Anesthesia management in a child with mucopolysaccharidosis

Paediatric anaesthesia · Jan 2024

Case Reports

Anesthesia management in a child with mucopolysaccharidosis and toxic epidermal necrolysis: A case report.

Toxic epidermal necrolysis and mucopolysaccharidosis are both rare diseases that pose significant airway maintenance challenges to anesthesiologists. In this report, we describe the anesthesia management in a 4-year-old male with mucopolysaccharidosis type II who developed toxic epidermal necrolysis and presented for ophthalmic surgical procedures. Combined use of propofol and ketamine with the support of high-flow nasal oxygen enabled adequate analgesia and sedation while maintaining spontaneous ventilation and airway patency. The strategy presented in this report may contribute to enhancing the safety of sedation in spontaneously breathing children with abnormal airways.

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- Akihisa Kawamura, Kaoru Tsuboi, Aiko Oka, Hirotoshi Sakaguchi, and Yasuyuki Suzuki.
- Department of Critical Care and Anesthesia, National Center for Child Health and Development, Tokyo, Japan.
- Paediatr Anaesth. 2024 Jan 1; 34 (1): 899189-91.
AbstractToxic epidermal necrolysis and mucopolysaccharidosis are both rare diseases that pose significant airway maintenance challenges to anesthesiologists. In this report, we describe the anesthesia management in a 4-year-old male with mucopolysaccharidosis type II who developed toxic epidermal necrolysis and presented for ophthalmic surgical procedures. Combined use of propofol and ketamine with the support of high-flow nasal oxygen enabled adequate analgesia and sedation while maintaining spontaneous ventilation and airway patency. The strategy presented in this report may contribute to enhancing the safety of sedation in spontaneously breathing children with abnormal airways.© 2023 John Wiley & Sons Ltd.

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Anesthesia management in a child with mucopolysaccharidosis and toxic epidermal necrolysis: A case report.

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