A case of Madelung's disease (benign symmetrical lipomatosis) with airway stenosis

Abstract

Madelung's disease (benign symmetrical lipomatosis) is characterized by symmetrical fat accumulation at multiple sites in the neck, trunk and limbs. We experienced a case of Madelung's disease in which tracheostomy and lipectomy were required to treat airway stenosis. The patient was a 64-year-old man with a long history of heavy alcohol consumption. He presented with complaints of hoarseness, neck swelling, and shortness of breath on exertion. CT and endoscopy revealed airway narrowing due to fatty deposits in the subcutaneous tissue and within the larynx. We therefore performed lipectomy and tracheostomy. To achieve a better long-term outcome, abstinence from alcohol is essential; however, he could only abstain during hospitalization and started drinking after discharge. Since he had no intention to continue to abstinence and indicated that he did not wish to receive additional treatment, we decided not to close the tracheostomy, and a tracheal cannula was placed. When treating patients with Madelung's disease, it is crucial to consider surgical treatment (lipectomy and tracheostomy) when functional abnormalities, such as airway obstruction, are observed.