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。     

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。     

10例马德龙病Ⅰ型临床分析并文献复习

目的探讨马德龙病（Madelung’s disease）的临床特点及诊断和治疗方法，减少该病的误诊误治。方法收集2016年12月—2018年6月笔者所在医院及外出会诊治疗的10例经外科手术治疗的马德龙病Ⅰ型患者的临床资料并结合文献进行复习。10例患者均为男性，年龄42~57岁，平均51岁。病变集中在颈项部、枕部，可伴有呼吸困难、吞咽不适、上臂疼痛、声音嘶哑及偏头困难等症状。所有患者均有长期饮酒史。结果所有患者均采取脂肪切除术，术后随访6个月至2年，随访期间无1例复发。结论马德龙病主要发生在长期饮酒史的中年男性，该病多伴有肝功能异常，手术治疗是马德龙病目前最有效的治疗方法，病变范围较大患者需采用分次手术治疗。     

多发性对称性脂肪瘤病临床分析

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。     

Madelung病伴阴囊脂肪堆积1例并文献复习

目的 探讨Madelung病的临床特点及诊断和治疗方法。方法 回顾性分析1例经外科手术治疗的Madelung病伴阴囊脂肪堆积患者的临床资料,并结合相关文献进行分析。结果 采取外科手术切除患者脂肪组织,术后随访半年,随访期间无复发。结论 Madelung病主要发生在有长期饮酒史的中年男性,其特点是存在进展性、过度和多发性脂肪瘤,呈对称分布,查体时要注意患者其他部位的异常脂肪团块蓄积,并及时联系相应科室干预;为了避免复发,外科手术切除是目前首选的治疗方法,手术的主要目的是改变患者外观形象并改善功能障碍,伴阴囊脂肪堆积的患者应避免出现隐匿阴茎综合征、性功能障碍或睾丸萎缩等并发症。     

良性对称性脂肪瘤病的诊断和治疗(附6例报告)

目的:探讨良性对称性脂肪瘤病(BSL)的诊断和治疗。方法:结合我院收治的6例BSL患者,回顾性分析BSL的临床症状、体征、诊断及治疗,并结合文献进行探讨。结果:6例均以颈项部大量皮下脂肪堆积为主要表现,其中1例伴有双侧上臂近肘窝处皮下脂肪堆积呈对称性隆起。6例患者伴左心舒张功能改变、糖耐量减低、阻塞性睡眠呼吸暂停低通气综合征、Ⅱ型糖尿病及高尿酸血症中的1项或多项,其中1例患者同时出现上述症状,除1例女性患者未见肝功能改变外其余5例男性患者均出现。5例男性患者均有长期酗酒史。除1例拒绝治疗外,其余5例行彻底切除术及戒酒,术后随访3个月～4年,1例术后3年后复发,未行再次治疗,其余4例无复发。所有患者术后病理诊断为无包膜脂肪组织。结论:BSL是一种脂肪代谢障碍引起的脂肪组织弥漫性、对称性沉积于颈肩部皮下浅筋膜间隙和(或)深筋膜间隙的良性疾病。患者以中年男性居多,长期的酗酒史及典型的临床表现对于该病的诊断有一定帮助,对于影响美观及功能的患者手术疗效较理想。     

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.     

鼻咽部淀粉样变性病的诊治分析

目的探讨鼻咽部淀粉样变性病的发病机制、分类、诊断及治疗。方法通过MRI、腹壁脂肪活检及病理学检查确诊的1例鼻咽淀粉样变性病患者，全麻鼻内镜下切除病变并结合文献进行分析。结果结合影像学资料经鼻内镜下完整切除病变，术后病检示黏膜下见大量无定型样物质，刚果红染色（＋＋），术后随访1年无复发。结论鼻咽部淀粉样变性病需病理切片确诊，MRI是诊断与鉴别诊断该病的最佳辅助检查，其首选治疗方式为鼻内镜下手术切除。     

婴幼儿腮腺血管瘤的手术治疗

目的 :探讨治疗婴幼儿腮腺血管瘤的有效方法。方法 :19例婴幼儿腮腺血管瘤 ,行腮腺浅叶切除 15例 ,全腮腺切除 3例 ,腮腺浅叶切除加深叶瘤体缝扎术 1例。 6例皮肤受累均同期切除 ,3例拉拢缝合 ,3例缺损较大 ,行颈项皮瓣转移修复 ,术中采用“假包膜”外剥离法及顺行解剖面神经主干法。结果 :3例出现轻度面瘫 ,均在 3～ 6个月内恢复。术中仅 1例输血 2 0 0ml。所有病例随访 1年以上 ,无肿瘤复发。结论 :外科手术中采用“假包膜”外剥离法 ,顺行面神经解剖及受累皮肤切除后缺损行颈项皮瓣转移修复等治疗 ,是根治婴幼儿腮腺血管瘤的有效方法     

鼻成形术后软骨肿胀增生:一种新的并发症

介绍鼻成形术后软骨瘤样增殖４例，讨论其组织学特征和治疗结果。男１例，女３例，年龄２１～４９岁，２例行大翼状软骨缘切除常规鼻成形术，另２例行耳软骨移植鼻成形术。术后１０个月，术区切除部软骨或移植软骨出现坚硬、光滑、卵圆形肿物，手术探查为软骨样增生，切除含有肿物的软骨片。病理学结果为肥大和增生软骨组织，１例伴轻微核异常。其中１例始发时仅行肿物刮除，但１年后在同一部位复发，连同软骨切除后无复发。鼻成形术后常见并发症为尖性隆起，但单侧尖性隆起应疑为软骨瘤样增生。与软骨移植相关的病变可能是软骨肿胀增生或移…     

An aesthetic deformity: Madelung's disease

Madelung's disease, also known as benign symmetric lipomatosis, multiple symmetrical lipomatosis or Lanois-Bensaude syndrome, consists of growth of fatty masses predominantly on the face, neck, shoulders, upper thorax and arms. These painless and symmetrical fatty deposits can cause important aesthetic changes in these patients. Middle-aged males are more commonly affected, with an increased prevalence in the Mediterranean area. Currently, the incidence in Italy is 1 in 25,000 males. The aetiology of Madelung's disease remains unknown, although approximately = 60% of affected patients are clinically overweight and there is an intimate association with chronic alcohol abuse. We report an unusual case of Madelung's disease in a 52-year-old male. The fatty deposits were completely resected by means of surgical lipectomy. The patient remains well and disease-free after 12 months of postoperative follow-up.     

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。     

Kimura's disease in children: a 9 years prospective study

Kimura's disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with lymphadenopathy and/or salivary gland enlargement. The nodular lesions are deep seated in subcutaneous tissue and clinically may mimic a neoplasm. Hence head and neck surgeons need to be aware of clinical presentation of Kimura's disease. OBJECTIVES: To study the clinical presentations, management and complications of Kimura's disease in pediatric age group. STUDY DESIGN: Prospective study. MATERIALS AND METHODS: The duration of study was 9 years (January 1998 to December 2006), comprising of 18 patients. Only histopathologically proven cases were included in this study. Blood eosinophil count and serum IgE estimation were done in all these patients. All the patients underwent fine needle aspiration cytology study. In 15 patients excision biopsy was done and resected specimen was sent for histopathological examination. In three cases, only biopsy was done to confirm the diagnosis. RESULTS: All the patients presented with painless swelling in the head and neck region. Post-auricular region was the commonest site (50%). Sixteen patients (88.8%) had blood eosinophilia and in 15 patients (83.3%) serum IgE level was elevated. Fifteen cases were treated by surgery and three cases were treated with steroids. Out of 18 cases, 15 cases were symptom free at the end of 1 year. Among 15 patients who underwent surgery, only one had recurrence (6.6 %). Out of three patients who were treated with corticosteroids, two came back with recurrence (66.6%). In our study, totally three patients had recurrence (16.6%). One patient had nephrotic syndrome (5.5%). CONCLUSION: Post-auricular region is the commonest site of involvement. Incidence is more common in the second decade of life. Recurrence rate is more with steroid therapy. Surgery is the best modality of treatment. The only systemic complication is nephrotic syndrome.     

Giant Madelung's disease. Report of a case and review of the literature

Madelung's disease, also known as benign symmetrical lipomatosis, is a rare proliferative disorder of unknown etiology that was first mentioned by Brodie in 1846.Characterized by multiple symmetrical deposits of unencapsulated fat in the head and neck region, the disease is most common in middle-aged men with a history of alcohol abuse. The only effective therapy in cases of dyspnea and dysphagia, indicating the necessity of treatment, is the surgical resection of the adipose tissue.The authors report on the evaluation and therapy in a case of giant Madelung's disease.     

A case of multiple symmetrical lipomatosis (Madelung's disease).

Multiple symmetrical lipomatosis (Madelung's disease) is a rare disease with multiple symmetrical unencapsulated fatty accumulation diffusely involving the neck, the shoulders and the upper extremities (Kohan et al. Otolaryngol. Head Neck Surg. 1993;108:156-159). We describe a 48-year-old Japanese man with a history of alcoholism and liver cirrhosis who reported gradually enlarging masses in his cervical region for 4 years. MRI revealed large masses suggesting lipomas in the neck. The patient underwent a two-stage lipectomy. This patient is the 13th case reported in Japan since 1978, though over 200 cases have been reported since 1846 in Europe, most of them from the Mediterranean (Kitano et al. ORL 1994;56:177 180; Kaku et al. Endocrinol. Diabetol. 1997;4:103-106).     

Rare case of Madelung disease

Madelung's disease, also known as benign symmetric lipomatosis (BSL), is a rare condition characterized by symmetric, painless fatty deposits arranged around the neck and shoulders girdle. This deformity is usually associated with chronic alcohol. The etiology remains unclear although some authors suggest that mitochondrial dysfunction represents the essential biochemical defect in BSL. This report describes a patient with this deformity. Lipomas were present in the pre-postauricular and nuchal area.     

'Defatting' tracheotomy in morbidly obese patients

OBJECTIVES/HYPOTHESIS: Standard-sized tracheostomy tubes often fit morbidly obese patients poorly because of increased submental and anterior cervical girth. The surgeon has two options to overcome this problem: Modify the tracheostomy tube to fit the patient or recontour the neck to accommodate a standard tube. The purpose of the study was to assess the safety and morbidity of the latter technique, the "defatting" tracheotomy. STUDY DESIGN: Retrospective cohort study of 23 patients treated between 1994 and 2001 with cervical lipectomy and tracheotomy. METHODS: Medical charts were reviewed for indications, demographics, body mass index, tracheotomy-related complications, and decannulation results. RESULTS: The average age of patients was 50 years (age range, 34-77 y). The mean preoperative body mass index was 55.9 (range, 39.2-73.5). Indications for the procedure were respiratory failure requiring chronic ventilation in 16 patients (70%) and obstructive sleep apnea in 7 (30%). Four patients died postoperatively from causes unrelated to tracheotomy. The mean follow-up time of survivors was 23 months. The overall tracheotomy-related complication rate was 43%. Four patients developed wound infections in the perioperative period, one patient developed a neck abscess, and one patient required neck exploration for control of hemorrhage. Four patients (22%) developed late complications including tracheitis (1), neck abscess (1), and stenosis of the tracheocutaneous tract (2). Eight patients (44%) ultimately had decannulation. CONCLUSIONS: Defatting tracheotomy is a safe technique that allows for the placement of a standard tracheostomy tube in morbidly obese patients. It is associated with a high rate of minor infectious complications. Even so, we think that cervical lipectomy with tracheostomy tube placement is the preferred surgical option for this patient population.     

Multiple symmetrical lipomatosis in the neck

Summary Multiple symmetrical lipomatosis (Madelung's disease) is a very rare condition that is commonest in countries bordering the Mediterranean. The disease is associated with symmetrical unencapsulated fat deposits over the neck and upper trunk. We present the first reported occurrence in the Chinese. Although lesions can initially mimic a head and neck malignancy, management is essentially symptomatic, with conservative removals done as indicated clinically or for cosmesis.