Rendu-Osler-Weber syndrome after this technique proved effective in other therapeutic settings. This method is known to be efficient in controlling tumor bleeding., Patients: Between March 1993 and September 1995, eight patients with an average age of 57 years were treated by super-selective intraarterial embolization., Results: In all cases epistaxis occurred on both sides. Unilateral embolization was performed in ten cases and bilateral embolization in five cases. In two of three patients with nasal packing the embolization permitted removal of the packing. Nevertheless nosebleeds reoccurred on an average of 3.5 days after therapy. Two of eight patients showed long-term improvement after therapy. They suffered fewer nosebleeds than before. Complications included one patient who suffered from partial hemiparesis after embolization, which lasted 1.5 hours and was completely reversible. Another patient died because of pulmonic bleeding caused by manifestation of the disease in this organ. Often patients complained of facial pain or headache which occasionally lasted for a few weeks., Conclusions: Endovasal embolization obviously is an alternative to artery ligation in emergency cases whereas the long-term success is currently impaired by formation of new anastomoses. Further development of this technique is necessary.

A case of hereditary hemorrhagic teleangiectasia (HHT, Morbus Rendu-Osler-Weber) in a 63-years-old patient presenting with recurrent gastrointestinal and nasopharyngeal bleeding who required transfusion of 100-200 units of red blood cell concentrate a year is reported. Endoscopically, one source of bleeding could be located in the upper part of the jejunum. Multiple Osler spots were found in the antrum, the bulbal and descending part of the duodenum without signs of bleeding. Colonoscopy revealed no further lesions. Selective mesentericography visualized dilatation of the arterial arcardes in the duodenal region with early venous filling. During coeliacography, parenchymal deposits of contrast medium appeared in the liver. Numerous angiodysplastic arterial alterations from the lower ethmoidal arteria were shown to be another source of bleeding. They were embolized using microspheres and thereby terminating epistaxis. Termination of epistaxis was the crucial prerogative for the consecutive aortal valve replacement. At abdominal ultrasound, the liver harbored multiple anechoic lesions and a bigger cystic formation with portal inflow and early venous distribution. This teleangiectatic portovenous-malformation was confirmed on abdominal computertomography and on T1-enhanced images of MRT, too. This case examplifies the universal character of organ distribution in HHT and underlines the value of complementary diagnostic and therapeutic modalities.

We report a synthesis of our experience about the Osler-Weber-Rendu disease, based on a retrospective study of 19 cases collected from 1978 to 1995. Except the almost systematic recourse to anterior or posterior packings in 31% of cases, management of epistaxis in urgency has consisted of arteriography with embolization of the maxillary arteries. In this way, in two third of cases, the control of the epistaxis was obtained. In the other third, it was necessary to associate arteriography to a ligature of the ethmoidal arteries. As part of a preventive treatment of epistaxis we prefer, presently, to inject in situ a fibrin glue, which can be reviewed at the demand. These injections bring only exceptionally complications. The quality and duration of remission is judged satisfactory by the patients in 80% of the cases. Although no physio-pathological explanation can be brought, the intramuscular injections of fibrin glue could increase the duration of remission, which in this way, could diminish the frequency of injections in situ.

This article describes a woman with hemorrhagic telangiectasia, or Osler-Weber-Rendu disease, with recurrent nosebleeds despite prior septal dermoplasty and laser treatments. Telangiectasias were clearly shown at the time of angiography. Embolization of abnormal vessels on both sides resulted in cessation of epistaxis for many months. Bilateral studies are important, even when the epistaxis seems to be unilateral.

The authors relate their one-year experience in percutaneous embolization of 12 patients presenting with severe epistaxis because of a Rendu Osler disease. Using Ethibloc a sclerosing substance derived from maize in direct intramucosal injection seems to be a promising technique. Ethibloc is still used successfully in percutaneous treatment of superficial venous angiomas.

Our experience of the control of epistaxis by "in situ" injections of tissucol is essentially based on the use of this procedure for the treatment of epistaxis associated with Rendu-Osler's disease. We reported our first trials of this technique at the meeting organised by Immuno France Laboratories in Bordeaux in November 1983. Since this time, our initial experience has been greatly enriched, with very good results. We have treated two unusual cases of very severe epistaxis which did not respond to any of the treatments administered. We feel that these special cases warrant being reported in order to demonstrate the value of this technique.