良性对称性脂肪瘤病4例

目的 探讨良性对称性脂肪瘤病(BSL)的诊断和治疗.方法 采取手术切除的方法治疗,回顾性分析4例BSL患者的病历资料及诊治过程,结合文献复习讨论BSL诊治特点.结果 术后病理诊断均为无包膜脂肪组织.所有病例术后外形恢复满意,无1例出现并发症.结论 BSL是一种脂肪代谢障碍引起的脂肪组织弥漫性、对称性沉积于颈项部等皮下浅筋膜间隙和(或)深筋膜间隙的良性疾病.以中老年男性居多,与酗酒有关,可伴有高尿酸血症、高胆固醇血症等代谢性疾病,CT检查有助于本病的诊断.对于影响美观及功能的患者手术疗效理想.     

酗酒患者良性对称性脂肪瘤5例

目的 探讨酗酒患者良性对称性脂肪瘤病(Madelung's disease) 的临床特点,提高其诊断和治疗水平。方法 对5例患者在全身麻醉下行颈部脂肪瘤切除术,采用低位领口颈部皮肤大弧形切口,在保护颈部重要组织结构的前提下,尽量将脂肪组织完整切除。结果 患者术后外形及功能均恢复满意,无并发症,术后禁酒并随访3～5年未见复发。结论 Madelung 病多见于中年男性,病因可能与酗酒有关,手术是最有效的治疗方法,术后禁酒可有效防止复发。     

良性对称性脂肪瘤的治疗

良性对称性脂肪瘤（benign symmetric lipomatosis,BSL）是一种脂肪代谢障碍引起的脂肪组织弥漫性、对称性沉积于颈肩部皮下或深部软组织的良性疾病.我科自1993年以来收治20例,现报告如下.     

Madelung病的诊断及手术治疗

目的　探讨Madelung病的诊断、外科手术方法。方法　回顾性分析2011年1月～2015年11月之间我科收住并接受手术治疗的Madelung病患者的临床资料。6例患者均为男性,年龄35～65岁,均表现为颈部项增粗,CT或MRI检查表现为大量脂肪堆积,1例CT显示伴有喉累及。均有大量嗜酒史,其中2例合并心脏、肺部或脑部疾患,1例糖耐量异常。1例因呼吸困难急诊就诊,5例均有不同程度睡眠呼吸暂停低通气综合征临床表现,所有患者均无吞咽困难。结果　6例患者均接受手术治疗,其中4例行择期双颈部增生脂肪切除术及气管切开术,1例行择期双颈部增生脂肪组织切除术,1例因呼吸困难急诊行颈前增生脂肪切除+气管切开术,术中术后均无严重并发症出现。5例择期双侧颈部手术者术者及患者均对术后颈部外形满意,并均于术后10天内拔除气管套管,1例急诊手术患者因合并心脏、肺部及脑部疾病,再次手术风险大,未再行彻底颈部增生脂肪组织切除术,因肺部同期有感染,术后第3天转入呼吸科继续治疗,出院后长期带管。随访15 d～48个月,颈部外形均可,无明显复发。所有患者术后病理诊断均为颈部脂肪组织瘤样增生或脂肪瘤样改变。结论　Madelung病是一种脂肪代谢障碍引起的脂肪组织弥漫性、对称性沉积于颈肩部筋膜间隙的良性疾病,常发生于有嗜酒史的男性,发病部位有特征性,易合并其他内科疾病,手术治疗是以改善外形及功能为目的,手术切除效果良好,术中注意保护颈部重要神经血管,以免损伤。术中选取合适的解剖标志为参考有利于手术的顺利进行。     

Madelung病的诊断和治疗

目的探讨Madelung病的临床特点、诊断和治疗方法。方法回顾性分析7例Madelung病患者的临床资料，结合文献进行探讨。结果7例均以颈项部大量皮下脂肪堆积为主要表现。1例伴有中枢和外周神经系统异常，1例伴有糖耐量异常，1例伴有慢性肝病和高尿酸血症，此3例同时合并睡眠呼吸暂停低通气综合征。另有1例合并自主神经系统功能异常。6例男性患者有长期酗酒史。5例行增生脂肪彻底切除手术并戒酒，随访6个月～5年无复发。1例行2次增生脂肪部分切除术，术后均复发。所有术后病理为无包膜脂肪组织。1例拒绝治疗。结论Madelung病是一种以大量无包膜脂肪团块呈对称性聚积在颈项部皮下组织内为主要特征的疾病，病因可能与慢性酒精中毒有关，常同时合并有多种内科疾病。彻底切除增生脂肪以改善外观、解除局部压迫和术后戒酒可能是治疗该病的较好方法。     

伴有神经系统损害的多发性对称性脂肪瘤病1例

多发性对称性脂肪瘤病又称马德龙病(Madelung's disease),是一种罕见的脂肪代谢异常性疾病,常见的临床表现为脂肪组织弥漫性、对称性的沉积于皮下浅筋膜间隙及深筋膜间隙,合并神经系统损害者少见[1],我科遇到1例此类患者,现报道如下.     

良性对称性脂肪瘤病28例报告

目的:探讨良性对称性脂肪瘤病的临床特点,提高诊断和治疗水平。方法:对经治的28例良性对称性脂肪瘤病的临床资料,结合文献进行分析。28例均行手术治疗。结果:28例术后无一例出现手术并发症或死亡,功能和外形均恢复正常。随访2～15年,平均6年5个月,5例复发。结论:良性对称性脂肪瘤多见于中年男性,具有典型的颈部肿块弥漫性对称性分布和“驼峰背”样外形。病因可能与嗜酒有关;手术是最有效的治疗方法,但以美容整形为主。故不宜过于强调手术彻底性,以免损伤颈部重要结构。     

头颈部良性对称性脂肪过多症的临床误诊分析

良性对称性脂肪过多症(benign symmetrical lipomatosis,BSL)又称马德隆病(Madelung's dis-ease),可分为Ⅰ型和Ⅱ型两种亚型<'[1]>,该症主要表现为躯体和肢体对称性的脂肪过度增生,甚至可生长至巨大体积.由于两种亚型在临床均少见,经治医师大多将其误诊为单纯脂肪增生或脂肪瘤,从而误导了患者的进一步治疗.我科2009-10-2010-09共收治3例BSL患者,其中Ⅰ型2例,Ⅱ型1例,门诊均以"多发脂肪瘤"诊断收入院,具体诊疗过程如下.     

良性对称性脂肪瘤病16例报告

目的探讨良性对称性脂肪瘤病的临床特征、诊断及治疗方法。方法结合文献回顾分析16例良性对称性脂肪瘤病患者的临床资料。16例均行手术治疗。结果16例患者术后功能和外形均较满意，13例切口Ⅰ期愈合，3例切口Ⅱ期愈合。随访1—8年，平均3年4个月，1例复发。结论良性对称性脂肪瘤病多见于中年男性，常表现为弥漫性对称性分布的颈部、双肩部肿块，病因可能与嗜酒有关。手术切除是其主要治疗方法，但以美容整形为主。     

多发性对称性脂肪瘤病临床分析CSCD

目的总结多发性对称性脂肪瘤病(Madelung病)的临床资料、治疗方法和随访资料,对其发病机制、临床表现及治疗方法进行讨论。方法分析中山大学附属肿瘤医院头颈外科16年间诊治的Madelung病患者的临床资料、颈部的治疗方案和随访资料,对其进行回顾性分析。结果 41例患者中女性1例,男性40例;27例患者有长期酗酒史,14例患者无饮酒史;患者无肥胖体征,颈部为最常见的发病部位;40例患者行外科手术切除,术后外观恢复8例出现皮下脂肪液化、术后出血及切口感染等并发症;随访时间内9例患者复发,所有复发患者均有术前及术后饮酒史。结论 Madelung病为临床少见病,饮酒为独立的危险因素,颈部病变手术为主要治疗手段,分区、分次手术是安全有效的治疗手段,术后需配合戒酒而避免复发。     

Correlation between preoperative clinical diagnosis and histopathological findings in patients with rhinosinusitis

BACKGROUND: The diagnosis of chronic rhinosinusitis (CRS) or chronic rhinosinusitis with polyposis (CRSP) is typically clinical and based on the combination of medical history, physical examination, and imaging. The recommendation to perform surgery and the type of surgery is tailored to the diagnosis. The objective of this study is to determine the accuracy of preoperative clinical CRS or CRSP diagnosis in patients who underwent endoscopic sinus surgery. METHODS: This is a retrospective review of a case series of 380 consecutive patients who underwent endoscopic sinus surgery for the preoperative clinical diagnosis of CRS or bilateral CRSP. Data regarding symptoms, nasal endoscopy, computed tomography findings, preoperative diagnosis, and postoperative histopathology results were collected. RESULTS: The preoperative diagnoses were CRS (n = 180) and CRSP (n = 200). Two of the 180 patients (1.1%) with the preoperative diagnosis of CRS had a different postoperative histopathological diagnosis. The histopathology in both patients showed noncaseating granulomata, leading to a diagnosis of sarcoidosis. Nine of the 200 patients (4.5%) with the preoperative diagnosis of CRSP had a different postoperative histopathological diagnosis. Of these nine patients, five had inverted papilloma (bilaterally in one instance); one had adenocarcinoma; one had squamous-cell carcinoma; one had chronic invasive granulomatous fungal sinusitis; and one had sinonasal sarcoidosis. CONCLUSION: The preoperative clinical diagnosis can be inaccurate in patients with CRS and bilateral CRSP. The misdiagnosis is more common in CRSP, including patients with recurrent polyposis.     

Otologic manifestations of relapsing polychondritis. Review of literature and report of nine cases

OBJECTIVE: Relapsing polychondritis (RP) is an episodic disease most likely of autoimmune etiology, characterized by recurrent inflammation of cartilaginous structures. METHODS: Retrospective case study at two tertiary referral centers with presentation of nine patients with otologic involvement of RP, review of the spectrum of otologic disorders seen, and treatment. RESULTS: The clinical course of otologic manifestations of RP was highly variable and ranged from mild to moderate. In 6/9 patients there was an association with other autoimmune disorders. In addition to recurrent auricular chondritis, which was present in 8/9 patients, our patients had otitis externa, chronic myringitis, Eustachian tube dysfunction, conductive hearing loss, sensorineural hearing loss, and tinnitus. All patients had their diagnosis of RP made on the basis of their otologic involvement and the response to systemic corticosteroids. CONCLUSION: The diagnosis of RP is primarily clinical, but laboratory studies and biopsy may contribute as well. Once the diagnosis is suspected, the otolaryngologist should consider consultation with a rheumatologist to assist in the management of additional systemic manifestations.     

Serology in facial paralysis caused by clinically presumed herpes zoster infection

Summary In some cases of peripheral facial palsy due to a clinically suspected varicella zoster virus (VZV) infection, the clinical diagnosis is not supported by serological tests. In a retrospective study, we examined the sera from 63 patients with clinical findings compatible with VZV infection: 57 had paired sera and 6 had single sera. In the paired-sera group, 18 cases were serologically negative initially by complement fixation (CF), while 9 of these cases were actually positive for VZV when CF was combined with ELISA. Moreover, evidence for a recent mumps virus infection was found in 6 patients and 1 patient was diagnosed as having recent mumps and cytomegalo-virus (CMV) infections. In the 6 single sera studied, the ELISA was suggestive of a recent infection with VZV (4 cases) and CMV (1 case).     

Serology in facial paralysis caused by clinically presumed herpes zoster infection

In some cases of peripheral facial palsy due to a clinically suspected varicella zoster virus (VZV) infection, the clinical diagnosis is not supported by serological tests. In a retrospective study, we examined the sera from 63 patients with clinical findings compatible with VZV infection: 57 had paired sera and 6 had single sera. In the paired-sera group, 18 cases were serologically negative initially by complement fixation (CF), while 9 of these cases were actually positive for VZV when CF was combined with ELISA. Moreover, evidence for a recent mumps virus infection was found in 6 patients and 1 patient was diagnosed as having recent mumps and cytomegalo-virus (CMV) infections. In the 6 single sera studied, the ELISA was suggestive of a recent infection with VZV (4 cases) and CMV (1 case).     

Laryngeal manifestations of paracoccidioidomycosis (South American blastomycosis)

OBJECTIVE: To report clinical manifestations, diagnosis, and epidemiologic characteristics of laryngeal paracoccidioidomycosis. DESIGN: Case series. SETTINGS: Tertiary care institutional hospital. PATIENTS: We reviewed the hospital records of 7 patients with laryngeal paracoccidioidomycosis diagnosed by histopathological examination. MAIN OUTCOME MEASURE: Clinical manifestations of laryngeal paracoccidioidomycosis. RESULTS: All patients were men and were middle-aged (range, 43-65 years), and most (86% [6/7]) were farm workers. All 7 patients regularly used tobacco, but only (43% [3/7]) were alcohol users. Clinical manifestations were dysphonia (86% [6/7]), dyspnea (71% [5/7]), dysphagia (43% [3/7]), and cough (29% [2/7]). Laryngeal examination revealed ulcerative lesions with a mulberrylike appearance in 3 patients and vegetative lesions in 4 patients. Many had multiple laryngeal lesions with involvement of the true and false vocal cords, the epiglottis, and the arytenoid and interarytenoid areas. The first diagnostic impression was carcinoma in all patients. CONCLUSIONS: Laryngeal paracoccidioidomycosis may be a difficult diagnosis for the unsuspecting clinician to make. Examination of the larynx can reveal lesions similar to laryngeal cancer; therefore, diagnosis of carcinoma must be ruled out by histopathological examination or culture of a specimen.     

When to suspect a perilymphatic fistula?

INTRODUCTION: The diagnosis of perilymphatic fistula (PLF) is difficult since no single clinical situation gives the diagnosis for sure. The goal of this study is to clarify the clinical situations where you must suspect a PLF. METHODS: Retrospective study of 20 patients that had an exploratory tympanotomy with a PLF confirmed peroperatively. An analysis of the symptoms, signs and complementary exams was done. The surgical findings and the postoperative evolution were noted. RESULTS: 100% of patients reported a hearing loss, 80% vertigo, 70% a tinnitus and 35% equilibrium problems. Every patient had an etiological event to explain the PLF (trauma 85%), stapedotomy (10%), other ear surgeries. Five patients had a positive fistula or Vasalva test. All patients except one had an hearing loss on the audiogram (sensorineural, mixte or conductive). 50% had a CT scan, 70% of which were abnormal. A VNG was done on 3 patients. The sites of the PLF were as follows: 90% oval window, 5% round window and 5% both windows. The hearing got better or was stabilised in 95% of patients after the operation. 64% saw an improvement of their tinnitus and 87% of their vertigo. CONCLUSION: The diagnosis of PLF is difficult and a high index of suspicion is mandatory. One must look for an etiologic situation to explain the PLF. The audiogram is almost always modified, a mixte hearing loss being common due to the high incidence of ossicular trauma associated with PLF. The clinical clinical situations where you must suspect a PLF were identified as follows: An old trauma, a recent trauma, a history of otologic surgery particularly on the stapes and a preexisting hearing loss that aggravates. A diagnosis scale to evaluate the risk of PLF, based on clinical situations, physical exam and complementary exams was done to help the clinician in the evaluation of PLF.     

The contribution of flexible endoscopy for diagnosis of acute bacterial rhinosinusitis

This prospective controlled study ascertained the contribution of flexible endoscopy (FE) for diagnosis of acute bacterial rhinosinusitis (ABRS) in a group of consecutive adult patients who had symptoms suggestive of ABRS and in whom sinus radiography (SRG) and FE were accomplished. We adopted for analysis the 2007 updated European guidelines on rhinosinusitis and computed the sensitivity and specificity of FE against SRG. Positive diagnosis was entertained when FE showed purulent material within sinus drainage area and/or SRG demonstrated air–fluid level, complete opacification, or at least 6 mm mucosal thickening. Of a total of 179 patients initially included in this study, 104 had clinical criteria compatible with guidelines for ABRS. Of them, 43 (41.3%) had positive FE and SRG, 17 (16.3%) had positive FE and negative SRG, and vice versa in 9 (8.7%); both modalities were negative in 35 (33.7%). FE yielded sensitivity of 82.7 ± 5.24% (95% CI: 72.41–92.97%) and specificity of 67.3 ± 6.50% (95% CI: 54.56–80.06%). Age, gender, symptom duration, pre-referral antibiotics, and treatment by primary/secondary physician were not associated with positive or negative diagnosis of ABRS. Of 75 patients who were excluded from the analysis, 33 (44%) had positive diagnosis of ABRS established by FE and/or SRG. The finding that clinical criteria had moderate predictive value (66.3%) highlights the need for objective measures for diagnosis of ABRS. In absence of feasible gold standard and considering that guidelines do not recommend SRG for routine diagnosis, FE serves as an indispensable ancillary tool for establishing ABRS.     

Wegener肉芽肿病的耳部表现

目的探讨Wegener肉芽肿病耳部表现的临床特征，组织病理学和实验室检测特点，做到早诊断和早治疗。方法回顾性分析14例Wegener肉芽肿病耳部表现的临床资料。结果14例中包括慢性化脓性中耳炎10例，其中合并周围性面神经麻痹2例；分泌性中耳炎3例；感音神经性聋1例。14例中3例慢性化脓性中耳炎，1例分泌性中耳炎和1例周围性面神经麻痹是Wegener肉芽肿病的首诊表现，开始表现为耳部单独受累，余9例开始即有多器官受累表现，全部病例均呈现多系统多器官受累。14例组织病理学诊断均符合Wegener肉芽肿病的特点。13例抗中性粒细胞胞质抗体阳性，14例有红细胞沉降率升高，13例C反应蛋白升高。所有病例经糖皮质激素和环磷酰胺治疗后病情均得到控制。11例随访时间3年以上，病情稳定无复发。结论Wegener肉芽肿病的耳部表现常缺乏特异性，早期诊断有赖于临床表现、组织病理学检查和抗中性粒细胞胞质抗体的及时检测。规范使用糖皮质激素和环磷酰胺可以有效地控制病情。     

Esthesioneuroblastoma: reflections of a 21-year experience.

OBJECTIVES: To evaluate the results of standardized treatment of esthesioneuroblastoma at a single institution during a 21-year period and calculate pertinent parameters, i.e., metastatic disease (local, regional, distant), disease-free survival, and complications of treatment. STUDY DESIGN: A retrospective review was conducted of all patients treated at a single institution from September 1976 through May 1998. METHODS: Only those patients who received their complete evaluation and treatment at our institution were included in this analysis. Thirty-five patients met this criterion. In addition, results of epidemiological, pathological, and molecular analyses were evaluated to seek accurate indicators for clinical outcomes. RESULTS: Six percent of patients presented with cervical metastatic disease, but ultimately 25.7% developed at least one episode of cervical metastases; 14.3% of patients developed a local recurrence an average of 6 years after diagnosis; and 37% of the patients ultimately developed at least one episode of metastatic disease. The disease-free survival for this cohort of 35 patients was 80.4% at 8 years. CNS complications occurred in 25.7% of the patients, 22.9% had orbital complications, 20% had systemic posttreatment problems, 18.2% had chemotoxic sequelae, 8.6% had infectious complications, and 14.3% had cosmetic sequelae. No epidemiological, pathological, or molecular factors appeared to be more accurate clinical indicators than the Kadish staging system. CONCLUSIONS: This series of esthesioneuroblastoma patients (N=35) reflects an 8-year disease-free survival of 80.4%, representing a significant number of patients treated and followed at one institution for an extended period of time. No valuable pathological or molecular indicators to predict aggressive clinical behavior were found. The average time interval before recurrent disease developed was more than 6 years, far greater than that expected for other sinonasal malignancies. Therefore, extended follow-up is necessary for this patient group.