Anaesthesia recommendations for Madelung's disease

Abstract

Madelung’s disease (MD) is a rare disorder affecting predominantly middle-aged males in Mediterranean areas, with a reported male to female ratio ranging from 15:1 to 30:1. It is characterised by diffused symmetrical non-encapsulated lipomas involving the maxillofacial region, neck, shoulders, thorax and head. Masses are normally asymptomatic, causing cosmetic deformity, neck immobility and compression symptoms, such as dyspnoea, dysphagia, dysarthria, hoarseness, and obstructive sleep apnoea. The distribution of lipomas is variable and often indistinguishable from other diseases, such as certain types of morbid obesity, encapsulated lipomas, Cushing syndrome, lymphoma or angiolipomatosis. Therefore, the prevalence may be higher than suggested by literature. The aetiopathogenesis remains unclear. Some factors, such as defects in lipolytic pathways in response to catecholamine, mitochondrial dysfunction, reduction of inducible nitric oxide synthase, and abnormal proliferation of brown fat cells may be involved in the development of the disease. One of the main predisposing factors is alcohol abuse, since approximately 60–90% of MD patients suffer from alcoholism. Chronic alcohol ingestion affects the betaadrenergic receptors, disrupts lipolysis and affects enzymatic processes in the mitochondria. Because of its association with alcohol consumption, MD may be associated with many comorbidities, such as hepatic disorders, arterial hypertension, dyslipidaemia, diabetes mellitus, polyneuropathy, hypothyroidism, macrocytic anaemia, nephropathy, hyperuricaemia, idiopathic thrombocytopaenic purpura or oral cancer. Its diagnosis is established through clinical history and physical examination. Imaging tests complete the diagnosis and exclude the presence of tumours affecting soft tissues. Surgical removal of the adipose masses remains the only effective treatment option. Nevertheless, because of the characteristics of the diffuse and infiltrative lipomas, their complete excision cannot be performed and recurrence is common after surgical treatment. No spontaneous regression of masses has been reported, but alcohol abstinence may delay the progression of the disease and prevent recurrences. The main anaesthetic concern in the treatment of patients with MD is the elevated risk of difficult intubation and a higher risk of postoperative bleeding, because of the difficult haemostasis and the involvement of vessels associated with big masses.