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.     

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.     

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.     

Surgical management of epistaxis in hereditary hemorrhagic telangiectasia

The most common manifestation of hereditary hemorrhagic telangiectasia (HH) is recurrent epistaxis. Numerous treatments have been used over the years, none proving entirely satisfactory. We have applied a new surgical treatment in seven selected cases of HHT with severe, life-threatening epistaxis. It consists of a near-total excision of the diseased nasal mucosa and its replacement by different skin flaps or a myocutaneous flap. This procedure has been performed since 1981. We stress the reliability and versatility of using the forehead myocutaneous island flap. Our original technique is described, follow-up of the patients is reported, and the criteria for selection of treatment are discussed.     

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.     

三维CT辅助下三线减张鼻中隔矫正术疗效观察

目的探讨三维CT辅助鼻内镜下三线减张鼻中隔矫正的手术技巧及其疗效观察。方法回顾性分析78例鼻中隔偏曲患者,全部行鼻内镜下三线减张鼻中隔矫正术,术前采用三维CT重建技术对鼻中隔偏曲部位进行评估,术中选择性地将鼻中隔方形软骨尾端、鼻中隔软骨与筛骨垂直板结合处、鼻中隔软骨下端与犁骨、上颌骨鼻嵴与腭骨鼻嵴结合处等部位形成的三条张力线分离,将应力区软骨和骨质按照事先设计方案进行切除,消除导致鼻中隔偏曲的应力,从而达到矫正鼻中隔的效果。结果所有患者术后均随访3个月以上,其中治愈57例,好转21例。结论鼻内镜下三线减张鼻中隔矫正术安全可靠,效果满意,最大限度保留了鼻中隔正常组织结构。术前利用三维CT重建技术对手术方式进行具有设计,具有手术精细,并发症少等优点,值得临床推广。     

The use of a nasal obturator in hereditary haemorrhagic telangiectasia: an alternative to Young's procedure

Epistaxis associated with hereditary haemorrhagic telangiectasia (HHT) is a challenging condition. Septodermoplasty, electrocautery and laser treatment often provide short-term relief only. The surgical closure of the nostrils ('Young's procedure') represents a longer term solution but has the disadvantage of causing permanent complete nasal obstruction. A Silastic obturator has the advantage of being a non-surgical intervention and allows temporary relief of the nasal obstruction.We present the cases of three patientswith HHT who used the obturator with good clinical effect, suggesting that this is a realistic non-surgical alternative to Young's procedure for patients with 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.     

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.     

Modified transnasal endoscopic medial maxillectomy with medial shift of preserved inferior turbinate and nasolacrimal duct

Although transnasal endoscopic medial maxillectomy (TEMM) is effective for the treatment of inverted papilloma (IP) in maxillary sinus (MS), it involves resection of the inferior turbinate (IT). TEMM also involves resection of the nasolacrimal duct (ND) in many cases to gain better access. Therefore, we developed a novel procedure in which the preserved IT and ND are shifted medially for a complete resection of IP in the MS. Incision was made in the mucosa of the lateral wall along the anterior margin of the IT. After removal of the medial maxillary wall except the ND and the lateral nasal mucosa, the anterior lateral mucosa of the nose, including the IT and the ND, was shifted in the medial direction to allow wider access to the MS. The tumor was removed together with the attachment through the anterior side of the ND. This modified TEMM was performed in 10 patients with IP. The IT and ND were preserved in all patients. We have not observed epiphora after this surgery. The advantages of the novel approach presented herein include: 1) preservation of the IT, ND, and lateral nasal mucosa; 2) wide access to the MS by shifting the IT, ND, and lateral nasal mucosa in the medial direction; and 3) direct access to the MS through anterior space of the ND, resulting in easier operation with a straight endoscope and instruments. This approach is a safe and effective method to obtain wide and straight access to the MS and to resect IP in the MS.     

Evaluation of calcium alginate nasal packing (Algostéril) versus Polyvinyl acetal (Merocel) for nasal packing after inferior turbinate resection

Nasal packing is commonly used to control postoperative bleeding in patients undergoing endonasal surgical procedures. Bleeding and pain on packing removal are frequently reported. The principal objective of this study was to investigate the efficacy and safety of Algosteril to control post-operative nasal bleeding. The secondary objective was to assess nasal bleeding and pain on packing removal, and to evaluate the endoscopic appearance of nasal mucosa 9 days after the procedure. This open, multicenter, randomized, controlled study was conducted on 50 patients undergoing partial bilateral inferior turbinectomy, packed with Algosteril on one side versus Polyvinyl acetal tampon (Merocel) on the other side following a left/right randomization. Both nasal packs effectively prevented post-operative bleeding. However, bleeding on packing removal was statistically less frequent and less severe with Algosteril than with Polyvinyl acetal (p = 0.016). In addition, pain was statistically lower with Algosteril than with Polyvinyl acetal (p = 0.0001). A trend to a better healing of the wound on day 9 was observed with Algosteril, leading us to further investigations. Algosteril nasal packing is as effective as Polyvinyl acetal in preventing postoperative bleeding following partial inferior turbinate resection. Its removal, however, is less traumatic for the nasal mucosa and less painful for the patient, therefore improving patient's comfort.     

Endoscopic resection of benign and malignant tumors in the nasal cavity and paranasal sinus

Endoscopic resection of nasal and paranasal sinus tumors is more aesthetic and less invasive than conventional resection, such as Luc's operation and lateral rhinotomy. We clarified the effect of radical endoscopic tumor excision and the control of local bleeding hazardous in endoscopic surgery. Subjects were patients with benign lesions in the nasal cavity, medial wall of the maxillary sinus, ethmoid sinus, and/or sphenoid sinus without concurrent malignant lesions. Although patients selection for malignant tumor excision was based on (1) possible en bloc resection, (2) low-grade malignant tumors, and (3) tumors in the nasal cavity and adjoining paranasal sinus, the final decision was made individual. Subjects were 23 patients with benign tumor (10 inverted papilloma, 9 hemangioma, 2 juvenile angiofibroma, and 2 other tumors) and 4 with malignant tumor (olfactory neuroblastoma, acinic cell carcinoma, squamous cell carcinoma, and chondroid chordoma) in the nasal and paranasal sinus. The tumor was resected en bloc except for patients with inverted papilloma (2 cases) and chondroid chordoma. Recurrence in benign tumors was zero during a mean observation of 21 months. One with chondroid chordoma, however, suffered a recurrent lesion 7 months after the initial operation. The lesion was successfully salvaged by a similar endoscopic procedure and subsequently treated with electron beam irradiation. Preoperative arterial embolization, laser coagulation, and ligation of the sphenopalatine artery were very useful in reducing blood loss during surgery and maintaining a clear endoscopic view. In intraoperative bleeding volume, less than 100 ml of bleeding occurred during surgery in 23 of 27 patients. The endoscopic excision of benign lesions in the nasal and paranasal sinus is thus as effective as conventional radical surgery. Endoscopic removal of malignant lesions remains controversial because of the small number of patients and short postoperative observation.     

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.     

Study on endoscopic sinus surgery management of chronic sinusitis with nasal polyps

Endoscopic sinus surgery is commonly used to treat chronic sinusitis. Subjects were 79 chronic sinusitis patients--50 men and 29 women aged 17 to 79 years (average: 50.6 years) undergoing endoscopic sinus surgery in our department from January 1993 to December 1997. Mean follow-up was 17.5 months. We evaluated preoperative staging of chronic sinusitis based on Kennedy staging. Most were stage 3. This type of staging was not effective in predicting nasal polyp relapse. We found that cases with diffuse polyposis and associated disease such as bronchial asthma or aspirin-induced asthma tended to experience a polyp relapse. Our results suggest that postoperative treatment is important in maintaining patency of the ostiomeatal complex, nasal polyp or edematous mucosa relapse must be treated early in on in occurrence.     

严重鼻衄治疗的相关问题研究

鼻衄是耳鼻咽喉科的常见疾病，治疗方法多样。报告63例严重鼻衄患者，引起贫血的48例（7．2％），鼻腔填塞2次以上的55例（873％），前后鼻孔填塞的34例（54．0％）。全部患者均行异内窥镜检查。其中在窥镜下重新止血的19例，行血管栓塞的2例。本文着重分析研究实际临床工作中鼻衄填塞治疗存在的问题，指出医源性鼻出血是严重鼻衄的原因之一。探讨鼻腔填塞、鼻内窥镜、血管栓塞及全身综合治疗在鼻衄治疗中的应用价值。     

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.     

Is oestrogen therapy justified in the treatment of hereditary haemorrhagic telangiectasia: a biochemical evaluation

Systemic and topical oestrogen can provoke squamous metaplasia of epithelium. In Hereditary Haemorrhagic Telangiectasia (HHT) the underlying telangiectasia may be protected from trauma and epistaxis reduced. Oestrogens have been advocated but their efficacy is unclear. Recent advances have now identified two oestrogen and one progesterone receptors. The aim of this study is to analyse the sex receptor status of HHT nasal mucosa to determine if oestrogen therapy is biochemically justified. Five HHT patients (three men, two women) and eight controls (four men, four women) underwent nasal mucosa biopsy. Samples were fixed in formalin and paraffin embedded. Alpha oestrogen (ERalpha) and beta oestrogen (ERss) and progesterone (PgR) receptors were identified using mouse monoclonal antibodies by the Streptavidin-biotin peroxidase method. ERss was detected in two HHT subjects (1 M: 1F) and two control subjects. ERalpha and PgR was absent in HHT subjects. This pilot study demonstrated that a subgroup of HHT patients were ERss positive. Oestrogen therapy therefore has a potential therapeutic role on a biochemical basis in these patients. ERss status should be determined before considering oestrogen therapy.     

难治性鼻出血患者出血部位各年龄段间的差异与特点

目的　探讨难治性鼻出血位点与年龄相关的分布特点及规律。方法　回顾性分析住院治疗的149例难治性鼻出血患者的临床资料,按年龄分为青年组（≤39岁）、中年组（40～59岁）、老年组（≥60岁）,分析其出血部位的特点及规律。结果　149例患者中,男性110例,女性39例;年龄 18～87岁,平均（53±14）岁;青年组36例,中年组52例,老年组61例。149例患者均行鼻内镜下鼻腔探查止血术。其中明确出血部位后行电凝止血或微填塞145例,并记录出血部位;未明确出血部位患者4例。三组患者最常见出血部位依次为：青年组：下鼻道穹窿22例（61%）,中鼻甲后段13例（36.1%）,嗅裂区中隔面1例（2.7%）;中年组：下鼻道穹窿23例（44.2%）,中鼻甲后段10例（19.2%）,嗅裂区中隔面17例（32.7%）;老年组：嗅裂区中隔面37例（60.6%）,中鼻甲后段15例（24.6%）,下鼻道穹窿7例（11.5%）。结论  难治性鼻出血患者中,青、中年患者出血部位以蝶腭动脉分支出血居多;老年患者出血部位以筛前动脉、筛后动脉分支出血居多。随年龄增长蝶腭动脉分支出血比例减少,筛前动脉、筛后动脉分支出血机会增加。     

鼻内窥镜下诊治难治性鼻出血68例分析

目的：探讨难治性鼻出血常见的出血部位及治疗方法。方法:回顾分析经内窥镜诊治的难治性鼻出血68例的临床资料，分析其出血部位及治疗效果。结果:出血部位在下鼻道顶部占27.9%(19/68)、嗅裂鼻中隔占20.7%(14/68)、中鼻道后上部占16.1%(11/68)、中鼻甲后端蝶腭动脉区和鼻中隔后端均为10.3%（7/68）、鼻咽顶占5.9%（4/68）、Woodruff静脉丛和部位不明均为4.4%(3/68)。1次治愈59例(86.7%)，2次9例(13.3%)，随访1～3个月均无复发。结论:应用鼻内窥镜检查鼻腔深部的出血并在镜下电凝或填塞止血安全有效。