Epistaxis In Hereditary Hemorrhagic Telangiectasia Research Articles

The use of thalidomide therapy for refractory epistaxis in hereditary haemorrhagic telangiectasia: systematic review.

Hereditary haemorrhagic telangiectasia is an autosomal dominant condition, characterised by mucocutaneous telangiectasia, aneurysm and arteriovenous malformations. Thalidomide has been used as a therapeutic strategy for refractory epistaxis in hereditary haemorrhagic telangiectasia patients. This review set out to examine the evidence for using thalidomide in the management of refractory epistaxis in hereditary haemorrhagic telangiectasia patients. A systematic search of the available literature was performed using Medline, Embase, Cochrane Library and NHS Evidence databases, from inception to December 2017. The search terms used included: hereditary haemorrhagic telangiectasia (HHT), Osler-Weber-Rendu syndrome, epistaxis, haemorrhage and thalidomide. All studies using thalidomide therapy showed a reduction in the frequency and duration of epistaxis, as early as four weeks post-therapy. In addition, thalidomide therapy was shown to increase median haemoglobin levels and reduce blood transfusion dependence. Current available evidence suggests that low-dose thalidomide is effective in transiently reducing epistaxis frequency and duration. Further studies are required to establish a treatment regimen to prevent side effects.

Bevacizumab for Epistaxis in Hereditary Hemorrhagic Telangiectasia: An Evidence-based Review.

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).

Medical treatment of epistaxis in hereditary hemorrhagic telangiectasia: an evidence-based review.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations. A search of the Ovid MEDLINE, Embase, and Cochrane databases was conducted by a research librarian. Abstracts in the English language and published in a peer-review journal were reviewed for relevance and inclusion. PRISMA guidelines were followed. Eighteen studies met the inclusion criteria. In a few small studies, thalidomide was shown to consistently improve severity and frequency of epistaxis and improve hemoglobin concentrations while decreasing the need for transfusion. Tranexamic acid appeared to only impact the epistaxis severity score and not other clinical outcomes. Selective estrogen modulators (SERMs), propranolol, rose geranium oil, and N-acetylcysteine, have demonstrated promising efficacy in small trials. Appropriate medical therapies for epistaxis outcomes in HHT remain undefined, and there is no "gold standard." Many of the studies are small and the data reported are heterogeneous, and therefore the ability to make strong evidence-based recommendations is limited. However, many different medications appear to be promising options.

Nintedanib as a novel treatment option in hereditary haemorrhagic telangiectasia

A 70-year-old patient with known hereditary haemorrhagictelangiectasia (HHT) was seen regularly in our outpatient clinic. He underwent multiple therapeutical interventions, including both surgical and medical, for the treatment of recurrent...

Bevacizumab injection for epistaxis in hereditary hemorrhagic telangiectasia

Bevacizumab injection for epistaxis in hereditary hemorrhagic telangiectasia

Efficacy and safety of propranolol for epistaxis in hereditary haemorrhagic telangiectasia: retrospective, then prospective study, in a total of 21 patients.

Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of angiogenesis associated with disabling epistaxis. Propranolol, a beta-blocker, exerts anti-angiogenic properties. In the retrospective study, nine of ten HHT patients receiving propranolol significantly improved their Epistaxis Severity Score. In the prospective study, after three months of propranolol treatment, the median duration of epistaxis per month significantly decreased as did the number of epistaxis episodes per month in 11 HHT patients. Tolerance of propranolol was quite satisfactory with only one hypotension among the overall 21 HHT patients. This article is protected by copyright. All rights reserved.

Is bevacizumab effective for reducing epistaxis in hereditary hemorrhagic telangiectasia?

The LaryngoscopeVolume 127, Issue 2 p. 289-290 Trio Society Best Practice Is bevacizumab effective for reducing epistaxis in hereditary hemorrhagic telangiectasia? Christopher J. Chin MD, FRCSC, Corresponding Author Christopher J. Chin MD, FRCSC christopher.chin@HorizonNB.ca Division of Otolaryngology–Head and Neck Surgery, Department of Surgery, Dalhousie Medicine New Brunswick, Saint John, New Brunswick, CanadaSend correspondence to Christopher J. Chin, MD, Division of Otolaryngology–Head and Neck Surgery, Department of Surgery, Dalhousie Medicine New Brunswick, 711 Millidge Avenue, Suite A, Saint John, NB, Canada E2K 2N7. E-mail: christopher.chin@HorizonNB.caSearch for more papers by this author Christopher J. Chin MD, FRCSC, Corresponding Author Christopher J. Chin MD, FRCSC christopher.chin@HorizonNB.ca Division of Otolaryngology–Head and Neck Surgery, Department of Surgery, Dalhousie Medicine New Brunswick, Saint John, New Brunswick, CanadaSend correspondence to Christopher J. Chin, MD, Division of Otolaryngology–Head and Neck Surgery, Department of Surgery, Dalhousie Medicine New Brunswick, 711 Millidge Avenue, Suite A, Saint John, NB, Canada E2K 2N7. E-mail: christopher.chin@HorizonNB.caSearch for more papers by this author First published: 31 October 2016 https://doi.org/10.1002/lary.26363Citations: 2 The author has no funding, financial relationships, or conflicts of interest to disclose. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume127, Issue2February 2017Pages 289-290 RelatedInformation

Intranasal topical estrogen in the management of epistaxis in hereditary hemorrhagic telangiectasia

Conclusion Application of topical estriol ointment is an effective treatment for hereditary hemorrhagic telangiectasia (HHT) epistaxis.Objective HHT is an autosomal-dominant disease characterized by epistaxis in more than 96% of patients. Management of this major symptom, epistaxis, has not been standardized. This study reports experience with topical application of estriol in patients with HHT.Methods Five patients with a confirmed diagnosis of HHT who first visited the hospital between 2012 and 2013 received 0.1% estriol ointment and were guided to apply the ointment twice daily to the anterior part of both nasal cavities. Severity of epistaxis was valued using epistaxis severity score (ESS) before and 3 months after initiating therapy.Results Five patients (three males, two females) received treatment. After the initiation of treatment, intensity and frequency of epistaxis became moderate in all patients. ESS decreased significantly from pre- to post-treatment (p = 0.043). No adverse events were recorded during follow-up.

Treatment of severe refractory epistaxis in hereditary hemorrhagic telangiectasia using a two-flap nasal closure method.

Nasal closure has been shown to effectively manage severe epistaxis refractory to other treatments in patients with hereditary hemorrhagic telangiectasia (HHT). The nasal closure procedure may be underutilized because of its surgical complexity and flap breakdown. This work is a retrospective review of 13 HHT patients treated for severe epistaxis with nasal closure between 2005 and 2013. Operating room (OR) time, need for revision surgery, preprocedure, and postprocedure epistaxis severity score (ESS), complete blood count values, and Glasgow Benefit Inventory (GBI) questionnaire results were collected for each patient. The technique is described. We characterize a typical nasal closure patient and compare outcomes based on our experience with the traditional 3-flap closure and a simplified 2-flap nasal closure procedure. The average candidate for nasal closure in this series had an ESS of 7.88, hemoglobin (Hgb) of 8.3 g/dL, and received multiple transfusions, iron therapy, and cautery/coagulation procedures. Average ESS subsequent to nasal closure using the 2-flap method is 0.92 and mean GBI score is 56.3. Comparison of 5 patients who underwent the traditional 3-flap nasal closure procedure and 8 patients receiving the 2-flap nasal closure showed no significant difference in postoperative ESS or GBI metrics. Mean operating room times of the traditional and simplified methods were 3.12 hours and 1.44 hours (p = 0.0001). Mean time to first revision for 8 nasal closure patients was 21.5 months. In short-term follow-up, the 2-flap procedure showed comparable effectiveness with significantly reduced complexity and operative time compared to the traditional nasal closure method.

Efficacy of KTP Laser Photocoagulation Combined with Cautery as Treatment for Epistaxis in HHT

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia characterized by spontaneous, recurrent epistaxis. Epistaxis ranges from sporadic, low volume bleeds to frequent hemorrhages causing anemia. In moderate-severe cases, KTP laser photocoagulation and/or electrocautery are commonly utilized to control epistaxis. Objective: This observational cohort study aims to evaluate the efficacy of laser therapy combined with cautery as treatment for epistaxis in HHT. Methods: Chart review and telephone interviews were conducted for all HHT patients at our center who had undergone laser therapy with or without concurrent electrocautery for intranasal lesions from 08/2011 – 12/2014. Data collected from chart included patient demographics, epistaxis severity score (ESS) at time of intervention, and any ESS recorded in the interim. Telephone interviews were conducted at two time points 18 months apart to collect ESS on subjects. Results: 35 subjects were included in the final analysis, 10 of which had >1 procedure during the time frame. The average pre-operative ESS was 7.1. Post-operative ESS were an average of 2.93 units lower than the pre-operative scores (p<0.05). On average, for each 1-unit increase in pre-operative ESS, the improvement in postoperative ESS increased by 0.72 units (p<0.05). There was no significant difference when comparing age with time between procedures (p=0.38). Conclusion: Laser therapy combined with cautery reduces epistaxis severity in HHT and provides greater improvement of symptoms in patients who present with more severe epistaxis.

Efficacy and safety of thalidomide for the treatment of severe recurrent epistaxis in hereditary haemorrhagic telangiectasia: results of a non-randomised, single-centre, phase 2 study

Hereditary haemorrhagic telangiectasia is a genetic disease that leads to multiregional angiodysplasia. Severe recurrent epistaxis is the most common presentation, frequently leading to severe anaemia. Several therapeutic approaches have been investigated, but they are mostly palliative and have had variable results. We aimed to assess the efficacy of thalidomide for the reduction of epistaxis in patients with hereditary haemorrhagic telangiectasia that is refractory to standard therapy. We recruited patients aged 17 years or older with hereditary haemorrhagic telangiectasia who had severe recurrent epistaxis refractory to minimally invasive surgical procedures into an open-label, phase 2, non-randomised, single-centre study at IRCCS Policlinico San Matteo Foundation (Pavia, Italy). We gave patients thalidomide at a starting dose of 50 mg/day orally. If they had no response, we increased the thalidomide dose by 50 mg/day increments every 4 weeks, until a response was seen, up to a maximum dose of 200 mg/day. After patients had achieved a response, they continued treatment for 8-16 additional weeks. The primary endpoint was the efficacy of thalidomide measured as the percentage of patients who had reductions of at least one grade in the frequency, intensity, or duration of epistaxis. We followed up patients each month to assess epistaxis severity score and transfusion need, and any adverse events were reported. We included all patients who received any study drug and who participated in at least one post-baseline assessment in the primary efficacy population. The safety population consisted of all patients who received any dose of study treatment. This trial is registered with ClinicalTrials.gov, number NCT01485224. Between Dec 1, 2011, and May 12, 2014, we enrolled 31 patients. Median follow-up was 15·9 months (IQR 10·1-22·3). Three (10%, 95% CI 2-26) patients had a complete response, with bleeding stopped, 28 (90%, 95% CI 74-98) patients had partial responses. Overall, all 31 (100%, 89-100) patients responded to therapy with a significant decrease in all epistaxis parameters (p<0·0001 for frequency, intensity, and duration). A response was achieved by 25 (81%) patients at 50 mg/day of thalidomide, five (16%) patients at 100 mg/day, and one (3%) patient at 150 mg/day. Patients had only non-serious, grade 1 adverse effects, the most common of which were constipation (21 patients), drowsiness (six patients), and peripheral oedema (eight patients). One patient died a month after the end of treatment, but this was not deemed to be related to treatment. Low-dose thalidomide seems to be safe and effective for the reduction of epistaxis in patients with hereditary haemorrhagic telangiectasia. Our findings should be validated by further studies with larger patient populations, longer follow-up, and that also assess the benefit for quality of life. Telethon Foundation.

Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia.

There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis. The Medline and Embase databases were interrogated on 15 November 2013 using the search items 'hereditary haemorrhagic telangiectasia' (title), 'epistaxis' (title) and 'treatment' (title and abstract), and limiting the search to articles published in English. A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3 in vitro studies. There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

Effect of Genotype and Antifibrinolytic Therapy on the Severity of Epistaxis in Hereditary Hemorrhagic Telangiectasia

▪Background: Hereditary Hemorrhagic Telangiectasia (HHT, or Osler Weber Rendu syndrome) is an inherited multiorgan disorder characterized by the development of abnormal blood vessels, resulting in the formation of telangiectasias on mucocutaneous surfaces (skin, lips, nasal and buccal mucosa, gastrointestinal mucosa) and arteriovenous malformations (AVMs) in certain visceral organs (brain, lungs, spinal cord and liver). Over 90% of affected individuals develop epistaxis, often leading to complications, such as iron-deficiency anemia and the need for oral and parenteral iron supplementation, blood transfusions, and associated decrease in quality of life. The management of epistaxis in HHT has consisted predominantly of invasive approaches like cautery, laser photocoagulation, and septodermopasty. Antifibrinolytic agents [aminocaproic acid and tranexamic acid] are frequently used in the management of patients with inherited and acquired mucocutaneous bleeding disorders; however data on the use of antifibrinolytics for the treatment of epistaxis in HHT have been limited to case reports. We hypothesize that antifibrinolytic therapy is effective for the treatment of moderate to severe HHT-related epistaxis.Methods: The medical records of patients with HHT evaluated at University of North Carolina from 2009 to October 31, 2013 were retrospectively reviewed. Patients with epistaxis requiring pharmacologic therapy in whom the epistaxis severity score (ESS) was available were included. The ESS is a clinical scoring system that utilizes six factors associated with self-reported epistaxis severity (frequency, duration, intensity, need for medical attention, anemia, and need for transfusion) to generate a clinical score. The response to antifibrinolytic therapy was evaluated by comparing pre-post ESS. Demographic data and HHT mutation status were also noted and the impact of genotype on the severity of epistaxis was analyzed. All statistical analyses were performed with SigmaStat 2.0 for Windows (SYSTAT Inc, Chicago, IL) using analysis of variance. Values are reported as +/- standard error of means. The study was approved by the Institutional Review Board.Results: Two hundred and twenty two patient records were reviewed. Of these, 126 were adults with epistaxis and were eligible for study evaluation. Seventy-one of these patients underwent genetic testing and 34 (47.9%) had an ACVRL1 (ALK-1 mutation while 22 (30.99%) had an ENG mutation. Five patients (7%) had a variant of uncertain significance (VUS), while no mutation was identified in 10 patients (14%) with a clinical diagnosis of HHT as per Curacao criteria. There was a correlation between the ESS and HHT genotype. Patients with an ACVRL1 (ALK-1) mutation (n=34) had significantly higher ESS (4.32 ± 0.455, p<0.05) compared to patients without mutations (2.08 ± 0.672). There was no significant difference in ESS between patients with an ENG mutation (n=22, ESS= 3.379 ± 0.455) or with a VUS (n=5, ESS= 5.5 ± 1.049) compared to those with negative genetic testing. Forty-nine HHT patients with moderate to severe ESS were started on an oral antifibrinolytic agent (aminocaproic acid 6 grams per day or tranexamic acid 3.9 grams per day). Patients were allowed to titrate the dose of medication down based on response. Follow-up over an average of 15 months (range 3-39 months) was available on 16 of the 49 patients. Of the 16 patients with follow up data, the average pre-treatment ESS score was 5.7 ± 0.466 and decreased to 4.8 ± 0.321 with antifibrinolytic therapy (p<0.05). All the 16 patients tolerated antifibrinolytic therapy well and without significant adverse effects.Conclusion: This study demonstrates for the first time that the severity of epistaxis in patients with HHT correlates with the HHT genotype. Epistaxis is more severe in patients with ACVRL1 (ALK-1) mutations. We also show that antifibrinolytic therapy is effective in improving the severity of epistaxis in individuals with HHT and that their chronic use is well tolerated in this population. We are currently in the process of expanding assessments of our study cohort. Larger, multicenter, randomized control trials are needed to study durability of treatment response and safety of these agents with long-term, continuous use. DisclosuresOff Label Use: off-label use of aminocaproic acid and tranexamic acid in patients with inherited and acquired mucocutaneous bleeding disorders.

The effects of epistaxis on health‐related quality of life in patients with hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease mainly characterized by epistaxis in more than 96% of patients. Recently, a validated questionnaire known as the HHT Epistaxis Severity Score (ESS) was developed. However, little is known about the relationship between epistaxis and quality of life. We hypothesize that epistaxis severity is a major factor predicting health-related quality of life (HR-QoL) in HHT patients. This is a cross-sectional study. The ESS questionnaire and Medical Outcomes Study 36-item short form (SF-36) were administered to subjects through an Internet survey. All participants had a definitive diagnosis of HHT through Curaçao criteria or genetic testing. Demographic information, genetics, and extensive histories were also collected. Descriptive analyses were performed with calculations of means and standard deviations (SDs) for continuous variables and proportions for categorical variables. Linear regressions were then performed to assess the association between HR-QoL and ESS. A total of 604 subjects participated between April and August 2008. All patients reported epistaxis, 285 (47.2%) had telangiectasias, and 545 (90.2%) had a family history of HHT; 167 (27.6%) patients had mild epistaxis (ESS <4), 285 (47.2%) reported moderate epistaxis (≥4 ESS <7), and 152 (25.2%) reported severe epistaxis (ESS ≥7). Patients with severe epistaxis had lower scores for both the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of HR-QoL when compared to those with mild epistaxis (p < 0.001, p < 0.001). The ESS is a major determinant of HR-QoL and should be considered as a measurement of treatment efficacy in HHT-related epistaxis.

Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - a double-blind placebo-controlled cross-over phase IIIB study

IntroductionEpistaxis is the most frequent manifestation in hereditary hemorrhagic telangiectasia, in which no optimal treatment exists. It can lead to severe anemia and reduced quality of life. Positive effects of tranexamic acid, an antifibrinolytic drug, have been reported on epistaxis related to this disorder. We sought to evaluate the efficacy of treating nosebleeds in hereditary hemorrhagic telangiectasia with tranexamic acid. Materials and MethodsIn a randomized, double-blind, placebo controlled, cross-over phase IIIB study, 1 gram of tranexamic acid or placebo was given orally 3 times daily for 3months for a total of 6months. Results22 patients were included in the intention-to-treat analysis. Hemoglobin levels, the primary outcome measure, did not change significantly (p=0.33). The secondary outcome measure was epistaxis score and patients reported a statistically significant reduction in nosebleeds, equaling a clinically relevant 54% diminution (p=0.0031), as compared to the placebo period. No severe side effects were observed. ConclusionTranexamic acid reduces epistaxis in patients with hereditary hemorrhagic telangiectasia.(Clinical trial registration numbers: BfArM 141 CHC 9008–001 and ClinicalTrials.gov NCT01031992)

Domiciliary floseal prevents admission for epistaxis in hereditary hemorrhagic telangiectasia

Laryngoscope 124:2238–2240, 2014

Intranasal submucosal bevacizumab for epistaxis in hereditary hemorrhagic telangiectasia: A double‐blind, randomized, placebo‐controlled trial

The purpose of this study was to investigate the efficacy of bevacizumab ("Avastin") for the treatment of epistaxis in hereditary hemorrhagic telangiectasia (HHT). In this double blind, placebo controlled trial, 15 adult patients with HHT with a minimum of 2 epistaxis episodes per week were randomized. A history of thromboembolic events or recent or planned surgery led to exclusion. Patients received a single intranasal submucosal injection (10 mL) of 100 mg bevacizumab or placebo. The primary outcome was the relative reduction of average daily epistaxis visual analog score (VAS). Average daily posttreatment VAS scores decreased by 27% in the bevacizumab group and by 3% in the placebo group (p = .57). The reduction in HHT epistaxis severity scores was -0.61 greater in the bevacizumab group compared to the placebo group (p = .34). Results show a trend toward reduced epistaxis with bevacizumab. This further supports the use of bevacizumab in HHT. Clincial Trials. gov number: NCT01314274

ELLIPSE Study

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

Pilot study of submucosal radiofrequency for epistaxis in hereditary hemorrhagic telangiectasia

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.

The effect of anterior palatine blocks on bleeding in hereditary hemorrhagic telangiectasia nasal surgery

When combined with local sphenopalatine (SP) injection and moderate hypotension, transoral or transcutaneous local injection of the anterior palatine (AP) vessels reduces intraoperative bleeding in hereditary hemorrhagic telangiectasia (HHT) patients undergoing nasal surgery. Retrospective chart review of 55 consecutive HHT patients undergoing a bevacizumab injection for recalcitrant epistaxis. Nineteen patients received local injections to only the SP vasculature, and 36 patients received AP and SP injections. Main outcome variable was estimated blood loss during nasal surgery. Independent variables included sex, age, epistaxis severity score, surgical techniques (including laser), and blood pressure parameters (baseline, preinduction, and postintubation). The mean blood loss in HHT patients receiving SP injections alone was 111 mL, whereas it was 22 mL for those receiving both AP + SP injections. This difference between groups approached statistical significance (P = .075). Blood pressure parameters were similar in both groups with no appreciable difference between intraoperative systolic and mean arterial blood pressure. The addition of either sublabial or transcutaneous AP injection to the standard SP block markedly reduces blood loss in HHT epistaxis nasal surgery.