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Aguilar Lizarralde 2 , A. This uncommon disease predominantly affects men between the ages of 30 and 60 and it has a ratio.
There is a strong correlation with alcohol abuse. Nonalcoholics and women can also be affected although this is rare. She had difficulty swallowing, dysarthria and dyspnea while sleeping. Key words: Madelung's disease; Benign symmetric lipomatosis; Macroglossia. Presentamos el caso de una mujer diagnosticada de Enfermedad de Madelung refiriendo engrosamiento progresivo de la lengua. It was first described by Brodie in and Otto Madelung in presented a series of 35 patients with cervical lipomatosis.
Towards the end of the 19th Century, Launois and Bensaude documented further series and they defined the syndrome as the presence of multiple accumulations of fat in a symmetric manner affecting the head, neck and upper half of the body. Non-alcoholics and women can also be affected, although this is rare. The disease is defined as the presence of multiple accumulations of fat in a symmetrical manner, and it generally affects the neck, abdominal wall, back, armpits, torso and arms.
Its etiology is unknown. Generally it is accompanied by a hepatic dysfunction due to alcohol4 and there is a lipogenic and anti-lipolytic effect. The diagnosis should essentially be established with clinical data and imaging studies such as the CAT scan, ultrasonography or MRI. She reported a progressive thickening of the tongue and she had difficulties swallowing. She also had dysarthria and dyspnea in decubitus position.
Her medical history included hypertension, a hemicolectomy of the right side as a result of a colo-rectal carcinoma five years previously. She was not obese and she denied alcohol abuse. On inspection multiple lipomatous masses were observed around her neck that had been operated on Fig. Her armpits and waist had been growing progressively over the previous five years. There was no lymph node swelling in the neck. There was a generalized swelling of the tongue that had produced a widening of the dental arches.
The midline raphe of the tongue had a normal appearance but the lateral areas had a surface that was smooth and yellow Fig.
A computed axial tomography was carried out that showed ill-defined adipose tissue invading the lingual muscles on both sides Fig. The patient was operated on under general anesthesia. A bilateral glossectomy was performed to reduce the tongue, and an effort was made to try and respect the tip and most of the back in order to maintain a perception of taste. The adipose tissue was intermixed with the muscles of the tongue, and capsules were not observed Fig.
The immediate postoperative period was incident-free. During the intervention a nasogastric feeding tube was positioned, and the inflammation was controlled with intravenous corticosteroids. She was discharged on day 7 of the postoperative period. The stitches were removed on day 14 Fig. The anatomopathological study revealed a diffuse proliferation of adipose tissue that was normal, and an increase in fibrous and vascular elements.
Capsules were not identified nor were atypical cells observed. During the postoperative period, the dyspnea in decubitus position and the dysphagia disappeared, although there was minimal dysarthria observed by the patient herself. Benign symmetric lipomatosis affecting the neck is well known, and around cases have been published. Over the years large amounts of fat deposits are accumulated, and they become esthetically deforming, and in advanced cases this may cause dyspnea and dysphagia.
The larynx may be affected not only as a result of direct compression by the fat, but also because of the recurrent infiltration of the laryngeal nerve. There is only one case published on malignant transformation to myxoid liposarcoma. On diagnosis it is important to keep in mind other tumoral entities. Macroglossia may be present in a large variety of conditions, such as congenital syndromes, metabolic and endocrine diseases, or it may appear in others such as amyloidosis, hyalinois or lingual tumours.
The clinical course of the disease starts with an initial period of rapid growth, followed by slow progression. There is no case published regarding the spontaneous regression of lipomas, in spite of abandoning alcohol. The cause of dyspnea, dysphagia and dysarthria should not be attributed to fat compression until other pathologies of the airways have been eliminated. Treatment is limited to the surgical resection of the adipose tissue, either directly or through liposuction.
Surgery is indicated for those patients with severe deformity, when there are psychological disturbances, and if there is compression of the aerodigestive tract causing dyspnea or dysphagia. Given that total excision of the adipose tissue cannot be carried out, relapse is common after the intervention, and patients should be warned of this.
When the tongue is affected, adipose tissue penetrates deeply and, as a result, the complete resection of the lesion is nearly impossible as this would result in the subtotal resection of the tongue. On planning the glossectomy, the volume to be resected should be carefully planned, bearing in mind the postoperative size and shape that the tongue is to have.
Treatment is surgical when there are obstructive complications given the size that the tongue can reach, but it should always be kept in mind that relapse is the norm. Parmar SC, Blackburn C. Madelung's disease: an uncommon disorder of unknown aetiology? Br J Oral Maxillofac Surg ; Enzi G. Multiple symmetrical lipomatosis: an updated clinical report. Medicine ; Enfermedad de Madelung. Rev Esp Cir Oral Maxilofac ; Benign Symmetric Lipomatosis of the Tongue: report of a case.
J Oral Maxillofac Surg ; Benign Symmetric Lipomatosis Madelung's disease : Case reports and current management. Aesthetic Plast Surg ; Longterm results following surgical treatment of benign symmetrical lipomatosis BSL. Int J Oral Maxillofac Surg ; Ann Dermatol Venereol ; Madelung's disease involving the tongue. J Am Acad Dermatol ; Madelung's disease: case report and discusion of treatment options.
Ann Plast Surg ; Benign symmetrical lipomatosis. Head Neck Surg ; Malignant degeneration in Madelung's disease benign lipomatosis of the neck : case report. Br J Plast Surg ; Head and neck cancers associated with Madelung's disease. Ann Surg Oncol ; Multiple symmetric lipomatosis: Korean experience. Dermatol Surg ; Servicios Personalizados Revista.
Multiple symmetric lipomatosis
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Aguilar Lizarralde 2 , A. This uncommon disease predominantly affects men between the ages of 30 and 60 and it has a ratio. There is a strong correlation with alcohol abuse. Nonalcoholics and women can also be affected although this is rare.
Lipomatosis simétrica múltiple
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly.
2017, Número 2
It should not be confused with the Madelung deformity of the wrist, which is an epiphyseal growth plate disturbance characterized by dorsal and radial bowing of the radius, or Madelung dyschondrosteosis , a dysplasia associated with the Madelung deformity. Madelung disease is most commonly seen in the Mediterranean population with a male to female ratio of The commonest age of onset is between the third and fifth decades. The fatty deposits are typically unencapsulated, with non-destructive infiltration and displacement of surrounding structures 9. Adipose cells usually have a benign microscopic appearance. Symmetric lobulated subcutaneous fatty deposition in a centripetal distribution.