Madelung’s disease was first described by Sir Benjamin Brodie 1 in 1846, but Otto Madelung 2 reported the first series of 35 patients in 1888. The following year, Launois and Bensaude 3 presented a new series of 30 patients, and since then approximately 200 patients have been described in literature. This uncommon disease predominantly affects men between the ages of 30 and 60 years. 4 The incidence is highest in the Mediterranean area, 5 and in nearly all patients there is a strong correlation with alcohol abuse. The disorder is defined as the presence of multiple and symmetrical fatty accumulations, usually involving the upper trunk, neck, and head. Several descriptive names have been associated with it, including benign symmetrical lipomatosis, multiple symmetrical lipomatosis, lipomatosis simplex indolens, and symmetrical adenolipomatosis. 6 Over the years the fat deposits achieve a large size, become cosmetically deforming, and in advanced cases, cause dyspnea and dysphagia. 7 Frequently associated findings include diabetes mellitus, hyperlipidemia, liver disease, hypothyroidism, and polyneuropathy of unknown origin. 8 The clinical course of the disease involves an initial period of fast growth, followed by many years of slow progression. There is no report of spontaneous regression, and the only effective therapy is the surgical reduction of the mass. 9
A 65-year-old man reported multiple, large disfiguring neck masses that had enlarged progressively during the last 5 years. Even without substantial weight gain, the patient complained that it was very difficult to find clothes that fit his neck and shoulders. Despite the large amount of swelling, there were no signs of dyspnea or dysphagia. Physical examination revealed multiple soft masses involving the submental and submandibular regions on both sides, extending to the level of the sternoclavicular joint and to the parotid glands bilaterally. A large posterior cervical lipoma was also present, in continuity with the anterior and lateral masses (Figs A, C). No cervical nodes were palpable and the mass did not transilluminate. Computed tomography confirmed the presence of a large lipoma infiltrating the muscular planes. The patient had no evidence of sensory or motor neuropathy, but he was a heavy drinker for 30 years.
Considering the extension and the infiltrating nature of the masses, surgical excision was planned instead of liposuction. Incisions were made bilaterally, following the regular facelift pattern, and the retroauricular extensions were joined posteriorly with a long horizontal cut. A separate incision was carried out anteriorly, a few centimeters higher than for a standard thyroidectomy. There was no evidence of capsule, and the fat blended diffusely with the surrounding tissues. The lesion was very adherent to the skin and to the carotid sheath. Intraoperative weight of the excised lipoma was 1,900 g. There was one posterior mass in continuity with the anterior and lateral masses. No separation was evident. The redundant skin was excised as in a regular facelift, and three suctions drains were left in place. The postoperative course was uneventful, and the patient was satisfied with the cosmetic result (Figs B, D). Even though part of the lipoma could not be removed completely, no additional surgery is planned at this time. The patient has resumed his previous lifestyle, including his heavy alcohol consumption.
Madelung’s disease is characterized by diffuse deposits of fat arranged symmetrically around the neck and shoulder girdle. It is more frequent in middle-age men of Mediterranean descent who have a history of heavy alcohol consumption during the previous decades. The masses are nonencapsulated and can eventually reach a very large size, diminishing the range of motion of the neck and upper extremities. Many patients present with cosmetic complaints and an inability to find clothes that fit. In a few patients, dyspnea and/or dysphagia have been reported as a result of the mediastinal involvement and compression of the upper aerodigestive tract 7.
The diagnosis of Madelung’s disease is usually made by physical examination and clinical history, but nuclear magnetic resonance imaging, computed tomography, and sonography may be helpful. 10 An incidence of 1 in 25,000 men is reported in the Italian population 6. A strong correlation exists with chronic alcohol abuse, and other less reliable connections have been described with glucose intolerance, hepatic disease, and hyperuricemia. Polyneuropathy, hypertension, hypothyroidism, and hyperlipidemia have been identified occasionally in patients with Madelung’s disease, but it must be noted that all of them can be caused or exacerbated by alcohol abuse. 11,12
Medical treatment with B2 agonist (salbutamol) has been advocated, 13 but its effectiveness is disputed and not proved clinically. Weight loss and abstinence from alcohol are still recommended; however, they do not reverse or stop the progression of the disease once it is established.
Surgery is the most effective treatment available for the disease, and several options need to be considered and evaluated properly. Liposuction can be performed in patients with masses of limited size. Because it can be performed safely under local anesthesia, it is possible to reduce the risks related to tracheal intubation in patients who are normally obese and who have a severely limited range of neck motion. In patients with severe cosmetic deformities, surgical excision of the lesion with removal of redundant skin is indicated. In our experience, the use of standard facelift skin incisions results in acceptable scars and guarantees good exposure of the operating field. Given the infiltrative nature of the unencapsulated lipomas, complete excision of the lesions is very difficult, and important structures could be compromised in a futile attempt to excise this benign tumor. Because only one case of malignant degeneration is reported in literature, 14 a debulking procedure can be considered the appropriate treatment for large lipomas of the neck.
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