The deformity of the chest, the enlarged abdomen, and the contractures, make it difficult to position the [MPS II] patient in the operating theatre.

- Dr Christina Lampe

Common Hunter syndrome symptoms include otitis media, abdominal hernia and enlarged tonsils/adenoids.1 These symptoms occur early in Hunter syndrome patients and in combination, and may be refractory to treatment, which means that patients often have multiple surgical interventions and associated anaesthesia procedures.2 For example, 40% of Hunter syndrome patients have had more than one hernia repair.2

Sedation and general anaesthesia are high-risk procedures, due to the anatomic abnormalities inherent to Hunter syndrome that lead to positioning problems, so these procedures require careful management.3 A flexible nasendoscopy, computed tomography scanning of the airway, or a video-recorded bronchoscopy of the airways with a flexible fibreoptic bronchoscope can be performed prior to surgery, to help anaesthetists prepare for the anatomical/positional obstacles that they will encounter.3

It is good practice to plan multiple surgical procedures for a single anaesthesia session and to monitor patients after a procedure, due to the risk of post-procedural oedema.3 It is valuable for anaesthetists to be able to consult with the other specialists (e.g. an ENT specialist or paediatric pulmonologist) during the induction of anaesthesia.3



Download an information leaflet for anaesthetists
For further information on the specialist management of Hunter syndrome patients by anaesthetists



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