One-step surgical approach of a thoracic aortic aneurysm in Wiskott-Aldrich syndrome.
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.
Bernabeu
Eduardo
E
Cardiovascular Surgery Service, Hospital Clinic, University of Barcelona, Barcelona, Spain.
Josa
Miguel
M
Nomdedeu
Benet
B
Ramírez
José
J
García-Valentín
Antonio
A
Mestres
Carlos A
CA
Mulet
Jaime
J
D000328:Adult; D000792:Angiography; D017545:Aortic Aneurysm, Thoracic / Q000000981:diagnostic imaging* / Q000209:etiology / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D002545:Brain Ischemia / Q000517:prevention & control; D002315:Cardiopulmonary Bypass; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010477:Perfusion / Q000379:methods; D013902:Radiography, Thoracic; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000175:diagnosis