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Revista Española de Cirugía Oral y Maxilofacial
versión On-line ISSN 2173-9161versión impresa ISSN 1130-0558
Rev Esp Cirug Oral y Maxilofac vol.28 no.2 Madrid mar./abr. 2006
CASO CLÍNICO
Benign symmetric lipomatosis of the tongue in Madelungs 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
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 Madelungs 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.
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.
Introduction
Madelungs 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 shoulders, 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 Madelungs 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 Madelungs 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
Madelungs 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 Madelungs 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 Madelungs 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 Madelungs 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 Madelungs 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
References
1. Parmar SC, Blackburn C. Madelung's disease: an uncommon disorder of unknown aetiology? Br J Oral Maxillofac Surg 1996;34:467-70. [ Links ]
2. Enzi G. Multiple symmetrical lipomatosis: an updated clinical report. Medicine 1984;63:56-64. [ Links ]
3. García JA, Dean A, Alamillos F, Ruiz JJ, Peñalba M, Zafra F, Barrios G. Enfermedad de Madelung. Presentación de un nuevo caso y revisión de la literatura. Rev Esp Cir Oral Maxilofac 2003;25:165-9. [ Links ]
4. Ogawa A, Nakamura H, Takahashi H. Benign Symmetric Lipomatosis of the Tongue: report of a case. J Oral Maxillofac Surg 1988;46:502-4. [ Links ]
5. González-García R, Rodríguez-Campo FJ, Sastre-Pérez J, Muñoz-Guerra MF. Benign Symmetric Lipomatosis (Madelung's disease): Case reports and current management. Aesthetic Plast Surg 2004;28:108-12. [ Links ]
6. Ujpal M, Nemeth ZS, Reichwein A, Szabo GY. Longterm results following surgical treatment of benign symmetrical lipomatosis (BSL). Int J Oral Maxillofac Surg 2001;30:479-83. [ Links ]
7. Duvar M, Pollet L, Herrenschmidt JC. Lipoma de la langue au cours d'une lipomatose symmétrique. Bull Mém Soc Med Hosp 1957;53:174-8. [ Links ]
8. Ghislain PD, Garzitto A, Legout L, Alcaraz I, Creusy C, Mediano P. Lipomatose bénigne symmétrique de la langue et lipomatose de Launoios´Bensaude. Ann Dermatol Venereol 1999;126:147-9. [ Links ]
9. Vargas-Díez E, Daudén E, Jones-Caballero M, García-Díez A. Madelung's disease involving the tongue. J Am Acad Dermatol 2000;42:511-3. [ Links ]
10. Adamo C, Vescio G, Battaglia M, Galleli G, Musella S. Madelung's disease: case report and discusion of treatment options. Ann Plast Surg 2001;46:43-5. [ Links ]
11. Palacín JM, Grande N, Rabell J, Sarobé N, Uribe I. Tratamiento quirúrgico de la lipomatosis cervical. Rev Esp Cir Oral Maxilofac 1998;20:257-63. [ Links ]
12. Argenta LC, McClatchey KD, Ferrel WJ, Newman MH. Benign symmetrical lipomatosis. Head Neck Surg 1981;3:240-3. [ Links ]
13. Tizian C, Berger A, Bukoupil KF. Malignant degeneration in Madelung's disease (benign lipomatosis of the neck): case report. Br J Plast Surg 1983;36:187-9. [ Links ]
14. Chan ESY, Ahuja AT, King AD, Lau WY. Head and neck cancers associated with Madelung's disease. Ann Surg Oncol 1999;6:395-7. [ Links ]
15. Lee HW, Kim TH, Cho W, Ryu BY, Kim HK, Choi CS. Multiple symmetric lipomatosis: Korean experience. Dermatol Surg 2003;29:235-40. [ Links ]