Korean J Anesthesiol.  1996 Oct;31(4):534-539. 10.4097/kjae.1996.31.4.534.

Epidural Anesthesia in a Patient with Pompe's Disease: A case report

• Kim JG
• Lim SY
• Shin KM
• Hong SY
• Choi YR

• KMID: 2240369
• DOI: http://doi.org/10.4097/kjae.1996.31.4.534

Abstract

A case report of a 19-year-old girl with the juvenile form of Pompe's disease, who underwent thoracic epidural anesthesia, is presented. Pompe's disease, glycogen storage disease type II, is an autosomal recessive disorder characterized by the lysosomal accumulation of glycogen. Patients with the juvenile form are distinguished by involvement of limb-girdle, and respiratory muscles without cardiac or nervous system manifestations. She had been managed with nasal intermittent positive pressure ventilation (NIPPV) and nightly ventilatory support. She had scoliosis with vertebral rotation and showed respiratory muscular weakness. In order to prevent postoperative respiratory complication and to achieve cardio- pulmonary stability, we chose the thoracic epidural block to perform appendectomy. Epidural injection of 0.8% lidocaine gave good sensory block without motor block and maintained cooperative state throughout the operation. Postoperatively, she remained well, but with NIPPV.

Keyword

Anesthetic techniques thoracic epidural; Metabolism Pompe's disease

MeSH Terms

Anesthesia, Epidural*
Appendectomy
Female
Glycogen
Glycogen Storage Disease Type II*
Humans
Injections, Epidural
Intermittent Positive-Pressure Ventilation
Lidocaine
Muscle Weakness
Nervous System
Respiratory Muscles
Scoliosis
Young Adult
Glycogen
Lidocaine