Awake intubation for anesthesia in a patient with mucopolysaccharidosis type vi (maroteaux-lamy syndrome)

Abstract

Mucopolysaccharidoses (MPS) are a group of rare, progressive hereditary diseases caused by the deficiency of 11 lysosomal enzymes responsible for the degradation of glycosaminoglycans (GAGs). Enzymatic dysfunction leads to the progressive accumulation of unmetabolized GAGs in tissues and organs, including the airways, making management difficult and constituting a challenge for the anesthesiologist. This article reports the anesthetic management of a patient with Mucopolysaccharidosis type VI (Maroteaux-Lamy Syndrome) who underwent laparoscopic herniorrhaphy. After pre-anesthetic evaluation, general anesthesia with awake nasotracheal intubation was chosen, performed via fiberoptic bronchoscopy.