Anesth Pain Med.  2013 Jan;8(1):64-67.

Experience of endotracheal intubation using video styletscope in a patient with mucopolysaccharidoses: A case report

• Joo HS
• Kim MH
• Lee AR
• Park HG

Abstract

Mucopolysaccharidoses (MPSs) are a group of inherited heterogenous metabolic disorders, caused by deficiency of an enzyme involved in the degradation of mucopolysaccharides, and thus deposition of mucopolysaccharides in all connective tissue. MPS type III is heparan sulphate storage disorder, especially marked central nervous system involvement. As with all the MPS disorders, this patient with MPS III presents coarse outlook features, such as macrocephaly, macroglossia, prominent mandible, short neck, and unstable atlantoaxial joints, as well as tracheal abnormality. These clinical findings give significant challenges to the anesthesiologist for adequate airway management and tracheal intubation. Recently, a number of new devices have been developed to facilitate intubation. We report an experience of facilitated tracheal intubation using video styletscope (OptiScope PM201(R), Pacific Medical, Seoul, Korea) in a 16 year old male patient with MPS III, who was undergoing inguinal hernia repair.

Keyword

Mucopolysaccharidoses; Tracheal intubation; Video styletscope

MeSH Terms

Airway Management
Atlanto-Axial Joint
Central Nervous System
Connective Tissue
Glycosaminoglycans
Hernia, Inguinal
Humans
Intubation
Intubation, Intratracheal
Macrocephaly
Macroglossia
Male
Mandible
Mucopolysaccharidoses
Neck
Glycosaminoglycans