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1/35. Non-insulin-dependent diabetes mellitus with type I multiple symmetrical lipomatosis: a case report.

    Type I multiple symmetrical lipomatosis (MSL; Madelung's disease) is characterized by lipomas in the nape of the neck and the supraclavicular and deltoid regions, resulting in a bull-necked appearance (Madelung's collar). It is most common in alcoholic men between 35 and 50 years of age. Type I MSL has been reportedly associated with hyperinsulinemia, but its association with diabetes mellitus is rarely discussed. We describe a case of non-insulin-dependent diabetes mellitus (NIDDM) associated with type I MSL. A 47-year-old alcoholic man presented with a seven-year history of hyperglycemia and progressive neck swelling with dysphagia for one year. physical examination showed diffuse and symmetrical swelling of the bilateral posterior aspects of the neck. biochemistry profiles revealed elevated concentrations of fasting serum glucose (276 /- 16 mg/dl), triglycerides (358 /- 79 mg/dl) and total cholesterol (323 /- 28 mg/dl). Endocrinologic studies showed normal thyroid function. neck sonography revealed diffuse thickening and swelling of the fatty structures of both sides of the neck. Normal sonography showed no fatty deposition in the liver. Maxillary and neck computerized tomography revealed diffuse fat accumulation in the submental and posterior neck regions, with no extension to the superior mediastinum. Fine needle aspiration cytology of the neck masses showed only fat cells. The patient received an oral hypoglycemic agent (glibenclamide 5 mg bid) for blood glucose control and lovastatin (20 mg before bed-time) for hyperlipidemia, and ceased drinking alcohol. The neck swelling resolved markedly after 15 months of medical treatment. This suggests that, in addition to the cessation of alcohol consumption, the reduction of blood glucose and lipid concentrations by medication may also assist in resolving the accumulated fat of type I MSL in patients with NIDDM.
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2/35. head and neck cancers associated with Madelung's disease.

    BACKGROUND: Madelung's disease is a rare lipodystrophy that presents with multiple fatty masses in the neck, trunk, and upper extremities. The fatty accumulation is considered a benign disease, but compression of the aerodigestive tract may occur in long-standing disease. methods: Eight Chinese patients with Madelung's disease were reviewed. All were male, aged 48 to 67 years, with a history of disease ranging from 4 to 20 years. Two of the eight patients developed aerodigestive symptoms and were subsequently found to have head and neck cancers. These two patients are described. RESULTS: The possible mechanism that may account for an increase in malignant tumors of the airway in this group of patients is the synergistic effect of smoking and alcohol abuse as risk factors for both Madelung's disease and malignant tumors of the airway. Currently it is recommended that these patients should have their fatty lesions removed surgically. The removal of fat facilitates examination of the neck for signs of cervical lymphadenopathy in malignant disease. CONCLUSIONS: patients with Madelung's disease should be followed regularly. The development of aerodigestive symptoms should be fully investigated with endoscopy and imaging. The cause of symptoms should not be attributed to fatty compression until a carcinoma of the upper airway has been excluded.
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3/35. A case of multiple symmetrical lipomatosis (Madelung's disease).

    Multiple symmetrical lipomatosis (Madelung's disease) is a rare disease with multiple symmetrical unencapsulated fatty accumulation diffusely involving the neck, the shoulders and the upper extremities (Kohan et al. Otolaryngol. head neck Surg. 1993;108:156-159). We describe a 48-year-old Japanese man with a history of alcoholism and liver cirrhosis who reported gradually enlarging masses in his cervical region for 4 years. MRI revealed large masses suggesting lipomas in the neck. The patient underwent a two-stage lipectomy. This patient is the 13th case reported in japan since 1978, though over 200 cases have been reported since 1846 in europe, most of them from the Mediterranean (Kitano et al. ORL 1994;56:177 180; Kaku et al. Endocrinol. Diabetol. 1997;4:103-106).
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4/35. Efficacy of lipectomy and liposuction in the treatment of multiple symmetric lipomatosis.

    BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by enlarging, symmetric, nonencapsulated, fat deposits mainly on the neck and upper trunk. Liposuction and lipectomy, although palliative, are the treatments of choice, especially indicated when vital structures are compromised. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of liposuction and lipectomy in the treatment of MSL. methods: We have examined two patients diagnosed with MSL who presented with symptoms derived from the compression of vascular, nervous, and/or respiratory tract structures. One was treated with lipectomy and the other with liposuction. RESULTS: A rapid resolution of the clinical symptoms was achieved with both therapies. The patient who was treated with lipectomy suffered from a compression of the left brachial plexus by scar tissue as an adverse effect, requiring a second surgical procedure. Liposuction only provoked a mild autoinvolutive hematoma in the other case. No clinical recurrences were observed at 3 and 2 years of follow-up respectively. CONCLUSIONS: We consider both lipectomy and liposuction as safe and effective techniques for the treatment of MSL patients. Although liposuction is usually associated with less adverse effects than lipectomy, location of the lipomas must be carefully considered before choosing one technique over another.
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5/35. Multiple Symmetric lipomatosis--MR appearances.

    Multiple Symmetric lipomatosis (MSL) is a rare disorder of lipid metabolism which is mainly seen in Mediterranean and eastern European populations, which results in massive fat accumulation mainly around the neck and back. The main differential diagnosis lies between MSL and the fat accumulation of Cushing's disease, and liposarcoma. This case demonstrates that MR imaging is a valuable aid to the diagnosis and treatment of this disease by giving excellent definition of soft tissue and vascular structures, allowing accurate assessment and preoperative planning of the disease.
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6/35. Multiple symmetric lipomatosis: an unusual cause of childhood obesity and mental retardation.

    Multiple symmetric lipomatosis (MSL), also known as Launois-Bensaude syndrome or Madelung's disease, is a rare disorder predominantly seen in middle-aged male patients. The disorder is characterized by large subcutaneous fat masses distributed around the neck, shoulders, and other parts of the trunk, often associated with nervous system abnormalities. A close relationship to alcoholism, metabolic disturbances and malignant tumours has been observed. Until now, MSL has only been described in adults. We report on the first two children, a 9-year-old girl and a 13-year-old boy, with the characteristic clinical findings of MSL. The girl presented with severe obesity, developmental delay, mild mental retardation, peripheral neuropathy, and latent hypothyroidism. In addition, she had elevated lactate concentrations in blood and cerebral spinal fluid suggesting mitochondrial dysfunction. Biochemical analyses of muscle showed a respiratory chain complex II deficiency. The boy suffered from severe obesity, mild mental retardation and insulin resistant diabetes mellitus. In both children, analyses of the mitochondrial genome did not reveal major deletions nor the MERRF 8344 point mutation. MSL seems to be a new neurometabolic disorder with heterogeneous clinical expression whose pathogenesis is still unknown.
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7/35. Benign symmetrical lipomatosis of the hands.

    Benign symmetrical lipomatosis (BSL) is a disease characterized by massively symmetrical fat deposits predominantly in the neck and shoulder girdle area. The disease is frequently associated with alcoholism, hepatopathy, glucose intolerance, hyperuricemia, and malignant tumors. We describe a patient with BSL of the dorsal surfaces of both hands.
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8/35. Madelung's disease: case report and discussion of treatment options.

    Madelung's disease was first described in 1846. Since then, approximately 200 patients have been reported in the literature. Its three main characteristics-typical location of the lesions, symmetry, and the diffusive nature of the fat-are more often found in men of Mediterranean descent. A clear association with alcohol abuse is reported, but the etiology remains uncertain. patients usually complain of their cosmetic appearance, but treatment can be rendered for decreased neck motion and/or aerodigestive problems. Given the benign nature of the lesion, surgical debulking is the treatment of choice, with liposuction reserved for smaller lesions. A standard facelift pattern can be used for skin incisions and removal, with good cosmetic results.
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9/35. Ultrasound assisted liposuction for the palliative treatment of Madelung's disease: a case report.

    Benign Symmetric lipomatosis (Madelung's disease) is a rare disease, characterized by massive fatty deposits in the neck, the shoulders, and the upper extremities. The deformity is associated with chronic alcohol use, malignant tumors of the upper airways, neuropathy, diabetes mellitus, hyperlipidemia, and other metabolic disorders. Although the deformity is prone to recurrence, surgical removal via lipectomy or liposuction provides the only way of palliation. This paper describes the treatment of a 51-year-old man with a history of alcoholism and liver cirrhosis. He reported masses in his cervical and facial regions that had gradually enlarged over a period of 6 years. He also developed respiratory symptoms due to the fatty compression of his upper airways. Our experience with ultrasound-assisted liposuction for the palliative treatment of this disease is reported.
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10/35. Multiple symmetric lipomatosis (Madelung's disease): report of two cases.

    Multiple symmetric lipomatosis (Madelung's disease) is a rare disease characterized by non-encapsulated adipose deposits in the neck, the superior part of the trunk and, very rarely, in the limbs. It is common in middle-aged Caucasian Mediterranean males but very rare in Chinese people. We describe two cases of Madelung's disease: both patients presented complaining of a development of a painless neck mass that was increasing in size. Panendoscopy was normal. Both underwent incisional biopsies that revealed benign adipose tissue characterizing Madelung's disease. We discuss the clinical presentation, associated morbidity, and treatments for Madelung's disease.
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