Differentials
Venous malformation
SIGNS / SYMPTOMS
Blue or purple color.
Present at birth, but may not be noticeable until later in life.
Soft and compressible on exam. Proportionate growth.
Nonpulsatile.
Size may readily increase with dependent positioning.
No spontaneous involution.
May ache but is not usually tender. Thrombosis may cause acute swelling and tenderness.
INVESTIGATIONS
Doppler ultrasound of lesion shows a sponge-like array of anechoic vessels with a venous waveform.[25]
MRI with and without contrast shows venous lakes and phleboliths.[35]
Laboratory studies may show low serum fibrinogen, elevated D-dimer, and low platelet counts indicating chronic intravascular coagulopathy.[2]
Arteriovenous malformation
SIGNS / SYMPTOMS
Blue or red color.
Present at birth, but may not be noticeable until later in life.
Proportionate growth.
No spontaneous involution.
May cause ulceration, pain, and claudication due to vascular steal.
Warm and pulsatile.[34]
Lymphatic malformation
SIGNS / SYMPTOMS
Large, translucent soft mass with normal overlying skin color.
Usually present at birth or before 2 years of age.
Proportionate growth.
No spontaneous involution.
May swell with prolonged dependent positioning.[34]
Rapidly involuting congenital hemangioma
SIGNS / SYMPTOMS
Fully formed at birth and involutes over the first year of life.[31]
INVESTIGATIONS
Standard histology shows prominent capillary lobules surrounded by fibrous tissue.
Central involuting zones with lobular loss, fibrous tissue, and abnormal draining channels.[36]
GLUT1 staining negative.[29][36] GLUT1 is an erythrocyte-type glucose transporter present in infantile hemangioma, brain, and placenta.[29][30][31]
Noninvoluting congenital hemangioma
INVESTIGATIONS
Standard histology shows prominent capillary lobules and associated thin-walled vessels within densely fibrotic stroma. These lobules also show thrombosis, hemosiderin deposits, and sclerosis.[31]
GLUT1 staining negative.[29][31] GLUT1 is an erythrocyte-type glucose transporter present in infantile hemangioma, brain, and placenta.[29][30][31]
Kaposiform hemangioendothelioma
SIGNS / SYMPTOMS
Congenital skin-colored, red or purple plaques or masses that resemble lymphatic malformations.
Life-threatening consumptive platelet trapping with acute tenderness, erythema, and swelling (Kasabach-Merritt phenomenon).
No spontaneous involution.[16]
INVESTIGATIONS
Standard histology shows infiltrative stands of thin endothelial cells that line slit-like vessels.
Eosinophilic epithelioid cell nests are interspersed.[10]
Fibrosarcoma
SIGNS / SYMPTOMS
Vascular-appearing tumor that mimics an infantile hemangioma.
Congenital, typically on extremities.
Displays rapid growth.
Capillary malformation
Tufted angioma
SIGNS / SYMPTOMS
Mottled red color with flat and papular areas.
Growth is slow over months to years.
Lesions extend laterally.
Associated with life-threatening platelet trapping, the Kasabach-Merritt phenomenon (consumptive platelet trapping with acute tenderness, erythema, and swelling).[39]
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