Why is Madelung’s disease a challenge for the anaesthesiologist?

Abstract

Source: Authors. The accompanying images exhibit a large round, subcutaneous fat mass protruding from the posterior cervical area and also several other masses along the back, in a patient with Madelung disease (Image 1). From an anterior view, it is also possible to see big bulky cheeks and another fat mass on the anterior neck (Image 2). Madelung’s disease, also known as multiple symmetric lipomatosis or Launois–Bensaude syndrome is a rare lipid metabolic disorder characterized by diffuse, progressive growth of encapsulated subcutaneous adipose tissue, in the neck, shoulder and other areas, as can be seen in the image. The exact cause of Madelung’s disease has not yet been fully understood, but there’s one theory that argues that a defect in the adrenergic-stimulated fat breakdown (lipolysis) process could result in improper fat deposits. Madelung’s disease affects more males than females (ratio of 15-30:1) and is usually diagnose between 30-70 years of age. For unclear reasons, this disorder appears to be more prevalent in Mediterranean and European population as compared to others regions in the world. (1) As described, the characteristics of Madelung’s disease imposes a careful anaesthetic approach because it makes