Anesthetic care for the child with Morquio syndrome: general versus regional anesthesia

Area of Science:

• Anesthesiology
• Genetics
• Pediatrics

Background:

• Morquio syndrome, a mucopolysaccharidoses (MPS IV) subtype, results from keratin sulfate accumulation.
• This accumulation causes anatomical changes, including airway distortion and cervical spine instability (odontoid hypoplasia).
• These complications pose significant risks during anesthesia, such as difficult intubation and atlanto-axial subluxation.

Observation:

• Two pediatric cases requiring anesthetic management for orthopedic procedures were analyzed.
• One patient received general anesthesia.
• The second patient underwent continuous spinal anesthesia, a novel approach for MPS IV.

Findings:

• Anesthetic management in Morquio syndrome must address potential airway difficulties and cervical spine instability.
• Continuous spinal anesthesia demonstrated feasibility in a pediatric patient with Morquio syndrome.

Implications:

• Highlights the critical need for careful pre-anesthetic evaluation in patients with Morquio syndrome.
• Suggests continuous spinal anesthesia as a potential alternative anesthetic technique in specific pediatric cases of MPS IV.
• Emphasizes the importance of multidisciplinary care for managing complex anesthetic challenges in rare genetic disorders.