Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Port-wine birthmark Capillary-venous malformation in the eye (choroidal angioma) and brain (leptomeningeal angioma) +++ Clinical Findings ++ In infancy, the eye may show congenital glaucoma, or buphthalmos, with a cloudy, enlarged cornea Facial nevus In early stages, may be the only indication, with no findings in the brain even on radiologic studies May be much more extensive than the first division of cranial nerve V; it can involve the lower face, mouth, lip, neck, and even torso Neurologic manifestations Focal seizures are common in infancy Cognitive impairment Headache and migraines Stroke-like symptoms CNS hemorrhage (rare) The characteristic cortical atrophy, calcifications of the cortex, and meningeal angiomatosis may appear with time, solidifying the diagnosis Physical examination may show hemiparesis on the side contralateral to the cerebral lesion Hemiatrophy of the contralateral limbs may occur Mental handicap may result from poorly controlled seizures +++ Diagnosis ++ CT scanning may show calcification of the cortex much earlier than radiographic films MRI often shows underlying brain involvement EEG Often shows depression of voltage over the involved area in early stages In later stages, epileptiform abnormalities may be present focally +++ Treatment ++ Early control of seizures is important to avoid consequent developmental setback If seizures do not occur, normal development can be anticipated Careful examination of the newborn, with ophthalmologic assessment to detect early glaucoma, is indicated Rarely, surgical removal of the involved meninges and the involved portion of the brain may be indicated, even hemispherectomy Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth