Mucopolysaccharidoses (MPSs) are a group of uncommon genetic diseases of connective tissue metabolism. It is well established\r\nthat the elective treatment of subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in\r\nhighly specialist centers. However, there is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists\r\nmight not have a large experience of MPS. Various attempts to produce guidelines have been made for MPS. There has been an\r\nincreasing interest in the topic as it is demonstrated by the number of scientific studies published in the last few years (peak in 2011).\r\nWe want to provide a summary of anesthetic management, reviewing the current literature on the topic in a procedural algorithm\r\nfor these high-risk patients, who require surgical procedures and diagnostic examinations under sedation with a higher frequency\r\nthan the general population.
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