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    ABSTRACT: Benign symmetric lipomatosis (Madelung's disease) is a rare disorder of fat metabolism that is characterized by progressive symmetrical formation of unencapsulated and painless excess fat masses around the neck and trunk that result in cosmetic disfiguration and functional impairment. Since the disorder is incompletely understood and causal therapy is unavailable, surgical removal of fatty masses is the mainstay of treatment. In this paper the authors describe their use of the classical facelift incision placement as well as a horizontal circum-occipital incision to approach and excise excess fat of the anterior and posterior neck in two patients. This method yielded satisfying results with a combination of good access to fat masses, smooth trimming and redraping of redundant skin, in addition to fairly inconspicuous scarring post-operatively. After removal of 1.5 kg of fat from each patient and a period of uncomplicated wound healing, both patients showed no signs of relapsing fatty growth. Although more challenging than most conventional approaches, the authors' technique has shown good outcomes in those treated with this condition.
    Journal of cranio-maxillo-facial surgery: official publication of the European Association for Cranio-Maxillo-Facial Surgery 06/2013; DOI:10.1016/j.jcms.2013.04.011 · 2.60 Impact Factor
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    ABSTRACT: Background Multiple symmetric lipomatosis (MSL) is a rare condition leading to grotesque disfigurement. Complete removal is almost impossible and recurrences inevitable. Objectives To evaluate powered-assisted liposuction (PAL) for the treatment of MSL. Setting The study was conducted at the University Hospital Basel, Switzerland. Methods Magnetic resonance imaging (MRI) was performed before and after surgery for quality assessment. In order to exclude malignancy incisional biopsies were taken before surgery. Outcome measures included aspiration volumes, duration of surgery, early morbidity, recurrence rates and overall patient satisfaction. Results Seven male patients aged between 43 and 70 years were identified. The mean liposuction volume equalled 2948±1566ml, the mean surgery time 74±28minutes. One hematoma was observed, whereas one recurrence rate was noticed after a mean follow-up of 14 months. Malignancy was excluded by histology in five patients. Preoperative MRI confirmed the diagnosis with no signs for malignancy and showed an efficient removal of the lesions. After a mean follow-up of 18 months a high patient satisfaction was achieved. Conclusions PAL can serve as an efficient method for the treatment of MSL. It has a significant effect on tightening of the skin, leading to a high patient satisfaction.
    Surgery for Obesity and Related Diseases 05/2014; 11(1). DOI:10.1016/j.soard.2014.05.004 · 4.94 Impact Factor
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    ABSTRACT: Aim of this study is an updated review of our case series (72 patients) as well as available literature on the Multiple Symmetric Lipomatosis (MSL), a rare disease primarily involving adipose tissue, characterized by the presence of not encapsulated fat masses, symmetrically disposed at characteristic body sites (neck, trunk, proximal parts of upper and lower limbs). The disease is more frequent in males, associated to an elevated chronic alcohol consumption, mainly in form of red wine. Familiarity has been reported and MSL is considered an autosomic dominant inherited disease. MSL is associated to severe clinical complications, represented by occupation of the mediastinum by lipomatous tissue with a mediastinal syndrome and by the presence of a somatic and autonomic neuropathies. Hyper-alphalipoproteinemia with an increased adipose tissue lipoprotein-lipase activity, a defect of adrenergic stimulated lipolysis and a reduction of mitochondrial enzymes have been described. The localization of lipomatous masses suggests that MSL lipomas could originate from brown adipose tissue (BAT). Moreover, studies on cultured pre-adipocytes demonstrate that these cells synthetize the mitochondrial inner membrane protein UCP-1, the selective marker of BAT. Surgical removal of lipomatous tissue is to date the only validated therapeutic approach. MSL is supposed to be the result of a disorder of the proliferation and differentiation of human BAT cells. Copyright © 2015 Elsevier B.V. All rights reserved.
    Nutrition Metabolism and Cardiovascular Diseases 02/2015; 25(4). DOI:10.1016/j.numecd.2015.01.010 · 3.88 Impact Factor