A 62-year-old man presented to the emergency department with severe dyspnea and stridor. He also reported the gradually progressive appearance of masses on his trunk and neck. He had a history of heavy alcohol consumption. Physical examination revealed painless multilobulated tumors of soft elastic consistency in the cervical and shoulder area. Computed tomography of the neck and thorax showed excess fat scattered throughout the neck, upper trunk and superior mediastinum (Fig. 1A–C), and a fatty mass in the larynx affecting the epiglottis (Fig. 1D, E). The final diagnosis was Madelung's disease. Due to the intense dyspnea, tracheotomy was performed and the laryngeal tumor was removed by microlaryngoscopy. Histological examination revealed mature adipose tissue. The patient's postoperative course was uneventful.
In A, chest computed tomography image obtained with three-dimensional coronal reconstruction showing the appearance of the patient's cervical region. In B, computed tomography image of the cervical region demonstrating the abnormal accumulation of lipomatous tissue, scattered particularly in the anterior neck region. In C, computed tomography scout image showing excess fat in the neck and upper trunk. In D, sagittally reconstructed image of the cervical region demonstrating a lipomatous mass in the larynx affecting the epiglottis (arrows). In E, detail of the mass in the larynx (arrows).
Madelung's disease, also known as multiple symmetrical lipomatosis, is a rare disease of unknown etiology characterized by symmetrical unencapsulated adipose tissue deposits in the neck and upper trunk. The diagnosis is clinical and based on typical adipose tissue distribution. The disorder usually occurs in middle-aged men with histories of alcoholism. It characteristically produces cosmetic deformities without functional impairment. However, it may cause symptoms related to upper airway obstruction. In these patients, tracheostomy combined with surgical lipectomy is the treatment of choice.1,2
Conflicts of interestThe authors declare that they have no conflicts of interest to express.
