Localized Marked Elongation of the Distal Internal Carotid Artery with or without PHACE Syndrome: Segmental Dolichoectasia of the Distal Internal Carotid Artery

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Intracranial dolichoectasia of the distal ICA was identified in 20 patients from 2005–2016 through a review of diagnostic cerebral angiography results. Images were reviewed to determine the vascular morphologic dispositions around the distal ICA, including dysplasia, mural calcification, vessel wall enhancement, lumen narrowing, and aneurysm formation. In this cohort, which had a strong female predominance (male/female ratio2:18), intracranial dolichoectasia had a more ipsilateral vascular morphologic disposition. Mural calcification was detected more frequently in elderly patients, whereas vessel wall enhancement was detected more frequently in younger patients. Follow-up images showed a slow progression of the lesions. The segmental nature of the striking elongation and tortuosity of the distal ICA suggest a type of congenital lesion representing either a sporadic phenomenon or an arterial change associated with PHACE syndrome.

Abstract

Figure 2 from paper
Representative images of ICDE in a patient without PHACE syndrome (patient 6, a 28-year-old woman). A and B, Bilateral ICA angiography shows a tangled arterial mass of the left distal ICA and no observable left A1 segment. C, Left vertebral artery angiography shows a dolichoectasia of the left P1 and P2 segments (arrow). D, CT angiography shows hypoplasia of the left A1 segment (arrow). E, T2-weighted image of the brain shows hypoplasia of the left midbrain. F, CT image of vessel wall calcification in the left distal ICA (arrow).

BACKGROUND AND PURPOSE

Segmental intracranial dolichoectasia of the distal ICA is a feature of PHACE syndrome or a sporadic phenomenon. We evaluated the relationship between intracranial dolichoectasia of the distal ICA and PHACE syndrome and illustrated the characteristic radiologic findings of the lesions.

MATERIALS AND METHODS

Intracranial dolichoectasia of the distal ICA was identified in 20 patients at our institution from 2005 to 2016 through a review of diagnostic cerebral angiography results. All radiologic images were reviewed to determine the vascular morphologic dispositions around the distal ICA, including dysplasia, mural calcification, vessel wall enhancement, lumen narrowing, and aneurysm formation. Medical records were reviewed to determine the symptoms of PHACE syndrome. Subsequently, the correlation between radiologic findings and PHACE syndrome was assessed.

RESULTS

In this cohort, which had a strong female predominance (male/female ratio= 2:18), intracranial dolichoectasia had a more ipsilateral vascular morphologic disposition. Mural calcification was detected more frequently in elderly patients, whereas vessel wall enhancement was detected more frequently in younger patients. Follow-up images showed a slow progression of the lesions. However, no significant differences in the vascular morphologic disposition and brain structural changes were observed between patients with (n = 11) and without (n = 9) PHACE syndrome.

CONCLUSIONS

The striking elongation and tortuosity of the distal ICA generally appeared to be a type of congenital lesion occurring early in embryogenesis as either a sporadic phenomenon or an arterial change associated with PHACE syndrome. Imaging findings revealed various mural abnormalities with a benign clinical course.

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Localized Marked Elongation of the Distal Internal Carotid Artery with or without PHACE Syndrome: Segmental Dolichoectasia of the Distal Internal Carotid Artery
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Jeffrey Ross
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