Lesson 292: PreAnesthetic Assessment of the Patient With Mucopolysaccharidosis

Authored By:

Jermale A. Sam, MD, Resident, Department of Surgery, Riverside Methodist Hospital, Columbus, Ohio. Dr. Sam is now an anesthesia resident at Medical University of South Carolina, in Charleston. Amir R. Baluch, MD, Attending staff physician, Metropolitan Anesthesia Consultants, Dallas, Texas; and Alan D. Kaye, MD, PhD, Professor and chairman, Department of Anesthesiology, Louisiana State University Health Sciences Center, New Orleans, Louisiana

Registration Fee: $15.00
2.0 AMA PRA Category 1 CreditsTM
available until June 30, 2012

Reviewed By:

Elizabeth A.M. Frost, MD, Clinical professor, Department of Anesthesia, Mount Sinai Medical Center, New York, New York

Review Date: May, 2011
REGISTRATION INFORMATION:

Participants who do not possess a copy of Anesthesiology News can download and print the course material in an easy to read convenient  format.  Participants must reflect on the information presented, and then register to complete the exam and course evaluation online before the availability date listed above.  (CME credit is not valid past this date).  

Click on the link below to download and print the course material and post-test exam.  Register for the exam to enter your responses to receive CME credit.  There are ten questions in the examination and you must achieve a score of 80% or better to earn CME credit.  Following successful completion, your certificate will be immediately available online.  In addition, a historical record of  completed CME courses is maintained online in an individualized profile.  This includes copies of course certificates which can be printed at any time.

The registration fee for this course is $15.00

Physicians are provided with two opportunities to successfully complete the exam presented here. 

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CLICK HERE TO VIEW / PRINT COURSE MATERIAL

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NEEDS STATEMENT

Mucopolysaccharidosis (MPS) describes a group of genetic disorders that can complicate the anesthetic care of patients—in particular, management of the airway. Patients with MPS should be managed by experienced anesthesiologists at centers that are familiar with these disorders. Rarely encountered disease states have been identified as important topics in the continuing education of clinical anesthesiologists.

CASE HISTORY

A 2-year-old boy with MPS type I (Hurler syndrome) presented for bilateral inguinal hernia repair. The child often made grunting sounds while asleep, according to his parents. He had been given several antibiotics in the past for frequent respiratory infections, and was found to have a mild heart defect. The patient had no allergies and had never received an anesthetic. He weighed 14 kg. A physical examination was remarkable for coarse facies, macroglossia, short neck, and hepatosplenomegaly. A cardiac examination was positive for a 2 of 6 murmur. Mild aortic regurgitation with normal left ventricular systolic function and no wall motion abnormalities were observed with 2-D echocardiography. Cervical spine and chest x-rays were within normal limits.

LEARNING OBJECTIVES

At the end of this activity, the participant should be able to:

    1. Define MPS.
    2. Describe the pathophysiology of MPS.
    3. Explain how tissue dysfunction develops in patients with such rare lysosomal storage disorders.
    4. Cite the incidence of MPS.
    5. List the clinical manifestations of MPS.
    6. List specific problems associated with the administration of anesthesia to patients with MPS.
    7. Present treatment options for patients with MPS.
    8. Define appropriate preoperative evaluation of these patients.
    9. Formulate an anesthetic plan.
    10. Anticipate potential problems in airway management, postoperatively.

TARGET AUDIENCE:  Anesthesiologists

Accreditation Statement

Mount Sinai School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

Credit Designation: The Mount Sinai School of Medicine designates each educational activity for a maximum of 2 AMA PRA Category 1 CreditsTM. Physicians should only claim credit commensurate with the extent of their participation in the activity.

It is the policy of Mount Sinai School of Medicine to ensure objectivity, balance, independence, and scientific rigor in all CME-sponsored educational activities. All faculty participating in the planning or implementation of a sponsored activity are expected to disclose to the audience any relevant financial relationships and to assist in resolving any conflict of interest that may arise from the relationship. Presenters must also make a meaningful disclosure to the audience of their discussions of unlabeled or unapproved drugs or devices.

Disclosures

The author, the reviewer, and the editor have no relation-ships with pharmaceutical companies or manufacturers of products to disclose. This educational activity may contain discussion of published and/or investigational uses of agents for the treatment of disease. Some uses of these agents have not been approved by the FDA. Please refer to the official prescribing information for each product for approved indications, contraindications, and warnings.

CONTACT INFORMATION

Questions regarding course content may be directed to Dr. Elizabeth Frost: ElzFrost@aol.com.

If you require technical assistance with completing this course, please contact Continuing Education Online Customer Service at 718-648-8080 or send e-mail to ceo.contact7@proceo.com.

CALL FOR WRITERS

If you would like to write a CME lesson in Anesthesiology News, please send an e-mail to Elizabeth A.M. Frost, MD, at ElzFrost@aol.com