Anesthetic Management in Mucopolysaccharidoses

Table 4

Anesthetic risk: levels and causes.

MPS	Risk level	Multisystem impairment
I H (Hurler) [56]; II (Hunter) [57]	Very high; High	(i) Tendency to obstruction of the upper airway; (ii) Difficult intubation; (iii) Presence of thick secretion in the airway; (iv) Possible cardiac impairment; (v) Possible odontoid hypoplasia
I S (Scheie) [56]; I HS (Hurler-Scheie) [56]	Mildly high; High	(i) Typical problems of I H (I HS); (ii) Risk of postoperative apnea; (iii) Heart disease (in particular aortic insufficiency)
III (Sanfilippo) [58]	Generally not increased
IV (Morquio) [59]; VII (Sly) [50]	Very high	(i) Odontoid hypoplasia; (ii) Difficult intubation; (iii) Possible alteration of the rib cage; (iv) Possible aortic insufficiency
VI (Maroteaux-Lamy) [23]	High	(i) Cardiomyopathy; (ii) Severe kyphoscoliosis; (iii) Thrombocytopenia; (iv) Possible odontoid hypoplasia; (v) Delayed awaking