Clinical and Imaging Characteristics of Diffuse Intracranial Dolichoectasia

The patient is a 47-year-old man. He presented in his 20s with 2 large fusiform aneurysms of his cavernous carotid arteries. He later developed an aneurysm of his basilar artery and had a subarachnoid hemorrhage from a dissecting aneurysm of his PICA (not shown). A, CTA image demonstrates a large fusiform aneurysm of the right cavernous carotid artery (long white arrow) and a thrombosed/calcified aneurysm of the left cavernous carotid artery (short white arrows). There is also an aneurysm of the basilar tip (curved black arrow). B, Right ICA cerebral angiogram shows a large fusiform aneurysm of the right petrocavernous carotid artery (straight black arrows) and a basilar tip aneurysm (curved black arrow). C, There was suspicion for underlying connective tissue disease. The patient underwent a skin biopsy. Electron microscopy of the skin biopsy shows multiple abnormally enlarged collagen fibers (black circles) consistent with collagen flowers. These are typically seen in Ehlers-Danlos syndrome. The patient later underwent genetic testing for Loeys-Dietz, Ehlers-Danlos, and Marfan syndromes. The findings of all tests were negative.

A 51-year-old man with a long history of headaches with associated nausea and vomiting. He had acute-onset left-sided weakness with a prominent left facial droop along with left face, arm, and leg numbness and slurred speech. The patient had no family history of cerebral aneurysms, though his father had an abdominal aortic aneurysm. A, MR imaging at the time of the initial evaluation showed a medial left pontine infarct (black arrow). There was evidence of a large dolichoectatic aneurysm of the basilar artery on MR imaging, and the patient underwent cerebral angiography for further evaluation. B, Cerebral angiography demonstrated a fusiform-type aneurysm of the basilar artery with a filling defect that was consistent with thrombus (black arrows). The patient also had diffuse arteriomegaly with dilation of the right supraclinoid ICA to 6 mm and dilation of the left supraclinoid ICA to 10 mm (white arrow, C). D, The day following the angiography, the patient had a 10/10 headache. Noncontrast CT at the time showed diffuse subarachnoid hemorrhage with most of the blood products surrounding the basilar artery aneurysm. He died the next day.

An 87-year-old man with a history of a right third-nerve palsy. A, Right ICA cerebral angiogram demonstrates a 20-mm cavernous carotid fusiform aneurysm with associated dilation of the supraclinoid ICA as well. B, Left ICA cerebral angiogram shows dilation of the left supraclinoid ICA to approximately 10 mm. C, Left vertebral artery cerebral angiogram shows diffuse dilation and tortuosity of the basilar artery measuring 9 mm in maximum diameter. The cause of the third-nerve palsy was thought to be the right cavernous aneurysm.