[A case report of anesthesia for a child with Pompe's disease (glycogen storage disease type II)].

Abstract

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.

• Authors: Michiko Sakakibara, Yuzo Teramoto, Tadashi Aoyama, Tetsuyuki Takahashi, Masato Hara, Megumi Maseki, Shinya Yamaguchi, Jun Nakata
• Journal: Masui. The Japanese journal of anesthesiology (Masui) Vol. 58 Issue 2 Pg. 219-22 (Feb 2009) ISSN: 0021-4892 [Print] Japan
• PMID: 19227183 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Anesthesia, General (methods)
• Catheterization, Central Venous
• Female
• Glycogen Storage Disease Type II (complications)
• Humans
• Infant
• Tracheotomy