Surgical Resection Of Thoracic Aortic Aneurysms In Wiskott–Aldrich Syndrome

  • Mehmet Akif Onalan Department of Cardiovascular Surgery, Sanlıurfa Mehmet Akif Inan Training and Research Hospital, Sanliurfa, Turkey
  • Omer Ali Sayin Department of Cardiovascular Surgery, İstanbul University Istanbul Medical Faculty, İstanbul, Turkey
  • Emin Tireli Department of Cardiovascular Surgery, İstanbul University Istanbul Medical Faculty, İstanbul, Turkey

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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Published
2018-07-02
Section
Articles