Madelung综合征六例诊治分析

Management of Madelung's Syndrome:Report of 6 Cases and Literature Review

目的探讨Madelung综合征的病因、诊断及治疗。方法回顾分析6例Madelung综合征患者临床资料,结合相关文献对本病进行分析。结果6例患者均以颈部增粗入院,4例男性患者有10年以上的酗酒史;B超和CT检查均发现颈项部皮下有大量堆积的脂肪组织。经内科综合治疗后肝功能、血压正常。在全麻下行增生脂肪切除,术后病理为无包膜脂肪组织,随访15～40个月,平均随访时间20个月,1例术后10个月复发,未行处理。结论Madelung综合征是一种罕见病,长期过度饮酒是公认的致病因素。CT检查对定性定量诊断和指导手术具有重要价值。治疗以综合治疗为主,须戒酒。手术切除局部增生的脂肪组织的根本目的在于缓解压迫症状,改善颈部外观。

Objective To investigate the etiology,diagnosis,and treatment of Madelung＇s syndrome.Methods The clinical data of six cases of Madelung＇s syndrome were analyzed retrospectively and relevant literatures were reviewed.Results All patients were presented with ＂fat neck＂.Four patients had a history of alcohol abuse for more than 10 years.B-ultrasound and CT examinations showed massive subcutaneous deposits of adipose tissuearound the neck.Liver function and blood pruessure retunred normal after comprehensive medical treatment.Hyperplasia adipose tissue was resected under general anesthesia.All samples were confirmed as no capsule hyperplasia adipose tissue by histopathology after operation.Patients were followed up for 15-40 months （mean：20 months）.One patient experienced recurrence 10 month after operation but did not undergo any further treatment.Conclusion Madelung＇s syndrome 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 hyperplasia adipose tissue helps alleviate the symptoms of oppression and improve the appearance of neck.