Approach

White ethnicity, female sex, prematurity, low birth weight, multiple gestation, and advanced maternal age are strong risk factors for development of hemangioma.

History and physical exam

Infantile hemangiomas may be present at birth, but they more typically present during the first few weeks of life as flat pink or blue macules or patches.[2][16] Alternatively, they may present as a red or blue papule or nodule. They undergo a period of accelerated growth, known as the “proliferative phase,” helping to differentiate them from other vascular entities. The duration of the proliferative phase varies depending on the morphology of the infantile hemangioma, but 80% of growth is usually reached by 3 months of age.[14] A superficial hemangioma develops a bright-red color: the surface appears tight and tense. Ulceration and bleeding may occur, particularly in areas subject to increased friction, maceration, and trauma (e.g., diaper area, neck, axilla, and scalp). Most lesions reach a period of stability by age 6 to 12 months, and then enter a period of spontaneous involution. Involution may take several years, during which the red color changes to blue, gray, and/or pink. Islands of normal-colored skin become apparent, and the hemangioma is palpably softer.[2][6][23]

Deep hemangiomas feel tense, and may swell with crying and dependent positioning. They lack the characteristic red or pink color, and may appear later in the first year of life. Their growth phase may not be as apparent and may occur later than superficial infantile hemangiomas, raising the possibility of confusion with a vascular malformation. Midline facial lesions, particularly those over the nose, need to be confidently differentiated from dermoid cysts, gliomas, and encephaloceles. Hemangioma overlying the lumbosacral spine may mimic meningocele and myelomeningocele.[10]

Imaging studies

Imaging studies are typically unnecessary but may differentiate infantile hemangioma from vascular or lymphatic malformations when diagnosis is uncertain, or for deep lesions which can be difficult to assess physically.[24]​ Doppler ultrasound of the lesion is quick, accurate, and cost effective. Ultrasound avoids the risks of anesthesia. However, interpretation is highly dependent upon the expertise of the technologist; and it may not conclusively distinguish proliferative vascular tumors (e.g., infantile hemangioma) from vascular malformations.

Magnetic resonance imaging (MRI) is indicated when clinical exam and ultrasound fail to provide a diagnosis, or when the extent of the lesion and relationship to adjacent structures needs to be defined more fully.[24]​[25]​​​​

MRI with contrast may differentiate infantile hemangioma from venous, arteriovenous, and lymphatic malformations.[26] MRI can also be used to distinguish an infantile hemangioma from an encephalocele or meningocele/myelomeningocele.[10] MRI studies are a useful adjunct to distinguish infantile hemangioma from malignant soft-tissue masses, including fibrosarcoma. Diagnostic specificity is reported to be up to 90%, with negative predictive value for malignancy up to 94%.[27] By contrast, the specificity for benign vascular tumors is 50%, and the specificity for malignant vascular tumors is 80%. A tissue biopsy is indicated when malignancy cannot be excluded.[1]

Computed tomography (CT) is not as useful as MRI because a CT scan cannot identify patterns of vascular flow.[10] Arteriography has been replaced in modern practice largely by noninvasive imaging techniques.[10]

MRI is also the test of choice to evaluate associated anomalies of the spine, brain, etc., when considering syndromic infantile hemangioma.[14]

Biopsy histology

Histopathologic findings vary greatly depending on the phase at which the infantile hemangioma is biopsied. Proliferative hemangiomas have a lobular architecture and are highly cellular. They involve the dermis and may extend into the subcutaneous tissues. Subtle vascular lumina appear slit-like and are surrounded by plump endothelial cells. Normal-appearing mitoses are frequent. Mast cells populate the surrounding stroma.[14][28] With involution, lumina enlarge and endothelial cells flatten. Vessels are replaced by fibro-fatty tissue.[28] Hemangiomas uniquely stain for GLUT1, an erythrocyte-type glucose transporter present in infantile hemangioma, brain, and placenta.[29][30][31] If a malignant tumor with a significant vascular component is considered a possibility, biopsy with histopathologic exam and special tissue stains, including GLUT1, is indicated.[28]

Variants and special considerations

Segmental hemangiomas may be associated with underlying abnormalities. Segmental cervicofacial, upper chest, shoulder, or arm hemangiomas may be associated with structural anomalies of the brain, cerebral vasculature, eyes, sternum, and/or aorta. This neurocutaneous disorder is known as PHACE(S) syndrome, with the acronym referring to posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities/aortic coarctation, abnormalities of the eye, and sternal clefting or supraumbilical raphe.[8] The syndrome is often incomplete.[32][Figure caption and citation for the preceding image starts]: Tender, ulcerated hemangioma on the left lower lipFrom the collection of Carla T. Lane, MD, PhD; used with permission [Citation ends].com.bmj.content.model.Caption@5d75b238 Infants with segmental cervicofacial hemangioma require ophthalmologic exam, echocardiogram, and possible central nervous system imaging.

Beard hemangioma: hemangiomas located on the lower face and neck have been associated with laryngeal hemangioma.[1] Progressive stridor is a worrisome sign. CT with intravenous (IV) contrast may be useful when optimal imaging of the airway is required.[24]​ Infants with hemangiomas in a beard distribution should be referred to an otolaryngologist for further evaluation and possible endoscopy.[1]

Lumbosacral hemangioma: hemangioma located in the lumbosacral area may signal underlying spinal dysraphism. Other associated malformations include tethered cord, renal, and skeletal anomalies. MRI is the test of choice.[9][10][24]​​ Segmental perineal, genital, buttock, or thigh hemangiomas should raise concern for LUMBAR syndrome, which refers to lower body infantile hemangioma and other cutaneous defects, urogenital anomalies and ulceration, myelopathy, bony deformities, anorectal malformations and arterial anomalies, and renal anomalies.[1]

Multifocal cutaneous infantile hemangiomas: infants with multiple cutaneous hemangiomas may have hemangiomas within their visceral organs. A prospective study revealed that 16% of infants who present with ≥5 infantile hemangiomas have hepatic hemangiomas.[12] In such patients a good physical exam is indicated. Hepatomegaly may indicate clinically significant liver hemangiomas and should be evaluated by ultrasound.[24]​ An abnormal cardiac exam may indicate high-output heart failure. Patients with multifocal cutaneous infantile hemangiomas or large visceral lesions are also at risk for hypothyroidism.[14]

Hemangiomas in certain locations can result in significant cosmetic or functional complications. Periorbital hemangiomas may result in ocular complications. Hemangiomas on the nasal tip or ear may cause cartilage destruction. Lesions on the face and ears may lead to permanent disfigurement. Bulky lesions on the scalp may result in alopecia. Lip hemangiomas can cause feeding problems and distort the normal contour of the mouth.[13] Genital and perineal hemangiomas are more likely to ulcerate and lead to associated complications. [Figure caption and citation for the preceding image starts]: Plaque-type cervicofacial ulcerated hemangioma (beard distribution)From the collection of Carla T. Lane, MD, PhD; used with permission [Citation ends].com.bmj.content.model.Caption@6ba32e04 Breast hemangiomas in females may result in permanent changes in breast development or nipple contour.

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