CASO CLÍNICO

 

Benign symmetric lipomatosis of the tongue in Madelung’s disease

Lipomatosis simétrica benigna de la lengua en la enfermedad de Madelung

 

 

A. López Ceres¹, Y. Aguilar Lizarralde², A. Villalobos García³, E. Prieto Sánchez⁴, A. Valiente Álvarez⁵

¹ Médico Residente de Cirugía Oral y Maxilofacial.
² FEA de Cirugía Oral y Maxilofacial.
³ Médico Residente de Medicina Interna.
⁴ Médico Residente de Anatomía Patológica.
⁵ Jefe de Servicio de Cirugía Oral y Maxilofacial. Complejo Hospitalario Carlos Haya, Málaga, España.

Dirección para correspondencia

 

 

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ABSTRACT

Madelung´s disease, or benign symmetric lipomatosis, is an uncommon disease characterized by non-encapsulated accumulations of fat in a symmetric manner around the neck and shoulders.
This uncommon disease predominantly affects men between the ages of 30 and 60 and it has a 15:1 ratio. There is a strong correlation with alcohol abuse. Nonalcoholics and women can also be affected although this is rare.
We report the case of a woman diagnosed with Madelung’s disease, who described a gradual swelling of the tongue. She had difficulty swallowing, dysarthria and dyspnea while sleeping.

Key words: Madelung's disease; Benign symmetric lipomatosis; Macroglossia.

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RESUMEN

La enfermedad de Madelung, o lipomatosis simétrica benigna es una enfermedad rara caracterizada por acúmulos grasos no encapsulados localizados de forma simétrica alrededor de cuello y hombros.
Esta enfermedad, afecta predominantemente a hombres en edades comprendidas entre los 30 y 60 años, con una relación hombre:mujer de 15:1. Existe una gran relación con el abuso del alcohol. Las personas no alcohólicas y las mujeres también pueden verse afectadas, aunque de forma más rara.
Presentamos el caso de una mujer diagnosticada de Enfermedad de Madelung refiriendo engrosamiento progresivo de la lengua. Presentaba dificultad para tragar, disartria y disnea con el decúbito.

Palabras clave: Enfermedad de Madelung; Lipomatosis simétrica benigna; Macroglosia.

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Introduction

Madelung’s disease, or benign symmetric lipomatosis, is a rare disease characterized by an accumulation of nonencapsulated fat that is located in a symmetric manner around the head and shoulder’s, rarely affecting the hips and lower limbs. It was first described by Brodie in 1846 and Otto Madelung in 1988 presented a series of 35 patients with cervical lipomatosis. Towards the end of the 19th Century, Launois and Bensaude (1898) 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.¹

This disease, which affects mainly men between the ages of 30 and 60, has a male/female ratio of 15:1. The greatest incidence is in the Mediterranean, and in Italy the incidence is of 1/25000 males.² In nearly all patients there exists a strong link with alcohol abuse. Non-alcoholics and women can also be affected, although this is rare.³

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.¹ Catecholamine-induced abnormal lipogenesis has been observed5 together with a possible association with hypothalamo-hypophysary lesions such as thyroid adenomas, hypothyroidism, renal tubular acidosis and hypertriglicaridemia.⁴

The diagnosis should essentially be established with clinical data and imaging studies such as the CAT scan, ultrasonography or MRI.⁶

Symmetric lipomatosis affecting the tongue as part of Madelung’s disease is very rare, and only three cases have previous been described.^7-9

 

Case report

A 53-year-old patient was referred to our department by the department of Internal Medicine with a diagnosis of Madelung’s disease. 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. 1). 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. 2).

A computed axial tomography was carried out that showed ill-defined adipose tissue invading the lingual muscles on both sides (Fig. 3).

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. 4).

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. 5).

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.

 

Discussion

Madelung’s disease is characterized histologically as multiple deposits of non-encapsulated adipose tissue. Benign symmetric lipomatosis affecting the neck is well known, and around 200 cases have been published.¹⁰

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.¹¹ After surgery or trauma there may be an increase in growth.

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.¹²

The histological examination shows lipomatous cells in abnormal areas that are totally indistinguishable from those that are in normal adipose tissue, although ultrastructural studies have demonstrated that adipose cells in Madelung’s disease are smaller and multivacuolated, which suggests that it has a possible brown fat origin. ² Likewise, the masses tend to be more vascularized than normal adipose tissue and, as a result, greater bleeding should be envisaged during the surgical act as well as during postoperative volemic replacement.

There is only one case published on malignant transformation to myxoid liposarcoma.¹³ The association of Madelung’s disease with other neoplasms appears to be due to the combination of chronic alcoholism and nicotine abuse that appears to be present in these patients, and who are susceptible to certain neoplasms.¹⁴

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.¹⁵

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.¹¹ Dietary restrictions do not cause any improvement. 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.⁶ ß2 agonists (salbutamol)^1,5,10 and intralesional injections of enoxaparin5 have been used as medical treatment but to no avail.

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.

 

Conclusions

We have presented the case of a patient diagnosed with Madelung’s disease that had symptoms of macroglossia as described in another three articles. This should lead one to think that the involvement of the tongue is another characteristic of Madelung’s disease, which is very rare but that does exist.

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.

 

 

Dirección para correspondencia:
Ana López Ceres
C/Luis Taboada 38, Blq 1, 1ºB
29017 Málaga, España
Email: analopezceres@gmail.com

Recibido: 03.11.2005
Aceptado: 23.03.2006

 

 

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