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E-048 moya moya syndrome: evaluation and treatment in patients with inflammatory conditions causing a puff of smoke
  1. R Shastri1,
  2. N Chaudhary1,
  3. A Pandey2,
  4. C Roark3,
  5. B Thompson2,
  6. J Gemmete1
  1. 1Radiology, University of Michigan, Ann Arbor, MI, USA
  2. 2Neurosurgery, University of Michigan, Ann Arbor, MI, USA
  3. 3Neurosugery, University of Michigan, Ann Arbor, MI, USA


Title Evaluation of Angiographic appearances of Moya Moya variants and their treatment options

Purpose To familiarize the viewer with many of the different disease entities known to cause the Moya Moya angiographic phenotype and help the viewer to identify additional salient imaging findings elsewhere in the body that may help with more precise diagnosis.

Approach/methods 1. Review relevant anatomy of the intracranial arterial circulation. 2. Describe the Moya Moya disease findings. 3. Describe several of the known diseases that cause Moya Moya appearance on angiography and give imaging examples of each by angiographic studies. 4. Imaging examples of pathognomonic findings in each disease entity.5. Review of imaging features that correlate with symptomatic disease where treatment is indicated. 6. Discussion of past and current treatment modalities including intracranial angioplasty, stenting and surgical bypass options.

Findings/discussion The “puff of smoke” imaging findings described for moya moya can be seen with the classic Moya Moya disease which was first described in Japanese families in the 1950’s. Since that time, we have identified other disease that has similar presentations such as neurofibromatosis, lupus erytmatosis and fibromuscular dysplasia. Evaluating perfusion in asymptomatic patients as well as stroke burden in symptomatic patients have been used to guide need for treatment. Treatment of this condition has been traditionally medical with aspirin as well as surgical through bypass surgery. Endovascular stent angioplasty has been utilized for intracranial stenosis associated with Moya Moya disease although evidence for its efficacy is thin in the current literature. After SAMMPRIS and COSS trial, evidence for benefit from intracranial stents or surgical bypass for intracranial atherosclerotic disease has come into question. Surgical bypass or intracranial angioplasty may offer additional treatment options for Moya Moya disease type patients in the future.

Conclusion By describing the angiographic patterns associated with Moya Moya phenomenon as well as associated symptoms we hope to elucidate the nuances of angiographic appearances in this broad clinical setting which may help narrow the differential diagnosis.. We will also discuss the treatment options and the current level of evidence for endovascular or surgical treatment.

Disclosures R. Shastri: None. N. Chaudhary: None. A. Pandey: None. C. Roark: None. B. Thompson: None. J. Gemmete: None.

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