类脂质蛋白沉积症1例并文献复习

1临床资料患者,女,32岁。因“自幼持续性声音嘶哑,加重1年”就诊于我院。患者出生时哭声弱,学语时出现声音嘶哑,发音费力,无构音障碍,无口腔溃疡,无癫痫发作,无脱发、无睫毛脱落,感冒时声音嘶哑加重,可出现失声。5岁出现双侧上眼睑淡黄色串珠样丘疹,无舌体变硬,口唇变厚。其父母体健,否认父母近亲结婚,家族中无类似患者。喉镜检查示鼻咽部可见淡黄色物沉积,双声带全长表面可见淡黄色沉积,延及声带下缘,黏膜波消失,双声带活动好（图1A）;软腭、舌系带、下唇处散在淡黄色沉积物（图1B,图1C）。皮肤：双侧上眼睑淡黄色串珠样丘疹（图2A）;肘、膝局部色素沉着（图2B）。全麻支撑喉镜下双侧声带肿物切除术。病理结果：双声带鳞状上皮黏膜组织,其内可见粉染均质物沉积,符合类脂质蛋白沉积症（刚果红-）（图3）。术后随访1年,声音嘶哑改善,喉镜见声带表面沉积物质基本消失（图4）。     

声带竹节病的临床研究

目的 探讨声带竹节病的临床特征、病理及治疗方法.方法 对2例声带竹节病患者的喉镜、病理和治疗等临床资料进行回顾性分析.结果 2例声嘶患者分别患有系统性红斑狼疮和未分化结缔组织综合征,声带病变表现为双侧声带中部黏膜下脂黄色条带状沉积物,激素、休声等治疗声嘶无改善.1例采用病变切除术,术后声音质量明显改善.声带切除物常规病理检查,可见Reinke间隙炎性细胞浸润,小血管增生及血管炎,结缔组织间隙可见嗜酸性均质物质沉积;免疫荧光检查证实患者声带固有层有IgA、C1q和IgM沉积.结论 声带竹节病为自身免疫病患者喉部特征性表现,病理改变为免疫复合物在声带固有层的沉积,外科干预可用于类固醇药物无效或病变导致声带闭合不全的情况.     

嗓音显微外科技术的临床应用

目的　对于喉部病变 ,在得到有效治疗的同时 ,最大限度地保留正常和高质量的发声功能。方法　 1986～ 2 0 0 0年中日友好医院耳鼻咽喉科应用嗓音显微外科技术治疗 115 3例喉部疾病。根据喉部各种良性病变 (声带小结、声带息肉、Reinke水肿、声带囊肿等 )和不同阶段早期恶性病变及声带癌前病变侵犯的组织学深度 ,分别采用显微切除技术、外侧微瓣技术、内侧微瓣技术、黏膜下注射技术、黏膜剥脱及激光切除术等 ,切除病变组织 ,最小伤及正常组织和对发音质量的影响。结果10 4 4例 (99 8% )声带小结、声带息肉、声带囊肿和 12例Reinke水肿在通过显微切除技术、外侧微瓣技术、内侧微瓣技术等治疗后均能在 1周后获得正常发音 ,2例巨大声带息肉患者半年内声嘶改善不满意 ;2 0例Reinke水肿、31例癌前病变经黏膜剥脱及黏膜表皮剥脱处理 ,除 3例癌前病变术后复发又经 2、3次手术 ,其余均在 1～ 3个月内恢复正常发音。 34例声带癌经黏膜剥脱处理 ,5例声带癌和 5例喉乳头状瘤经激光切除 ,术后 5年以上随访 5例声带癌复发 ,其中 2例再次行黏膜剥脱术 ,1例乳头状瘤反复 4、5次手术。治疗后的发音效果均比经颈部切除手术好。结论　嗓音显微外科技术是彻底治疗喉部疾病同时可以最大限度保留声带发声功能的重要手术方法     

丝裂霉素在喉部手术中的应用

目的:探讨丝裂霉素在喉部手术中的应用.方法:回顾性分析48例涉及双侧声带前1/3和(或)前连合的声带病变患者的临床资料,其中30例患者的治疗采用支撑喉镜下显微手术联合外用丝裂霉素,其余18例患者仅采用支撑喉镜下显微手术.结果:手术联合丝裂霉素组的30例患者中,除2例患者术后出现前连合轻微粘连外,其余均未发生声带粘连;单纯手术组的18例患者中,有6例患者术后前连合有粘连.结论:丝裂霉素可有效预防喉部手术后的声带粘连.     

喉显微手术及中西医结合治疗喉癌前病变临床观察

目的对比观察喉癌前病变显微手术及中西医结合疗法的疗效. 方法 33例喉癌前病变(声带白斑18例,声带角化症15例)随机分为中西医结合组(声带白斑10例,声带角化症7例)和西医对照组(声带白斑8例,声带角化症8例).对照组治疗方法为喉显微手术及术后使用抗生素;中西医结合治疗组为手术治疗加术后口服自拟中药汤剂. 结果中西医结合治疗组病变复发率为0%(0/17),单纯西医治疗对照组病变复发率为25.0%(4/16),差异有显著性意义(P＜0.05);治疗组喉显微术后声带黏膜及发音功能恢复正常时间平均为15.87±4.93天,对照组为26.56±13.08天,差异有显著性意义(P＜0.01). 结论中西医结合治疗喉癌前病变不仅创伤较小,且术后声带结构及功能恢复较快、原病变不易复发.     

甲状腺功能减退症致声音嘶哑误诊分析

甲状腺功能减退症(简称甲减)常引起内分泌系统症状,但累及喉部者较少见,特别是在早期,导致声音嘶哑现象较少.自2007年9月至2011年9月我科收治因甲减致声音嘶哑出现误诊3例,现报告如下. 例1 女,62岁.声音嘶哑2周于2007年9月13日入院.颜面部轻度浮肿,无发热、咳嗽、呼吸困难、吞咽困难、胸闷、怕冷、易疲劳等症状.既往史无特殊.入院时查体:血压85/55 mm Hg(1 mm Hg=0.133 kPa),脉搏55次/min,神志清楚,颜面部及眼睑皮肤轻度水肿;咽部慢性充血,双侧声带慢性充血,肥厚明显,右侧声带前中段见淡红色新生物,运动尚可,闭合欠佳(图1a).入院诊断:声带息肉(右),慢性肥厚性喉炎.行术前准备,血常规、尿常规、肝肾功能、血糖、血脂无异常.于2007年9月16日全身麻醉下行支撑喉镜下声带肿物切除术,术后采用庆大霉素及地塞米松雾化吸入,病理结果回报:声带息肉(病理改变与其他患者的息肉无明显区别).     

CO2激光喉显微外科技术治疗喉良恶性病变

目的回顾分析应用CO2激光喉显微外科技术治疗喉部良恶性病变的效果。方法自1999年1月1日至2003年6月30日，应用CO2激光喉显微外科技术治疗喉部良恶性病变313例，其中采用切割或汽化方法切除声带息肉236例，声带小结30例；采用声带黏膜剥脱术切除声带白斑、声带角化症、声带不典型增生27例；应用声带黏膜剥脱术或声带切除术切除声门型喉癌(T1aN0M0％)20例。结果所有患者均一次切除病变，术中出血少或不出血，术野清楚。术后无一例出现呼吸困难、出血等严重并发症。声带息肉和声带小结患者的有效率达100％；声带白斑、角化症、不典型增生患者的有效率为92．6％；声门型喉癌患者半年复发率10％。结论应用CO2激光进行喉显微外科手术术中出血少、术野清楚，有效率高，拓展了手术的适应证和范围，值得推广应用。     

电动切削及射频治疗在喉显微手术中的应用

支撑喉镜下将喉内镜连接电视显像录像、打印、电动切削系统 ,结合射频治疗仪用于喉部微创手术 ,是近年来喉手术方式的一大进步。我院自2 0 0 0年底以来对 16 0例喉部病变进行手术 ,取得了满意效果。现报告如下。1　资料和方法1.1　一般资料16 0例中 ,男 12 0例 ,女 4 0例 ;年龄 5～ 5 4岁。声音嘶哑 2个月～ 5年。病变部位 :声带前 1/3段97例 ,前连合 34例 ,占全声带 2 9例 ;其中 82例为双侧声带病变。病理诊断 :声带息肉 96例 ,声带囊肿 9例 ,任克水肿 11例 ,喉乳头状瘤 17例 ,原位癌6例 ,喉黏膜角化症 2 1例。 4例喉乳头状瘤患者曾行喉裂…     

频闪喉镜检查对声带白斑激光手术的价值

目的 探讨声带白斑手术前频闪喉镜检查与CO2激光手术切除范围及手术治疗效果的相关性.方法 1999～2002年72例在我科行CO2激光手术的声带白斑或声带角化患者,手术前进行频闪喉镜检查,观察病变范围及病变侧黏膜波的改变,采用不同的手术切除范围,术后随访3年以上.分析不同组织病理学结果 与黏膜波改变之间的差异.结果 72例声带白斑患者中,黏膜波正常者24例(33.3%),患侧黏膜波轻度减低者25例(34.7%),中度减低者10例(13.9%),重度减低者13例(18.1%).术后病理结果 ,51.4%(37/72)为角化不伴有不典型增生,19.4%(14/72)为角化伴有轻度不典型增生,12.5%(9/72)为角化伴有中度不典型增生,16.7%(12/72)为角化伴有重度不典型增生,其中3例可疑癌变.根据频闪喉镜检查结果采用不同的手术切除范围,72例患者一次治愈率为88.9%(64/72),癌变率为6.94%(5/72).结论 频闪喉镜检查可以作为声带白斑的术前评估手段,结合CO2激光手术对声带白斑进行早期干预治疗,降低癌变率,提高治愈率.     

喉部血管肉瘤一例

本文报道了我科收治的1例喉部血管肉瘤病例。患者老年男性,以声音嘶哑3年余入院,入院初步诊断为喉部新生物,术前病理提示:右侧声带考虑假肉瘤性改变。在全麻下经支撑喉镜CO2激光声带新生物切除术,术后病理示声带血管肉瘤,再次在全麻下经支撑喉镜CO2激光喉部分切除术,密切随访16个月无复发。     

Lipoid proteinosis (Urbach-Wiethe disease): a case report from India

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal-recessive anomaly that primarily affects the skin and the mucosa of the upper aerodigestive tract in children. It is caused by hyaline deposits in tissues. Hoarseness secondary to laryngeal involvement is frequently the first presenting feature. It is important to consider this disease in the differential diagnosis of hoarseness because it might lead to life-threatening airway compromise. We report a case of lipoid proteinosis in a 12-year-old girl who presented with hoarseness and skin lesions.     

Lipoid proteinosis of the larynx

Lipoid proteinosis is a rare disease that presents with hyaline deposits in many tissues. It involves predominantly the skin and upper aerodigestive tract, presenting with small yellowish papules and hoarseness. It may involve the central nervous system and cause intracerebral calcifications. Laryngeal lesions may resemble singer's nodule or chronic laryngitis. The pathogenesis of the disease is not clear although several studies suggest a defective collagen production and/or lysosomal storage disease. In this article two cases with skin and larynx involvement are reported.     

Lipoid proteinosis of larynx: review of four cases

Lipoid proteinosis is a rare autosomal recessive disorder characterized by intercellular deposition of an amorphous hyaline material. It mainly involves skin and mucosal membranes of upper aerodigestive tract as well as central nervous system, lung, lymph nodes and striated muscles. Etiology and pathogenesis are unknown. Infantile hoarseness is a common presenting feature of the disease due to infiltration of larynx. In two-thirds of the cases, voice changes are present at birth or in early infancy as the first manifestation. We present four patients with lipoid proteinosis involving skin, oropharynx and larynx.     

Radiological findings in lipoid proteinosis

The radiological findings in six patients suffering from lipoid proteinosis have been described and discussed. Laryngeal examination showed thickened epiglottis, swollen arytenoids and aryepiglottic folds, thickened irregular false and true vocal cords, and increased stiffness of the vocal cords. The ventricles and subglottic region were free. Bean-shaped suprasellar calcifications were found in two cases who were free of neurological symptoms. The chest and upper gastro-intestinal tract were radiologically free.     

Helicobacter pylori colonization in the larynges of patients with hoarseness

OBJECTIVES: Vocal nodules and polyps are two common noninfectious causes of hoarseness. Patients with persistent hoarseness often require microscopic laryngeal surgery to excise mass lesions of the larynx despite extensive voice resting and modification of voice use behavior. Helicobacter pylori has recently been reported to present in the upper aerodigestive tract. This study applies the rapid urease test to determine the colonization of Helicobacter pylori in surgical specimens of patients with vocal nodules and polyps. METHODS: In this prospective study, 53 consecutive patients with vocal nodules (n = 20) or vocal polyps (n = 33) were investigated from November 2004 to July 2005. Microscopic laryngeal surgery was performed in all cases. Tissue specimens harvested from the larynx were analyzed using the rapid urease test. RESULTS: The study population consisted of 33 females and 20 males with a mean age of 43.1 +/- 9.9 years. Thirteen (24.5%) of the 53 patients revealed Helicobacter pylori colonization, and all were histopathologically diagnosed with vocal polyps. The difference in incidence of Helicobacter pylori colonization between vocal nodules and vocal polyps was statistically significant (0% [0/20] vs. 39.4% [13/33], P = .001). CONCLUSIONS: Helicobacter pylori often colonizes in the larynxes of patients with vocal polyps. These results indicate the involvement of Helicobacter pylori in vocal polyps. However, the presence of Helicobacter pylori as an etiologic factor in vocal polyps remains inconclusive.     

Laryngeal lichen planus mimicking vocal fold dysplasia

Lichen planus (LP) is an immune-mediated chronic inflammatory disease affecting the skin and mucosal membranes, either alone or in combination. LP rarely occurs in the larynx, where it presents as leukoplakia. Additionally, due to its responsiveness to corticosteroid drugs, differential diagnosis from other causes of laryngeal leukoplakia is needed (e.g., dysplasia and squamous cell carcinoma [SCC]). A 71 years old male smoker presented with progressive hoarseness and cough without fever. Vocal folds’ motility was preserved. At fiberoptic laryngoscopy, he presented with bilateral cordal erythema and leukoplakia of the right true vocal fold. A sub-epithelial CO2 laser cordectomy was performed. Histological findings were consistent with mucosal LP. After surgical excision, the patient experienced symptomatic relief without medical treatment. A relapsing lesion became clinically evident six months later. A pinch biopsy was performed, and the histological examination confirmed the presence of laryngeal-LP. Hence, the patient underwent a 10-week corticosteroid-based treatment. After that, and during the subsequent 12 months of follow-up, the patient showed no signs of recurrence. LP is an exceedingly rare cause of leukoplakia in the larynx. Histological evaluation plays a key role in the diagnosis, in order to establish a proper therapeutic approach. Since LP is responsive to corticosteroid drugs and untreated disease tends to relapse, the medical treatment is advisable in such instances.     

Schwannoma laríngeo. Un tumor de laringe poco común

Schwannomas are benign tumours, rare among tumours of the larynx. They normally present as supraglottic masses (because they may arise from the internal branch of the superior laryngeal nerve), most commonly involving aryepiglottic folds or false vocal folds. Most patients present with a globus sensation, dysphagia or hoarseness. Conservative surgery is the treatment of choice. We report a case of a laryngeal asymptomatic neuroma that was diagnosed accidentally in an imaging test. Complete excision of the tumour was performed through a transoral CO₂ laser microsurgery without resorting to a tracheotomy. We discuss the clinical, pathologic and imaging findings and the management of this neoplasm. We also try to update the knowledge on the management of these tumours.     

Amyloidosis of the larynx

Amyloidosis of the larynx is a rare disease, accounting for less than 1% of all benign laryngeal 'tumours'. Three cases of this type of lesion are reported--one of the vocal cord, one of the false vocal cord and one of the subglottis and trachea. In 2 of the patients the amyloidosis was localized, while the third was later found also to have an epipharyngeal solitary plasmacytoma with amyloid deposits and in addition amyloidosis of the nasal cavity. However, the amyloidosis in this patient may still be regarded as being localized, as the clinical examination and laboratory tests afforded no evidence of generalized disease. Amyloidosis of the larynx may be manifested as a localized tumour or as a diffuse infiltration. The symptoms and signs will, of course, depend on the site of the amyloid deposit. When the vocal cords are involved hoarseness may result, and this was the most prominent sign in the present cases. The treatment of laryngeal amyloidosis is primarily by endoscopic excision. Amyloid substance has specific staining properties. The Congo red reaction with a green birefringence in polarized light and Phorwhite BBU using fluorescence microscopy are regarded as the most reliable staining reactions. Electron microscopy has revealed a typical fibrillar structure of amyloid.     

Solitary Extramedullary Plasmacytoma of the Apex of Arytenoid: Endoscopic, CT, and Pathologic Findings

Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that occurs mainly in the soft tissues of head and neck region, with the paranasal sinuses, nasal cavity and nasopharynx being the most common sites. Solitary EMP of the larynx is very rare but increasingly reported recently. Common sites of involvement in larynx in the order of frequency are the epiglottis, ventricles, vocal folds and ventricular folds. We report an extremely rare case of solitary EMP involving in the apex of arytenoids that was successfully treated by only surgical excision. Because solitary EMP of the apex of artytenoids is extremely rare, it should be included in the differential diagnosis for laryngeal mass. Also, solitary, small, pedunculated and localized EMP of the larynx could be completely removed by laryngeal microsurgery.