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  • Title: Surgical Resection Of Thoracic Aortic Aneurysms In Wiskott-Aldrich Syndrome.
    Author: Onalan MA, Sayin OA, Tireli E.
    Journal: Heart Surg Forum; 2018 Jul 02; 21(4):E305-E306. PubMed ID: 30084784.
    Abstract:
    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.
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