Kevin Ching MD, Presenter: Nothing to Disclose

Shobhit Madan MD,MPH, Abstract Co-Author: Nothing to Disclose

Sameh Tadros MD, MSC, Abstract Co-Author: Nothing to Disclose

Loeys-Dietz syndrome is a recently described connective tissue disorder that results in arterial tortuosity and aneurysms that may present in children.  While it predominately affects the heart and aorta, it may also affect the intracranial and peripheral vasculature at a young age.  This exhibit will review the imaging findings of Loeys-Dietz syndrome on Cardiac-MR, MRA, and CTA. 

1. Overview of Loeys-Dietz syndrome a. Genetic mutations. b. Unique clinical features with a focus on vascular pathology. c. Signs, symptoms, and clinical presentation.  2. Arterial tortuosity, dissection, and aortic root dilatation on Cardiac-MR and MRA. 3. Impact on the intracranial vasculature and imaging findings on CTA and MRA. 4. Treatment and recommendations for follow up imaging and multi-disciplinary care.   

Major teaching points include: 1. Unique genetic mutations in Loeys-Dietz syndrome affect the cardiac, cerebral, and peripheral vasculature in children leading to arterial tortuosity, aneurysms, and dissection. 2. Advanced imaging techniques(Cardiac-MR, MRA, and CTA) are important for initial diagnosis and long-term screening. 3. Treatment and follow up imaging in pediatric patients with Loeys-Dietz syndrome requires multi-disciplinary care. 

Ching, K, Madan, S, Tadros, S, 3D CT and Cardiac MR of Loeys-Dietz Syndrome in the Pediatric Patient.  Radiological Society of North America 2012 Scientific Assembly and Annual Meeting, November 25 - November 30, 2012 ,Chicago IL. http://archive.rsna.org/2012/12029366.html