Anesthetic management of a child with Hunter′s syndrome

• Main Authors: Jasmeet Kaur, Adarsh C Swami, Amit Kumar, Sneh Lata
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2012-01-01
• Series: Journal of Anaesthesiology Clinical Pharmacology
• Subjects: Difficult airway; Hunter′s syndrome; mucopolysaccharidoses; pediatric patient
• Online Access: http://www.joacp.org/article.asp?issn=0970-9185;year=2012;volume=28;issue=2;spage=255;epage=257;aulast=Kaur

Hunter′s syndrome is a member of a group of recessively inherited metabolic disorders termed mucopolysaccharidoses, caused by deficiency of lysosomal enzymes required for degradation of mucopolysaccharides or glycosaminoglycans, leading to accumulation of partially degraded glycosaminoglycans in various tissues. This leads to various anatomical abnormalities and systemic involvement, posing a challenge to an anesthetist. We present the anesthetic management of a 4-year old child with Hunter′s disease with anticipated difficult airway, who presented for adenotonsillectomy and repair of umbilical and inguinal hernia.