• H&P

    • 12-year-old child with a history of sickle cell disease, on exchange transfusion therapy, presents with progressive headaches over 1 year
    • With recent exchange transfusions, he developed bilateral arm weakness for 1-2 hours afterwards
    • Recent sports activity results in brief periods of profound arm weakness, especially on the right side, and word finding problems.

    Figure 1.

    1. What is your initial diagnosis?

    2. What test would you order next?

    3. What treatment would you offer?

    4. Which of the following describes you?

    5. I practice in one of the following locations.

    6. Please add any suggestions or comments regarding this case:
     
    • not sure about the answer to question 2. at this stage, with an arteriogram already done, maximal Rx of the sickle cell disease on-going, and neurological symptoms with exertion, I would probably go right to surgery, knowing that the other studies wouldn't add much. However, the bilateral arm weakness is unusual as an ischemic symptom, and there may be some type of peripheral vascular problem going on. I presume that the patient is on aspirin.
    • Symptomatic at various points in time, probably falsely correlated with transfusion reactions.
    • Would always try a direct bypass, but this is dependent on the size of the STA - if too small, would try EDAMS.
    • It seems the patient is the best candidate for EDAS surgery.
    • comments

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