A treatment algorithm for the management of epistaxis in hereditary hemorrhagic telangiectasia.

We examined the management options used for epistaxis of varying severity in patients with hereditary hemorrhagic telangiectasia (HHT) to develop a treatment algorithm. Fifty patients with HHT were studied from 1991 to 1997 to consider various treatment options and their relative efficacy. There were 28 females and 22 men; their ages ranged from 15 to 85. Follow-up ranged from 6 months to 6 years. A wide variety of treatments had been used (including various forms of cautery, septal surgery, topical and systemic hormones, antifibrinolytic therapy, arterial ligation, laser therapy, and closure of the nostrils). Treatments used during the follow-up period were argon laser therapy (40), systemic estrogen or progesterone (17), septodermoplasty (12), or closure of the nostrils (14). Patients were divided into those with mild to moderate disease (requiring one or no blood transfusions during the course of their illness) and those with severe disease (requiring more than one blood transfusion). Using this broad categorization, those with mild to moderate disease did well with sequential argon laser therapy with or without the adjunct of septodermoplasty or hormone therapy. Those with severe disease did not find argon laser therapy beneficial but found their epistaxis completely stopped with closure of the nasal cavities. Based on these findings, a simple treatment algorithm defines the optimum treatment options for epistaxis in this difficult-to-treat group of patients.     

Neodymium-YAG laser intranasal photocoagulation in hereditary hemorrhagic telangiectasia: an update report

Hereditary hemorrhagic telangiectasia is a challenging problem for the otolaryngologist since frequent, often severe epistaxis is the major symptom. Options for therapy in the past have included nasal packing, electrocautery, systemic estrogens, septal dermatoplasty, arterial embolization, and arterial ligation. Although successful treatment has been achieved with some of these methods, particularly septal dermatoplasty, other forms of therapy are needed prompting the use of laser photocoagulation. The neodymium yttrium-aluminum-garnet (Nd-YAG) laser was used to treat a group of 19 patients with hereditary hemorrhagic telangiectasia over a 4-year period without complications. Endonasal laser photocoagulation was effective in decreasing epistaxis in patients not requiring frequent transfusions prior to laser therapy. Three patients with the most severe epistaxis received minimal or no benefit from Nd-YAG laser photocoagulation. In most patients Nd-YAG photocoagulation is successful in treating epistaxis associated with hereditary hemorrhagic telangiectasia and should be a therapeutic option for this chronic disease having no available cure.     

Hereditary hemorrhagic telangiectasia: a review of 76 cases

OBJECTIVES/HYPOTHESIS: Hereditary hemorrhagic telangiectasia has long been viewed as a rare condition. Recent evidence indicates that the disorder is more frequent than previously thought. Recalcitrant epistaxis is a salient feature of this disease, and the otolaryngologist is often called on to make the diagnosis and guide the primary management of patients with hereditary hemorrhagic telangiectasia. Wider recognition of this condition, awareness of the natural history and associated findings, appropriate workup and screening for arteriovenous malformations (lungs, brain, liver), and knowledge of appropriate interventions can help avoid the considerable morbidity associated with hereditary hemorrhagic telangiectasia. STUDY DESIGN: Retrospective review. METHODS: Records of patients treated by the senior author (S.M.S.) for hereditary hemorrhagic teleangiectasia from 1993 to 2000 were reviewed. RESULTS: Seventy-six patients were identified, 98% of whom had epistaxis as their presenting complaint, with 75% having a family history of hereditary hemorrhagic telangiectasia. The severity of epistaxis varied in the patients: 66% had mild, 21% moderate, and 13% severe epistaxis. Sixty-four percent of patients had no transfusions, 25% had 1 to 10 transfusions, and 11% of patients had more than 10 transfusions. Complications of hereditary hemorrhagic telangiectasia were documented in 30% of patients. Screening for arteriovenous malformations was performed in only 34% of patients. Eighty-two percent of patients received a variable number of Nd:YAG laser treatments. CONCLUSIONS: The study presents the largest retrospective review of patients treated for hereditary hemorrhagic telangiectasia by a single otolaryngologist. The importance of a multidisciplinary approach facilitated by the otolaryngologist for evaluation of concomitant complications and morbidity (arteriovenous malformations) from hereditary hemorrhagic telangiectasia is demonstrated. An algorithm for controlling the epistaxis is presented.     

Avastin and diode laser: A combined modality in managing epistaxis in hereditary hemorrhagic telangiectasia

A patient with hereditary hemorrhagic telangiectasia is a rare presentation to the otolaryngologist in clinical practice. They almost present with epistaxis, which is recurrent, spontaneous and can be functionally and socially debilitating for the patient. Diode laser cauterization and Avastin intranasal injection showed significant improvement in epistaxis severity score. We report a case of hereditary hemorrhagic telangiectasia with chronic epistaxis (hemoglobin 3.4 mg/dl) managed with Diode laser and intranasal Avastin injection with great improvement in life quality and decrease in epistaxis attacks. Despite all treatment options absolute eradication of epistaxis attacks is difficult to obtain in these cases.     

Efficiency of laser treatment in patients with hereditary hemorrhagic telangiectasia

Earlier studies have shown the effect of laser treatment on epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). At the present time, only very few prospective trials have been performed, and many studies are based on patients’ subjective assessment of the severity of epistaxis. This prospective study measures the objective effect of laser treatment in HHT patients with mild to moderate epistaxis. We introduce an objective measure to assess the severity of epistaxis: the bleeding time (BT). Before and after treatment, the quality of life, as measured by the patient, was assessed and compared to normative data. In 30 patients, we measured the BT before laser treatment 1.5 and 6.5 months after treatment. The Short form 36 (SF-36), a validated health questionnaire, was completed before and 6.5 months after treatment. Compared to preoperative value, BT was significantly reduced 1.5 and 6.5 months after laser treatment (p < 0.05) in both cases. No significant difference in quality of life, before and after treatment, was found. The quality of life of the HHT patients was reduced in five out of eight dimensions when compared with the Danish background population. Laser treatment reduces epistaxis in HHT patients with mild to moderate epistaxis for at least 6 months; this group of patients have reduced quality of life compared to the background population.     

Nasal dermoplasty for Japanese hereditary hemorrhagic telangiectasia

OBJECTIVE: While generally considered an effective treatment for moderate to severe epistaxis in hereditary hemorrhagic telangiectasia (HHT), nasal dermoplasty (ND) has not been well established in Japan. This prompted the present Japanese assessment of clinical efficacy and patient satisfaction following this procedure. METHODS: Retrospective analysis of clinical records of 15 patients with HHT undergoing ND between August 1991 and May 2004 and survey of these patients as to postsurgical conditions. Main outcome measures were skin graft "take" frequency after surgery (all patients), reported patient satisfaction (eight recent patients), and reported volume and frequency of epistaxis after versus before surgery (eight recent patients). RESULTS: Graft take rate was 100%. Most patients experienced reduced frequency and volume of bleeding. One patient required an additional operation, total closure of the external nares, 2 years later. Overall patients felt satisfied with ND, experiencing less nasal obstruction than expected. CONCLUSIONS: ND is effective in Japanese patients with moderate and severe nasal bleeding from HHT, reducing their risk of bleeding.     

Outcome of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia

OBJECTIVES/HYPOTHESIS: Septal dermoplasty has been recommended as the treatment of choice for life-threatening epistaxis in patients with hereditary hemorrhagic telangiectasia. The purpose of the study was to evaluate the effectiveness and outcomes of septal dermoplasty for management of transfusion-dependent epistaxis. STUDY DESIGN: Retrospective study. METHODS: Between 1994 and 2004, septal dermoplasty was performed on 67 consecutive patients with severe epistaxis attributable to hereditary hemorrhagic telangiectasia. The numbers of units of blood received 1 year before and 1 year after septal dermoplasty were obtained. A subjective appraisal of the results of the surgery as well as second procedures after septal dermoplasty was determined. Patients were screened for pulmonary and cerebral arteriovenous malformations, gastrointestinal tract bleeding, and symptomatic liver disease. RESULTS: Data were obtained in 66 of 67 (98%) patients with a mean age of 61.5 years (mean follow-up, 3.9 y). Accurate transfusion requirements 1 year before and 1 year after septal dermoplasty were available in 32 of 66 (48%) patients. In these 32 patients, the mean units of blood received decreased from 21 units (range, 2-100 units) 1 year before septal dermoplasty to 1 unit (range, 0-10 units) in the year after septal dermoplasty (P < .001). Improved quality of life was claimed in 57 patients. Second therapies, ranging from cautery to repeat partial septal dermoplasty, were required in 15 patients during follow-up. Among the 67 patients, 31 (46%) had pulmonary arteriovenous malformation, 14 (21%) had gastrointestinal tract bleeding, 7 (10%) had symptomatic liver disease, and 5 (7%) had cerebral arteriovenous malformation. During the follow-up, 14 patients died of other complications of hereditary hemorrhagic telangiectasia (11 patients) and unrelated causes (3 patients). CONCLUSION: Septal dermoplasty remains an effective way of reducing transfusion requirements in patients with hereditary hemorrhagic telangiectasia and subjectively improves their quality of life. The otolaryngologist caring for patients with hereditary hemorrhagic telangiectasia should be familiar with other organ involvement by hereditary hemorrhagic telangiectasia to prevent complications during surgery.     

Treatment of hereditary hemorrhagic telangiectasia by ND-YAG laser photocoagulation

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) is an inherited abnormality of the vasculature characterized by abnormal subepidermal vessels. Frequent, sometimes severe, epistaxis often requiring blood transfusions is present in most patients. Septal dermatoplasty and estrogen have proved to be the most successful therapies. However, the frequency of failure in treatment has stimulated a search for an alternative therapy. The Nd-YAG laser was used at low-power settings (20-25 W) to treat 6 patients with hereditary hemorrhagic telangiectasia, all of whom had been treated previously using other modalities. Laser photocoagulation diminished the frequency and severity of hemorrhage in 5 of 6 patients without complications (6 to 9 month follow-up period). The Nd-YAG laser may be used repeatedly when new accessible lesions arise, and it appears to be a useful therapeutic option for this disease that is difficult to treat.     

Percutaneous embolization to control epistaxis in Rendu-Osler-Weber disease.

Recurrent epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease), a disorder characterized by widely scattered visceral, dermal, and mucosal vascular lesions. Emergency measures applied locally may control acute hemorrhage, but seldom result in long-term benefit. Recently, we have had the opportunity of performing bilateral carotid angiographic examination on a patient with hereditary hemorrhagic telangiectasia who was suffereing from severe intractable epistaxis. At the time of angiographic examination, embolization of both internal maxillary arteries was accomplished, and control of the epistaxis was achieved. In the patient with severe epistaxis that is unresponsive to local measures, percutaneous embolization offers substantial advantages over surgical intervention.     

Are patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia satisfied with nostril closure surgery?

Objective

Recurrent epistaxis as a manifestation of hereditary hemorrhagic telangiectasia (HHT) is usually difficult to control. Although no treatment is regarded to be completely efficacious, nostril closure is considered a modality of choice for the most severe cases. The cessation of airflow resulting from this procedure can stop bleeding by minimizing risk factors. However, loss of nasal functions is a disadvantage of nostril closure. We conducted a questionnaire survey of patients who underwent nostril closure surgery, regarding the effects and disadvantages of the operation.

Methods

Seven patients were asked questions on issues including frequency and severity of epistaxis pre- and post-operatively, satisfaction of treatment, and impairment in daily living activities.

Results

Most patients reported complete cessation of bleeding. Some still had bleeding, but the frequency and severity were far lower. No transfusions were required in any of the cases. Patients reported some disadvantages, for example, respiratory, olfactory, and phonatory issues. Six out of seven patients were very satisfied with the outcome of surgery.

Conclusion

Nostril closure surgery can remarkably reduce frequency and volume of epistaxis. Our survey indicated that satisfactory results were achieved. However, difficulties caused by complete nasal obstruction varied. Thus, individualized coping strategies are required.     

Intravenous and topical intranasal bevacizumab (Avastin) in hereditary hemorrhagic telangiectasia

Current treatment of severe epistaxis in patients with hereditary hemorrhagic telangiectasia is not durable in reducing the frequency and severity of bleeds. Recent reports have demonstrated marked improvement of epistaxis with administration of either intravenous or topical bevacizumab. We present the long-term outcome of a patient who received repeated treatments of intravenous bevacizumab followed by maintenance intranasal bevacizumab. We demonstrate durable control of epistaxis with intranasal bevacizumab. This allows delivery of bevacizumab effectively, reduces cost, and obviates the risk of systemic adverse effects related to bevacizumab.     

New classification of nasal vasculature patterns in hereditary hemorrhagic telangiectasia

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.     

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.     

A silicone nasal swab for the treatment of severe and recalcitrant epistaxis in hereditary hemorrhagic telangiectasia: an alternative to surgical nostril closure

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.     

Microvascular free flap in hereditary hemorrhagic telangiectasia

A 69-year-old male patient with hereditary hemorrhagic telangiectasia presented with severe epistaxis requiring repeated transfusions. Both nasal passages were densely populated with these vascular malformations that involved the entire nasal mucosa. A total rhinotomy was performed and all the nasal mucosa and turbinates were excised. Both nasal passages were completely resurfaced with a free radial forearm skin flap. The vascular pedicle was delivered from the nasal passage via the maxillary antrum to anastomose with the facial vessels in the cheek. Following surgery the patient had no further significant epistaxis.     

The natural history of epistaxis in hereditary hemorrhagic telangiectasia

The purpose of this retrospective study is to document the natural history of epistaxis in patients with hereditary hemorrhagic telangiectasia. A telephone questionnaire was administered to 73 patients who had been previously screened for pulmonary arteriovenous malformations (PAVMs). The incidence of epistaxis in this population was 93%, with a mean onset age of epistaxis of 12 years, a mean frequency of bleeding of 18 episodes per month, and a mean duration of bleeding of 7.5 minutes. More than 90% of patients experienced the onset of epistaxis before the age of 21 and symptoms were progressive with age. There were no differences in the age of onset, frequency of epistaxis, or duration of epistaxis between patients with PAVMs versus those without PAVMs. Although the natural history of epistaxis does not predict the presence or absence of pulmonary arteriovenous malformations, epistaxis is an early marker of the disease, hereditary hemorrhagic telangiectasia, and might guide screening for pulmonary and cerebral arteriovenous malformations in children of affected parents.     

Treatment of epistaxis in hereditary hemorrhagic telangiectasia patients by argon plasma coagulation with local anesthesia

BACKGROUND: Recurrent epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT). The aim of this study was to determine the role and efficacy of argon plasma coagulation (APC) in the management of epistaxis caused by HHT. METHODS: From 1997 to 2004, 43 patients with diagnosed HHT were treated for recurrent epistaxis with APC in our department. RESULTS: Thirty-six patients reported substantial reduction of bleeding after treatment. Of the 18 patients who previously needed blood transfusions, 13 reported substantial reduction of bleeding after treatment and no blood transfusions were necessary. CONCLUSION: APC allows a control of epistaxis in HHT patients and guarantees a long time free from blood transfusions. This treatment modality can be performed with local anesthesia, is not invasive, is well tolerated, is inexpensive, and can be used as a first step even in patients who need to undergo several blood transfusions for their epistaxis.     

The SF-36 health status questionnaire in assessing patients with epistaxis secondary to hereditary hemorrhagic telangiectasia

BACKGROUND: This study assesses the ability of the short form 36 (SF-36), a validated health status survey, to measure the health of patients with epistaxis due to hereditary hemorrhagic telangiectasia (HHT). METHODS: Thirty-eight patients completed the SF-36 and symptom-specific questionnaires. They were asked to rate their epistaxis as severe, moderate, or mild. The SF-36 data are compared with a reference population and analyzed with respect to the subgroups. RESULTS: The scores for each dimension of health were significantly reduced (p < 0.05) when compared with the reference population for all dimensions except pain. When compared with the patients' ratings of severity, a significant correlation was detected in five dimensions (p < 0.05). CONCLUSION: The SF-36 reflects the reduced health status of patients with epistaxis due to HHT. Changes in the SF-36 score could be used as an outcome measure in assessing efficacy of treatment of this condition.     

Management of epistaxis in hereditary hemorrhagic telangiectasia by Nd:YAG laser and quality of life assessment using the HR-QoL questionnaire

The purpose of this study was to describe the results of Nd:YAG laser application in hereditary hemorrhagic telangiectasia (HHT) patients and to measure the Health-Related Quality of Life (HR-QoL) in patients with HHT before and after Nd:YAG laser application in a prospective, clinical trial at a university hospital. Twenty-seven consecutive patients with HHT and mild to moderate degrees of epistaxis were followed-up for 2 years after Nd:YAG laser treatments. Recurrence of epistaxis after Nd:YAG laser application and measurement of HR-QoL using the International Quality of Life Assessment questionnaire, version 1.1 (IQOLA 1.1), was found. Eight patients (30%) received only one Nd:YAG laser treatment, 15 (56%) had a recurrence and received a second treatment and 4 (14%) had two recurrences and received three Nd:YAG laser treatments. HR-QoL was improved 2 years after the first Nd:YAG laser application in both its Physical Health Dimension (47.5±2.9 vs. 38.1±2.3 before treatment, P <0.05) and Mental Health Dimension (45.1±2.7 vs. 39.6±2.4 before treatment, P <0.05). Although no curative treatment for HHT exists, Nd:YAG laser treatment seems to constitute a simple and efficient method of epistaxis control, resulting in a significant improvement in quality of life.     

Natural history and control of epistaxis in a group of German patients with Rendu-Osler-Weber disease

INTRODUCTION: Epistaxis is the most common symptom of a complex, genetically determined vasculopathy, which is known under the notion hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber-syndrome). This study was initiated to gain more knowledge about the natural history of epistaxis in a German HHT-population. PATIENTS AND METHODS: Data of 49 HHT patients were ascertained by interviewing these patients with a standardized disease specific questionnaire. Patients' files were retrospectively reviewed for data concerning age, gender, past medical history, laboratory parameters, number of hospital admissions for epistaxis, conservative and operative types of therapy, treatment results and follow-up. MAIN RESULT: Epistaxis was the first and most prominent symptom in 93% of the patients and could be triggered most frequently by stress. Half of the patients had experienced first episodes of epistaxis in childhood, but usually epistaxis did not become troublesome before the age of 35 years. The effects of hormonal changes or therapies with systemic hormones were inconclusive with regard to impact on epistaxis. Patients with septal perforations had to be admitted for inpatient epistaxis treatment more frequently than patients with an intact nasal septum. An overall reduction of frequency and intensity of epistaxis could be achieved in 89% of the patients through the daily use of nasal lubricants and a minimum of two treatment sessions with the Nd:YAG laser. However in none of the cases the treatment results were permanent. More than 50% of the patients, who had been screened for visceral arteriovenous malformations, were positive for pathologic vascular lesions. PRINCIPAL CONCLUSION: The natural history of epistaxis in German HHT patients is similar to previously described entities from other parts of the world. First clinical signs of HHT may be present at an earlier age than previously thought.