Gastric pseudoaneurysm in the setting of Loey's Dietz Syndrome
We present a case of a 13 year old boy with Loey's Dietz syndrome and a history of a David procedure (valve sparing aortic root replacement) when he was 9 months old with aortic valve and aortic root replacement at 2 years of age with subsequent post-operative complete heart block requiring pacemaker insertion. He ultimately underwent a Bentall procedure at 12 years of age. Upon routine cardiology clinic follow-up an incidental finding of a cystic structure in the liver was noted during echocardiography ().\nHe was asymptomatic from a cardiac and gastrointestinal standpoint. The remainder of his echocardiogram showed normal mechanical aortic valve function and normal left ventricular function. His pacemaker function was normal.\nAn abdominal CT was obtained to further characterize the lesion. Compared to a CT obtained 4years previously a new, well demarcated, heterogenously enhancing mass or aneurysm was identified between the lesser curvature of the stomach and the liver measuring 3.9 cm × 5.3 cm × 4.3 cm ( and ). A small branch off the left gastric artery appeared to enter the left anterior aspect of the mass ().\nAn abdominal ultrasound with Doppler performed after the CT scan was consistent with a patent aneurysm or pseudoaneurysm impressing upon the left hepatic lobe ( and ). Color Doppler flow into the aneurysm was demonstrated from the left gastric artery. No thrombus was identified.\nSuccessful embolization of what was found to be a left gastric artery pseudoaneurysm () by interventional radiology was performed using 5 detachable GDC coils (Boston Scientific Corporation, Natick, MA) in the left gastric artery at the neck of the pseudoaneurysm ().\nHe was also noted to have a markedly ectatic superior mesenteric artery (SMA) with several irregular beaded second and third order vessels from the SMA thought to possibly represent manifestations of his disease. Two months following the coil embolization, an abdominal CT scan with contrast demonstrated the excluded large pseudoaneurysm with no enhancement noted within it. Nine months later, an abdominal ultrasound showed interval resolution of the embolized and thrombosed pseudoaneurysm without any residual abnormalities demonstrated in the area. There were no new aneurysms identified. He remains stable from a cardiac perspective as well.