Abstract
Mucopolysaccharidoses (MPSs) are a group of uncommon genetic diseases of connective tissue metabolism. It is well established that the elective treatment of subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in highly specialist centers. However, there is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists might not have a large experience of MPS. Various attempts to produce guidelines have been made for MPS. There has been an increasing interest in the topic as it is demonstrated by the number of scientific studies published in the last few years (peak in 2011). We want to provide a summary of anesthetic management, reviewing the current literature on the topic in a procedural algorithm for these high-risk patients, who require surgical procedures and diagnostic examinations under sedation with a higher frequency than the general population.
Highlights
The necessity of codified and univocal guidelines, suitable for all patients, is a common challenge in the field of rare diseases
There is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists might not have a large experience of MPS
Various attempts to produce guidelines of this kind have been made for mucopolysaccharidoses (MPSs)
Summary
The necessity of codified and univocal guidelines, suitable for all patients, is a common challenge in the field of rare diseases. The first difficulty arises from the lack of cases to study, so as to identify without excessive variety the clinical features of the conditions in study and from the consequent poor literature and the differences between worldwide centers in diagnosis, treatment, and management protocols. It is well established that the elective treatment for subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in highly specialized centers. There is the possibility to perform an anesthesia for these kinds of pediatric patients in a peripheral center, where anesthesiologists might not have a large experience of MPS. Our intent is to review the current literature on this topic in a procedural algorithm of anesthetic management for these high-risk patients, requiring surgical procedures and diagnostic examinations under sedation with a higher frequency than the general population. Our final aim is to improve the quality of care offered and, as a consequence, the quality of life of subjects affected by MPS
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