title: Hemangiomatosis of the spleen in a patient with Klippel-trénaunay syndrome.
abstract: Klippel-Trenaunay 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-Trenaunay 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.
authors.affiliation: Department of Radiology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium. sven.dekeyzer@Ugent.be
authors.forename: S
authors.initials: S
authors.lastname: Dekeyzer
authors.affiliation: Department of Radiology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium.
authors.forename: B
authors.initials: B
authors.lastname: Houthoofd
authors.affiliation: Department of Anatomical Pathology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium.
authors.forename: A
authors.initials: A
authors.lastname: De Potter
authors.affiliation: Department of Anatomical Pathology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium.
authors.forename: M
authors.initials: M
authors.lastname: Van Bockstal
authors.affiliation: Department of Radiology, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium.
authors.forename: P
authors.initials: P
authors.lastname: Smeets
authors.affiliation: General Internal Medicine, Infectious Diseases and Psychosomatic diseases, Universitair Ziekenhuis (University Hospital) Gent, Ghent, Belgium.
authors.forename: D
authors.initials: D
authors.lastname: Vogelaers
mesh_terms: D003287:Contrast Media; D003937:Diagnosis, Differential; D005260:Female; D006391:Hemangioma / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D008875:Middle Aged; D064847:Multimodal Imaging / Q000379:methods; D049268:Positron-Emission Tomography / Q000379:methods; D011856:Radiographic Image Enhancement / Q000379:methods; D013154:Spleen / Q000000981:diagnostic imaging; D013156:Splenectomy / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods; D014463:Ultrasonography; D051598:Whole Body Imaging / Q000379:methods
chemical_list: D003287:Contrast Media