Madelung's Disease: Revision of 59 Surgical Cases.

BACKGROUND: Madelung's disease (MD) is a rare disease, more common in Mediterranean countries and associated with alcohol abuse. However, MD etiology remains not completely understood. OBJECTIVE: The aim of this study was to investigate clinical features, treatment options and outcomes in patients with MD treated in our plastic surgery department. METHODS: We retrospectively reviewed 59 cases of MD operated on between 2004 and 2013. Demographic information, location of the deposits, associated pathology and habits, number and type of surgeries performed, surgical complications and disease evolution were analyzed. RESULTS: Ninety percent of the patients were males. Active or past history of alcohol abuse was reported by 86%. The main affected areas were anterior and posterior neck. A total of 230 surgical procedures were performed. Open surgery was used on 192 occasions (83.5%), liposuction alone on 30 procedures (13%) and lipectomy combined with liposuction on 8 interventions (3.5%). Surgical complications were found in 41 procedures (17.8%). Twenty-three patients (39%) were identified as having disease recurrence of the operated site in a mean time of 3.8 years. Alcohol consumption was not clearly associated with disease recurrence. CONCLUSIONS: Demographic characteristics of the studied cohort stand for published data. It is our opinion that lipectomy/dermolipectomy provides better aesthetic and functional results. Lipectomy procedures allow a thorough excision, correct identification of noble structures and careful hemostasis. Liposuction techniques, even ultrasound-assisted ones, had limited efficacy for the treatment of large masses. A long follow-up period is recommended considering the high propensity and mean time to recurrence. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Multiple symmetric lipomatosis - a reflection of new concepts about obesity.

Multiple symmetric lipomatosis (MSL) is characterized by subcutaneous accumulation of nonencapsulated adipose tissue. In type 2 MSL accumulation occurs on proximal limbs, upper back and hips. This sometimes unrecognized disease is similar to an exaggerated female fat distribution and can be confused with simple obesity. Obesity is a heterogeneous disorder and we suppose that type 2 MSL might have a place on the edge of the obesity spectrum. Several contemporary concepts about adipose tissue could be recognized in the model of MSL. Changes in fat distribution among different depots of adipose tissue in obesity have emerged as origin of its metabolic complications. Decreased insulin resistance and raised adiponectin have been found in MSL just as in some other conditions with accumulation of the subcutaneous adipose tissue (SAT). In that context, MSL may present as a model for possible favourable metabolic impact of SAT depots. Adipogenesis in MSL is not a consequence of energy excess but it is an active hyperplastic proliferation of SAT. This kind of behaviour of some adipocytes in several subcutaneous areas in MSL suggests that the energy unrelated adipogenesis could contribute to the expansion of at least a part of SAT depot in obesity in general. Contrary to current concept that the signals for adipogenesis are dependent only on the energy equation, allowing this additional mechanism would imply a new approach to issues of obesity, foremost to differentiate its particular types for which these concepts may be relevant.

[Multiple symmetrical lipomatosis: report of two different clinical types and review of the literature]. / Lipomatosis simétrica múltiple: presentación de dos formas clínicas diferentes y revisión de la literatura.

Multiple symmetrical lipomatosis or Lauwnois-Bensaude disease is a rare entity characterized by the development of subcutaneous fat masses around the neck shoulders and upper extremities. Peripheral neuropathy is a common finding as well as the association with alcholism, hepatopathy and glucose intolerance. Pathogenesis is unknown. We describe three cases with two different clinical types and review the data reported in medical literature.

Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study.

BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by the growth of uncapsulated masses of adipose tissue. MSL is associated with high ethanol intake and complicated by somatic and autonomic neuropathy and by the infiltration of the adipose tissue at the mediastinal level. To date, the disease is considered as slowly progressive, but long-term longitudinal data are still lacking. In this study, a long-term follow-up of a large series of MSL patients is presented. METHODS: We studied 31 patients with MSL (30 males and one female) first evaluated at our institution from 1973 to 1992. All patients were followed until 1998-1999 or until death, with a mean follow-up of 14.5+/-5.0 y (range 4-26 y). Both at baseline and during follow-up, the location and the size of the subcutaneous lipomatous fat depots, the presence and the extension of deeply localized lipomatous tissue, and the presence and the severity of both somatic and autonomic neuropathy were evaluated. RESULTS: Eight MSL patients died during follow-up (25.8% of patients). A sudden death was proved to be the cause of death in three patients. All these three patients had severe autonomic neuropathy and none had coronary disease, acute myocardial infarction or other cardiac abnormalities. No signs or symptoms of coronary heart disease were present in the whole series. In addition to this high fatality rate, a substantial morbidity related to the occupation of the mediastinal space by the lipomatus tissue and to somatic neuropathy was also observed. CONCLUSIONS: MSL is associated with a significant morbidity and mortality. Therefore, the definition of 'benign symmetric lipomatosis', still adopted by several authors, cannot be justified.

Long-term results following surgical treatment of benign symmetric lipomatosis (BSL).

Benign symmetric lipomatosis is a rare disease. Its main characteristic is the symmetric deposition of fat, in an unencapsulated form, at typical sites in the body, but primarily in the head and neck region. Its aetiopathogenesis is unknown. A disturbance of lipid metabolism is involved, and there is a proven connection with chronic alcoholism. Problems with the differential diagnosis of the characteristic clinical picture arise from the fact that this disease is not widely recognized. Over a period of 30 years, the authors treated 31 patients with benign symmetric lipomatosis. They review the pertinent literature and report retrospectively on the fates of the 11 surviving patients.

Mitochondrial toxicity induced by nucleoside-analogue reverse-transcriptase inhibitors is a key factor in the pathogenesis of antiretroviral-therapy-related lipodystrophy.

Highly active antiretroviral therapy (HAART) can induce a characteristic lipodystrophy syndrome of peripheral fat wasting and central adiposity. HIV-1 protease inhibitors are generally believed to be the causal agents, although the syndrome has also been observed with protease-inhibitor-sparing regimens. Here, we postulate that the mitochondrial toxicity of the nucleoside-analogue reverse-transcriptase inhibitors plays an essential part in the development of this lipodystrophy, similar to the role of mitochondrial defects in the development of multiple symmetrical lipomatosis.

[Lipoma, multiple lipomas and lipomatosis]. / Lipomes, lipomes multiples et lipomatoses.

Lipomatous tumors are very frequent; simple lipoma is the most common variety. According to Enzinger, lipomatous tumors are classified into five different groups: simple lipomas, variant lipomas forms, heterotopic lipomas, infiltrating lipomas and lipomatosis, and finally hibernomas. Usually, lipomatous tumors are characterized by a slow, unpainful, growth. Classical treatment includes surgical resection. The clinical diagnosis is confirmed by histology. Liposucion can be a therapeutic option in certain cases.

Síndrome de Madelung - relato de um caso e revisäo de literatura / Madlung's syndrome - case report and literatura review

A Síndrome de Madelung é um tipo raro de lipodistrofia descrita pela primeira vez em 1888 por Otto Madelung. Deste entäo, apenas 200 casos foram relatados na literatura. A condiçäo, de etiologia ainda desconhecida, caracteriza-se por uma deposiçäo de tecido adiposo simetricamente em: pescoço, ombros, regiöes supraclaviculares, suboccipital, tronco e regiäo proxima dos membros superiores, determinando um desfiguramento progressivo do doente. Embora usualmente assintomática, a doença pode causar complicaçöes severas, tais como: compressäo traqueal, laríngea ou mediastinal. O diagnóstico diferencial deve incluir outras lesöes do tecido celular cutâneo, a saber: lipomas, angiolipomas, neurofibromas, sarcomas, bem como outras lipomatoses. A excisäo cirúrgica é o único método efetivo de tratamento, sendo a operaçäo em geral trabalhosa devido à natureza hipervascularizada e fibrosa do tecido lipomatoso. Relata-se um caso de Síndrome de Madelung que está sendo acompanhado pelo serviço de Cirurgia Geral do HC-UFPR. O paciente, alvo de olhares curiosos pelo seu aspecto, vem sendo submetido a dermolipectomias e lipoaspiraçöes periódicas

[Launois Bensaude disease. Focus apropos of 16 cases]. / Maladie de Launois Bensaude. Mise au point à propos de 16 cas.

After a review of the clinical, pathological and aetiological features, the authors describe the various surgical techniques used (conventional surgery or liposuction) to restore a normal social life to these patients, deformed by their disease. They confirm the predominant role of conventional surgery and define the limited indications in which liposuction can be used. Based on a review of 16 cases, the surgeon must make sure that the patient has ceased his or her alcohol abuse before performing this difficult treatment associated with a number of complications.

[Classification and terminological designation of Madelung's lipomatosis]. / Klassifikatsiia i terminologicheskoe oboznachenie lipomatoza Madelunga.

From experience in the treatment of 18 patients with Madelung's lipomatosis the authors suggest a system of designation of the spread of the disease embracing the possible localizations of the tumor. The system of recording the spread and marked character of the adipose tumors makes it possible to appraise the developmental stage of the disease. It is suggested that 4 stages of the disease are distinguished. According to the type of growth, two forms of tumors are distinguished: nodular and diffuse. Systemic designation and the form of the tumor give a more complete idea of the disease and thus allow the optimal tactics of surgery to be chosen. Among the numerous terms suggested for the disease, the most appropriate, reflecting the essence of the disease is "Madelung's lipomatosis". The authors suggest it for unification of the designations of the disease and easier literature search and generalization of information on this rare disease.

[Benign symmetrical lipomatosis with predominant localization in the neck area (Madelung's disease)]. / Dobrokachestvennyi simmetrichnyi lipomatoz s preimushchestvennoi lokalizatsiei v oblasti shei (bolezn' Madelunga).

Eighty-eight males with benign symmetrical lipomatosis (BSL) were subjected to complex examination, 68 of them were operated on. BSL is a slowly progressing disease marked by the development of nonencapsulated symmetrical fatty masses mainly in the region of the neck. It usually occurs in males at the age of 35-50 years. Chronic alcoholic intoxication was found in 46 (48%) patients, 36 (41%) patients had various functional disorders which caused invalidation in 5 (5.7%) cases. A familial character of the disease was revealed in four (4.5%) cases. A classification of the disease is suggested which takes into account the functional disorders and the degree of development of the lipomatous masses in the neck. The author discusses the stages of the treatment, the surgical anatomy, a method for revealing the salivary glands in the lipomatous tissue by staining them with methylene blue solution, the operative techniques, the complications and their prevention, and the late results of surgical treatment. Satisfactory esthetic and functional results were achieved in 94.2% of patients who were operated on.

[Benign symmetric Launois-Bensaude type III lipomatosis and Bureau-Barrière syndrome]. / Benigne symmetrische Lipomatose Launois-Bensaude Typ III und Bureau-Barrière-Syndrom.

We report on a patient with an unusual distribution of benign symmetric Launois-Bensaude lipomatosis. In addition, the patient presented with Bureau-Barrière syndrome. With regard to our own observations and those reported in the literature, we propose a new classification of the disease referred to the distribution of the fatty tissue.

Lipomatosis: a propósito de nuestras observaciones / Lipomatosis: for the purpose our observations

Los lipomas son tumores únicos o múltiples, desarrollados a partir del tejido adiposo. En sus formas múltiples constituyen las "lipomatosis", dando origen a cuadros clínicos raros y complejos. Se reconocen los siguientes cuadros: 1) Enfermedad de Dercum, afecta fundamentalmente a mujeres perimenopáusicas obesas, con disendocrinias, presentando tumores dolorosos en tronco y miembros; 2) Adenolipomatosis o Enfermedad de Launois-Bensaude, con afectación de hombres, disponiéndose en cuello, ingle y axilas, remedando adenopatías, 3) Lipomatosis mesosomática de Roch-Lery, con compromiso exclusivo de tronco y 4) Enfermedad de Touraine-Renault, disponiéndose en forma segmentaria o zoniforme

Lipomatosis: a propósito de nuestras observaciones / Lipomatosis: for the purpose our observations

Los lipomas son tumores únicos o múltiples, desarrollados a partir del tejido adiposo. En sus formas múltiples constituyen las "lipomatosis", dando origen a cuadros clínicos raros y complejos. Se reconocen los siguientes cuadros: 1) Enfermedad de Dercum, afecta fundamentalmente a mujeres perimenopáusicas obesas, con disendocrinias, presentando tumores dolorosos en tronco y miembros; 2) Adenolipomatosis o Enfermedad de Launois-Bensaude, con afectación de hombres, disponiéndose en cuello, ingle y axilas, remedando adenopatías, 3) Lipomatosis mesosomática de Roch-Lery, con compromiso exclusivo de tronco y 4) Enfermedad de Touraine-Renault, disponiéndose en forma segmentaria o zoniforme