Madelung病的诊断及手术治疗

Diagnosis and surgical treatment of Madelung's disease

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。

OBJECTIVE To discuss t he diagnosis and surgical methods of the Madelung＇s disease.＆amp;nbsp;METHODS Detailed clinical data of 6 patients with Madelung＇s disease who was received surgical treatment in our department from January 2011 to November 2015 were reviewed. RESULTS All patients were male, aged from 35 to 65 years. The 6 patients had excessive subcutaneous fat deposit predominantly around the neck. One of the 6 patients had excessive fat deposit in larynx. All patients had history of heavy drinking. Two patients had the complication of cardiac disease, pulmonary disease and cerebral disease. One patient had the complication of glucose intolerance. Five patients were associated with sleep apnea syndrome. One patient came to hospital with dyspnea in emergency. All patients had received surgical treatment and abstinence from alcohol, 4 patients had the total neck lipectomy and tracheotomy. One patient had the total neck lipectomy with no tracheotomy. One patient had received emergency surgery-partial neck lipectomy and tracheotomy because the patient came to hospital with dyspnea in emergency. This case was referred to respiratory department without a second surgery for his severe complications and high surgical risk. This case discharged with tracheal tube. No obvious recurrence was seen during a follow-up of 15 days to 48 months. The pathological results were nonencapsulated fat. CONCLUSION Madelung＇s disease is a kind of benign disease arised from lipodystrophy which characterized by massive accumulation of nonencapsulated subcutaneous fat mainly located symmetrically in the fascial space of neck and shoulder. Most patients had heavy drinking history, chronic alcoholism may be a major risk factor. It may be associated with some complications. Total neck lipectomy has good effect. The aim of the operation is to improve outline appearance and function of the neck, which is good for patient＇s quality of life.