Preliminary Diagnosis: Sturge-Weber syndrome
I. What imaging technique is first-line for this diagnosis
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MRI of the brain with and without IV contrast is the preferred first-line imaging for Sturge-Weber syndrome.
II. Describe the advantages and disadvantages of this technique for diagnosis of Sturge-Weber syndrome.
Advantages
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Ideal for demonstrating the changes of Sturge-Weber including pial angiomatosis, gyral calcification and gliosis in the affected lobes, ipsilateral choroid plexus enlargement and hyperenhancement, and prominent deep veins.
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When combined with clinical findings of seizure disorder and port wine stain in the trigeminal nerve distribution, findings are diagnostic.
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Does not use ionizing radiation
Disadvantages
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Expensive
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Time-consuming
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Requires significant patient cooperation, as the patient must be able to remain still for prolonged periods of time.
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IV contrast requires good renal function with GFR > 60. Administration of contrast in patients with GFR of 30-60 is decided on a case-by-case basis.
III. What are the contraindications for the first-line imaging technique?
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Contraindicated in patients with non-MR compatible metallic hardware or foreign bodies
IV. What alternative imaging techniques are available?
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Non-enhanced and enhanced head CT
V. Describe the advantages and disadvantages of the alternative techniques for diagnosis of Sturge-Weber syndrome.
Non-enhanced and enhanced head CT
Advantages
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May depict gyral calcifications better than MRI
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Exam can be performed quickly and therefore requires less patient cooperation to obtain a diagnostic-quality exam
Disadvantages
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Uses ionizing radiation
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Provides significantly less anatomic detail than MRI
VI. What are the contraindications for the alternative imaging techniques?
Non-enhanced and enhanced head CT
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Contraindicated in pregnant patients
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Requires adequate renal function, generally a creatinine of <1.7 although the actual value varies from institution to institution.
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