Open AccessManagement of an anticipated difficult airway in Hurler′s syndrome
Author(s) -
T Gurumurthy,
S Shailaja,
Shetty Kishan,
M. James Stephen
Publication year - 2014
Publication title -
journal of anaesthesiology-clinical pharmacology/journal of anaesthesiology clinical pharmacology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.466
H-Index - 32
eISSN - 2231-2730
pISSN - 0970-9185
DOI - 10.4103/0970-9185.142862
Subject(s) - medicine , hurler syndrome , mucopolysaccharidosis , mucopolysaccharidosis i , airway , umbilical hernia , airway management , intubation , tracheal intubation , ventilation (architecture) , mucopolysaccharidosis type i , anesthesia , pediatrics , surgery , enzyme replacement therapy , hernia , disease , mechanical engineering , engineering
Management of an anticipated difficult airway in Hurler syndrome. Hurler syndrome is a subtype of Mucopolysaccharidosis (MPS) type 1. Mucopolysaccharidosis (lysosomal storage diseases) are a group of inherited disorders caused by deficiency of specific lysosomal enzyme required for a normal degradation of glycosaminoglycons (GAGs). Administration of general anaesthesia in patients who have congenital syndromes such as Hurler's is often a challenge because of progressive airway, craniofacial and skeletal abnormalities that may make both the ventilation and intubation difficult. We encountered difficult mask ventilation and endotracheal intubation was not possible and finally ventilated with laryngeal mask airway in a known case of Hurler syndrome posted for umbilical hernia repair.