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

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