Successful Operation for Thoracoabdominal Aortic Aneurysm in a 5-Year-Old Boy With Tuberous Sclerosis.

ABSTRACT

The association of abdominal aortic aneurysm (AAA) and tuberous sclerosis (TS) is rare. A 5-year-old boy was diagnosed with a 7-cm calcified thoracoabdominal aortic aneurysm (TAAA), and the clinical evaluation revealed TS. The patient underwent an open repair with a 14-mm polyester tube graft prosthesis. The pathologic examination showed nonspecific dystrophic changes with loss of elastin fibers in the media of the aorta. The graft was patent on computed tomographic angiography performed 1 month after the operation. Because of the high risk of rupture, early elective repair is suggested.