Cerebral venous sinuses thrombosis and pulmonary embolism due to antiphospholipid syndrome
Presentation
Severe headache , altered behavior and photophobia for 3 days. Reduced consciousness
Patient Data
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Ill-defined hypodensity at the right parieto-temporal lobe with partial effacement of adjacent cerebral sulci and gyri as well as the right lateral ventricle. The hypodensity involves both grey and white matter (cytotoxic edema). Another ill-defined hypodensity is noted involving the left thalamus.
Widespread increased attenuation within the dural venous sinuses, namely superior sagittal sinus, right transverse sinus, right sigmoid sinus, straight sinus, vein of Galen, in keeping with "cord sign". Bilateral internal cerebral veins and right temporoparietal cortical veins have high attenuation too.
CT venogram
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In CT venogram, definite filing defects are noted within the aforementioned high-attenuating dural venous sinuses, internal cerebral veins and cortical veins.
Admission chest radiograph
Chest radiograph on admission showed normal diameter of right interlobar artery. No focal lung consolidation or pleural effusion.
After 3 days of hospital admission, patient has sudden onset of chest pain and developed shortness of breath.
It showed enlargement of the pulmonary artery (well seen for the right interlobar/descending pulmonary artery) in keeping with Fleischner sign.
Bulging of left heart border. Small focal lung consolidation at the right mid-zone.
This is highly suspicious for acute pulmonary embolism from the chest radiograph in this clinical context as patient already had dural venous sinuses thrombosis.
Urgent CT pulmonary angiogram performed.
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Saddle-shaped filling defects within the bifurcation of main pulmonary trunk, bilateral main pulmonary arteries and further extending to the lobar, segmental and visualized subsegmental branches in bilateral lung.
Peripheral focal lung consolidation at the posterobasal segment of right lower lobe.
Flattening of interventricular septum, right ventricular enlargement (Increased right ventricle to left ventricle ratio is 1.1 >1.0) and mild cardiomegaly represent CT features of right heart strain.
After 5 months of treatment
The previously seen enlarged pulmonary artery (right interlobar artery) and bulging at the left heart border have resolved. No focal lung consolidation.
After 5 month of treatment
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Encephalomalacia at the right parieto-temporal lobe.
Resolved dural venous sinuses thrombosis as evidenced by normal attenuation of the dural venous sinuses and resolved filling defects in dural venous sinuses on CT venogram
Case Discussion
Further investigations taken to find the etiology of multiple sites of thrombosis. Results as followings:
- lupus anticoagulant positive (two samples taken in 4 months apart)
- ANA negative
- C3/C4 normal
- anti-cardiolipin negative
- cANCA and pANCA negative
According to revised Sapporo criteria1, this patient fulfills the clinical and the laboratory criteria for the diagnosis of antiphospholipid syndrome. The patient has been put on life-long anticoagulation.
Follow-up chest radiograph showed resolution of the pulmonary artery enlargement and resolution of cerebral venous sinuses thrombosis in CT venogram of the brain.
This is a great radiology case for demonstrating important radiological signs to identify cerebral venous sinuses thrombosis and massive pulmonary embolism.
The patient initially presented with sudden onset of central nervous system symptoms (headache, photophobia and altered behavior), where plain CT brain performed showed multiple sites of acute infarctions which are not confined to one particular arterial vascular territory, ie: left thalamus and right temporal-parietal lobes. The presence of abnormally high attenuation of the dural venous sinuses (cord sign) should raise the suspicion of venous thrombosis and lead to performing urgent CT venogram by radiologists/physicians.
Fleischner sign is one of the signs of pulmonary embolus on chest radiographs , which is closely related to massive pulmonary embolism. It has a low sensitivity but high specificity.