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Ann Thorac Surg 2007;83:1537-1538
© 2007 The Society of Thoracic Surgeons

Case Reports

One-Step Surgical Approach of a Thoracic Aortic Aneurysm in Wiskott-Aldrich Syndrome

Eduardo Bernabeu, MDa, Miguel Josa, MDa,*, Benet Nomdedeu, MD, PhDb, José Ramírez, MD, PhDc, Antonio García-Valentín, MDa, Carlos A. Mestres, MD, PhDa, Jaime Mulet, MD, PhDa

a Cardiovascular Surgery Service, Hospital Clinic, University of Barcelona, Barcelona, Spain
b Haematology Service, Hospital Clinic, University of Barcelona, Barcelona, Spain
c Department of Pathology, Hospital Clinic, University of Barcelona, Barcelona, Spain

Accepted for publication October 2, 2006.

* Address correspondence to Dr Josa, Servicio Cirugía Cardiovascular, Hospital Clinic Barcelona, C/Villarroel, 170, E-08036 Barcelona, España (Email: mjosa{at}clinic.ub.es).

Wiskott-Aldrich syndrome is a primary immunodeficiency characterized by infections, thrombocytopenia, and eczema. We present a 33-year-old man with this syndrome who underwent a one-stage ascending aorta, aortic arch and descending aortic aneurysm repair under moderate hypothermia and continuous visceral and cerebral perfusion. Histologic examination showed the presence of an aortitis with granulomatous inflammatory response and multinucleated cells.






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