Abstract
Mucopolysaccharidoses (MPS) are rare, inherited, lysosomal storage diseases that cause accumulation of glycosaminoglycans, resulting in anatomic abnormalities and organ dysfunction that can increase the risk of anesthesia complications. We conducted a systematic review of the literature in order to describe the anesthetic management and perioperative outcomes in patients with MPS. We reviewed English-language literature search using an OVID-based search strategy of the following databases: 1) PubMed (1946-present), 2) Medline (1946-present), 3) EMBASE (1946-present), and 4) Web of Science (1946-present), using the following search terms: mucopolysaccharidosis, Hurler, Scheie, Sanfilippo, Morquio, Maroteaux, anesthesia, perioperative, intubation, respiratory insufficiency, and airway. The review of the literature revealed nine case series and 27 case reports. A substantial number of patients have facial and oral abnormalities posing various challenges for airway management, however, evolving new technologies that include videolaryngoscopy appears to substantially facilitate airway management in these patients. The only type of MPS that appears to have less difficulty with airway management are MPS III patients, as the primary site of glycosaminoglycan deposition is in the central nervous system. All other MPS types have facial and oral characteristics that increase the risk of airway management. To mitigate these risks, anesthesia should be conducted by experienced anesthesiologists with expertise in using of advanced airway intubating devices.
Highlights
Mucopolysaccharidoses (MPS) are rare, inherited, lysosomal storage diseases characterized by deficiencies in 11 different lysosomal enzymes involved in the metabolism of glycosaminoglycans, previously known as mucopolysaccharides
The systematic review of the literature identified nine case series, and their airway management is summarized in Table 2 [5,8,9,10,11,12,13,14,15]
We identified 27 individual case reports and these patients’ characteristics and their airway management is summarized in Tables 3 and 4 [7,8,9,10,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38]
Summary
Mucopolysaccharidoses (MPS) are rare, inherited, lysosomal storage diseases characterized by deficiencies in 11 different lysosomal enzymes involved in the metabolism of glycosaminoglycans, previously known as mucopolysaccharides These enzyme deficiencies result in progressive, widespread accumulation of partially degraded glycosaminoglycans in the lysosomes of various tissues and organs; the characteristic patterns of accumulation form the basis of MPS classification into seven types of progressive MPS diseases (Table 1) [1,2,3,4,5]. Glycosaminoglycan accumulation in the upper airway results in hypertrophy of adenoids, tonsils, tongue, and laryngopharynx, which may all pose difficulty for anesthetic airway management This is especially important because MPS patients frequently require surgical interventions with anesthesia.
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