Advanced laryngeal compression due to diffuse, symmetric lipomatosis (Madelung's disease).

Abstract

Massive fat deposition in the neck is an exceedingly rare cause of laryngeal compression but one with a specific constellation of radiographic findings. We have recently seen such a case, which we believe to be of general interest. The differential diagnosis of symmetrical narrowing of the laynx or laryngotracheal airway in adults is limited. When there are no associated intraluminal abnormalities, the process is generally one which weakens the supporting cartilagenous structure, such as traumatic fracture or ischaemia from prolonged use of a cuffed endotracheal tube (Jameset al., 1970), infiltration by primary or metastatic neoplasm (Janower et al., 1970) or inflammation of the cartilage itself (as with relapsing polychondritis) (Horn and O'Loughlin, 1962). In Madelung's disease the larynx and trachea are compressed by prodigious, local adipose proliferation. The patient was first seen at the Massachusetts General Hospital at age 53 in 1967 because of painless, progressive swelling of the neck of six months' duration. There had been some recent exertional fatigue, a sensation of pressure in the ears, and a feeling of “tightness” upon turning the neck. There were no complaints of dysphonia, dysphagia, or dyspnoea. His past history was one of heavy alcohol consumption, but good physical health. He was a heavy smoker throughout his adult life and had had minimal nonpurulent morning sputum production for several years. Some 15 years prior to admission, he had received three “X-ray treatments” to the neck because of “neurodermatitis”; no details of this procedure could be obtained.