General Anesthesia Management for Adult Mucopolysaccharidosis Patients Undergoing Major Spine Surgery.

OBJECTIVE

Mucopolysaccharidosis (MPS) are a group of rare systemic lysosomal storage diseases associated with severe airway obstruction and cardiac disease, making anesthesia management difficult. Contemporary treatment extends the lifespan of affected individuals, increasing the need for major surgery in adulthood.

CLINICAL PRESENTATION AND INTERVENTION: We provided general anesthesia for 6 adult MPS patients undergoing spine surgery. The airway was assessed as difficult in all, with 2 receiving awake fiberoptic intubation and 1 successfully undergoing video-laryngoscopy, while 3 video-laryngoscopy procedures failed and required conversion to fiberoptic intubation. One patient developed ventricular fibrillation.

CONCLUSION

Adult MPS patients have substantial anesthesia risk.