E5040. Small Patients, Big Images: Navigating Renal Involvement in Children with Tuberous Sclerosis
  1. Valentina Ferrer Valencia; Boston Children's Hospital
  2. Carol Barnewolt-; ; Boston Children's Hospital
Tuberous sclerosis is a multisystem genetic disorder with various clinical manifestations. Renal involvement is common and often presents a diagnostic challenge. This educational exhibit aims to enhance the understanding of pediatric radiologists and clinicians regarding the radiologic manifestations of renal tuberous sclerosis, their correlation with pathology, and their implications for patient management.

Educational Goals / Teaching Points
To provide a comprehensive review of the radiologic signs, imaging techniques, and relevant anatomical and pathophysiological considerations related to renal tuberous sclerosis in pediatric patients. By illustrating key imaging findings and their clinical significance, this exhibit aims to facilitate accurate diagnosis and guide appropriate monitoring strategies.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
1. Pathogenesis and clinical presentation. Briefly introduce the genetic basis of tuberous sclerosis and highlight its renal manifestations in pediatric patients. Teaching point: understand the genetic basis of tuberous sclerosis and its implications for renal involvement in pediatric patients. 2. Imaging techniques. Describe the imaging modalities commonly used for evaluating renal involvement in tuberous sclerosis, including ultrasound, CT, and MRI. Highlight the strengths and limitations of each modality. Teaching point: differentiate between various imaging modalities used to assess renal manifestations of tuberous sclerosis. 3. Radiologic signs and imaging findings. Present a series of illustrative images showcasing characteristic radiologic signs, such as renal cysts, angiomyolipomata, and renal cell carcinoma associated with tuberous sclerosis. Provide side-by-side comparisons with pathological specimens to underscore the correlation between imaging and histopathology. Teaching point: recognize and interpret characteristic radiologic signs, such as renal cysts, angiomyolipomata, and renal cell carcinoma, associated with tuberous sclerosis. 4. Differential diagnosis. Discuss the differential diagnosis of renal lesions in pediatric patients and highlight key differentiating features that aid in distinguishing tuberous sclerosis-related findings from other renal conditions. Teaching point: identify key differentiating features for distinguishing tuberous sclerosis-related renal findings from other pediatric renal conditions. 5. Management implications. Outline the clinical significance of accurate radiologic diagnosis, emphasizing the impact on management and patient outcomes. Teaching point: understand the critical role of accurate radiologic diagnosis in guiding appropriate treatment strategies for pediatric patients with tuberous sclerosis-related renal involvement.

This educational exhibit serves as a concise and informative resource for pediatric radiologists, clinicians, and medical students seeking to enhance their knowledge of the renal manifestations of tuberous sclerosis. By focusing on well-defined teaching points and utilizing various imaging modalities, this exhibit aims to provide a comprehensive understanding of the uroradiologic aspects of this genetic disorder.