Open AccessAnesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery
Author(s) -
Nidhi Gupta,
Girija Prasad Rath,
Renu Bala,
Bapura Kiran Reddy,
Arvind Chaturvedi
Publication year - 2012
Publication title -
saudi journal of anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.416
H-Index - 24
eISSN - 1658-354X
pISSN - 0975-3125
DOI - 10.4103/1658-354x.97036
Subject(s) - medicine , hurler syndrome , hydrocephalus , shunt (medical) , complication , surgery , hunter syndrome , anesthetic , anesthesia , mucopolysaccharidosis , airway , disease
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.