A 7 year old 35 kg girl with Hurler syndrome for adenotonsillectomy is at increased risk compared to a healthy 7 year old for the following:
a) neurological injury from neck extension
b) postoperative respiratory events
c) difficult face mask ventilation on induction
d) all of the above
Answer d.
Hurler syndrome, or mucopolysaccharidosis I, is an autosomal recessive disorder where the congenital absence of the lysosomal enzyme alpha- L-iduronidase leads to the build-up of the glycosaminoglycans heparan sulfate and dermatan sulfate in the tissues; the tongue, pharyngeal connective tissue and skeletal structures are involved, leading to propensity for upper airway obstruction and development of an unstable cervical spine.