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AJNR - American Journal of Neuroradiology

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Agenesis of Bilateral Internal Carotid Arteries and Posterior Fossa Abnormality in a Patient with Facial Capillary Hemangioma: Presumed Incomplete Phenotypic Expression of PHACE Syndrome

Young-Cheol Weon^a, Jin-Il Chung^b, Hyung-Jin Kim^a and Hong sik Byun^a

^a Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
^b Department of Radiology, Medimoa Hospital, Seoul, Korea

View larger version (120K): [in a new window]   FIG 1. On axial (A) and coronal (B) T2-weighted images, hypoplasia of the right cerebellar hemisphere and absence of the inferior cerebellar vermis are present. Note that the abnormally dilated basilar artery with numerous small signal intensity void structures corresponds with the collateral networks at the prepontine cistern. On MR angiography (C, -D), both internal carotid arteries are not seen, and the distal portion of internal carotid arteries is reconstituted from the external carotid arteries.

View larger version (97K): [in a new window]   FIG 2. Injection of right external carotid artery (A and B) and left external carotid artery (C and D) demonstrates the reconstitution of the distal internal carotid artery by branches of the internal maxillary arteries, middle meningeal arteries, deep temporal arteries, artery of the foramen rotundum, ascending pharyngeal arteries, and collaterals of the occipital artery. Injection of the left vertebral artery (E and F) shows the discontinuation between the vertebral arteries and the abnormally dilated proximal basilar artery with collateral branches mainly from the left vertebral artery.

View larger version (93K): [in a new window]   FIG 3. Normal internal carotid arteries are not seen in the carotid spaces, and no carotid canals are noted on the bone window setting of the brain CT (A) and T1-weighted brain MRI (B).

View larger version (81K): [in a new window]   FIG 4. Injection of the innominate artery (A) and CT scan obtained at the level of thoracic inlet (B) demonstrate the unusual subclavian artery turning like a screw.