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...

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Bibliographic Details
Published in:Pediatric anesthesia 2024-01, Vol.34 (1), p.89-91
Main Authors: Kawamura, Akihisa, Tsuboi, Kaoru, Oka, Aiko, Sakaguchi, Hirotoshi, Suzuki, Yasuyuki
Format: Article
Language:English
Subjects:
airway management
anesthesia
Anesthesia - methods
Child
Child, Preschool
Genetic disorders
Humans
Ketamine
Male
Metabolic disorders
Mucopolysaccharidosis II - complications
mucopolysaccharidosis type II
ophthalmic surgical procedures
Propofol
Stevens-Johnson Syndrome - complications
Stevens-Johnson Syndrome - therapy
toxic epidermal necrolysis
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Summary: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.
ISSN:1155-5645
1460-9592
DOI:10.1111/pan.14746