CASE REPORT
Year : 2012  |  Volume : 6  |  Issue : 2  |  Page : 178-180

Anesthetic management in children with Hurler's syndrome undergoing emergency ventriculoperitoneal shunt surgery


Department of Neuroanaesthesiology, All India Institute of Medical Sciences (A.I.I.M.S.), New Delhi, India

Correspondence Address:
Girija Prasad Rath
Department of Neuroanaesthesiology, Neurosciences Centre, 6th Floor/ Room No. 9, A.I.I.M.S., New Delhi - 110 029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-354X.97036

Rights and Permissions

Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler's syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with increased intracranial pressure is a frequent complication seen in these patients. The rarity of the syndrome accounts for the small number of case reports and anecdotal information on the intracranial manifestations of this syndrome. We describe the successful anesthetic management of 2 children with Hurler's syndrome who underwent ventriculoperitoneal shunt surgery for acute hydrocephalus.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed2744    
    Printed110    
    Emailed0    
    PDF Downloaded120    
    Comments [Add]    

Recommend this journal