Madelung病的诊断和治疗

Diagnosis and treatment of Madelung's disease

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。

Objective To review the characteristics of Madelung＇s disease which is rare and unfamiliar to clinicians and to find the method of diagnosis and treatment. Methods Detailed clinical data of 7 patients with Madelung＇s disease were reviewed and analyzed. And related literatures were discussed together. Results All of 7 patients have excessive subcutaneous fat deposit predominantly around neck. One patients had the complication of central and peripheral neuropathy, One patients had the complication of glucose intolerance, One patients had the complication of chronic hepatopathy and hyperuricaemia. These 3 patients were associated with sleep apnea syndrome simultaneously. Another one patient was accompanied by autonomic nervous system disease only. Total neck lipectomy and abstinence from alcohol were performed on 5 patients. No recurrence was seen during a follow-up of 6 months to 5 years. One patient received partial lipectomy twice and relapsed again. All pathological results were nonencapsulated fat. One patient refused treatment. Six men patients were alcohol abusers. Conclusions Madelung＇s disease is characterized by massive accumulation of nonencapsulated subcutaneous fat mainly located symmetrically in the neck. Chronic alcoholism may be a major risk factor. It may be associated with some internal diseases. Total neck lipectomy to improve figuration and relieve pressure and abstinence from alcohol may be the main effective therapy.