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    Infant’s growing birthmark causes blurry vision

    Infant presents with a repidly growing birthmark on the left upper eyelid.

    The Case

    You are asked to see a healthy 3-month-old boy with a rapidly growing lump on his left upper eyelid. At birth there was a red macule that was diagnosed as a small port-wine birthmark. The infant was seen by a pediatric ophthalmologist who noted significant astigmatism of the left eye.

    DERMCASE diagnosis } Infantile hemangioma

    Pathogenesis, epidemiology, and classification

    Infantile hemangiomas are benign vascular tumors that result from the proliferation of endothelial-like cells that express high levels of glucose transporter isoform 1 (GLUT1) and placenta-associated vascular antigens.They develop early in life in 4% to 5% of infants.2,3 There is a higher prevalence in females, non-Hispanic whites, premature infants (<37 weeks gestational age), infants of low birth weight (<2500g), and infants of multiple gestations.There also seem to be associations with older maternal age (≥30 years), placenta previa, and preeclampsia during the prenatal period.4

    Hemangiomas are often classified morphologically as superficial, deep, or mixed.5,6 A superficial hemangioma is red, nodular, and raised above the normal skin. A deep hemangioma typically presents as a subcutaneous skin-colored nodule or tumor with overlying bluish discoloration, with or without associated telangiectasia. Mixed hemangiomas contain both superficial and deep components.

    Hemangiomas can be focal (localized and usually round or oval and relatively small); multifocal (same as focal but multiple hemangiomas); and segmental (covering a specific territory and usually large).6

    Segmental hemangiomas are more likely to be associated with developmental abnormalities, including PHACE syndrome (posterior fossa malformations, large segmental hemangiomas, and arterial/cardiac/eye abnormalities).Therefore, these children require more intensive monitoring; are more likely to require medical therapy; and tend to have more complications (eg, ulceration) than children with localized hemangiomas.6

    Children with multiple lesions can have as many as 30 focal hemangiomas or more, but visceral involvement appears to be rare and rates of complications in these children are comparable to those with localized types.6

    Natural course

    The natural progression of hemangiomas includes 2 phases: proliferation and spontaneous involution. The average hemangioma will reach 80% of its full size by 3 months with the majority of growth completed by 5 months of age.Following proliferation, the majority of hemangiomas begin to regress by 1 year of age. Involution is characterized by a change in color from bright red to dark, dull red with central graying, and softening and flattening of the lesion. Although most hemangiomas do not require treatment, half will leave some residual change such as telangiectasias, loose baggy skin, and/or fibrofatty tissue.

    Hemangiomas must be distinguished from vascular malformations. A hemangioma is a vascular neoplasm that grows by cellular hyperplasia; a vascular malformation is a result of defective vascular morphogenesis.The differences are outlined in Table 1.8-10


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