Anesthetic management of a child with Hunter′s syndrome

Abstract

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.

Keywords

• Difficult airway
• Hunter′s syndrome
• mucopolysaccharidoses
• pediatric patient