Childhood Hemangiomas and Vascular Malformations




Patient Story



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A baby girl is brought to the office because her mother is concerned over the growing strawberry hemangioma on her face. Her mother is reassured that most of these childhood hemangiomas regress over time and that there is no need for immediate treatment (Figure 93-1).




FIGURE 93-1


Strawberry hemangioma on the face causing no functional problems. Treatment is reassurance and watchful waiting. (Used with permission from Richard P. Usatine, MD.)






Introduction



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Hemangiomas are the most common benign tumors of infancy. They can be problematic if they block vision or interfere with any vital function. Most hemangiomas are small and of cosmetic concern only.




Synonyms



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Infantile hemangiomas, angiomas. Strawberry hemangiomas are also called superficial hemangiomas of infancy. Cavernous hemangiomas are also called deep hemangiomas of infancy.




Epidemiology



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  • Approximately 30 percent of hemangiomas are present at birth; the other 70 percent appear within the first few weeks of life.



  • Hemangiomas occur more commonly in fair-skinned, premature, female infants. In one study, the mothers of children with hemangiomas are of higher maternal age, have a higher incidence of preeclampsia and placenta previa, and are more likely to have had multiple gestation pregnancies.1



  • There is an increased incidence of vascular anomalies in the families of children born with hemangiomas.1



  • The data are mixed as to whether chorionic villus sampling may play a role in the formation of hemangiomas.1



  • Females are affected more often than males (2.4:1).1





Etiology and Pathophysiology



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  • Hemangiomas consist of an abnormally dense group of dilated blood vessels. Most childhood hemangiomas are thought to occur sporadically.



  • Hemangiomas are characterized by an initial phase of rapid proliferation, followed by spontaneous and slow involution, often leading to complete regression. Most childhood hemangiomas are small and innocuous, but some grow to threaten a particular function (Figure 93-2) or even life.



  • Rapid growth during the first month of life is the historical hallmark of hemangiomas, when rapidly dividing endothelial cells are responsible for the enlargement of these lesions. The hemangiomas become elevated and may take on numerous morphologies (dome-shaped, lobulated, plaque-like, and/or tumoral). The proliferation phase occurs during the first year, with most growth taking place during the first 6 months of life. Proliferation then slows and the hemangioma begins to involute.



  • The involutional phase may be rapid or prolonged. No specific feature has been identified in explaining the rate or completeness of involution. However, in one type of hemangioma, the rapidly involuting congenital hemangioma, the proliferation phase occurs entirely in utero such that the lesion is fully developed at birth, followed by complete involution during the second year of life.1



  • A good rule of thumb is 50 percent of childhood hemangiomas will involute by age 5 years, 70 percent by age 7 years, and the remainder of childhood hemangiomas will take an additional 3 to 5 years to complete the process of involution.1



  • Of the lesions that have involuted by age 6 years, 38 percent will leave residual evidence of the hemangioma in the form of a scar, telangiectasia, or redundant, “bag-like” skin. The chance of a permanent scar increases the longer it takes to involute. For example, of the lesions that involute after age 6 years, 80 percent may exhibit a cosmetic deformity.1





FIGURE 93-2


Large hemangioma on the face needing immediate treatment to prevent amblyopia in the left eye. Although this hemangioma follows the V1 dermatome, this is not a port-wine stain and the patient does not have Sturge-Weber syndrome. (Used with permission from Richard P. Usatine, MD.)






Diagnosis



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Clinical Features


Early lesions may be subtle, resembling a scratch or bruise, or alternatively may look like a small patch of telangiectasias or an area of hypopigmentation. Hemangiomas can start off as a flat red mark, but as proliferation ensues, it grows to become a spongy mass protruding from the skin. The earliest sign of a hemangioma is blanching of the involved skin with a few fine telangiectasias followed by a red macule. Rarely, a shallow ulceration may be the first sign of an incipient hemangioma.1 Hemangiomas are typically diagnosed based on appearance, rarely warranting further diagnostic tests.



Hemangiomas may be superficial, deep, or a combination of both. Superficial hemangiomas are well defined, bright red, and appear as nodules or plaques located above clinically normal skin (Figures 93-1 to 93-3). Deep hemangiomas are raised flesh-colored nodules, which often have a bluish hue and feel firm and rubbery (Figure 93-4).




FIGURE 93-3


Strawberry hemangioma present since birth on the face of a 22-month-old girl. Although it is close to her eye, her vision has never been occluded. She has been followed by ophthalmology and no active treatment was recommended. The hemangioma grew larger during the first year of life and is now beginning to involute without treatment. (Used with permission from Richard P. Usatine, MD.)






FIGURE 93-4


Deep (cavernous) hemangioma on the arm in a 9-month-old child. Treatment is watchful waiting. (Used with permission from Richard P. Usatine, MD.)


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Dec 31, 2018 | Posted by in PEDIATRICS | Comments Off on Childhood Hemangiomas and Vascular Malformations

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