Multiple Symmetric Lipomatosis-Reports of Two Cases and Review of the Literatures

多發性對稱性脂肪瘤－病例報告及文獻回顧

歐冠彣(Kuang-Wen Ou) ； 陳錫根(Shyi-Gen Chen) ； 陳天牧(Tim-Mo Chen)

《臺灣整形外科醫學會雜誌》 23卷2期 (2014/06) Pp. 161-168

摘要

背景：多發性對稱性脂肪瘤，又名madelung's disease，為一罕見疾病，典型的特徵是在頸部、肩膀、前胸、背部及上臂有對稱性巨大脂肪瘤，病患常有嚴重的酒精濫用病史，並常併發神經病變。這樣的病人在整形外科門診可能被當作簡單的脂肪瘤而手術切除，而忽略潛藏可能的疾病及適當衛教。目的及目標：我們報告兩例多發性對稱性脂肪瘤患者伴隨不同程度的血小板缺乏，這是一個不常被提及的併發症但卻可能造成手術出血的機會提高。材料及方法：我們紀錄兩名多發性對稱性脂肪瘤患者其發病特徵、病史、手術方式、併發症及術後追蹤，並針對多發性對稱性脂肪瘤作文獻回顧。結果：兩名患者術前的血小板分別為52,000/μL及101,000/μL，前者在術前接受血小板輸血矯正。我們使用直接切除及抽脂的方式來減少脂肪瘤的體積，直接切除的患者發生皮下積液的手術併發症，而另一名抽脂的患者在兩年後有復發情形而再次接受直接切除手術。兩名患者皆對術後外觀感到滿意。結論：多發性對稱性脂肪瘤是一個罕見疾病但容易被誤診，雖然其手術方式與脂肪瘤相同但復發率高，且病人潛在疾病及病程發展都需要不同於一般脂肪瘤的關注與追蹤，在門診遇到這樣的病人，手術是其次，最重要的是衛教其戒除酒精並改善生活方式

並列摘要

Background: Multiple symmetric lipomatosis (MSL)-also known as Madelung's disease-is a rare disease characterized by the typical appearance of multiple huge symmetric lipomas over the neck, upper arms, shoulder, and back. It is reportedly strongly associated with alcohol abuse and neuropathy. Aim and objectives: In the present report, we describe two cases of MSL complicated with thrombocytopenia, a less mentioned comorbidity in previous literatures, which could lead to higher operative risk Materials and Methods: We describe the distinguishing clinical features, images, medical history, surgical procedures, and follow-up outcomes of the two cases of MSL. In addition we reviewd the literatures and describe the findings. Results: Thrombocytopenia was noted in both cases and one recieved platelet transfusion for hematology correction before surgery. Lipectomy and liposuction were used to decrease tumor volume. Seroma formation was noted postoperatively with lipectomy. However recurrence within 2 years was noted in patient who underwent liposuction. Conclusion: MSL is a rare disease and is easily misdiagnosed as simple lipoma. Although the treatments for these conditions are similar, a thorough assessment of the underlying disease should be performed. Moreover, it is essential to educate such patients to abstain from consuming alcohol and to change their lifestyle.

並列關鍵字

multiple symmetric lipomatosis ； Madelung's disease ； thrombocytopenia