. 2019 May 29;14:118. doi: 10.1186/s13023-019-1080-y

Table 13.

Guidance statements for tonsillectomy and/or adenoidectomy, tracheostomy and insertion of ventilation tubes

Statement	Percentage consensus
Tonsillectomy and/or adenoidectomy is recommended in patients with MPS VI who display upper airway obstruction, recurrent otitis media, snoring and/or OSA, as early as possible following diagnosis without waiting for disease progression Evidence Grade: C (level 2/3/4 studies)	91%
Tracheostomy is recommended in patients with MPS VI who exhibit severe upper airway obstruction that cannot be treated by an alternative approach, and in patients with severe sleep apnoea that is not treatable by CPAP or tonsillectomy and/or adenoidectomy Evidence Grade: D (limited published evidence)	95%
Insertion of ventilation tubes is recommended for patients with MPS VI with otitis media with effusion and/or recurrent otitis media to maintain hearing and/or prevent recurrent acute otitis media Evidence Grade: D (limited published evidence)	96%

Statement

Percentage consensus

Tonsillectomy and/or adenoidectomy is recommended in patients with MPS VI who display upper airway obstruction, recurrent otitis media, snoring and/or OSA, as early as possible following diagnosis without waiting for disease progression

Evidence Grade: C (level 2/3/4 studies)

91%

Tracheostomy is recommended in patients with MPS VI who exhibit severe upper airway obstruction that cannot be treated by an alternative approach, and in patients with severe sleep apnoea that is not treatable by CPAP or tonsillectomy and/or adenoidectomy

Evidence Grade: D (limited published evidence)

95%

Insertion of ventilation tubes is recommended for patients with MPS VI with otitis media with effusion and/or recurrent otitis media to maintain hearing and/or prevent recurrent acute otitis media

Evidence Grade: D (limited published evidence)

96%