Approach

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

History and physical examination

Infantile haemangiomas 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 haemangioma, but 80% of growth is usually reached by 3 months of age.[14] A superficial haemangioma develops a bright red colour: the surface appears tight and tense. Ulceration and bleeding may occur, particularly in areas subject to increased friction, maceration, and trauma (e.g., nappy 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 colour changes to blue, grey, and/or pink. Islands of normal-coloured skin become apparent, and the haemangioma is palpably softer.[2][6][23]

Deep haemangiomas feel tense, and may swell with crying and dependent positioning. They lack the characteristic red or pink colour, 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 haemangiomas, 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. Haemangioma overlying the lumbosacral spine may mimic meningocele and myelomeningocele.[10]

Imaging studies

Imaging studies are typically unnecessary but may differentiate infantile haemangioma 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 anaesthesia. However, interpretation is highly dependent upon the expertise of the technician; and it may not conclusively distinguish proliferative vascular tumours (e.g., infantile haemangioma) from vascular malformations.

Magnetic resonance imaging (MRI) is indicated when clinical examination 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 haemangioma from venous, arteriovenous, and lymphatic malformations.[26] MRI can also be used to distinguish an infantile haemangioma from an encephalocele or meningocele/myelomeningocele.[10] MRI studies are a useful adjunct to distinguish infantile haemangioma 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 tumours is 50%, and the specificity for malignant vascular tumours 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 non-invasive imaging techniques.[10]

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

Biopsy histology

Histopathological findings vary greatly depending on the phase at which the infantile haemangioma is biopsied. Proliferative haemangiomas 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] Haemangiomas uniquely stain for GLUT1, an erythrocyte-type glucose transporter present in infantile haemangioma, brain, and placenta.[29][30][31] If a malignant tumour with a significant vascular component is considered a possibility, biopsy with histopathological examination and special tissue stains, including GLUT1, is indicated.[28]

Variants and special considerations

Segmental haemangiomas may be associated with underlying abnormalities. Segmental cervicofacial, upper chest, shoulder, or arm haemangiomas 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, haemangioma, 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 haemangioma on the left lower lipFrom the collection of Carla T. Lane, MD, PhD; used with permission [Citation ends].com.bmj.content.model.Caption@35ff3403 Infants with segmental cervicofacial haemangioma require ophthalmological examination, echocardiogram, and possible central nervous system imaging.

Beard haemangioma: haemangiomas located on the lower face and neck have been associated with laryngeal haemangioma.[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 haemangiomas in a beard distribution should be referred to an otolaryngologist for further evaluation and possible endoscopy.[1]

Lumbosacral haemangioma: haemangioma 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 haemangiomas should raise concern for LUMBAR syndrome, which refers to lower body infantile haemangioma and other cutaneous defects, urogenital anomalies and ulceration, myelopathy, bony deformities, anorectal malformations and arterial anomalies, and renal anomalies.[1]

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

Haemangiomas in certain locations can result in significant cosmetic or functional complications. Periorbital haemangiomas may result in ocular complications. Haemangiomas 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 haemangiomas can cause feeding problems and distort the normal contour of the mouth.[13] Genital and perineal haemangiomas are more likely to ulcerate and lead to associated complications. [Figure caption and citation for the preceding image starts]: Plaque-type cervicofacial ulcerated haemangioma (beard distribution)From the collection of Carla T. Lane, MD, PhD; used with permission [Citation ends].com.bmj.content.model.Caption@7aac26d4 Breast haemangiomas in females may result in permanent changes in breast development or nipple contour.

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