Multiple Interventions to Thoracoabdominal Aortic Aneurysm in a Child With Tuberous Sclerosis

Thoracoabdominal aortic replacement in a 6-year-old boy with Loeys-Dietz syndrome

“…Patients with LDS syndrome may experience aortic dissection and aneurysm at a very young age, and compared to the disease presentation in adults, the onset of aortic dissection and aneurysm is more abrupt and progresses rapidly in children [3]. The indications for prophylactic treatment of the aorta in patients with LDS include: adult patients with an aortic diameter exceeding 4 cm; aortic root diameter growth > 0.5 cm/year; pediatric LDS syndrome patients with an aortic diameter exceeding the 99th percentile for the average age of children.…”

“…The indications for prophylactic treatment of the aorta in patients with LDS include: adult patients with an aortic diameter exceeding 4 cm; aortic root diameter growth > 0.5 cm/year; pediatric LDS syndrome patients with an aortic diameter exceeding the 99th percentile for the average age of children. For pediatric and adolescent patients, the diameter of the arti cial blood vessel selected for surgery should be slightly larger than the measured aortic diameter obtained from CT scans, allowing the arti cial blood vessel to meet the growing needs during the developmental process [3]. It is not recommended to use endovascular stent intervention therapy in patients with genetic aortic syndromes (LDS syndrome and Marfan syndrome).…”

Open Emergency Repair of a Thoracoabdominal Aortic Aneurysm on a 12-Year-Old Boy With Tuberous Sclerosis

“…4 Multiple case reports have associated TSC with AA since 1966 5 however only 4 cases involved the thoracoabdominal aortic segment (Table 1). [6][7][8][9] Most AA in patients with TSC occurs in the abdominal aorta and less frequently in the thoracic aorta. 10 The age of presentation is variable, however, Salerno et al showed that the age of presentation of AA was before 5 years of age, and mortality due to aneurysmal rupture was 29%.…”

“…4 Multiple case reports have associated TSC with AA since 1966 5 however only 4 cases involved the thoracoabdominal aortic segment (Table 1). 6-9…”