(Launois-Bensaude benign symmetric lipomatosis, cephalothoracic lipodystrophy)

Very rare: 1/25.000. Much more common in men than in women. There is an important and symmetric accumulation of fatty tissue (uncapsulated lipomas) at the level of the cervicothoracic region. The cause is unknown but there is a strong association with excessive consumption of alcohol. A deficit of lipolysis of adrenergic origin and accumulation of brown fat of fetal origin have also been reported.

Treatment: surgical removal, liposuction.

Anesthetic implications:

alcoholism, risk of difficult ventilation by mask or intubation, potentially difficult central venous access via the jugular or subclavian veins (depending on the location of the masses)

References : 

• Ampollini L, Carbognani P. 
  Madelung’s disease. 
  NEJM 2011; 364: 465 (3 Feb)

• Becerra-Bolaños A, Valencia L,  Cabrera-Ramírez L,  Rodríguez-Pérez A.
  Madelung’s disease and airway management.
  Anesthesiology 2019 ; 130 : 313-4
• Weng ZC, Tapia Salinas B, Noguera Tomas J, Del Castillo Pardo De Vera JL, De la Morena Molina JD.
  Anesthetic management of a patient with Madelung’s disease presenting with a giant cervical lipoma: a case report.
  A&A Practice 2024;18:e01779

Updated: April 2024