Difficult tracheal intubation is the
rule and fiberoptic intubation should be considered. In spite of the
atlantoaxial instability, mucopolysaccharides may accumulate in the tongue
and nasopharyngeal tissues, making visualization of the glottic opening
difficult during direct laryngoscopy. Hence, patients may also present with
obstructive apnea and increased sensitivity to the ventilatory depressant
effect of narcotics, so regional anesthesia for postoperative pain control
is recommended, as well as observation in the intensive care unit for 24 to
48 hours. Finally, the heart may be affected by mucopolysaccharide
accumulation in different ways, so the dose of anesthetics should be
adjusted accordingly.