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] 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
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.