Idiopathic Dilatation of the Aorta with Dissection in a Family without Marfan Syndrome

Dissection of the aorta is very rare in children, but classically occurs in the presence of Marfan syndrome or other connective tissue disorder. We present a case of spontaneous dissection in a 12-year-old boy whose half brother has an idiopathic dilated aorta and whose mother has also required surgery for dissection of a dilated aorta. No features of connective tissue disorder were present in any family member.