Imaging

Sturge-Weber Syndrome

Preliminary Diagnosis: Sturge-Weber syndrome

I. What imaging technique is first-line for this diagnosis

  • 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

  • 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.

  • When combined with clinical findings of seizure disorder and port wine stain in the trigeminal nerve distribution, findings are diagnostic.

  • Does not use ionizing radiation

Disadvantages

  • Expensive

  • Time-consuming

  • Requires significant patient cooperation, as the patient must be able to remain still for prolonged periods of time.

  • 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?

  • Contraindicated in patients with non-MR compatible metallic hardware or foreign bodies

IV. What alternative imaging techniques are available?

  • 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

  • May depict gyral calcifications better than MRI

  • Exam can be performed quickly and therefore requires less patient cooperation to obtain a diagnostic-quality exam

Disadvantages

  • Uses ionizing radiation

  • Provides significantly less anatomic detail than MRI

VI. What are the contraindications for the alternative imaging techniques?

Non-enhanced and enhanced head CT

  • Contraindicated in pregnant patients

  • Requires adequate renal function, generally a creatinine of <1.7 although the actual value varies from institution to institution.

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