Anaesthetic management of a preadolescent child with Mucopolysaccharidosis type II (Hunter’s syndrome) for insertion of a ventriculoperitoneal shunt

Mucopolysaccharidosis type II or Hunter’s syndrome is a rare, X-linked genetic lysosomal storage disorder, which results in multiorgan dysfunction and accumulation of glycosaminoglycans in various tissues, including the airway. Because of this, airway management in these patients has been a challenge among anaesthesiologists. In this report, we describe an awake tracheal intubation of a preadolescent-aged child with Hunter’s syndrome scheduled for ventriculoperitoneal shunt insertion. The preservation of ventilatory drive and airway tone, combined with continuous passive oxygen supplementation during airway manipulation, allowed for safe use of advanced airway techniques in an anticipated difficult paediatric airway. Moreover, understanding of the full range of issues surrounding the condition helped mitigate complications and successfully deliver anaesthesia for the patient.