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.

A retrospective comparison of propofol to dexmedetomidine for pediatric magnetic resonance imaging sedation in patients with mucopolysaccharidosis type II

“…Mucopolysaccharidosis (MPS) type II, also known as Hunter syndrome, is a rare X‐lined recessive disease caused by deficiencies of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (GAGs) . Accumulated GAGs in the upper airway can lead to severe airway obstruction due to macroglossia, tonsil and adenoid hypertrophy, vocal cord enlargement, supraglottic narrowing, and tracheomalacia . In particular, MPS II patients have a higher incidence of difficult airway than other types of MPS patients .…”

“…1 Accumulated GAGs in the upper airway can lead to severe airway obstruction due to macroglossia, tonsil and adenoid hypertrophy, vocal cord enlargement, supraglottic narrowing, and tracheomalacia. 2,3 In particular, MPS II patients have a higher incidence of difficult airway than other types of MPS patients. 4,5 Therefore, it can be challenging to achieve the desired level of sedation while maintaining a patent airway and ensuring ventilation during deep sedation of such patients.…”

Pediatric syndromes with noncraniofacial anomalies impacting the airways

“…As there is a phenotypic variety, the anesthesiologist may use one or more devices to obtain the optimal ventilation (Figure ) …”

“…Anesthetic airway management in a series of 49 patients (Mayo Clinic) with MPS. Reproduced with permission Clark et al…”

Successful spinal anesthesia in a patient with mucopolysaccharidosis type I under femoral fracture reduction and external fixation

“…Therefore, airway‐related anesthetic complications in patients with MPS I are also a part of pediatric anesthesia. Clark et al found that the overall incidence of difficult tracheal intubation in a case series of various types of MPS ranged from 28% to 44%. Regional anesthesia seems to be a logical choice for these patients.…”