[MIM 607 015]

(Mucopolysaccharidosis type I H/S)

Mucopolysaccharidosis. Autosomal recessive transmission of a mutation at locus 4p16.3 causing a deficiency in α-L-iduronidase.

Similar clinical picture as Hurler's disease but with a later onset and a slower evolution with a conserved intelligence.

Anesthetic implications: 

difficult intubation (macroglossia). Valvular heart failure with a risk of cardiac failure.

References : 

-        Nicolson SC, Black AE, Kraras CM. 
Management of a difficult airway in a patient with Hurler-Scheie syndrome during cardiac surgery.
Anesth Analg 1992; 75: 830-2.

Updated: March 2019