Traumatic aortic dissection in a boy with Loeys-Dietz syndrome.

Abstract

A 9-year-old boy was admitted to the emergency department for interscapular chest discomfort. Bidimensional echocardiography revealed a severe aortic root and sino-tubular junction dilatation with a suspicion of intimal flap at the aortic isthmus and a pseudo-aneurysm which was confirmed by a computerized tomography scan. The patient was immediately transferred to the operating room for an emergent replacement of the aortic isthmus. His recovery after surgery was uneventful, and he was discharged home 12 days later on beta blockers and angiotensin II receptor antagonist. A genetic recognition confirmed the diagnosis of Loeys-Dietz syndrome type I.