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
- Scarpa M et al. Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease. Orphanet J Rare Dis 2011; 6: 72.
- Mendelsohn NJ et al. Importance of surgical history in diagnosing mucopolysaccharidosis type II (Hunter syndrome): Data from the Hunter Outcome Survey. Genet Med 2010; 12(12): 816–822.
- Muenzer J et al. Multidisciplinary management of Hunter syndrome. Pediatrics 2009; 124(6): e1228–e1239.