Doença de Madelung: abordagem cirúrgica combinada de lipectomia e lipoaspiração / Madelung's disease: combined surgical approach of lipectomy and liposuction

A doença de Madelung (DM) ou lipomatose simétrica múltipla é uma patologia caracterizada pelo acúmulo de tecido adiposo não encapsulado e depositado simetricamente ao redor do pescoço e tronco superior (tipo I - forma mais comum). Sua etiologia ainda é pouco esclarecida, porém apresenta evidente associação com o consumo crônico excessivo de bebidas alcoólicas. As deformidades físicas são o que levam o paciente a buscar serviço médico, juntamente com eventuais sintomas de acometimento cervical como redução de mobilidade e afecções respiratórias. O diagnóstico da lipomatose simétrica múltipla é clínico, podendo ser complementado com exame de imagem para afastar demais hipóteses diagnósticas e avaliar a extensão do acometimento. O tratamento pode ser realizado por duas modalidades: clínico ou cirúrgico (lipectomia ou lipoaspiração). Relata-se o caso de paciente com lipomatose simétrica múltipla tipo I abordado cirurgicamente com ambas as técnicas: lipectomia cervical e lipoaspiração abdominal. Paciente evoluiu de maneira satisfatória, com redução de queixas e sem recidivas até o presente momento.

Madelung's disease or Multiple Symmetric Lipomatosis is a condition characterized by the accumulation of unencapsulated adipose tissue deposited symmetrically around the neck and upper trunk (type I - most common form). Its etiology is still unclear, but it is clearly associated with chronic excessive consumption of alcoholic beverages. Physical deformities lead the patient to seek medical care, along with possible symptoms of cervical involvement such as reduced mobility and respiratory disorders. Multiple Symmetric Lipomatosis diagnosis is clinical and can be complemented with imaging to rule out other diagnostic hypotheses and assess the extent of involvement. Treatment can be performed in two ways: clinical or surgical (lipectomy or liposuction). We report the case of a patient with Multiple Symmetric Lipomatosis type I surgically treated with both techniques: cervical lipectomy and abdominal liposuction. The patient evolved satisfactorily, with a reduction in complaints and no recurrences so far.

Two Cases of Multiple Symmetric Lipomatosis in Women with Alcoholism / 대한피부과학회지

Madelung's disease, also known as multiple symmetric lipomatosis (MSL) is a rare disease characterized by the presence of multiple, symmetric, loose adipose tissues distributed around the neck, shoulder, back or chest. MSL is different from simple obesity, which is characterized by the presence of well-distributed total body fat. It is rarely reported in Asia and most commonly seen among middle-aged men. Although the etiology of MSL is unknown, the disease is associated with alcoholism in 60% to 90% of patients. The diagnosis is usually made on the basis of the history, and clinical appearance. Also, imaging such as CT or MRI could confirm the diagnosis. Herein, we experienced an uncommon case of MSL in middle-aged women with alcoholism.

Do you know this syndrome? Type 2 benign symmetric lipomatosis (Launois-Bensaude)

Abstract A 57-year-old female showed bulky, loose tumors, which progressively spread to her arms, anterior chest, and back. She reported dysphagia and dyspnea after mild exertion. She denied alcohol consumption. CT scan of her chest showed no internal lesions. Benign symmetric lipomatosis is a rare syndrome, clinically described as multiple nonencapsulated lipomas of various sizes and symmetrical distribution. This syndrome has three known phenotypes; in type 2 (Launois-Bensaude syndrome), lesions occur primarily on the shoulders, upper arms, and chest, and is unrelated to alcoholism. It causes aesthetic deformities and might block the upper airways. Mediastinal invasion might occur as well.

Fast-growing multiple symmetric lipomatosis / 영남의대학술지

Multiple symmetric lipomatosis (MSL) is a rare disease characterized by symmetrical massive fatty deposits on the face, neck, shoulders, and upper trunk. We report a 74-year-old man who complained of painless subcutaneous nodules on his posterior neck that developed 3 weeks earlier. In a week, variably-sized similar lesions developed on both his shoulders and upper extremities. At the time of his hospital visit, several firm nodules as big as a walnut to a child's fist and with the normal-skin-hue were observed on his posterior neck, both shoulders, upper extremities, and trunk. The histological examination of his upper left arm revealed more mature adipocytes without encapsulation in the subcutaneous tissue. MSL was generally known to occur slowly over months or years. However, this is an unusual case that showed a fast-growing nature.

Multiple Symmetric Lipomatosis (Madelung's Disease) of the Neck and Pharynx / 대한이비인후과학회지

Multiple symmetric lipomatosis (Madelung's disease) is an infrequent disease of unknown etiology. The typical characteristic of the disease is bulging, symmetric masses of fat tissue on the neck, upper extremities, and upper parts of the trunk. The disease strongly affects middle-aged men, people from Mediterranean regions and with alcohol abuse history. We report three cases of middle to old age males with long-standing multi-lobulated bulging of the neck, which gradually enlarged over many years. Of peculiar interest is one patient who presented with snoring and apnea together with typical findings of Madelung's disease. The case had involvement of the hypopharynx, which was successfully managed with microscopic laryngeal surgery with CO2 laser. The authors elucidate three cases of Madelung's disease found in the pharynx and neck, and report a review of the literature.

Lipomatose simétrica múltipla: relato de dois casos / Multiple symmetric lipomatosis: report of two cases

Multiple symmetric lipomatosis is an unusual disease of unknown etiology. It is characterized by symmerical accumulation of fat tissue deposits in neck, face, trunk and proximal limbs. It is more common in mole adult subjects with a history of alcohol intake. Paliative surgery is the main treatment and it can be performed by conventional open excision or liposuction. Recurrence is common. The objetive of this article is to report two cases of multiple symmetric lipomatosis treated by liposuction in Regional Hospital of Asa Norte, in Brasilia - Federal District...

A lipomatose simétrica múltipla é uma doença incomum, de etiologia desconhecida, caracterizada pelo acúmulo simétrico de depósitos de tecido adiposo em face, pescôço, tronco e região proximal dos membros. É mais frequente em indivíduos adultos do sexo masculino, geralmente com história de etilismo. O tratamento é cirúrgico, com ressecção paliativa por via aberta ou por lipoaspiração. A recidiva é comum. O presente artigo tem por objetivo relatar dois casos de lipomatose simétrica múltipla, tratados por lipoaspiração no Hospital Regional da Asa Norte, em Brasília-DF...

Doença de Madelung: relato de caso e revisão da literatura / Madelung's disease: a case report and literature review

A doença de Madelung é caracterizada por massas simétricas de tecido adiposo no pescoço, ombros, braços e parte superior do tronco. A tomografia computadorizada demonstra achados de imagem característicos e é considerada o método de escolha para diagnóstico, estadiamento pré-operatório e acompanhamento pós-cirúrgico. Relatamos o caso de um homem com aspectos tomográficos típicos da doença de Madelung.

Madelung's disease is characterized by the presence of symmetric masses of adipose tissue on the neck, shoulders, arms and the upper trunk. Computed tomography demonstrates characteristic imaging findings, and it is considered to be the method of choice for diagnosis, pre-operative staging, and postoperative follow-up. The authors report the case of a man with typical tomographic findings of Madelung's disease.

Multiple Symmetric Lipomatosis: Characteristics and Treatment in Korean Patients

PURPOSE: Multiple symmetric lipomatosis (MSL) is a relatively rare disorder characterized by presence of multiple, symmetric, nonencapsulated fat masses in face, neck, shoulder and other areas. There has been only a few cases reported in Korea. The main purpose of this research is to examine the Korean patients to see what kinds of special characteristics occurred due to this disease and to decide the proper treatment. METHODS: A total of 16 patients were evaluated retrospectively. 5 patients were treated at our hospital. The other patients were reviewed from literature. We analyzed the biological characters of patients, location of fat deposit, morphologic characters of patients, clinical evidence of neuropathy, associated metabolic disorders and treatment modality. RESULTS: All cases were male patient. The mean age of onset was 47.43 years. All patients were moderate to heavy alcoholics. The most common location of fat deposition was posterior neck and abdomen. In neurologic exam of 9 patients, 5 patients showed muscle weakness, tremor, pain and autonomic nerve dysfunction. In metabolic studies of 9 patients, total cholesterol values were higher in 1 patient. A glucose tolerance test was abnormal in 1 patient. In treatment modality, 14 patients were treated with surgical resection, 1 patient was treated with liposuction and surgical excision, 1 patient was treated only with liposuction. CONCLUSION: To treat MSL patients successfully, we should concentrate not only on the removal of the fatty tissue but also on neurologic abnormities, metabolic disorders and associated diseases.

Multiple Symmetric Lipomatosis with Peripheral Neuropathy: A case report

Multiple symmetric lipomatosis is a rare disorder characterized by massive fatty deposits arranged symmetrically around the neck, shoulder, abdomen and back. It is typically associated with high alcohol consumption and a high prevalence of peripheral neuropathy. The pathogenesis of the syndrome is still unknown, but mitochondrial abnormality or metabolic abnormalities are occasionally found in the affected patients. In our patient, clinical and electrophysiologic signs of a generalized peripheral sensorimotor neuropathy and a multiple bilateral lumbosacral radiculopathy were observed. Sural nerve biopsy demonstrated many small unmyelinated fibers with complete loss of axoplasm and a extensive loss of myelinated fibers. Lipoma biopsy demonstrated non-capsulated mature adipose cells in the subcutaneous tissue. Serum lipid studies were normal. MERRF point mutation of mitochondrial DNA were negative in blood. We reported a case of multiple symmetric lipomatosis and peripheral polyneuropathy with the review of literature.

A case of multiple symmetric lipomatosis with acute renal failure due to pulmonary thromboembolism / 대한내과학회지

Multiple symmetric lipomatosis (MSL) is a rare disease characterized by the presence of diffuse, non-tender, symmetric deposits of adipose tissue on the neck, back and upper part of the trunk. It has been reported that this disease could be associated with various metabolic abnormalities such as impaired glucose tolerance, hyperuricemia, alcohol consumption, type 4 hyperlipidemia, renal tubular acidosis and thyroid disorder. We present a case of type 2 MSL with pulmonary thromboembolism. He suffered sudden dyspnea and decreased urine output. The causes of dyspnea and acute renal failure (ARF) in this case were dehydration and hemodynamic disturbance due to pulmonary thromboembolism. After thrombolytic and fluid therapy, his condition was improved. He was discharged on warfarin medication and has remained asymptomatic for 12 months.

A Case of Multiple Symmetric Lipomatosis / 결핵

Multiple symmetric lipomatosis ( Madelung's disease ) seems to represent a relatively common disease in Europe but has not been described in the Korean literature. It is characterized by highly typical symmetric deposits of subcutaneous fat on the neck, arms, and upper part of the trunk. The localized increase of adipose tissue is hyperplastic in nature and probably results from a local defect in adrenergic stimulated lipolysis. Multiple symmetric lipomatosis is frequently, but inconstantly, associated with alcoholism, liver disease, glucose intolerance, and hyperuricemia, but their etiologic role remains to be established. Clinical importance is the apparent high incidence of malignant tumors of the upper airways requiring through ear, nose, and throat and internal examination of all patients. Another importance is possibility of airway obstruction due to mass effect. Dietary treatment and weight loss are of limited value. Surgical treatment gives only temporary relief and should be restricted to patients with functional impairment. We report a case of a 63-year-old man with multiple symetric lipomatosis and alcoholism.

Multiple Symmetric Lipomatosis (Cervical Lipomatosis): Two cases report

Multiple symmetric lipomatosis is a rare disease and affects almost exclusively middle aged man, usually with a background of excessive a alcohol intake. The disease is characterized by progressive growth of subcutaneous fat masses which are located symmetrically at neck, shoulders, chest, abdomen and groin, and which subsequently penetrate deeply into the surrounding spaces and structures with symptomatic compression of deep organs, such as trachea. A recent survey revealed a high incidence of sometic and autonomic neuropathy. The exact cause of the disease is not known, but a hyperplastic mechanism has been postulated, with in vitro studies demonstrating a defect in adrenergic-stimulated lipolysis of lipomatous tissue. We have experienced two cases of multiple symmetric lipomatosis. Case 1 was a 59-year-old male, complaining of slowly enlarging doughunt ring-shaped mass at his neck. He had a habit of excessive alcohol intake for many years. The subcutaneous mass at the neck was excised. The pathology report described the specimen as "normal adipose tissue". Case 2 was a 49-year-old male, complanining of slowly enlarging multiple symmetric masses at the neck, shoulders, chest, abdomen, flank and groin over a period of 6 years. He also complained of mild muscular weakness. He had a habit of excessive alcohol intake for many years. The subcutaneous mass in the neck was excised. The specimen had a tendency to form globular masses and microscopically indistinguishable from mature adipose tissue.

Multiple symmetric lipomatosis

Multiple symmetric lipomatosis (MSL) is an extremely uncommon disorder. In the medical literatures about 200 cases have been reported. MSL is not associated with other generalized lipomatous disorders, nor are these patient to be necessarily obese. The cause of MSL is unknown. The disorder usually occurs in middle-aged males and there is frequently a history of alcoholism. Some instances of familial occurrence have been reported, but the majority of cases are sporadic. Two cases of MSL are presented.