Sagittal sinus thrombosis in a teenager: homocystinuria associated with reversible antithrombin deficiency.
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A comprehensive synopsis on cerebral venous thrombosis is presented. It emphasizes the various aetiologies, the wide clinical spectrum and the unpredictable outcome. Imaging techniques and pitfalls are reported and the therapeutic options are discussed.
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An alcoholic man with uncontrolled diabetes mellitus had right conjunctivitis, facial numbness, and periorbital edema progressing to bilateral visual loss, and left ptosis in association with a large necrotic palatal ulcer due to zygomycosis. The infection progressed to bilateral retinal vein engorgement; left-sided ophthalmoplegia, fixed dilated pupil, and absent corneal reflex; and right-sided ophthalmoplegia, ptosis, and facial nerve paralysis. Work-up revealed disease of both ethmoid sinuses and the right maxillary sinus, with bilateral thromboses of the cavernous sinuses. An aggressive combined therapeutic attack (three Caldwell-Luc procedures, exploration of orbit walls, control of diabetes, systemic and local amphotericin therapy) led to survival with a three-year follow-up thus far.
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We report the rare occurrence of fatal cardiac tamponade due to coronary sinus and right atrial thrombosis associated with central venous catheterization. The clinical course was extremely rapid, with death occurring a few minutes after the acute clinical event. Other causes of acute cardiac tamponade were ruled out by the postmortem studies.
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An autopsy case of chronic thrombotic occlusion of the superior sagittal sinus (SSS) with an unusually protracted clinical course is reported. The patient was an 84-year-old man without any predisposing conditions for thrombosis. The clinical features were atypical and were characterized by recurrent cerebral subcortical hemorrhages. The autopsy revealed a large, organized thrombus in the SSS and extensive hemorrhagic infarction of the bilateral parasagittal region of the cerebral hemispheres. The hemorrhagic infarction was comprised of the aggregation of numerous minute foci of coagulation necrosis associated with petechial hemorrhages. Marked phlebosclerotic changes were observed in the subarachnoid and intracerebral veins, which were considered to be reactive changes of the venous walls against a persistent elevation in peripheral venous pressure caused by thrombosis of the SSS.
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A 23-year-old man was admitted because of vomiting and severe, progressive headache. After admission, he suffered from a generalized clonic seizure, and developed right hemiparesis. Contrast-enhanced CT of the brain showed empty delta sign in the posterior part of the superior sagittal sinus and filling defect in the straight sinus. T2-weighted MRI demonstrated high intensity area in the left parieto-occipital subcortical region. Delayed venous phase of the right carotid angiography confirmed the diagnosis of thrombosis of the superior sagittal sinus. Coagulation studies gave a protein C activity of 35.3% (normal range 55-140%), protein C antigen of 45% (normal range 70-150%). Same results were obtained from the studies of his father and one of his sisters, indicating hereditary protein C deficiency. We started warfarin therapy under the administration of heparin for a week, then he has been followed up with no subsequent problems.
The present report describes a case of cerebral venous thrombosis occurring in a patient suffering from refractory anaemia with excess of blasts (RAEB). This unusual complication suggests that RAEB could be considered a potential prothrombotic state.