Surgical Resection of Thoracic Aortic Aneurysms in Wiskott-Aldrich Syndrome

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 this condition. The risk of death from aneurysmal rupture in patients with Wiskott-Aldrich syndrome is high, and surgery is required for the resection of these aneurysms. We report a case wherein a successful resection of a descending thoracic aneurysm was performed. A 12 year-old child with this syndrome underwent a one-stage descending aortic aneurysm repair under continuous visceral perfusion. Histological examination showed the presence of an aortitis with granulomatous inflammatory response and multinucleated cells.