Clinical 22-case study of hereditary hemorrhagic telangiectasia subjects undergoing nasal dermoplasty

Hereditary hemorrhagic telangiectasia (HHT)-a systemic autosomal dominant disorder adversely affecting skin, mucous membranes, and visceral blood vessels-typically shows epistaxis. In this pathological condition, even minor stimulation may cause bleeding and hemostasis is unlikely to occur. Subjects with HHT showing severe nasal bleeding require nasal dermoplasty (ND). We report our experience in 22 cases of HHT undergoing ND seen from January 2000 to November 2009. Average age at surgery was 55.8 years. Since April 2008, we modified ND, cut the harvested skin graft from the lateral thigh in halves and spread each strip on the anterior halves of the nasal cavity. Placement was circumferential in the sequence of agger nasi, nasal valve, septum, floor, and inferior turbinate. Before this technique was used, bleeding could not be controlled in 3 of 14 cases. Two of the 3 had histories of arterial embolization. Subjects undergoing the new technique have enjoyed improved quality of life.     

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.     

Clinical strategy in hereditary hemorrhagic telangiectasia

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a recurrent bleeding tendency caused by vascular malformations and preferentially involving the mucous membrane of the nose. The rhinological management of epistaxis is a challenge in which the frequency of bleeding has to be reduced without damage to the nasal mucosa, despite the fact that therapy necessarily has to be repeated. METHODS: The clinical course in 30 patients with HHT was monitored prospectively. Nasal mucosal efflorescences underwent Nd:YAG laser therapy at individually defined intervals, and the effect on the frequency and duration of bleeding was documented, as were adverse effects. RESULTS: No serious adverse effects (e.g., septal defects or synechiae) were observed as a consequence of therapy. During the course of laser therapy and ongoing compliance with nasal mucosal care instructions, the frequency of bleeding fell from "several times daily" to "every 2 weeks." CONCLUSION: In conjunction with Nd:YAG laser therapy, ongoing and consistent care of the nasal mucosa is a proven and effective treatment regimen in HHT. As an integral element in an interdisciplinary strategy for diagnosis and therapy, this regimen yields satisfactory quality of life while avoiding local complications.     

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.     

鼻内镜下蝶腭动脉电凝术治疗严重后段鼻出血

目的 评价鼻内镜下蝶腭动脉电凝术治疗严重后段鼻出血的疗效。方法 对25例后鼻孔填塞治疗无效的鼻出血住院患者,行鼻内镜下蝶腭动脉电凝术,其中24例全身麻醉,1例局部麻醉。25例中有2例患者同时行双侧蝶腭动脉电凝术。结果 术中所有蝶腭动脉均能明确识别,并在出蝶腭孔处将其成功电凝。22例术后未再出血;1例术后6h再次出血,经后鼻孔填塞2d后出血治愈;2例术后当天轻微鼻出血,药物治疗后停止。出院后随访3~6个月,患者均无再次鼻出血,除2例出现轻度鼻腔黏连外无其他并发症。结论 鼻内镜下蝶腭动脉电凝术治疗严重后段鼻出血是一种安全、有效的治疗方法。     

Argon plasma coagulation and topically applied estriol. Long-term results in the treatment of hereditary hemorrhagic telangiectasia of the nasal mucosa

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.     

Office treatment by cryotherapy for severe posterior nasal epistaxis--update

We have used cryotherapy as an office treatment for severe posterior nasal epistaxis for 15 years. In 1971 we reported a retrospective study on 42 consecutive patients with severe posterior nasal bleeding; 26 had cryotherapy and 16 had anterior and posterior nasal packing. Our data showed that cryotherapy controlled epistaxis with less morbidity. We have now used cryotherapy for posterior epistaxis in over 450 patients. In this presentation we review the history of cryotherapy, rationale for treatment with cryotherapy, experimental tissue changes in cryotherapy, and the method for freezing in cryotherapy. We then discuss 50 recent patients with severe posterior nasal bleeding whom we have treated with cryotherapy. We have found that cryotherapy is a successful way to manage patients with severe posterior nasal bleeding.     

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.     

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.     

Septectomy and septal dermoplasty for the treatment of severe transfusion-dependent epistaxis in patients with hereditary hemorrhagic telangiectasia and septal perforation

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.     

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.     

A new method of nasal dermoplasty (MW grafting method) in patients with large septal perforation

Nasal dermoplasty is effective in controlling epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). Skin graft take failure occurs mostly in cases of large septal perforation. The MW method was developed as a modification of nasal dermoplasty designed for patients with HHT having a large septal perforation. It seems to be a safe and effective approach and should be tried for recurrent bleeders with septal perforation.     

Selective embolization in the treatment of intractable epistaxis

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.     

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.     

Contact endoscopy of the nose in patients with Rendu-Osler-Weber syndrome

OBJECTIVE: Teleangiectases are the source of hemorrhage in many HHT patients. Most frequent site of bleeding is the nose and more than 90% of all individuals with HHT suffer from recurrent epistaxis. Despite all efforts, treatment of epistaxis in HHT continues to be a problem for many otorhinolaryngologists, who can alleviate recurrent nosebleeds by Septodermoplasty or laser therapy, but rarely can stop nasal hemorrhages permanently. Recurrence is almost inevitable, but the mechanisms of recurrence are not fully understood. METHODS: Prior to routine Nd:YAG laser therapy of nasal telangiectases the nasal mucosa of 17 patients with HHT according to the clinical diagnostic criteria of the HHT Foundation International was examined with a 0 degrees contact rhinoscope in areas with clinically visible telangiectases as well as in clinically normal mucosa. The digitally recorded images were compared to findings of a group of five healthy volunteers and the findings of five patients with polypoid sinusitis. RESULTS: Visualization of subepithelial vessels was feasible in all individuals of the study group as well as the control groups. Dilated vascular loops and tortuous vessels could be found in the study groups as well as in the control group, but the overall density of telangiectatic vessels was on an average higher in the HHT group. The process of vessel dilatation and tortuous configuration seemed to progress with age. CONCLUSION: Contact endoscopy allows the investigation of the angioarchitecture of capillaries of the nasal mucosa in vivo. This observation may be of significance for studies of nasal diseases, which are accompanied by epistaxis. With this regard it seems to be of special interest for studies of HHT.     

Use of a sprayed fibrin hemostatic sealant after laser therapy for hereditary hemorrhagic telangiectasia epistaxis

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a relatively common autosomal dominant condition. Epistaxis is a frequent manifestation, often occurring daily and requiring iron and blood transfusions. Surgery often is bloody and difficult. The aim of this study was to evaluate the effectiveness of a sprayed fibrin, hemostatic sealant in preventing postoperative epistaxis after laser treatment of nasal mucosa in HHT. Fibrin sealant was compared with nasal packing for likelihood of postoperative epistaxis and financial impact including material costs and hospitalization fees. METHODS: Retrospective review was performed of 64 individual laser treatments for epistaxis in HHT patients at the University of California, San Diego, Medical Center between 2002 and 2005. Nasal packing was used in 30 procedures and fibrin sealant was used in 34 procedures. RESULTS: Six of 30 (20%) procedures using postoperative nasal packing required admission with an average hospital expense of $5914. One of 34 patients (3%) in the fibrin sealant group required hospitalization (p = 0.04). CONCLUSION: Aerosolized fibrin sealant prevents postoperative epistaxis after nasal laser treatment in HHT patients. Compared with traditional nasal packing we found improved patient comfort and recovery with substantial cost savings.     

Osteonecrosis after intranasal injection with bevacizumab in treating hereditary hemorrhagic telangiectasia: A case report

Intranasal bevacizumab injections have been used in treating hereditary hemorrhagic telangiectasia (HHT)‐related epistaxis since 2009. It is believed to be a safe and effective treatment for a selected group of HHT patients in reducing frequency and intensity of epistaxis, with few or none adverse effects. In this case report, however, we will describe a patient who developed bilateral osteonecrosis in the knees while undergoing regular intranasal submucosal bevacizumab injections. Although osteonecrosis previously has been documented in patients receiving bevacizumab intravenously in oncologic doses, thus far it has not been reported in patients treated with intranasal submucosal injections. Laryngoscope, 128:593–596, 2018     

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.     

Early operative intervention versus conventional treatment in epistaxis: randomized prospective trial

OBJECTIVE: This prospective randomized trial was designed to compare intranasal endoscopic sphenopalatine artery ligation (ESAL) with conventional nasal packing in the treatment of recurrent epistaxis. METHODS: Patients were registered in the study databank following referral for epistaxis control to the otolaryngology service at the University of Alberta. All patients were initially packed using Merocel (Xomed Surgical Products, Jacksonville, FL) nasal dressings bilaterally. Patients were enrolled in the study following failure of Merocel packings. Informed consent was obtained in accordance with the Health Research Ethics Board. The patients were then managed with Vaseline nasal packs or ESAL. Patient demographics, treatment characteristics, number of hospitalization days, and rates of recurrence were recorded prospectively. The total cost of treatment for each patient was calculated. RESULTS: Nineteen patients were enrolled in the study. There was a significant reduction in cost and length of hospitalization of the patients undergoing ESAL compared with the conventional nasal packings. ESAL was also 89% effective in controlling the bleeding and had minimal sequelae or complications. The overall calculated cost of patients undergoing ESAL was dollars 5133 compared with dollars 12213 in the conservative group, resulting in an average saving of dollars 7080 per patient. There was overwhelming patient satisfaction with ESAL compared with nasal packings. CONCLUSION: ESAL is an excellent, well-tolerated, and cost-effective method of treating recurrent epistaxis.     

Epistaxis and its management: an observational pilot study carried out in 23 hospital centres in France

OBJECTIVE: The purpose of this study is to describe the treatment of epistaxis in hospital emergency departments and to identify the principal risk factors for more severe episodes of bleeding. STUDY PROTOCOL: Prospective cross-sectional epidemiological study MATERIAL AND METHODS: This study was carried out in 23 hospital centres in France, most of them teaching hospitals. Every patient presenting non-traumatic epistaxis or else associated with hereditary hemangioma during two consecutive and separate 24-hour periods were included. RESULTS: Fifty patients were included in the study. Nasal bleeding was stopped within 30 minutes for 47 patients. Fourteen patients were hospitalized. The risk factors for severe epistaxis included either copious bleeding or else bleeding for more than 6 hours or patients aged 65 and over. A history of repeated nasal packing and/or taking medication with a known hemorrhagic risk was associated with the amount and duration of bleeding (p < 0.05). CONCLUSION: Risk factors for severe epistaxis should be identified as to improve patient care and avoid treatment failure or useless hospitalization.