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Benign Symmetrical Lipomatosis Treated with Tumescent Liposuction

INTERNATIONAL JOURNAL OF COSMETIC SURGERY AND AESTHETIC DERMATOLOGY
Volume 2, Number 2, 2000

ABSTRACT: Multiple symmetrical lipomatosis is a rare disease of unknown etiology with significant cosmetic effects and occasionally with disabling symptoms. This is a case report of treatment with aggressive liposuction and oral salbutamol with moderately good long-term results.

Introduction

THERE ARE APPROXIMATELY 160 CASES reported in the literature of multiple symmetrical lipomatosis (symmetric adenolipomatosis, Madelung's syndrome, Buschke's syndrome, and Lanois-Bensaude syndrome).(1-3) This condition was first described in the late 1800s and involves unencapsulated symmetrical lipomas, usually if the neck, shoulders, and trunk. Hematoxylin-eosin microscopic examination shows normal fat tissue. (4)

Clinical Syndrome

Clinically, the onset is between 35 and 40 years and is most prevalent in the male. (5) A familiar pattern has been suggested. (6) At first the patient notices difficulty in buttoning the collar of his shirt and symmetrical masses appear in the posterior part of the neck. Then the masses occur in the submandibular region, the chest, and the rest of the body except for the extremities. Asthenia and apathy are usually present, compression of peripheral nerves can result in pain but not paresis, and dyspnea and cough may develop from airway compromise and mediastinal compression. (7, 8) Alcohol overuse has been frequently noted.

Liver function tests may be slightly abnormal. Hyperuricemia, glucose intolerance, and high blood lipids have been reported. (9) Defects in lipless (10) and mitochondrial genetic errors (11) have been demonstrated.

Treatment of the disorder has been mainly aggressive surgical resection, (12, 13) but liposuction has been attempted as well. (14) Oral salbutamol (12 mg daily in divided doses) has been reported as beneficial. (15)

This 40-year-old man with a history of untreated hypertension gave a history of increasing size of lipoma of the neck and shoulders girdle over a 10-year period. The patient was not obese but had large lipomatous masses in the neck and shoulders on examination. He had been on salbutamol (3 mg three times daily) for 1 year, which appeared to limit the rate of growth of the lipomas. He had no symptoms from the disorder. There had been three previous attempts at surgical removal of the masses.

Aggressive tumescent liposuction of the neck was performed in May 1997. The tumescent solution consisted of a total of 1800 mL of physiological solution containing 0.1 mg lidocaine and 1:1,000,000 epinephrine. A total of 3400 mL of fat and fluid was removed from the shoulder girdle. Following an uneventful recovery, the neck and back lipomas were removed by liposuction in July 1997 using 1200 mL tumescent fluid and removing 1500 mL of fat and fluid.

Neck Liposuction Patient Photos

The patient was continued on salbutamol. The results were excellent at 7 weeks postoperatively (Figure 1), but some of the areas of fatty deposit recurred over the following 2 years.

liposuction in two sessions

Discussion

Although there is no capsule around the fatty deposits in this syndrome, the liposuctioned areas respond like lipomas with the capsule left in situ in that there is a high likelihood of recurrence. In the areas where aggressive surgery had been performed, the long-term cosmetic results were better, but aggressive liposuction was more difficult because of the fibrosis.

Salbutamol had a definite effect because the patient noted a significant worsening of the fat deposits when he was off the drug.

References

  1. Agrez, M., Hellew, A., and Barrie, P.: Benign symmetric lipomatosis. Aust NZ J Surg 1995;65:616-618.
  2. Enzi, G.: Multiple symmetric lipomatosis, an updated clinical report. Medicine 1984;63:56-64.
  3. Martin, D.S., Sharafuddin, M., Boozan, J., Sundaram, M., Archer, C., et al.: Multiple symmetric lipomatosis (Madelung's disease). Skeletal Radiol 1995;24:72-73.
  4. Ruzica, T., Vieluf, D., Landthaler, M., and Braun-Falco, O.: Benign symmetric lipomatosis Launois-Bensaude: Report of ten cases and review of the literature. J Am Acad Dermatol 1987;17:663-674.
  5. Stavropoulos, P., Zouboulis, C.C., Trautmann, C., and Orfanos, C.E.: Symmetric lipomatosis in female patients. Dermatology 1997;194:26-31.
  6. McKusick, V.A.: Mendelian Inheritance in Man. Baltimore: Johns Hopkins University, 1978, p. 242.
  7. Luscher, H.J., Prein, J., and Spiessi, B.: Lipomatosis of the neck (Madelung's neck). Ann Plast Surg 1986;16: 502-508.
  8. Katou, F., Shirai, N., Motegi, K., Satoh, R., and Satoh, S.: Symmetriclipomatosis of the tongue presenting as macroglossia: Report of two cases. J Craniomaxillofac Surg 1993;7:298-301.
  9. Green, M.L., Glueck, C.J., Fujimoto, W.Y., and Seegmiller, J.E.: Multiple symmetric lipomatosis with gout and hyperlipoproteinemia. Am J Med 1970;48:239-246.
  10. Enzi, G., Inelman, E.M., Baritussio, A., Dorigo, P., Prosdocimi, M., and Mazzoleni, F.: Multiple symmetric lipomatosis: A defect in adrenergically stimulated lipolysis. J Clin Invest 1977;60:1221-1229.
  11. Klopstock, T., Naumann, M., Schalke, B., Bischof, F., Seibel, P., et al.: Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. Neurology 1994;44:862-866.
  12. Selvaag, E., Schneider, M., Wereide, K., and Kviem, M.: Benign symmetric lipomatosis Launois-Bensaude successfully treated with extensive plastic surgery. Dermatol Surg 1998;24:379-380.
  13. Springer, H.A., and Whitehouse, J.S.: Launois-Bensaude adenolipomatosis. Plast Reconstr Surg 1972;50:291-294.
  14. Carlin, M.C., and Ratz, J.L.: Multiple symmetric lipomatosis: Treatment with liposuction. J Am Acad Dermatol 1988;18:359-362.
  15. Leung, N.W.Y., et al: Multiple symmetric lipomatosis (Launois-Bensaude syndrome): Effect of oral salbutamol. Clin Endocrinol 1987;7:601-606