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Published online by Cambridge University Press: 22 December 2025
This case report details the diagnosis and surgical management of a 7 cm aortic root aneurysm occurring in a 12-year-old with cutis laxa type B, an extremely rare connective tissue disorder. Our patient underwent a Bentall procedure as primary treatment for the aneurysm and had a successful interventional treatment of a postoperative pseudoaneurysm.
Addendum: “Seven months after device closure of an aortic fistula, our patient presented with a pulsatile, painless erythematous sternal mass. He reported no history of fever or other symptoms. The white blood cell count was 3,810/mm³ (neutrophils 54%, lymphocytes 28%, monocytes 14%, eosinophils 3%), hemoglobin 9.5 g/dL, hematocrit 32.2%, and platelet count 310,000/mm³. A COVID-19 test was positive. Computed tomography angiography revealed the previously seen thrombosed pseudoaneurysm measuring 13.7 × 13.1 cm. Inside it, two new pseudoaneurysms were identified, both showing contrast enhancement similar to the aorta. One was located near the prosthetic aortic valve, measuring 7 × 4 cm, and the other near the closure device at the ascending aorta, measuring 3.1 × 2.5 cm. Additionally, a well-defined, encapsulated mass measuring 3.5 × 3.2 cm was observed in the thoracic wall. The patient underwent emergency surgery. Femoral bypass was established, and cooling was initiated. During cooling, the sternum was reopened, and a large amount of fibrin and clot was removed. A significant flow of oxygenated blood began to flow from the mediastinum, preventing further cooling on CPB. As a result, at 30°C, CPB was stopped to examine the anatomy and locate the bleeding site. After removing all tissue from the pseudoaneurysm, we observed that large sections of the native aorta in the proximal arch and at the junction with the graft were missing. The graft was completely loose within the chest cavity and beyond repair. Based on these findings and the impossibility of maintaining cardiopulmonary bypass with an aorta beyond repair, support was discontinued, and the patient died intraoperatively. Presumed cause of death was infected pseudoaneurysm with aortic disruption, with underlying severe aortopathy of cutis laxa syndrome. We submit this followup to reinforce the unpredictability of this disease despite comprehensive surgical treatment.