Paediatric surgeon

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

 

Abdominal hernia and ear and respiratory infections are very often the first presentation of the disease, in fact by quite a long way. Most of the other features will not occur until 1 to 2 years of age...enlarged tonsils and adenoids, the large liver and spleen.

- Dr David Whiteman

 

Tympanostomy, adenoidectomy and tonsillectomy are among the most common surgical procedures performed to relieve symptoms in Hunter syndrome patients. 2 Overall, 57% of Hunter syndrome patients undergo surgery before a diagnosis of Hunter syndrome is made and 84% of all Hunter syndrome patients undergo surgery at some stage. 2 Preparation before surgery is key; patients should be assessed by a multidisciplinary team that includes a cardiologist, an ENT specialist and an anaesthetist with experience in MPS disorders, to evaluate any anatomical obstacles to anaesthesia or problems such as valve degeneration. 1,3

An ENT specialist or paediatric pulmonologist can provide support during surgery in the event of complications, and it is good practice to plan multiple surgical procedures for a single anaesthesia session, however, extending the operation time increases the risk of respiratory complications. 3 After surgery in Hunter syndrome patients, there is still a great risk of airway complications, so monitoring is important during this period. 3

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References

  1. 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.
  2. 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.
  3. Muenzer J et al. Multidisciplinary management of Hunter syndrome. Pediatrics 2009; 124(6): e1228–e1239.

Nurses

Rheumatologist