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.

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Baastrup disease is a condition where spinous processes form painful pseudoarticulations. We present a patient with midline axial back pain consistent with Baastrup disease confirmed by computed tomography, which revealed degenerative changes along the opposing surfaces of the spinous processes at L1-2 and L2-3. Local anesthetic and steroid interspinous injections lost their effectiveness over time. She underwent successful interspinous radiofrequency lesioning, which has not been previously described in the literature. At 4 months follow-up, she reported complete pain relief.

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Intraoperative tourniquet-related pain is well recognized by anesthesiologists, but postoperative tourniquet-related pain has received little attention. We present a patient who underwent a total knee arthroplasty under general anesthesia and an adductor canal nerve block. She complained of intractable thigh pain postoperatively. Lidocaine 5% patches completely resolved her postoperative tourniquet-related pain.