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- W2272191201 abstract "Abstract : Ullrich syndrome is a rare congenitalhypotonic-sclerotic muscular disorder in which affectedchildren develop a slowly progressive scoliosis andcontractures and limpness of joints. The disease causesincreasingly invalidating contractures and hardening ofthe muscles of the neck and trunk. While this neuromuscular type of scoliosis is progressive, patients rarely attain the point of surgery due to their compromisedgeneral medical condition. This may explain the currentlack of outcome data and the paucity of information onperioperative management for patients with Ullrichsyndrome undergoing major surgery. The purpose ofthis report was therefore to describe our first experiencewith the perioperative and anesthetic management of a15-year old boy presenting with Ullrich syndrome and asecondary invalidating scoliosis. The specific challengesof this condition characterized by severe restrictive lungdisease and a challenging airway abnormality arediscusse" @default.
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- W2272191201 date "2010-01-01" @default.
- W2272191201 modified "2023-09-27" @default.
- W2272191201 title "Anesthesia and perioperative management for a patient with Ullrich syndrome undergoing surgery for scoliosis." @default.
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