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Anaesthetic problems in Hurler‐Scheie syndrome. Report of two cases
Author(s) -
Sjøgren P.,
Pedersen T.
Publication year - 1986
Publication title -
acta anaesthesiologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.738
H-Index - 107
eISSN - 1399-6576
pISSN - 0001-5172
DOI - 10.1111/j.1399-6576.1986.tb02459.x
Subject(s) - medicine , hurler syndrome , mucopolysaccharidosis i , mucopolysaccharidosis , sedation , pneumonia , anesthesia , intubation , tracheal intubation , surgery , hunter syndrome , pathology , disease , enzyme replacement therapy
Hurler‐Scheie syndrome is an intermediate form of mucopolysaccharidosis. Affected patients characterstically present in infancy with serious abnormalities including the upper airways and the lungs. We present two patients with Hurler‐Scheie syndrome and describe their anaesthetic management. One caw was complicated by difficult endotracheal intubation and postoperative pneumonia. The second casr was successfully miinagtd using spinal anaesthesia and mild sedation.