Certain steps should be taken to prevent serious problems while administering anesthesia to a patient with mucopolysaccharidosis.


Preoperative evaluations:

(1) ECG and echocardiography

(2) cervical spine for stability

(3) respiratory function with arterial blood gases

(4) evidence of obstructive sleep apnea with polysomnography

(5) severity of spinal deformities

(6) range of motion in the cervical spine and temporomandibular joints

(7) other risk factors for difficult intubation

(8) identify supine positions where the airway is open or obstructed

(9) amount of nasopharyngeal secretions



(1) Anesthesia should be administered by physicians familiar with administering anesthesia to patients with mucopolysaccharidosis.

(2) The parents and patient should be given careful informed consent.

(3) A difficult intubation should be assumed and prepared for.

(4) Consider use of a laryngeal mask airway (LMA).

(5) Resolve any active infections prior to surgery.

(6) Stabilize the neck during intubation if atlanto-axial instability is present. Apply a halo frame prior to surgery and leave in place until the patient has adequately recovered.

(7) Review previous operative records but be prepared for changes due to disease progression.

(8) Monitor pulse oximetry before and after surgery.

(9) Recovery should be performed in the ICU and the patient should be monitored until fully recovered.

(10) Chest physiotherapy should be carefully applied in the post-operative period to reduce the risk of pneumonia.

(11) Be prepared for a cardiac ischemic event.


Recommendations if sleep apnea present:

(1) Nasal CPAP with sleep apnea.

(2) Use local regional block if possible to help control pain.

(3) Avoid excessive doses of opiates.


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