E2362. Tuberous Sclerosis: Radiologic Manifestations and Recommended Imaging Surveillance
Authors
Gabriel Duhancioglu;
University of Arizona
Conner Reynolds;
University of Arizona
Hina Arif;
University of Arizona
Background
Tuberous sclerosis complex (TSC) is a rare autosomal dominant neurocutaneous disorder characterized by development of benign tumors in multiple organs, including the kidneys. Several complications of this disorder, if not properly identified and managed, carry a high morbidity. In fact, renal angiomyolipoma (RAML)-associated hemorrhage remains the leading cause of death in patients with TSC. Therefore, a fundamental understanding of TSC imaging features, as well guidelines for image-guided diagnostic screening, surveillance, and complication management is critical.
Educational Goals / Teaching Points
Review the key neurological, cardiac, and pulmonary imaging features in patients with TSC. Provide consensus guidelines for image-guided diagnostic screening and surveillance of complications in patients with TSC. Review the key imaging features of renal angiomyolipoma (RAML) and image-guided management of hemorrhagic complications in patients with TSC.
Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Imaging findings in this presentation include subcortical tubers/SEGAs, cardiac rhabdomyomas, lymphangiomyelomatosis, and pulmonary artery aneurysms, renal artery aneurysms, bilateral hemorrhagic renal angiomyolipomas with image-guided coil embolization.
Conclusion
This presentation provides a foundational radiographic understanding of the constellation of disease seen in TSC. Prompt identification, referral for diagnostic screening, and continued surveillance are paramount to providing high-quality healthcare for patients with TSC.
