CASE PRESENTATION: The patient was a 17-month-old boy who weighed 6.5 kg and was 71 cm tall. He presented for
strabismus surgery. He had
muscular hypotonia,
mental retardation, hepatic dysfunction, mild cerebellar hypoplasia, and some dysmorphic features including inverted nipples and abnormal subcutaneous fat distribution of the hips. Gene analysis revealed a compound heterozygous mutation in the gene encoding PMM2 and the patient was diagnosed as having
PMM2-CDG.
General anesthesia was performed with
sevoflurane,
nitrous oxide, and
rocuronium. Neuromuscular monitoring was performed during
anesthesia using train-of-four (TOF)-Watch® (MSD, Japan). As postoperative
analgesia, the surgeon performed sub-Tenon's
anesthesia. We did not use any intravenous
analgesic. After completion of the operation, residual
rocuronium was competed by administration of
sugammadex. The patient gained consciousness and spontaneous breathing was established shortly thereafter, and the trachea was smoothly extubated. He was transported to an inpatient ward and was discharged to his home the next day without any complication.
CONCLUSIONS: We performed safe
anesthetic management in a 17-month-old boy with
PMM2-CDG using
rocuronium under neuromuscular monitoring.A patient with
PMM2-CDG may show nearly normal susceptibility to nondepolarizing NMBAs.