Abstract
Pelizaeus-Merzbacher disease (PMD) is a genetic leukodystrophy, which is a progressive and degenerative central nervous system abnormality caused by dysmyelination. Because the incidence of PMD is extremely low, only a few case reports have been published regarding its anesthetic management. In particular, epidural anesthesia has only been reported in one case of general anesthesia combined with caudal anesthesia. We performed general anesthesia combined with epidural anesthesia for the soft-tissue release surgery for bilateral hip subluxation in a six-year-old male patient diagnosed with PMD. General anesthesia was induced with sevoflurane in nitrous oxide and oxygen. Rocuronium was administered to facilitate tracheal intubation. After intubation, general anesthesia was maintained with sevoflurane in the air and oxygen. An epidural catheter was placed from L3/4. For epidural anesthesia and analgesia, 1% mepivacaine was used as needed, and 2 ml/h of 0.2% ropivacaine was started one hour before the end of surgery. During surgery, only epidural analgesia was provided as postoperative analgesia, and the patient did not complain of pain after extubation. Anesthesia lasted three hours and 55 minutes. No significant hemodynamic or respiratory changes occurred. Postoperatively, the patient received continuous epidural analgesia and regular oral acetaminophen, and pain control was good. The epidural catheter was removed on the second postoperative day. The postoperative course was good, and the patient was transferred to a pediatric rehabilitation hospital on the fifth postoperative day. No adverse events occurred and no neurological deficits were observed during hospitalization. In conclusion, anesthesiologists should pay attention to the possibility of perioperative aspiration, spasticity, and seizure, even with mild PMD. Proper preoperative evaluations, intraoperative monitoring, and anesthetic techniques will ensure safe anesthesia for PMD patients. Although regional anesthesia in patients with pre-existing neurologic deficits is controversial, we were able to safely perform epidural anesthesia and postoperative continuous epidural analgesia in a pediatric patient with PMD.