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A 10 year old boy with no prior medical history suddenly developed severe pain between the shoulder blades, dyspnoea, and cyanosis, and lost consciousness within 24 hours. Computed tomographic (CT) and three dimensional imaging reconstructed from spiral CT (Vitrea) revealed an aneurysmal dilatation of the ascending aorta with a diameter of 81 mm, extending from the aortic valve to just proximal of origin of the left subclavian artery (right, white arrow). The right pleural space contained an amount of free blood. A huge mediastinal haematoma surrounding the ascending aorta, compressing both the heart and the lungs and displacing the oesophagus laterally, is seen. In addition, CT angiography revealed a contrast leak in the posterior wall just behind the cerebral vessels (below, black arrow). Furthermore, severe calcifications of the aneurysmal aortic wall were evident, indicating pre-existing aortic pathology.
Emergency surgery included replacement of the ascending aorta and the inferior part of the aortic arch by a 22 mm Vascutec prosthesis, leaving an island of autologous tissue containing the head vessels intact. The postoperative course was uneventful.
Pathological examination of the resected aneurysmal tissue showed severe scarring and degeneration of the aortic wall with dystrophic calcifications, indicating a systemic cause. Predisposing factors, including hereditary connective tissue disease (Marfan's syndrome, Ehlers-Danlos syndrome), congenital heart abnormalities, hypertension, trauma, infection (syphilis), and autoimmune disease, were excluded, making this case extremely rare.