Scrotum involvement in Madelung's disease: a case report / 中华泌尿外科杂志

Madelung's disease is more common in male patients who drink alcohol. It can affect many parts of the body, but rarely affects scrotum. A case of Madelung's disease involving the scrotum was reported. The scrotum tumor was removed by operation and good results were obtained. No recurrence was found in the follow-up of 14 months. Surgical resection could be an effective treatment for this disease.

A case report of Type Ⅰ Madelung disease and literature revive / 中华整形外科杂志

A male patient, with diagnose of Madelung disease, was admitted in September 2009. He has been a heavy drinker for decades before onset of the disease. This patient was characterized by the large amount of symmetrical deposits of adipose tissue in the subcutaneous layer around neck, and without obesity on other sites. The excessive adipose tissue was surgically removed by three steps. Appearance almost returned to normal. No recurrence happened after 8 years of follow-up.

Research progress in Madelung′s disease / 中华整形外科杂志

Madelung′s disease is a rare lipid metabolic disorder with unclear mechanism, characterized by the formation of diffuse uncapsulated lipomas in face, neck, shoulder and other body areas. This disease mainly affect middle-aged male, and is related to alcohol abuse. The treatment nowadays is only palliative surgery with a high recurrence rate, including lipectomy and liposuction. Both of them have advantages and disadvantages.

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.

Relato de caso: doença de Madelung / Case report: Madelung's disease

A síndrome de Madelung ou também conhecida por lipomatose simétrica múltipla (LSM) é uma lipodistrofia benigna rara que se caracteriza por múltiplas massas adiposas não encapsuladas. Ainda de etiologia desconhecida, existe a suspeita de uma possível herança autossômica dominante. Está fortemente associada ao abuso de álcool (60-90% dos casos). Apresenta curso clínico variável, de crescimento rápido com posterior estabilização ou progressão lenta. Pode ser assintomática ou causar sintomas de compressão de estruturas mediastinais e do trato aerodigestivo. O tratamento de escolha é cirúrgico (AU)

Madelung syndrome, also known as multiple symmetric lipomatosis (MSL), is a rare benign lipodystrophy that is characterized by multiple non-encapsulated adipose masses. Although of unknown etiology, there is a suspected autosomal dominant inheritance. It is strongly associated with alcohol abuse (60-90% of cases). It presents a variable clinical course, of fast growth with later stabilization or slow progression. It may be asymptomatic or cause symptoms of compression of mediastinal structures and aerodigestive tract. The treatment of choice is surgical (AU)

Imaging Features of Madelung's Disease: Case Report

Madelung's disease, or benign symmetric lipomatosis, is an uncommon disorder that is characterized by massive symmetrical deposits of adipose tissue in the upper trunk, neck and head, and is usually associated with alcohol abuse; as such, patients usually complain of cosmetic issues. Historically, Madelung's disease is usually encountered in men between 30 and 60 years of age, and is more prevalent in the Mediterranean population. In this case study, we describe a rare case of Madelung's disease, in an Asian patient who presented with symmetrically located bilateral masses in the anterior neck, which grew progressively larger over a period of seven years.

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.

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

Relata-se o caso de um paciente portador da doença de Madelung, definida clinicamente pela presença de múltiplos acúmulos de tecido adiposo não encapsulados, usualmente envolvendo a região cervical e superior do tórax, de distribuição simétrica. O exame de imagem eleito para confirmação do diagnóstico foi tomografia computadorizada, por ser menos dispendiosa e mais acessível, comparando-se à ressonância magnética.

The authors report the case of a patient with Madelung's disease, which is clinically described as the presence of multiple and symmetric non-encapsulated masses of fatty tissue, usually involving the neck and the upper region of the trunk. Computed tomography was selected as the imaging method for diagnosis confirmation, considering its lower cost and higher availability as compared with magnetic resonance imaging.

Doença de Madelung. Relato de caso / Madelung's disease. Case report

JUSTIFICATIVA E OBJETIVOS: A doença de Madelung, também conhecida como lipomatose simétrica benigna (LSB), é um tipo raro de lipodistrofia que se caracteriza por deposição simétrica de tecido adiposo no pescoço, porção superior do tronco e membros, determinando um desfiguramento progressivo do paciente. De etiologia desconhecida, a doença acomete principalmente homens, etilistas e, embora usualmente assintomática, pode causar complicações graves, tais como compressão traqueal, laríngea ou mediastinal. O objetivo deste estudo foi relatar um caso de doença de Madelung, bem como sua possível patogênese e tratamento. RELATO DO CASO: Paciente do sexo masculino, 45 anos, etilista, cardiopata, com lipomas distribuídos simetricamente na região cervical e superior do tórax. Exames gerais sem alterações e tomografia cervical com diminuição discreta da luz traqueal. CONCLUSÃO: A LSB, relatada como doença benigna, pode originar massas volumosas e sintomas compressivos de vias aéreas, conferindo prognóstico reservado. Ressalta-se a importância da suspeição diagnóstica, que é feita à ectoscopia.

BACKGROUND AND OBJECTIVES: Madelung's disease, also known as benign symmetric lipomatosis (BSL), is a rare type of lipodystrophy characterized by symmetric deposits of adipous tissue in neck, upper trunk and limbs, determinating a progressive disfigurement of the patient. Of unknown etiology, this disease occurs usually in men, alcoholist and, despite asymptomatic, it may lead to severe complication - as tracheal, laryngeal and mediastinal compression. The objective of this study was report a Madelung's disease case, as well as the possible pathogenesis and treatment. CASE REPORT: An alcoholist and cardiopatic 45 year-old male patient, with symmetric lipomas in the cervical region and upper part of trunk, and a light tracheal compression, shown in cervical tomography. CONCLUSION: The BSL, although considered a benign disease, it can generate bulky masses and airways compression symptoms, and so a bad prognosis. Because of it, the clinical suspection becomes important. It is suggested by ectoscopy.

Lipomatose simétrica benigna: doença de Madelung: relato de caso / Benign symmetric lipomatosis: Madelung's disease: case report

JUSTIFICATIVA E OBJETIVOS: A lipomatose simétrica benigna (LSB) foi descrita inicialmente por Madelung em 1888 e possui patogênese ainda desconhecida. É caracterizada por múltiplos depósitos de gordura indolor não encapsulado com disposição simétrica, localizados preferencialmente na região cervical e tronco superior, podendo causar sintomas compressivos e aparência pseudoatlética. O tratamento cirúrgico é o mais efetivo, por ressecção ou por lipossucção. O objetivo deste relato foi apresentar a importância do caso descrito, devido à sua exuberância clínica, apesar de assintomática e sem alterações significativas laboratoriais.RELATO DO CASO: Paciente do sexo masculino, 77 anos, agricultor, natural de Pernambuco. Relatou que há oito meses apresentava crescimento progressivo de massa indolor na região cervical, occipital e superior do tórax, negando outros sintomas que pudessem acompanhar o crescimento e sem história familiar. Hipertenso, etilista e tabagista. Ao exame constatou-se massa volumosa simétrica cervical fibroelástica, na região cervical, não aderida a planos profundos e sem sinais flogísticos.CONCLUSÃO: A LSB foi classificada por Enzi e col. em dois tipos, o paciente em questão foi classificado como tipo I, lipoma predominantemente cervical. A ingesta de álcool consumida pelo paciente tem forte associação com a etiologia da doença de acordo com Enzi e col. Foi realizada lipectomia, com abordagem em dois tempos cirúrgicos devido a invasão de estruturas nobres. O paciente evoluiu bem clinicamente, sem complicações, com bom resultado estético. O presente caso apresentava uma exuberância clínica não condizente com os parâmetros laboratoriais e sintomatologia descrita na literatura.(AU)

BACKGROUND AND OBJECTIVES: Benign symmetric lipomatosis (BSL) was first described by Madelung in 1888 and still has unknown pathogenesis. It is characterized by multiple unencapsulated painless fat deposits with symmetrical disposition, preferably located in the cervical region and upper trunk and may cause compressive symptoms and pseudoathletic appearance. Surgical treatment is most effective, and it could be done by resection or liposuction. The objective of this study was to present the importance to describe the case due to your clinical exuberance without symptoms or significant changes in laboratory tests.CASE REPORT: Male patient, 77 years old, farmer, born in Pernambuco. Reported that 8 months ago began progressive growth of a painless mass in the cervical region, occipital region and upper chest, with no family history. He has high blood pressure, alcoholic and smoker. On examination, it could be noticed a large fibroelastic symmetric mass, asymptomatic, in the cervical region, not adhered to deep planes and with no signs of inflammation.CONCLUSION: The benign symmetric lipomatosis was rated by Enzi et al. in two types, the patient in this case was classified as type I, predominantly cervical lymphoma. The intake of alcohol consumed by the patient is strongly associated with the etiology of this disease, according to Enzi et al. The patient underwent a lipectomy with a two steps approach due to surgical invasion of vital structures. The patient was stable clinically, without complications, with good aesthetic results. The clinical case described had an exuberance of clinical manifestations, not consistent with laboratory parameters and symptoms described in the literature.(AU)

Benign symmetric lipomatosis (madelung’s disease): A case report of new variant.

Benign symmetric lipomatosis (BSL) is characterized by the presentation of numerous symmetric non-tender fatty tumors in the neck, suboccipital region, proximal extremities and upper parts of the trunk, typically noted as a “pseudoathletic” appearance. The etiology of BSL remains unknown but mitochondrial mutations have been found in some patients. The mitochondrial cytopathy was suggested to be studied in all BSL patients especially those with neuropathy. We have presented a case of BSL with reverse fat distribution, which we named the “Pop-eye” appearance in which the fatty tumor was distributed at both the distal part of the upper extremities and the proximal part of the lower extremities. A study of the mitochondrial tRNA lysine gene did not reveal mutation at position 8344.

Madelung's disease: A case report and literature review / 实用口腔医学杂志

A patient suffering Madelung's disease was treated and the literatures were reviewed in this article. The cause, the clinical features and treatment were discussed. The cause of this disease is unknown, and it is characterized by symmetrical accumulation of fat around the neck,nape and armpit having a appearance of "horse collar"or "hump back". Histological analysis revealed typical fat tissue depositing around the structures. Surgery is the main means of treatment, and prognosis is good.

Lipomatose simétrica múltipla (doença de Madelung): relato de caso / Multiple symmetrical lipomatosis (Madelung's disease): a case report

Os autores relatam um caso de um paciente com doença de Madelung com os resultados do seu tratamento. A doença de Madelung é uma doença rara de etiologia desconhecida. Esta condição é caracterizada pela deposição de múltiplas massas de tecido adiposo localizadas na face, região cervical e tronco superior. Essas massas benignas não possuem cápsula e estão distribuídas de forma simétrica. Existem apenas 200 casos notificados na literatura médica e, geralmente, afeta homens com antecedentes alcoólicos. A maioria dos pacientes afetados apresentam possível herança autossômica e são de países do Mediterrâneo. O diagnóstico é feito pela história clínica, constatação física das massas com a característica distribuição, biópsia e tomografia computadorizada. A cirurgia é o tratamento de escolha, com lipectomia e/ou lipoaspiração (AU)

The authors report the case of a patient with Madelung's disease and the treatment results. Madelung's disease is a rare disease of unknown etiology. This condition is characterized by the growth of fatty masses around the face, back of the head, neck and upper arms. These benign fatty masses are not enclosed within a membranous capsule and are symmetrically distributed. There are only 200 cases reported in the medical literature and it usually affects males with an alcoholic background. Affected patients present possible autosomal inheritance and come from Mediterranean countries. The diagnosis is made through clinical history, physical evidence of fatty masses with the characteristic distribution, biopsy and CT scan. Surgery is the treatment of choice, with lipectomy and/ or liposuction (AU)

Experience of Benign Multiple Symmetrical Lipomatosis

Benign symmetrical lipomatosis, also known as Madelung's disease, is a rare condition characterized by massive fatty deposits arranged symmetrically around the neck, shoulders, and arms. Patients usually complain of their cosmetic appearance, but treatment can be considered for decreased neck motion and/or aerodigestive problems. Surgical lipectomy and liposuction are treatment of choice, but recurrence is common. Because the distribution of the lesion tends to be wide and diffuse, single operation is not sufficient for improving contour and functional problems, especially in severely affected case. We experienced a progressed patient who suffered from limitation of neck motion, dyspnea including sleep apnea due to massive deposits on whole neck. We performed 2 staged operations for the purpose of removing as much lipomatous tissue as possible. After 2 staged excisions, patient's appearance and aerodigestive problems are much improved and recurrence is not observed.

Treatment of Beign Multiple Symmetrical Lipomatosis with Liposuction

PURPOSE: Benign symmetrical lipomatosis(Madelung's disease) is a disease of uncertain etiology that manifests as massive lipomatous deposits in specific area of the body. It is usually located on the neck, shoulder, proximal regions of the extremities and the abdomen. Madelung's disease is found many in middle-aged, Mediterranean man. Imaging using either computed tomography or magnetic resonance imaging is often recommended. Dietary restriction provides no relief of disease. Abstinence with alcohol may delay further progression but does not regression tumor mass. The objective of this study is to prove effectiveness of liposuction for treatment of benign lipomatosis. METHODS: We conducted liposuction as a treatment for benign lipomatosis. After general anesthesia, We conducted power assisted liposuction and amount of suctioned volume was about 2500 cc. After surgery, we did compressive dressing and then followed by pressure garment. RESULTS: Six months after surgery, patient was examined for follow up at outpatient department. Recurred lipoma was not observated. Patient was satisfied with result. CONCLUSION: The only effective therapy for Madelung's disease is surgical removal but recurrence could be occurring. Also multiple surgical scars will be visible. We report a patient with multiple large lipomatosis successfully treated with liposuction.

Surgical Treatment of Two Cases of Benign Symmetrical Lipomatosis Causing Dyspnea / 대한이비인후과학회지

Benign symmetrical lipomatosis, known as Madelung's disease, is a rare disease characterized by non-encapsulated fat tissue accumulation in a symmetrical manner around the neck, shoulders, and upper extremities, rarely involving the lower limbs and the lower body. To date, there have been approximately 200 cases reported. We experienced two cases of Madelung's disease, 57 and 59 year-old man with neck and shoulder swelling and the symptom of dyspnea. Laryngoscopic and CT scan findings showed severe narrowing of supraglottic and paraglottic area, caused by fat tissue accumulation. The fat tissues were surgically excised, one by external approach and the other by laryngomicrosurgery using LASER under direct laryngoscope, with successful result. We report it with a review of the current literatures.

Benign Symmetrical Lipomatosis

Benign symmetrical lipomatosis(BSL) is a rare disease of unknown origin characterized by symmetrical and diffuse deposits of nonencapsulated fatty tissue. It is located on the neck, the shoulder, the proximal regions of the extremities and the abdomen. It is strongly associated with alcoholism. Other associated diseases include liver disease, dyslipidemia, gout, abnormal glucose tolerance, and malignancies of the upper airways. BSL is found mainly in middle-age, mediterranean men. Imaging using either computed tomography or magnetic resonance imaging is often recommended. Dietary restriction provides no relief. Abstinence from alcohol may delay further progression but does not cause regression of the deformity. The only effective therapy for BSL is surgical removal but recurrence is common. Physicians should be aware of this condition because of its association with metabolic disease and malignancies