Concern for PHACES syndrome: vascular lesions on the right side of the face affecting the lower lip, medial aspect of right upper lip, right side of the oral cavity, right lateral face, right inferior eyelid, and right ear. MRI: There is an enhancing lesion along the right posterolateral aspect of the spinal cord at the C2 level, which measures up to 8 mm. There appear to be superficial enhancing areas in the right lips and the right auricle. There is no evidence of intracranial hemorrhage, mass, or acute infarct. The brain parenchyma and pituitary gland appear unremarkable. The ventricles and basal cisterns are normal in size and configuration. There is no midline shift or herniation. The major cerebral flow voids are intact. The orbits, skull, paranasal sinuses, and scalp soft tissues are grossly unremarkable.MRA: There is aplasia of the right common carotid artery and deficiency of the right external and internal cervical carotid arteries. The superior portions of the right internal carotid artery are supplied by retrograde flow via the circle of Willis via a hypoplastic intracranial internal carotid artery and there is a communication with the cervical internal carotid artery with the hypoplastic extracranial vertebral artery via a proatlantal type artery. In addition, there appears to be communication between the right superior cervical artery and right vertebral artery. The right vertebral artery otherwise terminates as the PICA. The left carotid and vertebral arteries, on the other hand, are diffusely enlarged and there are bilateral posterior communicating arteries. The bilateral anterior, middle, and posterior cerebral arteries are patent. There is no evidence of cerebral aneurysms.
1. A subcentimeter enhancing lesion along the right posterolateral aspect of the spinal cord at the C2 level may represent a hemangioma. Furthermore, enhancing areas in the right lips the right auricle likely correspond to the known cutaneous hemangiomas. Otherwise, no evidence of intracranial hemangiomas.2. Aplasia of the right common carotid artery and deficiency of the right external and internal cervical carotid arteries. The superior portions of the right internal carotid artery are supplied by retrograde flow via the circle of Willis via a hypoplastic intracranial internal carotid artery and there is a communication with the cervical internal carotid artery with the hypoplastic extracranial vertebral artery via a proatlantal type artery. In addition, there appears to be communication between the right superior cervical artery and right vertebral artery. 3. Compensatory enlargement of the left carotid and vertebral arteries.