Indian Journal of Anaesthesia

CASE REPORT
Year
: 2016  |  Volume : 60  |  Issue : 5  |  Page : 349--351

Anaesthetic management of a patient with Pompe disease for kyphoscoliosis correction


Vaishali Kumbar, Jayashree Simha, Parameswara Gundappa 
 Department of Anaesthesiology, Manipal Hospital, Bengaluru, Karnataka, India

Correspondence Address:
Vaishali Kumbar
Department of Anaesthesia, Manipal Hospital, 98, Hal Airport Road, Bengaluru, Karnataka
India

Pompe disease (PD) is a type II glycogen storage disease, characterised by abnormal glycogen deposition, mainly in heart and skeletal muscles, leading to progressive loss of muscle function. The infantile variety is associated with severe hypertrophic cardiomyopathy and generally do not reach adulthood. The juvenile variety presents with progressive muscle weakness and respiratory failure. Anaesthetic management concerns in the patient reported here were mainly due to respiratory failure, myopathy and sensitivity to muscle relaxants and significant haemodynamic changes perioperatively. We successfully managed a 13-year-old girl with juvenile PD on respiratory support scheduled for thoracolumbar kyphoscoliosis corrective surgery. Ketamine and dexmedetomidine were used for induction of anaesthesia and maintenance. Muscle relaxants were diligently avoided in this case.


How to cite this article:
Kumbar V, Simha J, Gundappa P. Anaesthetic management of a patient with Pompe disease for kyphoscoliosis correction.Indian J Anaesth 2016;60:349-351


How to cite this URL:
Kumbar V, Simha J, Gundappa P. Anaesthetic management of a patient with Pompe disease for kyphoscoliosis correction. Indian J Anaesth [serial online] 2016 [cited 2020 Nov 24 ];60:349-351
Available from: https://www.ijaweb.org/article.asp?issn=0019-5049;year=2016;volume=60;issue=5;spage=349;epage=351;aulast=Kumbar;type=0