Anesthetic management in children with Hurler's syndrome undergoing emergency ventriculoperitoneal shunt surgery

Area of Science:

• Anesthesiology
• Genetics
• Pediatrics

Background:

• Mucopolysaccharidoses (MPSs) are lysosomal storage disorders.
• MPS I (Hurler's syndrome) poses significant airway management challenges for anesthesiologists.
• Hydrocephalus and increased intracranial pressure are common complications in MPS I.

Observation:

• Limited case reports exist on intracranial manifestations of Hurler's syndrome.
• Successful anesthetic management of two pediatric patients with Hurler's syndrome is described.
• Both patients required ventriculoperitoneal shunt surgery for acute hydrocephalus.

Findings:

• The anesthetic approach prioritized airway security and hemodynamic stability.
• Careful preoperative assessment and planning were crucial for successful outcomes.
• Ventriculoperitoneal shunt placement was safely achieved under general anesthesia.

Implications:

• This case series provides valuable insights for managing complex pediatric anesthesia cases.
• Highlights the importance of multidisciplinary collaboration in rare genetic disorders.
• Contributes to the limited literature on anesthetic considerations for Hurler's syndrome with hydrocephalus.