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Original Article

Hemangiomatosis of the spleen in a patient with Klippel-Trénaunay syndrome

Authors:

S Dekeyzer ,

Department of Radiology, General Internal Medicine, Infectious Diseases and Psychosomatic diseases
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B Houthoofd,

Department of Radiology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium, BE
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A De Potter,

Department of Radiology, Anatomical Pathology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium, BE
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M Van Bockstal,

Department of Radiology, Anatomical Pathology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium, BE
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P Smeets,

Department of Radiology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium, BE
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D Vogelaers

Department of General Internal Medicine, Infectious Diseases and Psychosomatic diseases
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Abstract

Klippel-Trénaunay syndrome is a rare disorder characterized by a triad of port-wine stains, varicose veins, and bony and soft tissue hypertrophy usually involving an extremity. Visceral involvement in Klippel-Trénaunay syndrome is rare, but has been described in the colon, small bowel, bladder, kidney, spleen, liver, mediastinum and brain. In this paper we present the case of a 45-year-old woman with Klippel-Trénaunay syndrome in whom routine physical examination unexpectedly revealed the presence of a left upper quadrant mass. Abdominal US, contrast enhanced CT and whole-body PET-CT demonstrated multiple large cystic lesions within a massively enlarged spleen. Based on the clinical history and imaging findings diffuse hemangiomatosis of the spleen was suspected. This diagnosis was confirmed by pathologic examination after splenectomy.

How to Cite: Dekeyzer, S. et al., (2013). Hemangiomatosis of the spleen in a patient with Klippel-Trénaunay syndrome. Journal of the Belgian Society of Radiology. 96(6), pp.357–359. DOI: http://doi.org/10.5334/jbr-btr.457
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Published on 01 Nov 2013.
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