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Facial capillary angiomatosis presents as a port-wine stain. The port-wine stain in Sturge-Weber syndrome usually involves the whole area of skin innervated by the ophthalmic branch of the trigeminal nerve, but occasionally it involves a smaller area in the distribution of the ophthalmic branch, such as the inner corner of the upper eyelid, or a larger area that extends beyond the limits of the ophthalmic branch of the trigeminal nerve. Every neonate with a port-wine stain on the face should have an ophthalmological evaluation (Figure 296.1)
unless the port-wine stain is light colored and in the middle of the forehead just above the nose.

Figure 296.1.Facial hemangioma involving the ophthalmic division on the right trigeminal nerve and maxillary divisions of the trigeminal nerves; buphthalmos on the right.

Glaucoma is the most frequent ocular pathology. Glaucoma is usually detected in the course of a routine ophthalmologic evaluation in a neonate with a facial port-wine stain. Glaucoma may produce buphthamos (Figures 296 and 296.2). Buphthalmos may be the presenting sign of Sturge-Weber syndrome. Buphthalmos is readily diagnosed by observation and by MRI (Figure 296.2[B]).

A
B

Figure 296.2. [A] Facial hemangioma involving the ophthalmic and maxillary divisions of the trigeminal nerve. [B] MRI appearance of buphthalmos (left eye). Compare the distance between the lens and the cornea in the left eye and right eye.


Seizures are an unusual initial manifestation of Sturge-Weber syndrome in the neonatal period. Hemiparesis and mental retardation do not usually occur in the neonatal period. Arachnoid and piamater angiomatosis usually occurs in the parietal occipital region ipsilateral to the facial angioma. Cerebral cortical atrophy often occurs in the same hemisphere as meningeal angiomatosis. Hydrocephalus due to sagittal sinus and venous obstruction may also occur. Contrast MRI of the brain may show meningeal angiomatosis even in the neonatal period. The skull x-ray finding of “double contour” convolutional calcifications do not occur in neonates. Sturge-Weber syndrome has a sporadic occurrence.

 

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right eye temporal muscle temporal muscle temporal lobe temporal lobe zygomatic bone zygomatic bone left eye cornea lens lens ethmoid sinus ethmoid sinus nasal cavity superior orbital fissure superior orbital fissure optic nerve mandibular branch of CN V (V3) ophthalmic branch of CN V (V1) maxillary branch of CN V (V2) Gomez, 1987 Wiedmann, 1997 Spitz, 1996 Click on figure for animated labels.  Pause pointer on different areas of the figure for labels. Figure must be centered.