Aortic aneurysms in children and young adults with tuberous sclerosis: Report of two cases and review of the literature

Abdominal aortic aneurysms (AAAs) in children and young adults are rare; so me have been observed in patients with tuberous sclerosis (TS). We report t wo cases and review the literature. A 9-year-old girl with TS was diagnosed with a 3-cm calcified AAA, and a 41-year-old man with TS was diagnosed wit h a 7.5-cm thoracic aortic aneurysm (TAA). Both patients underwent open rep air with a tube polyester graft without complication. They are both doing w ell at 7 and 8 years after surgery Pathologic evaluation revealed medial at rophy and focal medial disruption in the aortic wall in both patients. With our two cases, 15 patients with TS and aneurysms have been reported; 12 ha d AAA, and four had TAA tone patient had both). Three AAAs and two TAAs rup tured. Six patients died because of aneurysmal disease. There is an associa tion between TS and aortic aneurysms. Patients should be screened for aorti c aneurysms at the time TS is diagnosed and annually thereafter. Because of the high risk of rupture, early elective repair is suggested. New aortic a neurysms after repair map also develop.