Clinical vignettes
Percutaneous closure of false aneurysms of the aorta in Wiskott–Aldrich syndrome
Giorgio Faganello*, Mark Hamilton, Peter Wilde, and Mark S. Turner
Bristol Heart Institute, Dolphin House, King Edward Building, Upper Maudlin Street, Bristol Royal Infirmary, Bristol BS2 8HW, UK
*Corresponding author. Tel: +44 01173420457, Fax: +44 01173420472. Email: giorgio.faganello@libero.it
We report a 27-year-old man with Wiskott–Aldrich syndrome and aortitis causing severe aneurysmal dilatation. He underwent a two-stage composite graft replacement of aortic root and ascending aorta in 1998 and replacement of part of the distal aortic arch and descending aorta in 2003. Three years after the previous surgery, he was admitted with haemoptysis due to an aorto-bronchial fistula from a false aneurysm of the ascending aorta (Panel A).
Redo surgery was considered high risk, so he underwent percutaneous implantation of three IDC coils in the aneurysm and closure of the neck with a 13 mm Amplatzer septal occluder. CT scan at 4 weeks showed complete occlusion (Panel B).
Four months later, he had another haemoptysis and a second aneurysm in a different position had developed (Panel C).
The neck of the aneurysm was too large for coils, so an 18 mm Amplatzer septal occluder was implanted (Panel D).
Three weeks later, a routine CT scan showed appearance of air inside the 18 mm occluder, indicating patency of the fistula between the aneurysm and the bronchus (Panel E).
Twelve hours after CT, he had a catastrophic haemoptysis and died within an hour, without time for surgical or catheter intervention.
Autopsy revealed extension of the neck of the second aneurysm along the suture line. Both the Amplatzer devices were positioned correctly (Panel F).
The first aneurysm had organized thrombus in the cavity around the coils and no evidence of fresh bleeding.
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