Launois-Bensaude Syndrome Research Articles

Central form of multiple symmetric lipomatosis: a case report

Multiple symmetric lipomatosis (also known as Madelung's disease, Launois-Bensaude syndrome or benign symmetric lipomatosis) is a rare disease, the etiology of which is unknown. Multiple, symmetric, non-encapsulated lipomatous masses on the face, neck, upper arms, and upper trunk are typical in most cases. Five out of the 200 cases reported in the literature were the distal form of the disease; the rest were proximal. We describe, for the first time, a central form of the disease, simultaneously involving the lower trunk, arms and upper legs.

La maladie de Launois-Bensaude : description, prise en charge. À propos de dix patients opérés

Launois-Bensaude syndrome is a rare pathology. The presence of multiple, symmetric, non encapsulated lipomatous masses in the face, neck, upper arms and upper trunk is typical. Men are especially affected between age of 35 and 50. The disease is frequently associated with alcoholism, hepatopathy, glucose intolerance, hyperuricemia, and malignant tumors of the upper airways, requiring thorough clinical evaluation of all patients. Cosmetic deformity and compression lead patients to seek treatment. Therapy is difficult. Dietary treatment and weight loss are of limited value in the management of this pathology. Surgery is the only effective treatment. The surgeon must make sure that the patient has ceased his alcohol abuse before performing this treatment. Conventional surgery or liposuction are used to restore a normal social life to these patients, deformed by their disease. This surgical management has a severe tendency to haemorrhage, dissection is complicated by infiltrations of neighbouring tissue and is frequently followed by recurrence, mainly after liposuction. Nevertheless, based on a review of ten cases, good results of surgical removal must prompt to operate them on.

A case of obstructive sleep apnea syndrome: unusual comorbidity

Launois-Bensaude syndrome (LBS) is a rare disease, characterized by the accumulation of fatty tissue predominantly in the neck, shoulders and thorax, whose diagnostic is clinical. We describe a new case in a 73 year-old man. As the patient was obese, complained of snoring and was treated for hypertension, we looked for a morbid association with an obstructive sleep apnea syndrome (OSAS). A polysomnography (PSG) and a cervical magnetic resonance imaging (MRI) were performed. PSG demonstrated OSAS with an apnea-hypopnea index of 43/h. Cervical MRI showed fatty infiltration resulting in airway narrowing at the pharyngeal but not at the tracheal level. Only, 3 cases of such a morbid association have already been published; in 2 of these patients was a tracheal compression. More over a metabolic syndrome was present. This observation draws attention to the need for seeking OSAS among patients affected by LBS, even in a moderate form, and emphasises the roles of the upper airway narrowing by the fat infiltration as well as the role of the metabolic syndrome in the genesis of OSAS.

Launois-Bensaude Syndrome: An Unusual Localization of Obesity Disease

Launois-Bensaude syndrome is a rare pathology consisting of adipose masses symmetrically distributed mainly in the superior part of the body. Men are especially affected between age of 30 and 60 as well as chronic alcohol abusers. Etiopathogenesis is attributable to mutations or deletions of mitochondrial DNA, and alcohol is a possible cofactor. The current treatment of the disease is described based on the authors' experience. Four cases treated in our department are retrospectively reviewed regarding comorbidities and type of surgery performed. A relevant and long-lasting reduction of fat bulges has been obtained in all cases with no major complications except for a mild anemia. Launois-Bensaude syndrome causes a functional rather than esthetic concern due to the peculiar localization of fat bulges. Currently, the only effective therapy is surgery, through lipectomy or liposuction of adipose bulges.

Diffuse Lipomatosis of Thyroid Gland

A 64-yr-old man with chronic renal failure and psoriasis presented with enlargement of thyroid gland and dyspnea. Thyroid function was within normal limits, and no antithyroid antibodies were detected. Nonsteroid therapy was done. Ultrasonography detected diffuse goiter with heterogeneous echogenicity. Scintigraphy revealed enlargement of thyroid, with irregularly reduced uptake, without cold nodules. Computed tomography, performed to evaluate tracheal diameters, showed a goiter, slightly compressing the airway. Thyroid had a negative density, in the range of fat tissue (Fig. 1). Computed tomography also detected mediastinal lipomatosis, clearly separated from thyroid. Fine-needle thyroid cytology showed abundant fat cells between normal follicles, without any sign of hyperplasia, malignancy, or amyloid deposition. Diffuse thyroid lipomatosis is a rare entity, initially reported by Dhayagude in 1942 (1). A few cases have been reported in the literature (2–4). It is characterized by diffuse proliferation of adipose tissue in the gland, sometimes associated with amyloid deposition. This condition has been explained in different ways; the prevailing hypothesis suggests that fat remains included in the gland during the embryogenesis (2). Adenolipoma and other intrathyroid fat-containing masses were easily excluded, because these rare entities appear as focal nodules, well circumscribed, within an otherwise normal gland. A benign, symmetrical lipomatosis of the upper trunk, neck, and head, known as Launois-Bensaude syndrome, should spare the thyroid gland (5). In conclusion, this is an example of idiopathic diffuse lipomatosis of thyroid gland. Because the natural history of this rare condition is unknown, further follow-up is warranted.

Tumescent Liposuction in a Patient with Launois—Bensaude Syndrome and Severe Hepatopathy

GRASSEGGER, ALFRED MD*; HÄUSSLER, RUDOLF MD†; SCHMALZL, FRANZ MD, PHD‡ Author Information

Multiple symmetric lipomatosis (MSL) of neck in a child (Madelung's disease): report of a rare presentation

A case of multiple symmetric lipomatosis (MSL) of neck, also known as Madelung's disease or Launois-Bensaude syndrome, is reported in an 8-year-old girl who presented with bilateral swellings in the neck. MSL is characterized by large subcutaneous fat masses distributed around the neck, shoulders and other parts of the trunk, often associated with abnormalities of the nervous system. This is commonly seen in middle-aged men with a history of alcohol abuse. Metabolic disturbances and malignant tumours have also been associated with this disorder. MSL is rare in children and there was only one prior report in the literature describing MSL that affected two children. With an initial clinical suspicion of bilateral cystic hygroma, imaging was sought. CT demonstrated multiple compartmentalized, unencapsulated fat-containing lesions on either side of the neck extending into the mediastinum. This radiographic appearance was strongly suggestive of lipomatous tissue within the neck and eventually confirmed by histopathology. The recognition of this disease is important as, in addition to cosmetic abnormality, it can compress the important neurovascular structures. These fatty deposits were later surgically excised.

Scrotal involvement in Madelung disease: clinical, ultrasound and MR findings

Multiple symmetrical lipomatosis, also known as Madelung disease, Launois-Bensaude syndrome, and benign symmetrical lipomatosis, is an uncommon disease that is characterized by the presence of multiple and symmetrical deposits of nonencapsulated adipose tissue in the neck, upper trunk, arms, and legs. It affects mainly men, with a higher incidence in the Mediterranean area, and is typically associated with high alcohol consumption and a high prevalence of peripheral neuropathy that usually appears long after the development of lipomatous masses. We report the case of a patient with involvement of the scrotum. We present clinical, ultrasound, and magnetic resonance appearances of these scrotal lipomatous masses for the first time.

Unklare Gewichtszunahme?

In the case of a 49 year old patient, a weight gain of 37 kg occurred during the first weeks of the year 2003, accompanied by a painless increase in the diameter of the upper arms and thighs. This process did spontaneously cease in the summer of 2003, but weight reduction could not be achieved. The diagnosis of multiple symmetrical lipomatosis, also known as Launois-Bensaude syndrome, the first authors to describe the condition in detail in 1898, could be established due to the unique appearance of the patient. Typical features of the disease are the accumulation of multiple lipomata in the shoulder girdle, upper arms, thorax and thighs, whereas the face, the forearms and the shanks are typically excluded. The etiology of the disease is obscure, dietetic intervention is futile, surgical approaches are liposuction or excision of the lipomata. Cessation of alcohol consumption may also be helpful, since the condition is typically associated with present or past alcohol abuse. The disease is usually reported to be rare, but there is reason enough to assume, that it is frequently misdiagnosed as simple truncal obesity.

Four different airway-management strategies in patient with Launois-Bensaude syndrome or Madelung's disease undergoing surgical excision of neck lipomatosis with a complicated postoperative course

Launois-Bensaude syndrome, otherwise known as Madelung's disease, is a rare disorder characterized by fatty accumulations of the upper trunk, neck and head, causing serious cosmetic deformity and neck immobility. We report a case of a patient with Launois-Bensaude syndrome who required 4 different airway management strategies during his hospitalization for postoperative complications after an elective surgical excision of a severe neck lipomatosis. Anesthesiologists who treat patients with Madelung's disease should be aware of 2 major problems: difficult airway and increased frequency of postoperative bleeding. A proper choice of airway management technique is therefore necessary, including a safe intubation and extubation plan to reduce the consequences of airway complications.

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.

Surgical management of Launois-Bensaude syndrome.

The presentation, physical findings, and surgical management of a patient with extensive Launois-Bensaude syndrome are reported.

Madelung's disease: an uncommon disorder of unknown aetiology?

A case of Madelung's disease (benign multiple symmetrical lipomatosis, Launois-Bensaude syndrome) is described. The characteristic clinical features, associations with chronic alcoholism and a review of the current literature is reported.

Multiple Familial Angiolipomatosis: Treatment of Liposuction

Lipomas constitute the most common soft-tissue tumors [11] and may occur sporadically or as one of several inherited disorders. Benign fatty tumors can be classified into three major categories: solitary lipomas, familial multiple lipomatosis, and congenital diffuse lipomatosis [4]. The latter has also been referred to as multiple symmetric lipomatosis, Madelung's disease, or Launois-Bensaude syndrome. Within the categories a more vascular varient of the lipoma, the angiolipoma, has been described separately by some authors, but in many reports it is not distinguished from a lipoma. Liposuction has been used for the treatment of solitary lipomas, but to our knowledge liposuction has not previously been reported for excision of angiolipomas or in familial cases involving multiple lipomas as described here.