A patient with a mucopolysaccharidosis may present with a number of risk factors that make administration of anesthesia difficult. Problems may include a difficult or failed intubation.


Risk factors for difficult administration of anesthesia:

(1) obstructive sleep apnea

(2) atlanto-axial subluxation

(3) heart disease (cardiomyopathy, septal hypertrophy with outflow obstruction, infiltration of a coronary artery with ischemia, mitral or tricuspid valve incompetence)

(4) chronic sinonasal infections with excessive upper respiratory secretions

(5) infiltration of soft tissues with enlargement of tongue, adenoids and/or tonsils

(6) short and immobile neck

(7) limited range of motion in cervical spine and temporomandibular joint

(8) tracheal narrowing

(9) tracheal collapse on neck flexion

(10) short-trunk dwarfism

(11) kyphoscoliosis and/or lumbar lordosis

(12) obstructive lung disease with reduced gas diffusion


Children with Hurler's syndrome and coarse facial features present the greatest difficulty.


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