Anesthetic Management in a Child with Pelizaeus-Merzbacher-like Disease: A Case Report

Background Pelizaeus-Merzbacher-like disease (PMLD), a leukodystrophy, is a rare inherited condition primarily affecting the central nervous system. Clinical features such as seizures, hypotonia, spasticity, contractures, respiratory difficulty, dysphagia, and gastroesophageal reflux raise significant anesthetic concerns. Case We report the successful management of a 14-year-old child with PMLD scheduled for bilateral foot reconstruction for equinovarus deformity. General anesthesia was induced using rapid sequence induction and maintained with sevoflurane, remifentanil, and dexmedetomidine infusions. Neuromuscular blockade was not repeated, and the train-of-four count recovered to 4 prior to emergence. Body temperature and fluid balance were closely monitored. Conclusions Potential difficult airway, aspiration risk, perioperative seizures, respiratory depression, hypothermia, spasticity, hemodynamic instability, prolonged neuromuscular blockade, positioning challenges, and pain control pose significant challenges to anesthesiologists. Detailed assessment, comprehensive anesthetic planning, and vigilant monitoring are essential for safe anesthetic management in patients with PMLD.