Surgical Resection Of Thoracic Aortic Aneurysms In Wiskott-Aldrich Syndrome
Onalan, M.Akif.; Sayin, O.Ali.; Tireli, E.
Heart Surgery Forum 21(4): E305-E306
2018
ISSN/ISBN: 1098-3511 PMID: 30084784 Document Number: 696185
Aortic aneurysms are a rare condition in children. Wiskott-Aldrich syndrome is a primary immunodeficiency characterized by infections, thrombocytopenia, and eczema. Aortitis and aneurysm formation seem to be progressive in patients with Wiskott-Aldrich syndrome. The risk of death from aneurysmal rupture in patients with Wiskott-Aldrich syndrome is high and surgery is required for resection of aneurysms. We report a case where a successful resection of a descending thoracic aneurysm. We present a-12 year-old child with this syndrome who underwent a one-stage descending aortic aneurysm repair under continuous visceral perfusion.Histologic examination showed the presence of an aortitis withgranulomatous inflammatory response and multinucleated cells.