An infantile hemangioma (IH), sometimes called a strawberry mark due to appearance, is a type of benign vascular tumor or anomaly that affects babies.
It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in extracutaneous locations including the liver and gastrointestinal tract.
[5][1] Hemangiomas may grow rapidly, before stopping and slowly fading, with maximum improvement typically occurring by the age of 3+1⁄2 years.
[11][12] While this birthmark may be alarming in appearance, physicians generally counsel that it be left to disappear on its own, unless it is in the way of vision or blocking the nostrils.
Superficial lesions can be flat and telangiectatic, composed of a macule or patch of small, varied branching, capillary blood vessels.
[2] Superficial IHs in certain locations, such as the posterior scalp, neck folds, and groin/perianal areas, are at potential risk of ulceration.
[18] Deep IHs present as poorly defined, bluish macules that can proliferate into papules, nodules, or larger tumors.
Larger or segmental hemangiomas that span a large area can sometimes have underlying anomalies that may require investigation, especially when located on the face, sacrum, or pelvis.
Large IHs can leave visible skin changes secondary to severe stretching that results in altered surface texture.
[27] Also, a hypothesis was presented by researchers that maternal placenta embolizes to the fetal dermis during gestation, resulting in hemangiomagenesis.
[28][29] However, another group of researchers conducted genetic analyses of single-nucleotide polymorphism in hemangioma tissue compared to the mother's DNA which contradicted this hypothesis.
[33][37] On Doppler ultrasound, an IH in the proliferative phase appears as a high-flow, soft-tissue mass usually without direct arteriovenous shunting.
On MRI, IHs show a well-circumscribed lesion with intermediate and increased signal intensity on T1- and T2-weighted sequences, respectively, and strong enhancement after gadolinium injections, with fast-flow vessels.
[38] Under the microscope, IHs are unencapsulated aggregates of closely packed, thin-walled capillaries, usually with endothelial lining.
[42] Indications for treatment include functional impairment (i.e. visual or feeding compromise), bleeding, potentially life-threatening complications (airway, cardiac, or hepatic disease), and risk of long-term or permanent disfigurement.
When they interfere with vision, breathing, or threaten significant disfigurement (most notably facial lesions, and in particular, nose and lips), they are usually treated.
[45][46][47] Following the serendipitous observation that propranolol, a nonselective beta blocker, is well tolerated and effective for treatment of hemangiomas,[48][49] the agent was studied in a large, randomized, controlled trial[50] and was approved by the U.S. Food and Drug Administration for this indication in 2014.
[43] Since that time, topical timolol maleate in addition to oral propranalol has become a common therapy for infantile hemangiomas.
According to a 2018 Cochrane review,[53] both of these therapies have demonstrated beneficial effects in terms of clearance of hemangiomas without an increase in harms.
All of these results were based on moderate- to low-quality evidence, thus further randomized, controlled trials with large populations of children are needed to further evaluate these therapies.
Other systemic therapies which may be effective for IH treatment include vincristine, interferon, and other agents with antiangiogenic properties.
Vincristine, which requires central venous access for administration, is traditionally used as a chemotherapy agent, but has been demonstrated to have efficacy against hemangiomas and other childhood vascular tumors, such as kaposiform hemangioendothelioma and tufted angioma.
[54][55] Interferon-alpha 2a and 2b, given by subcutaneous injection, has shown efficacy against hemangiomas,[56] but may result in spastic diplegia in up to 20% of treated children.
Intralesional corticosteroid (usually triamcinolone) injection has been used for small, localized hemangiomas, where it has been demonstrated to be relatively safe and effective.
[68] PDL is most often used for treatment of ulcerated hemangiomas, often in conjunction with topical therapies and wound care, and may speed healing and diminish pain.
[75] Hemangiomas, especially those that have gotten very large during the growth phase, may leave behind stretched skin or fibrofatty tissue that may be disfiguring or require future surgical correction.
For example, a patient with a large facial hemangioma who is found to meet criteria for PHACE syndrome will require potentially ongoing neurologic, cardiac, and/or ophthalmologic monitoring.
For these reasons, infants with infantile hemangiomas should be evaluated by an appropriate clinician during the early proliferative phase so that risk monitoring and treatment be individualized and outcomes can be optimized.
The term hemangioma was originally used to describe any vascular tumor-like structure, whether it was present at or around birth or appeared later in life.
[78] The classification of vascular anomalies is now based upon cellular features, natural history, and clinical behavior of the lesion.