Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management

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.

FIGURE 1

Anesthetic airway management in a series of 49 Mayo Clinic patients with various types of mucopolysaccharoidoses.

FIGURE 2

(A and B) A 15-year-old girl with mucopolysaccharidosis Type VI. Despite the fact that her facial characteristics gave impression that her airway is “manageable,” she had difficult mask ventilation, and 3 failed attempts to place a laryngeal mask airway. Placement of endotracheal tube was successful with a video laryngoscope aided by fiberoptic bronchoscope (see discussion for details). Published with the consent of patient’s legal representative.