HHT-associated Epistaxis Research Articles

Pazopanib in treatment of Hereditary Hemorrhagic Telangiectasia-related epistaxis and gastrointestinal bleeding.

BackgroundHereditary hemorrhagic telangiectasia (HHT) is a bleeding disorder characterized by arteriovenous malformations (AVMs), commonly presenting with epistaxis and gastrointestinal bleeding. Bleeding symptoms may be difficult to manage and may become life-threatening, with many patients developing dependence on parenteral iron and/or blood transfusion. There is a growing body of evidence that antiangiogenic therapies may be effective in management of bleeding symptoms, presumably targeting pathogenic HHT pathways such as vascular endothelial growth factor (VEGF) receptor. ObjectivesTo report single-center, retrospective real-world use of pazopanib, an orally administered tyrosine kinase inhibitor that blocks VEGF receptors, in six patients with HHT-associated epistaxis and/or gastrointestinal (GI) bleeding. Patient/MethodsA retrospective observational analysis was performed to assess the safety/efficacy of pazopanib use in patients with confirmed HHT-associated epistaxis and/or GI bleeding between 01 January 2019 and 14 June 2023The Indiana Hemophilia and Thrombosis (IHTC) institutional EMR was queried for HHT patients who were treated with pazopanib for >3 months.Patient data were obtained from patient documentation, physician/nursing notes, and on-call documentationInstitutional IRB approval was obtained for data pull as an exempt study Results and ConclusionsOur observations on the real-world use of pazopanib in six HHT patients with moderate to severe bleeding showed improvement in hemoglobin levels, with reduction in iron infusions and red blood cell transfusion requirement. Pazopanib may be a reasonable option for patients with HHT with epistaxis or gastrointestinal bleeding that are refractory to standard treatment.

Innovations in the management of epistaxis secondary to hereditary hemorrhagic telangiectasia: our evolution to injection sclerotherapy as the treatment of choice.

We compared the efficacy of intralesional sclerotherapy using 3% sodium tetradecyl sulfate with non-sclerotherapy-based treatments for Hereditary Hemorrhagic Telangiectasia-associated epistaxis management. This is a retrospective study of patients who underwent surgical intervention for HHT-associated epistaxis management from 01/2010-02/2024. Patients undergoing sclerotherapy with intralesional 3% sodium tetradecyl sulfate were included in the sclerotherapy group and others undergoing conventional non-sclerotherapy-based procedures in the non-sclerotherapy group. Outcomes like breakthrough epistaxis, emergency visits, intra-op blood loss, blood transfusions, and procedure complications in the 3-month perioperative period were compared. Twenty-three patients who underwent 74 intranasal procedures were identified. In the sclerotherapy group, 17 patients underwent 47 procedures. In the non-sclerotherapy group, 10 patients underwent 27 procedures. Till the 3rd post-treatment month, fewer breakthrough epistaxis episodes were observed after sclerotherapy procedures (13/47) vs. non-sclerotherapy procedures (14/27); (p = 0.037). Intraoperative blood loss was significantly lower during sclerotherapy (median: 10 ml) vs. non-sclerotherapy procedures (median: 50 ml); p < 0.001. The time interval between successive procedures was not significantly different in the sclerotherapy (median 6.5 months) vs. the non-sclerotherapy group (median 3.5 months); p = 0.13. Nasal crusting was the most common complication in the sclerotherapy group (36.9%). Two patients in each group had new onset septal perforation, none of the patients had vision loss or cerebrovascular accident. One emergency department visit was reported in the sclerotherapy group vs. 7 (in 3 patients) in the non-sclerotherapy group. Compared to non-sclerotherapy treatments, intralesional sclerotherapy for epistaxis in HHT is more effective in decreasing breakthrough epistaxis, and has lower intraoperative blood loss.

Validation of epistaxis severity score for hereditary hemorrhagic telangiectasia in Japan

ObjectiveThis 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. MethodsThe 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. ResultsIn 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. ConclusionThe Japanese version of the ESS was considered valid and may be useful as an outcome measure of future HHT-associated epistaxis trials in Japan.

A comparative study of two grading systems for epistaxis in hereditary haemorrhagic telangiectasia.

Different institutions use different grading systems for hereditary haemorrhagic telangiectasia (HHT)-associated epistaxis. It is important to have a universal, standardized grading system to compare and evaluate the effectiveness of different treatment options. We introduced the "Intensity, Frequency and need for Blood Transfusion" (IFT) grading system for HHT-associated epistaxis in 2008. Hoag et al. proposed the "Epistaxis Severity Score" (ESS) for the International HHT foundation in 2010. This study aimed to evaluate the potential correlation between the ESS and IFT grading systems. The study included 354 simultaneous reports using the IFT and ESS from 106 patients. The correlation between the ESS, IFT and haemoglobin levels was measured using Pearson's correlation coefficient. The ESS and IFT were scored simultaneously by the patient and doctor in 48 cases to evaluate if there was a discrepancy in the scoring applied by either set of responders. The measured correlation between the two grading systems was good (0.75). The grade of epistaxis reported by patients and doctors respectively showed no significant difference. Both the IFT and ESS grading systems correlate significantly to the haemoglobin level. Both the IFT and ESS scores correlate to each other, and their results are comparable. Whether the IFT or ESS scoring was performed by the patient or doctor had no significant impact.

Development and Validation of the Nasal Outcome Score for Epistaxis in Hereditary Hemorrhagic Telangiectasia (NOSE HHT)

Epistaxis is the greatest cause of morbidity in patients with hereditary hemorrhagic telangiectasia (HHT); because of this, a validated epistaxis-specific quality-of-life instrument for HHT should be made available. To develop and validate an epistaxis-specific quality-of-life patient-reported outcome measure for HHT. This survey study focused on the development and validation of the Nasal Outcome Score for Epistaxis (NOSE) in HTT (NOSE HHT) outcome measure with data prospectively collected from December 10, 2019, to March 15, 2020. A total of 401 patients were recruited from within the Cure Hemorrhagic Telangiectasia online patient advocacy social media network, the Washington University HHT Center of Excellence, and a randomized clinical trial investigating an intranasal timolol gel for HHT-associated epistaxis. Face and content validity, factor analysis, internal consistency as measured through Cronbach α, construct validity, responsiveness to change, and minimal clinically important difference. The NOSE HHT was developed and validated with a possible score ranging discretely from 0 to 4 for each of the 29 items and a total score ranging continuously from 0 to 4 after dividing by the total number of items answered. A total of 401 participants completed the NOSE HHT. Factor analysis identified 3 factors that matched the a priori specified subgroups of particular aspects of life affected by HHT-associated epistaxis: physical problems (mean [SD] magnitude, 1.59 [0.83]), functional limitations (mean [SD] magnitude, 1.28 [0.84]), and emotional consequences (mean [SD] magnitude, 1.95 [1.02]). The instrument had high internal consistency with an overall Cronbach α of 0.960. Convergent validity determined the total NOSE HHT score to be a strong predictor of disease severity; total NOSE HHT score can be split up into the following epistaxis severity categories: mild (0-1), moderate (1.01-2), and severe (>2). The instrument was found to be sensitive to change, and the minimal clinically important difference for the total NOSE HHT score was 0.46. Evaluation of the consistency, reliability, and responsiveness of the NOSE HHT survey found it to be a valid instrument to assess severity and change in epistaxis. Study results suggest that the NOSE HHT survey is clinically applicable and useful as an outcome measure of future HHT-associated epistaxis trials.

Efficacy of Timolol in a Novel Intranasal Thermosensitive Gel for Hereditary Hemorrhagic Telangiectasia–Associated Epistaxis

Other than nasal moisturizers, no standard-of-care medical therapy exists for epistaxis in hereditary hemorrhagic telangiectasia (HHT). With epistaxis as the greatest cause of morbidity in patients with HHT, there is a need to identify effective topical therapies. To determine the efficacy and safety of an intranasal timolol thermosensitive gel vs placebo thermosensitive gel in treating HHT-associated epistaxis. This double-blind, placebo-controlled randomized clinical trial was conducted from October 29, 2019, to May 20, 2020, at a tertiary care center. A total of 27 patients with HHT and moderate-to-severe epistaxis were recruited and included in this prespecified analysis: 14 in the timolol group and 13 in the placebo group. Inclusion criteria included (1) age 18 years or older, (2) clinical or genetic diagnosis of HHT, (3) screening Epistaxis Severity Score (ESS) of 4 or greater and 2 or more nosebleeds cumulatively lasting at least 5 minutes per week, (4) stable epistaxis pattern over the preceding 3 months, and (5) no change in epistaxis treatment or nasal hygiene regimen in the preceding month. Exclusion criteria included (1) contraindications to systemic β-blocker administration, (2) use of medications interacting with timolol, (3) use of antiangiogenic medications in the last month before recruitment, and (4) use of anticoagulants, antiplatelets, or fibrinolytic therapies within the last month. Novel thermosensitive intranasal timolol (0.1%) gel vs placebo thermosensitive gel applied twice daily to each nostril for 8 weeks. The primary outcome was the median change in ESS and percentage of participants reaching the minimal clinically important difference in ESS. Secondary outcomes were changes in Clinical Global Impression-Severity and Clinical Global Impression-Improvement scores, Nasal Outcome Score for Epistaxis in Hereditary Hemorrhagic Telangiectasia, and hemoglobin level. Of 27 participants randomized (median [range] age, 55 [20-76] years; 14 women [52%]; 25 White [93%]), a total of 23 patients with HHT completed the primary outcome measure. Within the timolol gel and placebo gel groups, respectively, the median change (range) in ESS was 2.32 (0.22 to 5.97) vs 1.96 (-0.91 to 5.98), and 9 of 11 (82%) vs 9 of 12 (75%) participants experienced a clinically meaningful improvement in ESS. Twenty-two of the 23 participants (96%) reported improvement via the Clinical Global Impression-Improvement score, with 81% vs 58% of participants reporting reduced severity of epistaxis in the timolol vs placebo group, respectively. Of participants completing the Nasal Outcome Score for Epistaxis in HHT at follow-up visit, 7 of 10 (70%) in the timolol group achieved a clinically important difference vs 5 of 10 (50%) in the placebo group. There was no change in hemoglobin level between or within groups. Zero participants in the placebo group and 2 of 13 (15%) in the timolol group withdrew because of adverse events. Thermosensitive gel, alone or in combination with timolol, was highly effective in reducing HHT-associated epistaxis. The timolol group had greater improvement in epistaxis and quality of life than the placebo group, but effect estimates were imprecise, and no definitive conclusions on the superiority of timolol can be drawn. Physicians treating patients with HHT-associated epistaxis should consider a thermosensitive gel (with or without timolol) for their patients. ClinicalTrials.gov Identifier: NCT04139018.

Intranasal bevacizumab injections improve quality of life in HHT patients.

Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT), with the greatest negative impact on quality of life (QoL). Repeated intranasal submucosal bevacizumab injections (RISBI) is a relatively new treatment option for moderate or severe grades of epistaxis in HHT. However, the effect of RISBI on QoL is not fully evaluated. Prospective, non-comparative study. Patients treated by RISBI for HHT-associated epistaxis between June 2011 and August 2013 were prospectively invited to the present study. The end of follow-up was October 2013. The patients were requested to answer QoL questionnaires before the first treatment, and 6-8 weeks after the last treatment. Three levels of QoL were assessed: Overall QoL using Cantril's Self-Anchoring Ladder; Health-related QoL using Short Form 36 (SF-36), and Disease-specific QoL. Psychological distress was measured with the Hospital Anxiety and Depression scale (HADS). Thirty-three patients were treated with RISBI during the period referred to above. Twenty-three patients completed the QoL questionnaires. The average number of treatments per patient was 2.15 ± 1.3 (Range: 1-5). The mean overall QoL improved from 6.47 ± 1.9 to 7.26 ± 1.6 (P < .05). Several dimensions measured by SF-36 were significantly improved with a medium to strong effect size. HADS demonstrated a significant decrease in psychological distress after the last treatment. HHT patients treated by RISBI improved in several aspects of quality of life, and psychological distress decreased. RISBI was an effective treatment option for moderate and severe grades of HHT-associated epistaxis. 4 (case series). Laryngoscope, 130:E284-E288, 2020.

Treatment with low-dose tacrolimus inhibits bleeding complications in a patient with hereditary hemorrhagic telangiectasia and pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) can be found in patients suffering from a loss-of-function mutation of the gene encoding for the activin receptor-like kinase 1 (ALK-1), a bone morphogenetic protein (BMP) type 1 receptor. Interestingly, ALK-1 mutations also lead to hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disease characterized by arteriovenous malformations (AVMs) leading to potentially life-threatening bleeding complications such as epistaxis. Current therapeutic options for both diseases are limited and often only temporary or accompanied by severe side effects. Here, we report of a patient with a mutation of the ALK-1 gene suffering from both HHT and PAH. Recently, it was shown that tacrolimus increased ALK-1 signaling and had beneficial effects in selected end-stage PAH patients. We thus hypothesized that treatment with tacrolimus may prevent disease progression in this patient. Surprisingly, treatment with low-dose tacrolimus dramatically improved his HHT-associated epistaxis but did not attenuate progression of PAH.

Long-term experience with intranasal bevacizumab therapy.

Long-term follow-up of intranasal bevacizumab therapy in hereditary hemorrhagic telangiectasia (HHT). Prospective, noncomparative study. Patients treated for HHT-associated epistaxis by intranasal submucosal bevacizumab injections between June 2011 and August 2013 were included and followed prospectively. The effectiveness of the treatment was evaluated by the epistaxis severity score (ESS); the epistaxis intensity, frequency, and the need of blood transfusion (IFT) score; and hemoglobin levels. Thirty-three patients were included. The total number of treatments with intranasal bevacizumab injection was 210. The mean number of treatments per patient was 6.2 ± 4.6 (range, 1-16), and the mean treatment and observation period was 38.8 ± 21.8 months (range, 2-66 months). Four patients showed no improvement after treatment. Eleven patients (33.3%) showed initial improvement in both ESS and IFT, but the treatment was discontinued before the end of the study because the effect became gradually shorter lasting despite repeated injections. Twelve patients (36.3%) continued to have a positive response to the treatment at the end of the study. No local adverse effects were observed, but one patient developed osteonecrosis in both knees during the treatment period. Intranasal bevacizumab injection is an effective treatment for most of the moderate and severe grades of HHT-associated epistaxis. The duration of the effect of the treatment was variable. Primary and late resistance phenomena to the treatment were quite common. 4. Laryngoscope, 128:2237-2244, 2018.

Novel treatments for epistaxis in hereditary hemorrhagic telangiectasia: a systematic review of the clinical experience with thalidomide

Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant bleeding disorder that affects one in 5,000–8,000 individuals [1, 2]. It is characterized by mucocutaneous telangiectasia and arteriovenous malformations in the pulmonary, cerebral and hepatic circulations [3]. The disorder is primarily caused by mutations in genes involved in vascular development and repair, e.g. the endoglin gene (ENG) located on chromosome 9 and the gene encoding activin receptor-like kinase-1 (ALK-1) located on chromosome 12 [4]. Recurrent and severe epistaxis is the most common presentation of HHT, frequently leading to severe anemia requiring blood transfusions [3]. Several approaches have been tried for the management of nosebleeds in HHT, including compression techniques, bilateral embolization, surgical arterial ligature and other techniques such as laser therapy, sclerosing agents, electrocautery and septodermoplasty [5]. Adjuvant medical treatments include estrogens, progestogens and antifibrinolytic agents such as tranexamic acid [6, 7]. However, most of these therapeutic measures are only palliative and largely unsatisfactory, creating therapeutic challenges and worsening the quality of life of HHT patients, especially those with severe bleeding who experience important impediments to daily activities. Recently, angiogenesis has been implicated in the pathogenesis of HHT [3]. Circulating concentrations of both transforming growth factor-b and vascular endothelial growth factor are significantly increased and anti-angiogenic drugs such as bevacizumab and thalidomide have, therefore, been tried in the treatment of vascular malformations in this disease [8–12]. The clinical experience on the role of thalidomide, a potent immunosuppressive and anti-angiogenic agent, for the pharmacological management of HHTassociated epistaxis is systematically reviewed in this paper. We performed an extensive literature search through MEDLINE, EMBASE, OVID, and SCOPUS using ‘‘Hereditary hemorrhagic telangiectasia’’, ‘‘HHT’’, ‘‘Osler-WeberRendu syndrome’’, ‘‘vascular malformation’’, ‘‘epistaxis’’, ‘‘nosebleeds’’, ‘‘thalidomide’’ and ‘‘antiangiogenic agents’’ as search terms. Further references not initially identified in the search but referenced within these articles were also reviewed. Unpublished works were identified by searching the abstract books of the most important conferences on hematological diseases. Overall, 29 cases from seven studies on the use of thalidomide for epistaxis in HHT patients refractory to standard medical and local surgical treatments were collected [13–19] (Table 1). After the first report by Kurtin [13] of improvement of nosebleed in a patient with epithelioid leiomyosarcoma and concomitant HHT, a few investigators have assessed the potential role of this anti-angiogenic drug in this clinical setting. The largest case series available in the literature is that including the results of an interim analysis of an ongoing prospective trial conducted by Balduini and colleagues and recently published in an abstract format [19]. Thalidomide was administered to eight patients at a starting dose of 50 mg/day and increased by 50 mg/day every 4 weeks until response to a maximum of 200 mg/day. A complete or partial response, defined as cessation or reduction in the severity of epistaxis, was observed in all patients but one, with significant decreases of transfusion requirements and improvement of quality of life. Six of the seven patients who had a clinical response to this drug responded within 1 month of starting treatment. Interestingly, an experimental study conducted by Lebrin and colleagues [17] showed that thalidomide M. Franchini (&) F. Frattini S. Crestani C. Bonfanti Department of Transfusion Medicine and Hematology, Carlo Poma Hospital, Mantua, Italy e-mail: massimo.franchini@aopoma.it

Bevacizumab in hereditary hemorrhagic telangiectasia‐associated epistaxis: Effectiveness of an injection protocol based on the vascular anatomy of the nose

To evaluate the effectiveness of a standardized intranasal bevacizumab injection in treating hereditary hemorrhagic telangiectasia (HHT)-associated epistaxis. Prospective pilot study. A total dose of 100 mg bevacizumab (25 mg/mL Avastin) was injected submucosally, 50 mg on each side. A total of 0.5 mL was injected in the sphenopalatine area, upper part of bony septum, upper part of the later nasal wall, and the anterior part of nasal floor. No cauterizations or laser therapy were done during or after the procedure. The hemoglobin level and grades of epistaxis were recorded before and monthly after the procedure. The IFT grading system (intensity [I], frequency [F] of epistaxis, and the amount of blood transfusion [T]) and epistaxis severity score (ESS) for hereditary hemorrhagic telangiectasia system were used. Quality of life (QoL) was evaluated before and 4 weeks after the procedure using the Short Form-36 Health Survey questionnaire, Cantril's Self-Anchoring Ladder questionnaire, and Slotosch disease-specific QoL questionnaire. A significant improvement was found in IFT grading (P = .007), ESS grading (P = .001), and hemoglobin level (P = .01). The QoL differences were statistically not significant. The four-injection site technique of intranasal administration of bevacizumab is an effective treatment option in HHT-associated epistaxis, at least on the short-term effect. Long-term and comparative studies are needed to further evaluate the significance of this treatment modality.

Treatment of hereditary hemorrhagic telangiectasia with submucosal and topical bevacizumab therapy

Bevacizumab delivered as a submucosal and topical intranasal therapy effectively controls hereditary hemorrhagic telangiectasia (HHT)-associated epistaxis. Prospective institutional review board-approved study. Between December 2009 and December 2010, 19 patients with HHT-associated epistaxis were treated with 100 mg of intranasal submucosal bevacizumab. Following treatment, patients were contacted monthly to report their epistaxis severity score (ESS) for 9 or more months after their initial treatment. If a patient had a significant increase in their ESS, they were offered treatment with 100 mg of topical bevacizumab, administered via a metered dose atomizer. All treatments were recorded. All 19 patients had a postinjection ESS documented through 9 months, whereas 17 patients had completed ESS data between months 10 and 12. Six of the 19 patients received eight additional 100 mg of topical bevacizumab treatments because they had an increase in their ESS. Results demonstrated a mean preinjection ESS of 8.12, with an ESS nadir of 2.00 reached at 2 months following submucosal injection (P < 0.0001). Over the following 12 months, the mean ESS steadily increased back to a maximum of 3.6 reached at 11 months following injection (P < .0001). Intranasal submucosal bevacizumab effectively treats HHT-associated epistaxis for up to 12 months following treatment.

The Natural History of Epistaxis in Patients with Hereditary Hemorrhagic Telangiectasia in the Norwegian population: A Cross-sectional Study

Epistaxis is usually the first and most common symptom in hereditary hemorrhagic telangiectasia (HHT), which is known also as Rendu-Osler-Weber syndrome. The severity of HHT-associated epistaxis is highly variable and can affect the patient's quality of life. In the literature, the natural history of epistaxis in HHT patients has been described in a few countries but not from the Norwegian population. This work focused on the natural history of epistaxis in the Norwegian population in a cross-sectional study. Ninety-eight patients with three or four Curaçao criteria were included. The severity of epistaxis was graded depending on epistaxis intensity, frequency, and the amount of blood transfusion during a period of 4 weeks. The epistaxis grades were studied in association with age, gender, gene mutation, age of onset, and whether the patient had or had not been treated for epistaxis during the last 2 years. Most of the HHT patients (90%) complained of mild-to-moderate epistaxis. Seventy-seven percent of the patients started epistaxis by or before the age of 20 years. The progression of HHT-associated epistaxis with age could not be proved statistically in this study. There was no statistically significant difference in the grades of epistaxis between HHT1 and HHT2 type, neither between female and male patients. Most of the patients started epistaxis by or before the age of 20 years. There was a significant difference in the grade of epistaxis between non-ENG, non-ALK1 carrier patients, and ENG or ALK1 carrier patients. Compared with other populations, the grading of epistaxis in Norwegian patients with HHT gave generally similar results. A multicenter epidemiological study is required to get a larger study population. A common internationally accepted grading or classification system for epistaxis in HHT is highly recommended.

Antiestrogen therapy for hereditary hemorrhagic telangiectasia: A double‐blind placebo‐controlled clinical trial

Hereditary hemorrhagic telangiectasia (HHT) is associated with recurrent epistaxis in 90% of cases. Good response to hormone treatment has been documented, although its use remains controversial. The aim of this study was to examine the efficacy of an antiestrogenic agent, Tamoxifen, in the treatment of HHT-associated epistaxis. Twenty-five patients (11 men, 14 women; mean age 51 years) with a diagnosis of epistaxis due to HHT were randomly assigned to receive treatment with oral tamoxifen 20 mg/d or placebo for 6 months. Follow-up consisted of physical examination and once-monthly blood tests. The groups were similar in age and sex distribution. Of the 21 participants who completed the trial, alleviation of the epistaxis was noted in 9 of 10 tamoxifen-treated patients and 3 of 11 placebo-treated patients (including 2 with only temporary improvement). The difference between the groups at the trial end point was significant for both frequency (P = .01) and severity (P = .049) of the disease. Hemoglobin concentration rose in 4 tamoxifen-treated patients and decreased in 5 controls. Tamoxifen appears to be an effective agent for the treatment of epistaxis due to HHT.

The utility of bipolar electrocautery in hereditary hemorrhagic telangiectasia

The surgical treatment of epistaxis associated with hereditary hemorrhagic telangiectasia (HHT) is varied. Laser therapy is often inadequate for larger complex lesions. This study sought to determine if bipolar cautery can be effectively and safely used in treating HHT-associated epistaxis. Records from all patients with HHT treated surgically over 8 years were reviewed retrospectively. Outcomes or complications were noted in the clinic on follow-up evaluation. Twenty-seven patients with HHT who underwent surgical treatment of epistaxis were evaluated; 18 were treated with bipolar cautery. Forty-two separate bipolar treatments were performed. No new septal perforations or synechiae were noted. Twenty-two of 42 treatments were coupled with ancillary laser treatments. The bipolar was also used as the sole technique in 20 procedures. Bipolar electrocautery is a safe and effective tool for the intraoperative control of HHT-related epistaxis. Bipolar electrocautery may be used as an adjunct to laser techniques or as a stand-alone technique. C-4.

New Classification of Nasal Vasculature Patterns in Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is a disorder characterized by the triad of recurrent epistaxis, telangiectasia, and a family history of the disease. Management of recalcitrant epistaxis in HHT remains a challenging problem for otolaryngologists. The precise coagulation of telangiectasias with the Nd-YAG laser has shown efficacy in the treatment:of HHT-associated epistaxis, but results can be variable and patient selection is critical in ensuring a successful outcome. We propose a new classification of nasal vasculature patterns in HHT as a means for selecting the Nd-YAG laser for photocoagulation treatment. The records of 40 patients who underwent Nd-YAG laser photocoagulation for HHT were reviewed retrospectively. Outcomes after Nd-YAG laser treatment were correlated with three observed nasal vasculature patterns: (I) isolated punctate telangiectasias or individual small arteriovenous malformation; (II) diffuse interconnecting vasculature with "feeder" vessels; and (III) large solitary arteriovenous malformation, which may be associated with scattered telangiectasia. Types I and II were the most common vasculature patterns seen in this patient population. Patients with patterns I and III showed greater improvement in epistaxis after Nd-YAG laser photocoagulation. Patients with pattern II fared better with septodermoplasty. These findings suggest that analysis of nasal vasculature patterns can improve therapeutic stratification of.patients with HHT. Proper patient selection using this new classification scheme may improve the management of epistaxis in patients with HHT.