Picture Of Port Wine Stain Of Newborn

Figure 1.115. This infant shown in Figure 1.114, now at the age of 3 months, developed a cystic swelling over the scalp in the first few weeks of life. The cystic swelling increased gradually in size; note the bluish hue. On removal this was confirmed to be a strawberry hemangioma. Note that strawberry hemangiomas may have a large component visible on the surface of the skin, or may be covered by the skin, thus obscuring their characteristic appearance.

Figure 1.116. In some infants the lesion may appear to be a hemangioma but, as in this example, on biopsy the diagnosis was a hemangioen-dothelioma of the right chest.

Capillary Bleeding Images

Figure 1.117. The capillary hemangioma involving the right side of the face in this infant demonstrates the fact that hemangiomas may expand sufficiently to interfere with function or may evidence bleeding or superficial infection. In this infant there would be marked interference with development of normal vision and, if untreated, this would lead to astigmatism and other problems. In such instances, treatment with steroids or laser surgery may be indicated.

Figure 1.118. Port wine stain (nevus flammeus) is another macular hemangioma which represents a regional dilatation and enlargement of mature capillaries. It usually affects the skin of the face and neck, and the lesions are sharp-bordered and range in color from pale purple to deep burgundy. It usually does not indicate underlying abnormalities unless it extends into the area of distribution of the ophthalmic branch of the trigeminal nerve that serves the facial skin over the eyelid and up into the brow where, as in this infant, it may signal the presence of the intracranial vascular anomalies of Sturge-Weber syndrome (enceph-alotrigeminal angiomatosis).

Figure 1.118. Port wine stain (nevus flammeus) is another macular hemangioma which represents a regional dilatation and enlargement of mature capillaries. It usually affects the skin of the face and neck, and the lesions are sharp-bordered and range in color from pale purple to deep burgundy. It usually does not indicate underlying abnormalities unless it extends into the area of distribution of the ophthalmic branch of the trigeminal nerve that serves the facial skin over the eyelid and up into the brow where, as in this infant, it may signal the presence of the intracranial vascular anomalies of Sturge-Weber syndrome (enceph-alotrigeminal angiomatosis).

Figure 1.119. Another example of SturgeWeber syndrome in which the distribution involves both the 1st and 2nd branch of the trigeminal nerve. Note the glaucoma of the right eye. In Sturge-Weber syndrome, lesions stop at the midline. When extensive facial involvement is present, there may be an associated glaucoma (buphthalmos). As this is one of the neurocutaneous syndromes, a CT scan of the head should be done to exclude intracranial involvement. Radiographs of the skull may reveal unilateral curvilinear, double-contoured lines of calcification in the cerebral cortex ("railroad track calcification"). The intracerebral vascular abnormalities lead to brain atrophy and ocular lesions (optic atrophy).

Figure 1.119. Another example of SturgeWeber syndrome in which the distribution involves both the 1st and 2nd branch of the trigeminal nerve. Note the glaucoma of the right eye. In Sturge-Weber syndrome, lesions stop at the midline. When extensive facial involvement is present, there may be an associated glaucoma (buphthalmos). As this is one of the neurocutaneous syndromes, a CT scan of the head should be done to exclude intracranial involvement. Radiographs of the skull may reveal unilateral curvilinear, double-contoured lines of calcification in the cerebral cortex ("railroad track calcification"). The intracerebral vascular abnormalities lead to brain atrophy and ocular lesions (optic atrophy).

Figure 1.120. This infant with involvement of the trunk and limbs is another example of Sturge-Weber syndrome. There were bilateral congenital glaucoma, seizures at the age of 6 days, and an abnormal CT scan. In SturgeWeber syndrome, seizures occur in 80% of infants, mental retardation in a high percentage, and an associated glaucoma in 40 to 50% of the infants, especially if bodi the ophthalmic and maxillary branches of die trigeminal nerve are involved.

0 0

Post a comment