Together for Short Lives
Call the Helpline 0808 8088 100

Surgical Resection Of Thoracic Aortic Aneurysms In Wiskott-Aldrich Syndrome

Journal title
The heart surgery forum
Publication year
2018
Author(s)
Onalan, M. A.; Sayin, O. A.; Tireli, E.
Pages
E305-e306
Volume
21
Number
4

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.

Research abstracts