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BJO Online First, published on March 13, 2014 as 10.1136/bjophthalmol-2013-304460 Clinical science

Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature Dan B Rootman,1 Manraj K S Heran,2 Jack Rootman,3,4 Valerie A White,3,4 Panitee Luemsamran,5 Yeni H Yucel1,6 1

Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, Canada 2 Department of Radiology, University of British Columbia, Vancouver, Canada 3 Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, Canada 4 Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada 5 Orbit and Oculoplastic division, Ophthalmology department, Siririaj Hospital, Mahidol University, Bangkok, Thailand 6 Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada Correspondence to Dr Jack Rootman, Eye Care Centre, 2550 Willow St. Vancouver, BC, Canada V5Z 3N9; [email protected] Received 10 October 2013 Revised 21 January 2014 Accepted 17 February 2014

ABSTRACT Purpose The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma. Methods In this clinicopathologic series, clinical features were obtained from patient records. All imaging studies were reviewed. All specimens were reviewed with haematoxylin and eosin, and 10 were subject to a staining protocol including: Movat Pantachrome, periodic acid Schiff, D2-40, CD31, GLUT-1, Ki-67, vascular endothelial growth factor receptor 1 (VEGF-r1) (flt-1), VEGF-r2 (Flk-1), VEGF, anti-smooth muscle actin (SMA), CD20, CD4, CD8 and CD68. Imaging and pathology were reviewed in a systematic fashion. Results Clinically, lesions were more common in middle-aged females presenting with axial proptosis and pain. One-third demonstrated signs of optic nerve dysfunction. Dynamic imaging revealed focal early and diffuse late enhancement. Lesions demonstrated slow growth at 0.2 cm3/year. Histologically, all lesions demonstrated large vascular channels with matureappearing endothelium and abundant stroma. Three salient features were noted and characterised: thrombosis, nests of perivascular hypercellularity and expanded stromal elements. Acute thrombosis was a feature of each specimen (

Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature.

The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma...
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