Benign Symmetric Lipomatosis

良性對稱型脂肪增生症

廖文傑(Wen-Chieh Liao) ； 常玉琴(Yuh-Chirn Charng) ； 林素娥(Su-Er Lin) ； 馬旭(Jin-Teh Lin) ； 林進德(Hsu Ma) ； 葉發來(Fa-Lai Yeh)

《臺灣整形外科醫學會雜誌》 20卷1期 (2011/03) Pp. 1-8

https://doi.org/10.7096/TJTSPS.201103.0001

摘要

背景： 良性對稱型脂肪增生症又稱爲Madelung's disease，是一種罕見之脂肪增生疾病，一般好發於中年男性。主要的特徵爲無痛，多發性無包膜脂肪組織，對稱廣泛地沉積於頭枕部下方、頸部及上半身。其起因尚未明確，但文獻報告與酗酒、及代謝性疾病有關連。 目的及目標： 我們提出五位病患臨床治療經驗並討論評估該疾病臨床狀況、外觀型態及臨床生化等因素，同時將文獻整理回顧。 材料及方法： 本文報告五位符合診斷標準之病患。依據病患之臨床表現、症狀和徵象、影像學特徵、治療方法及合併出現之全身系統性疾病加以分析。五位病患皆爲無家族史男性，平均47.2歲。皆因腫塊效應造成之美觀及脂肪沉積影響頭頸部活動來求診。五位病患中，四位有酗酒習慣，同時合併出現酒精性肝病、肝硬化、高尿酸血症、高血脂及糖尿病等相關疾病。病患均接受外科手術，局部切除脂肪或合併脂肪抽吸。 結果： 平均追蹤88.6月。病患對術後相關功能及美觀之改進均滿意。有一位病患因繼續酗酒習慣，術後六個月於原先手術部位出現脂肪沉積。除了一位病患手術後出現血腫，並無嚴重之合併症。 結論： 依據我們經驗，治療良性對稱型脂肪增生症採用切除合併脂肪抽吸手術，在病患功能及外觀之改進的確有效。

並列摘要

Background:
Madelung's disease, benign symmetric lipomatosis, is a rare proliferative disease predominantly seen in middle-aged male patients. The disorder is characterized by multiple symmetrical, painless deposits of non-encapsulated fat around the neck, upper arms and upper part of the thorax. No clear etiology has been recognized while a close correlation to alcohol and metabolic disturbances has been observed.
Aim and Objectives:
To evaluate the clinicopathologic, morphologic and biochemical findings in this disease, we present and discuss various factors in this disease of 5 cases and give a survey of the literature.
Materials and Methods:
Five patients with benign symmetric lipomatosis were reviewed. All were male, aged 38 to 73 years, with a history of disease ranging from 6 months to 7 years. Patients usually complain of their cosmetic appearance, limited range of motion of the neck, difficulty in swallowing and compression of the airway. We performed the surgical excision via lipectomy with or without liposuction in all patients for the neck tumors.
Results:
The mean follow-up period was 88.6 months. The functional results were satisfactory in all patients. A recurrence was observed in one of our patients six months after the operation. Severe complications were not observed.
Conclusions:
According to our experience, surgical removal of lipomatous tissues in BSL can yield satisfactory functional and cosmetic results.

並列關鍵字

benign symmetric lipomatosis ； madelung syndrome ； lipectomy ； liposuction