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E5411. A Puff of Smoke: Imaging Workup in Moyamoya
Authors
  1. Fabianna Himet Coll; San Juan Bautista School of Medicine
  2. Felipe Barreras Galindo; San Juan Bautista School of Medicine
  3. Pablo Barreras Galindo; San Juan Bautista School of Medicine
  4. Mario Polo Asenjo; Hospital Menonita; San Juan Bautista School of Medicine
  5. Joel Colón Torres; San Juan Bautista School of Medicine
  6. Karl Schreiber; San Juan Bautista School of Medicine
  7. Juan Vicenty-Padilla-; ; University of Puerto Rico Medical Sciences Campus
Background
Moyamoya disease is a progressive, noninflammatory, nonatherosclerotic, vaso-occlusive process affecting primarily the supraclinoid internal carotid arteries. The proximal anterior intracranial circulation is predominantly affected, with involvement of the posterior intracranial circulation being rare. It typically affects children, adolescents, and young adults and may present with ischemic or hemorrhagic stroke. The correct diagnosis of Moyamoya, as well as an appropriate imaging work-up, are crucial to begin prompt treatment. Treatment of Moyamoya is aimed at improving flow to the most affected and eloquent cortical territories by means of surgical revascularization and procedures that promote neoangiogenesis. This exhibit will discuss the angiographic findings in Moyamoya using CTA, MRA, and DSA and highlight important collateral circulation pathways that develop. CT perfusion (CTP) is also an important tool that allows us to quantify and map the areas with decreased cerebral blood flow (CBF), which is crucial in presurgical planning. Common revascularization techniques will be discussed and correlated with CTA images.

Educational Goals / Teaching Points
The target audience of this educational exhibit is general radiologists, neuroradiologists, radiology residents, and medical students. The primary goal of the exhibit is to review common angiographic findings of Moyamoya using CTA and DSA, describe collateral circulation pathways in Moyamoya, understand the importance of CTP in the imaging work-up of Moyamoya, and be familiarized with common surgical revascularization techniques in Moyamoya disease.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
The development of collateral circulation pathways in Moyamoya represents the body’s physiological response to reestablish blood flow to hypoperfused brain tissue. Knowledge of these collateral routes is crucial to adequately interpret angiographic studies in moyamoya. CTP quantifies and maps CBF and helps to select those patients that would benefit the most from revascularization. The pathognomonic ‘puff of smoke' sign will be viewed through CTA with maximal intensity projection (MIP), as well as a 3D-rendered image showing arterial occlusions. In addition, CTP images will be facilitated to capture CBF in individuals with the disease.

Conclusion
The correct diagnosis of moyamoya, as well as an appropriate imaging work-up, are crucial to begin prompt treatment. Treatment of Moyamoya is aimed at improving flow to the most affected and eloquent cortical territories by means of surgical revascularization and procedures that promote neoangiogenesis. This exhibit will discuss the angiographic findings in Moyamoya using CTA, MRA, and DSA and highlight important collateral circulation pathways that develop. CTP is also an important tool that allows us to quantify and map the areas with decreased CBF, which is crucial in presurgical planning. Common revascularization techniques will also be discussed and correlated with CTA images.