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BACKGROUND: Madelung's disease (MD), alternatively referred to as benign symmetric lipomatosis, multiple symmetric lipomatosis, or Launois-Bensaude syndrome, is an uncommon benign disorder marked by symmetric proliferation of adipose tissue in the head, neck, and torso regions. CASE DESCRIPTION: In this case, the patient was a female with the late middle-aged demographic, diagnosed with Type I MD. Notably, she exhibited no prior history of alcohol consumption, and there was no family history of MD. Subsequent to the clinical diagnosis, the patient underwent medical imaging assessments to delineate the surgical parameters. Post-surgery, she demonstrated a favorable recovery trajectory, marked by the absence of any surgical complications. RESULT: The patient underwent successful surgical excision of the lipomatous mass. Postoperatively, she had an uneventful recovery with no complications and no recurrence observed during the follow-up period of seven months. CONCLUSION: Timely diagnosis and early surgical intervention play a pivotal role in enhancing the quality of life for individuals with MD. Preoperative medical imaging examinations function as highly effective tools, contributing to heightened surgical safety and a decreased probability of encountering complications during the surgical procedure.
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Lipomatose Simétrica Múltipla , Humanos , Feminino , Lipomatose Simétrica Múltipla/cirurgia , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/patologia , Pessoa de Meia-IdadeAssuntos
Lipomatose Simétrica Múltipla , Neoplasias Hepáticas , Humanos , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/patologia , Lipomatose Simétrica Múltipla/cirurgia , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/complicações , Masculino , Pessoa de Meia-Idade , Carcinoma Hepatocelular/cirurgia , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/patologia , FemininoRESUMO
Madelung's disease is characterized by the presence of multiple symmetric and nonencapsulated facial, neck, and upper trunk lipomas. Depending on the location and size of lipomas, there may be concerns for difficult airway management and cardiovascular collapse. We present the anesthetic management case of a patient with cervical and mediastinal lipomas who underwent elective cervical lipoma resection.
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Anestésicos , Lipoma , Lipomatose Simétrica Múltipla , Neoplasias do Mediastino , Humanos , Lipomatose Simétrica Múltipla/cirurgia , Lipoma/cirurgia , Pescoço/cirurgiaRESUMO
<b><br>Introduction:</b> Madelung's disease is a rare condition characterised by the symmetric growth of fatty tumours (lipomas) around the neck, shoulders, upper arms and trunk.</br> <b><br>Case report:</b> We present a description of a male patient with extensive adipose tissue overgrowth around the neck. Once the possibility of malignancy was excluded, the patient's history and clinical and radiological findings led to the diagnosis of Madelung's disease. A two-stage surgery was planned and the patient underwent lipectomy of the lipomas around the neck.</br> <b><br>Conclusions:</b> This article analyses the clinical data with Madelung's disease; discusses its aetiology, clinical manifestations, diagnosis and treatment methods; and provides help with clinical diagnosis and treatment.</br>.
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Lipoma , Lipomatose Simétrica Múltipla , Humanos , Masculino , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/cirurgia , Lipomatose Simétrica Múltipla/patologia , Lipoma/diagnóstico , Lipoma/diagnóstico por imagemRESUMO
Multiple symmetric lipomatosis (MSL) is a rare disease associated with metabolic disorders and alcoholism. In this report, we describe the clinical outcome and surgical protocol for eight men with Madelung collar who underwent treatment in a craniofacial and plastic surgery facility in Hanoi, Vietnam, between May 2018 and December 2020. We discuss the patients' clinical symptoms, subclinical signs, epidemiology, magnetic resonance imaging, computerized tomography, surgical protocol, complications, and postoperative indicators that we collected and evaluated. Each patient underwent surgery in two stages (i.e., in supine and prone positions). We injected a tumescent solution 10 min before the incision. Two surgeons performed each operation simultaneously. After surgery, we followed the patients for 6-27 months. All of the patients had a history of long-time alcohol abuse and had associated comorbidities that included liver disease, blood disorders, restricted neck movement, and orthopnea. We did not have to open the trachea or transfuse blood during any of the surgical procedures. Postoperatively, all patients were satisfied with their functional results and aesthetic appearance. One patient experienced a recurrence of his MSL. We believe our surgical protocol provides optimal results for patients with MSL and Madelung collar.
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Abdominoplastia , Alcoolismo , Lipectomia , Lipomatose Simétrica Múltipla , Cirurgiões , Masculino , Humanos , Lipomatose Simétrica Múltipla/cirurgia , Contenções , Alcoolismo/complicaçõesRESUMO
Madelung's disease is a lipodystrophy of unknown etiology. This article reports a case of Madelung's disease complicated with laryngeal cancer. The clinical manifestations of the patient were progressive hoarseness and dyspnea, dysphagia, and diffuse symmetrical swelling of the neck, submental, and submandibular. Dynamic laryngoscopy revealed a giant cauliflower-like neoplasm in the throat, with the left vocal cord fixed. Laryngeal CT showed laryngeal carcinoma ï¼transglottic typeï¼, signs of lymph node metastasis in the left jugular chain region, and Madelung syndrome in the neck. Biochemical tests showed albumin 38.7 g/L, globulin 27.5 g/L, prealbumin 160 g/L, aspartate aminotransferase 14 IU/L, γ-transpeptidase 80 IU/L, alanine aminotransferase 7 IU/L, Creatinine 43 µmol/L. Preoperative pathology suggested squamous cell carcinoma. Admission diagnosis included laryngeal cancer ï¼transglottic T4N1M0ï¼, â ¢ degree laryngeal obstruction, Madelung's disease and fatty liver. The patient recovered well after surgery.
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Obstrução das Vias Respiratórias , Neoplasias Laríngeas , Lipomatose Simétrica Múltipla , Humanos , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/patologia , Lipomatose Simétrica Múltipla/cirurgia , Neoplasias Laríngeas/cirurgia , Laringoscopia , Obstrução das Vias Respiratórias/etiologia , Dispneia/etiologiaRESUMO
Unlike simple obesity, Madelung's disease (MD) is a rare disease characterized by symmetric accumulation of massive adipose tissue on the neck, the superior part of the trunk and limbs, leading to a pathognomonic cosmetic deformity. Here, we report an extremely rare case of MD associated with bilateral gynecomastia in a 61-year-old man, with a history of recent liver transplantation for alcoholic liver disease (ALD).
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Lipomatose Simétrica Múltipla , Transplante de Fígado , Humanos , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/etiologia , Lipomatose Simétrica Múltipla/cirurgia , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-IdadeRESUMO
Madelung's disease is a rare benign systemic lipomatosis, which often presents in the head, neck and upper trunk regions. The appearance of symmetrical, excessive adipose tissue in the subcutaneous layer is its clinical characteristic. Orbital involvement is unusual with only a few cases reported previously. In this study, we describe the clinical and radiological features of Madelung's disease in the orbits. A 42-year-old man with alcohol addiction presented with chronic bilateral masses of the lower eyelids and proptosis. Computed tomography (CT) scan showed excessive symmetrical non-encapsulated fat deposition in the orbital fat, lower eyelids, salivary glands, subcutaneous tissue along the neck and under the sternocleidomastoid muscles and supraclavicular areas bilaterally. Histopathological study of the orbital masses revealed mature adipose tissue interspersed with thin fibrous septae. He developed recurrent lipomatosis 1 year after surgical excision.
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Lipomatose Simétrica Múltipla , Lipomatose , Tecido Adiposo/patologia , Adulto , Humanos , Lipomatose/diagnóstico por imagem , Lipomatose/patologia , Lipomatose/cirurgia , Lipomatose Simétrica Múltipla/diagnóstico por imagem , Lipomatose Simétrica Múltipla/patologia , Lipomatose Simétrica Múltipla/cirurgia , Masculino , Pescoço/patologia , Órbita/patologiaRESUMO
BACKGROUND: Madelung disease is a rare lipid metabolic disorder, and most cases are sporadically reported. There are currently no systematic reviews summarizing the epidemiological and clinical characteristics of this disease. The purpose of the current article is to extract and analyze the existing evidence concerning Madelung disease derived from case series in order to provide adequate treatments for patients based on a more comprehensive understanding of the disease. METHODS: PubMed, Embase, and Web of Science databases were queried for relevant articles using the search terms "Madelung disease," "multiple symmetric lipomatosis," "Launois-Bensaude syndrome" and synonyms until Aug 31,2020. Data statistics of Madelung disease epidemiology and clinical characteristics are summarized in different tables or charts with Microsoft Office software. RESULTS: Patients exhibiting Madelung disease were mostly located in Europe, although some records existed in Asia as well. Average patient age was between 45 and 65 years old. Type I was the most common form of the disease, and the neck was the most common location for tumors. Madelung disease is associated with various metabolic disorders, and hematoma and seroma were the most common complications. Overall recurrence rate was 18.3%, with similar recurrence rates after lipectomy and liposuction. Fewer complications occurred after liposuction compared with lipectomy, but relapse was more common after liposuction. CONCLUSION: Madelung disease consists of specific epidemiological and clinical characteristics, knowledge of which can be helpful for diagnosis and cognition. Lipectomy and liposuction are considered to be the most effective treatment methods for Madelung disease; however, choice of surgery should be based on comprehensive consideration of the disease, such as severity, mass location, and patient expectations. 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 .
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Abdominoplastia , Lipectomia , Lipomatose Simétrica Múltipla , Idoso , Humanos , Pessoa de Meia-Idade , Lipomatose Simétrica Múltipla/diagnóstico , Lipomatose Simétrica Múltipla/epidemiologia , Lipomatose Simétrica Múltipla/cirurgia , Pescoço , Resultado do TratamentoRESUMO
BACKGROUND: Multiple Symmetric Lipomatosis (MSL) is a rare disorder related to fat metabolism and lipid storage. The condition results in characteristic depositions of fat, especially around the cephalic, cervical, and upper thoracic subcutaneous. It is much more common in adult males who live in the Mediterranean region and has only rarely been reported in Asian females. In this report, we present a case of an Asian female with MSL and also review the clinical features of the condition, including radiological and histological findings required for proper diagnosis and management. CASE PRESENTATION: A 59-year-old Korean female came in with a chief complaint of palpable mass present in shoulder and upper back regions. Images showed diffuse non-encapsulated adipose tissue in the subcutaneous layer of the suboccipital, posterior neck area. The patient wanted to remove the mass for cosmetic reasons and discomfort. Excisional biopsy was planned. Preoperative blood analyses showed deteriorated liver function, and the computed tomography findings were consistent with liver cirrhosis. Detailed history taking revealed that she consumed highly levels of alcohol. Lipectomy was performed and the histological findings demonstrated large dystrophic adipocyte morphology. The patient was recovered uneventfully. CONCLUSION: When patients have multiple symmetric lipomatous lesions, clinicians should suspect MSL and survey possible associated conditions, such as alcoholism, liver cirrhosis, dyspnea, and neuropathy in detail.
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Tecido Adiposo/diagnóstico por imagem , Lipectomia , Lipomatose Simétrica Múltipla/diagnóstico por imagem , Lipomatose Simétrica Múltipla/cirurgia , Cirrose Hepática/diagnóstico por imagem , Biópsia , Feminino , Humanos , Lipomatose Simétrica Múltipla/patologia , Pessoa de Meia-Idade , Doenças Raras , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
La deformidad de Madelung es una alteración poco común de la articulación de las muñecas. Se vincula a mutaciones del gen SHOX y se caracteriza por alteraciones en el radio, carpo y cúbito, con predominio bilateral. Afecta principalmente a pacientes de sexo femenino y aparece al inicio de la adolescencia. Se presenta una paciente de 15 años de edad, con antecedentes de problemas de salud. Al entrar en la adolescencia comenzó a presentar deformidad en ambas muñecas, más marcada en el lado derecho acompañado de dolor. El diagnóstico de deformidad de Madelung se concluyó mediante la clínica asociado a la positividad de los estudios imagenológicos, basados en los criterios radiográficos de Dannenberg y otros. Se decidió tratamiento quirúrgico, mediante osteotomía doble correctora para longitud y fijación externa de la mano derecha, con la resolución completa de la deformidad y seguimiento en la Consulta Externa de Ortopedia(AU)
Madelung's deformity is a rare alteration of the wrist joint. It is linked to mutations of the SHOX gene. It is characterized by alterations in the radius, carpus and ulna, predominantly bilateral. It mainly affects female patients; signs and symptoms are evident at the beginning of adolescence. To present a case of a patient with a diagnosis of Madelung deformity. The case of a 15-year-old female patient with a health history and family history of interest of an equine clubfoot father is presented. When she entered adolescence, she began to present deformity in both wrists, more marked in the right side accompanied by pain. This is a patient with a Madelung deformity. The diagnosis was concluded by the clinic associated with the positivity of the imaging studies(AU)
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Humanos , Feminino , Adolescente , Anormalidades Congênitas , Lipomatose Simétrica Múltipla/cirurgia , Lipomatose Simétrica Múltipla/congênito , Lipomatose Simétrica Múltipla/diagnóstico por imagem , Sinais e SintomasRESUMO
INTRODUCTION: Multiple symmetric lipomatosis (MSL) is an uncommon medical condition characterized by symmetric fat accumulation mainly in the neck and other upper body regions. The involvement of the larynx is rare according to the literature, and we present a case of MSL with larynx involvement treated with a surgical approach. PATIENT CONCERNS: A 55-year-old male was admitted to our hospital due to progressively aggravated breathing difficulty, and tracheotomy was performed before transfer. When he tried to block the cannula, the breathing difficulty returned. The patient's neck had been thickening for the past 2 years. DIAGNOSIS: Pathological examination confirmed the diagnosis of MSL. INTERVENTIONS: The patient underwent lumpectomy and neck exploration. OUTCOMES: The lipoma was removed, the patient was free of any dyspnea symptoms and recovered well, and the tracheal cannula was removed at a local hospital. CONCLUSION: MSL can infiltrate the larynx and grow into the preepiglottic space and paraglottic spaces, resulting in breathing difficulties. Lipomas present in the spaces described above must be removed at the same time; otherwise, symptoms of dyspnea cannot be alleviated.