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Cerebral arteriovenous malformation with lobar hemorrhage

Case contributed by Peter Mitchell
Diagnosis certain

Presentation

Headache and reduced GCS to 11.

Patient Data

Age: 90 years
Gender: Female

Left frontal lobe intraparenchymal hemorrhage, with perifocal edema.

Diffuse hypodensity at periventricular white matter and centrum semiovale, suggestive of chronic small vessel disease.

Large tortuous vessel over the hematoma and left frontal lobe with focal dilatation. This is consistent with an enlarged vein with venous varix, most probably secondary to an arteriovenous malformation, the major differential diagnosis is a dural arteriovenous fistula with cortical venous drainage and varix (Cognard 4).

Large left frontal intraparenchymal acute hemorrhage (involving the cerebral cortex) measuring 36x36 x33 mm in maximal dimensions of surrounding region of hypodensity compatible with vasogenic edema.

There is moderate secondary mass effect with compression of adjacent sulci (resulting in crowding of the sulci at the vertex), partial compression of the frontal horn of the left lateral ventricle and mild subfalcine herniation. There is also approximately 3 mm rightward midline shift measured at the septum pellucidum.

No evidence of intraventricular or extra-axial hemorrhagic extension. No further evidence of acute hemorrhage. Deep white matter/periventricular foci of low attenuation is compatible with chronic small vessel ischemia.

Conventional aortic arch anatomy. Bilateral vertebral arteries are of subclavian origin.

Overlying the left frontal and temporal lobes are very large caliber arteries and draining veins (seen draining into the sigmoid sinus on the left and the sagittal sinus superiorly). There is a more focal bulbous aneurysmal dilatation measuring 7x6mm in size overlying the left frontal lobe.

Conclusion

Large left frontal intraparenchymal acute hemorrhage measuring 36x36x33mm in maximal dimension veins with evidence of surrounding vasogenic edema and secondary mass effect as described above. The CT angiogram study demonstrates enlarged draining vessels overlying the left frontal and temporal lobes (combination of arteries and veins).

Findings are most compatible with an underlying arteriovenous malformation, with suggestion of arterial supply from the left external carotid artery.

A digital subtraction angiogram study is recommended for further evaluation.

Superficial pial AVM with rapid arteriovenous shunting, a (predominantly) single draining vein with large venous varicosity.

Note the 4D DSA showing time resolved filling of the vein, varicosity, and showing the associated stenosis.

Case Discussion

Lobar hematoma occurring spontaneously in a 90-year-old still most commonly represents hypertensive hemorrhage or hemorrhage related to underlying amyloid angiopathy. Bleeding diathesis - especially anticoagulation - needs to be excluded. However underlying causes such as tumor, vascular malformation, aneurysm, and venous sinus thrombosis need to be considered even in a 90-year-old with hypertension. CT, post-contrast CT (looking for "spot sign"), and CTA would have sufficient sensitivity as first-line investigations, with catheter angiography or MRI depending on the findings.

Selective DSA with microcatheter was performed, one branch occluded at the nidus, but the diffuse nature of the nidus, multiple "en passant" arterial feeders, and consideration of hematoma removal meant further endovascular intervention was not proceeded with, and surgical evacuation planned.

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