59 Behçet disease with CNS thrombophlebitis

Abstract

Abstract Background Behçet's disease is a peculiar vasculitis that affects both small and large vessels of the arterial and venous circulations. Objectives We present the case of two school-aged boys who presented with acute cerebral venous thrombosis, leading to the diagnosis of Behçet’s disease. Case presentation - An 8-year-old boy is admitted for severe headaches with nausea. The clinical examination notes a febrile child with marked aphtosis (with a history of genital “burns”). ESR and CRP are slightly elevated, while cerebral CT angiography reveals superior sagittal sinus occlusion. Angio-MRI confirms thrombophlebitis. - Another 9-year-old boy is referred for abdominal and spinal pain with intense headaches; accompanied with a subacute fever for >15 days. CT angiography revealed (reveals) stenosis of the longitudinal sinuses. For both children, the pathergy test, skin biopsy, and ophthalmic examination completed the whole picture of the Behçet disease. Discussion Major vascular manifestations of Behçet's disease include venous and/or arterial occlusion and aneurysm. Cerebral venous thrombosis (CVT) is very particular and might lead to an intracranial hyper-pressure, as in our cases. Steroids remain the initial reference treatment, with particular attention to anticoagulation and the addition/relay with colchicine. Conclusion Cerebral venous thrombosis may reveal Behçet's disease, especially in the pediatric population. The prognosis is poor and requires a rigorous, multidisciplinary follow-up.