Madelung病11例回顾分析  

Management of Madelung's disease:report of 11 cases and literature review

作　　者：宇尧 杜心如[1] 骆辉[1] 徐子彧[1] 

机构地区：[1]首都医科大学附属北京朝阳医院骨科,北京100020

出　　处：《中国骨与关节杂志》2016年第8期616-622,共7页Chinese Journal of Bone and Joint

摘　　要：目的探讨Madelung病的病因、发病机制、影像学表现、实验室检查、病理学表现、诊断及治疗。方法回顾分析11例Madelung病患者的临床资料,并结合相关文献对本病进行分析。结果 11例均以无痛性肿块来院就诊,9例单发于颈背部,2例多发于颈背部及四肢。2例男性患者有20年以上的酗酒史。B超及MRI均发现颈背部及四肢皮下有大量的脂肪组织堆积。经内科治疗血压、血糖及肝功能正常后,9例在全麻下行肿物切除术,术后病理为无包膜的脂肪组织。术后随访6-36个月,平均15个月,3例在术后36个月内复发。结论 Madelung病是一种罕见的疾病,长期酗酒被认为是一种潜在的危险因素。MRI检查对定性定量诊断及指导手术治疗具有重要价值。治疗常以综合治疗为主,戒酒可能有效。手术切除局部堆积的脂肪组织的根本目的在于改善外观并缓解局部压迫症状。Objective To investigate the etiology, pathogenesis, imaging characteristics, laboratory examination, pathology, diagnosis, and treatment of Madelung＇s disease. Methods Clinical data of eleven cases of Madelung＇s disease were analyzed retrospectively and relevant literature was reviewed. Results Nine patients were presented with ＂fatty neck＂,2 patients were presented with ＂fatty neck＂ and ＂fatty limbs＂. Two patients had a history of alcohol abuse for more than 20 years. B-ultrasound and MRI examinations showed massive subcutaneous deposits of adipose tissue around the neck and limbs. Blood pressure, blood glucose and liver function returned to normal after comprehensive medical treatment. Hyperplasia of adipose tissue was resected under general anesthesia. Nine samples were confirmed as no capsule hyperplasia of adipose tissue by histopathology after the operation. Patients were followed up for 6- 36 months（ mean： 15 months）. Three patient experienced recurrence 36 months after the operation. Conclusions Madelung＇s disease is a rare disease and the recognized etiology is the long-term excessive drinking. A comprehensive management plan is required to treat this disease. Surgical resection of local hyperplasic adipose tissue helps alleviate the symptoms of oppression and improve the appearance of neck and limbs.

关 键 词：脂肪瘤样病 多发性对称性 营养和代谢性疾病 磁共振成像 病理状态 体征和症状 诊断 鉴别 

分 类 号：R597[医药卫生—内科学]