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Pediatric Anesthesia
Anesthesia and Pain Medicine 2013;8(1):64-67.
Published online January 31, 2013.
Experience of endotracheal intubation using video styletscope in a patient with mucopolysaccharidoses: A case report
Hyo Sung Joo, Myung Hee Kim, Ae Ryung Lee, Hui Gyeong Park
Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. mhsmc.kim@samsung.com
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.
Key Words: Mucopolysaccharidoses, Tracheal intubation, Video styletscope


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