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Aneurysm of the aortic root in the setting of Wiskott-Aldrich syndrome

Published online by Cambridge University Press:  01 April 2009

Masamichi Ono*
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
Heidi Goerler
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
Thomas Breymann
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
*
Correspondence to: Masamichi Ono, MD. Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. Tel: +49-511-532 9397; Fax: +49-511-532 9832; E-mail: Ono.Masamichi@mh-hannover.de

Abstract

Wiskott-Aldrich syndrome is a rare X-linked disease, associated with immunodeficiency, infections, thrombocytopaenia, and eczema. Aortitis and formation of aneurysms have also been described. We describe here our experience with a 7-year-boy with this syndrome. He survived replacement of the aortic root because of an aneurysmal ascending aorta, and subsequent bone marrow transplantation.

Type
Brief Report
Copyright
Copyright © Cambridge University Press 2009

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References

1.Perry, GS, Spector, BD, Schumann, LM. The Wiskott-Aldrich syndrome in the United States and Canada (1892–1979). J Pediatr 1980; 97: 7278.CrossRefGoogle ScholarPubMed
2.McCluggage, WG, Armstrong, DJ, Maxwell, RJ, Ellis, PK, McCluskey, DR. Systemic vasculitis and aneurysm formation in the Wiskott-Aldrich syndrome. J Clin Pathol 1999; 52: 390392.Google Scholar
3.Johnston, SL, Unsworth, DJ, Dwight, JF, Kennedy, CT. Wiskott-Aldrich syndrome, vasculitis and critical aortic dilatation. Acta Pediatr 2001; 90: 13461348.Google Scholar
4.van Son, JAM, O’Marcaigh, AS, Edwards, WD, Julsrud, PR, Danielson, GK. Successful resection of thoracic aortic aneurysms in Wiskott-Aldrich syndome. Ann Thorac Surg 1995; 60: 685687.CrossRefGoogle Scholar
5.Narayan, P, Alwair, H, Bryan, AJ. Surgical resection of sequential thoracic aortic aneurysms in Wiskott-Aldrich syndrome. Interact Cardiovasc Thorac Surg 2004; 3: 346348.Google Scholar
6.Bernabeu, E, Josa, M, Nomdedeu, B, et al. One-step surgical approach of a thoracic aortic aneurysm in Wiskott-Aldrich syndrome. Ann Thorac Surg 2007; 83: 15371538.Google Scholar
7.Boztug, K, Baumann, U, Ballmaier, M, et al. Large granular lymphocyte proliferation and revertant mosaicism: two rare events in a Wiskott-Aldrich syndrome patient. Haematologica 2007; 92: e43e45.CrossRefGoogle Scholar
8.Rose, C, Castillo, E, Wessel, A, et al. Morphological and functional MRI studies after correction of aortic isthmus stenosis. Z Kardiol 2002; 91: 161168.Google Scholar
9.Mullen, CA, Anderson, KD, Blaese, RM. Splenectomy and/or bone marrow transplantation in the management of the Wiskott-Aldrich syndrome: long term follow up of 62 cases. Blood 1993; 82: 29612966.Google Scholar
10.Vricella, LA, Williams, JA, Revekes, WJ, et al. Early experience with valve-sparing aortic root replacement in children. Ann Thorac Surg 2005; 80: 16221627.CrossRefGoogle ScholarPubMed