[A case report of anesthesia for a child with Pompe's disease (glycogen storage disease type II)].
A case report of a child with Pompe's disease (glycogen storage disease type II), who underwent two general anesthetics, is presented. The progressive infiltration of heart and skeletal muscle with glycogen results in a severe form of cardiomyopathy and respiratory muscle weakness. Death usually occurs by 1 year of age from respiratory insufficiency or end-stage cardiomyopathy. Consequently, there are significant problems in the anesthetic management of these patients. The patient, a female child of 6 months presented signs of cardiac failure and took treatment with olprinone and diuretic. The initial surgical procedure was placement of a subcutaneous central venous catheter. Anesthesia was induced with ketamine and midazolam intravenously, and was maintained with nitrous oxide (50%) and intravenous ketamine. The dosage of the dopamine and olprinone was necessary to maintain circulation. For tracheotomy, the second procedure, anesthesia was induced and maintained with sevoflurane and dopamine. Recently a promising enzyme replacement therapy has resulted in improved clinical outcomes, and the number of anesthesia for infants of with Pompe's disease will increase in future.
Sakakibara
Michiko
M
Department of Anesthesiology, Toyohashi Municipal Hospital, Toyohashi 441-8570.
Teramoto
Yuzo
Y
Aoyama
Tadashi
T
Takahashi
Tetsuyuki
T
Hara
Masato
M
Maseki
Megumi
M
Yamaguchi
Shinya
S
Nakata
Jun
J
D000768:Anesthesia, General / Q000379:methods*; D002405:Catheterization, Central Venous; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000150:complications*; D006801:Humans; D007223:Infant; D014140:Tracheotomy