Chen Xing-Min, Li Wu-Yi, Ni Dao-Feng, Wei Bo-Jun, Xu Chun-Xiao, Gao Zhi-Qiang, He Lin, Chen Yong-Ning
Department of Otorhinolaryngology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2006 Jul;41(7):524-7.
To review the characteristics of Madelung's disease which is rare and unfamiliar to clinicians and to find the method of diagnosis and treatment.
Detailed clinical data of 7 patients with Madelung's disease were reviewed and analyzed. And related literatures were discussed together.
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.
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.
回顾马德隆氏病的特征,该病较为罕见,临床医生对此并不熟悉,并探寻其诊断和治疗方法。
回顾并分析7例马德隆氏病患者的详细临床资料,并结合相关文献进行讨论。
7例患者均有主要位于颈部周围的皮下脂肪过度沉积。1例患者出现中枢和周围神经病变并发症,1例患者出现葡萄糖耐量异常并发症,1例患者出现慢性肝病和高尿酸血症并发症。这3例患者同时合并睡眠呼吸暂停综合征。另1例患者仅伴有自主神经系统疾病。5例患者接受了全颈部脂肪切除术并戒酒。在6个月至5年的随访期间未见复发。1例患者接受了两次部分脂肪切除术,再次复发。所有病理结果均为非包膜脂肪。1例患者拒绝治疗。6例男性患者为酗酒者。
马德隆氏病的特征是主要对称位于颈部的非包膜皮下脂肪大量堆积。慢性酒精中毒可能是主要危险因素。它可能与一些内科疾病有关。全颈部脂肪切除术以改善外形和减轻压迫并戒酒可能是主要的有效治疗方法。