We searched PubMed with the term “infantile haemangioma” for articles published in English between Jan 1, 2010, to Dec 31, 2015. We also included highly cited older publications because of their relevance. For the sections on treatment we gave priority to randomised controlled studies, and meta-analyses or large series of patients in the absence of randomised controlled data.
SeminarInfantile haemangioma
Introduction
Infantile haemangiomas are the most common soft-tissue tumours of infancy. They arise in the first few weeks of life, then display a period of active growth followed by spontaneous involution. Most infantile haemangiomas do not require therapy and regress spontaneously; however, about 10–15% result in complications, such as obstruction, ulceration, or disfigurement, and require treatment. This Seminar provides an update on new insights into the pathogenesis and treatment of infantile haemangioma, which has arisen as a result of the discovery that β blockers are an effective treatment. It will cover the different clinical aspects of infantile haemangioma, their possible associated complications, and current approaches to management.
Section snippets
Epidemiology
The prevalence of infantile haemangioma in mature neonates is around 4·5%,1 with a female (2·3–2·9 times higher) and white predominance. It increases with low birthweight and decreasing gestational age, and is as high as 23% in premature babies smaller than 1000 g birthweight.2 Additionally, family history of infantile haemangioma, intrauterine complications such as eclampsia, and placental anomalies are also important risk factors.1
Pathogenesis
Infantile haemangioma is the result of dysregulation of both vasculogenesis and angiogenesis (figure 1); however, the triggers that initiate development of infantile haemangioma are still a matter of debate. No single hypothesis is sufficient to describe all features of infantile haemangioma. The most likely scenario would involve hypoxic stress as the triggering signal,3 inducing overexpression of angiogenic factors such as VEGF via the HIFα pathway.4 In response to VEGF overexpression, stem
Clinical characteristics
Infantile haemangiomas exhibit a characteristic growth pattern (figure 2); precursor lesions (figure 3) are either present at birth or manifest during the early neonatal period (as a pale area of vasoconstriction or a telangiectatic red macule. After a latent period of 1–3 weeks, the haemangiomas typically start to proliferate. Superficial infantile haemangiomas are located in the upper dermis and typically appear as elevated red papules, nodules, or plaques (figure 4). Deep infantile
Obstruction and functional impairment
Visual obstruction usually manifests during the early proliferation phase (ie, within the first 2–3 months of life). Haemangiomas located on the eyelid or close to the eye can lead to permanent amblyopia, astigmatism, or strabismus (figure 7). Additional potential complications include proptosis, poor eyelid closure, and optic nerve injury.13 Obstruction of the nostrils or auditory channel is less commonly observed. Paraglottic or intratracheal infantile haemangioma, often heralded by
Diagnosis and assessment
Infantile haemangiomas are usually diagnosed clinically. Imaging studies and other investigations are required in the special situations listed in the table. Ideally, a patient with infantile haemangioma who is at risk of complications should be referred to a multidisciplinary team for evaluation and for specific diagnostic measures (eg, MRI, screening for hypothyroidism, or coagulation abnormalities) and initiation of specific treatment.
Several scores have been established to assess severity,
Vascular anomalies
Vascular anomalies are classically divided into vascular tumours and vascular malformations (panel 1), and both can resemble infantile haemangioma. Usually a vascular malformation (such as a port-wine stain or lymphatic malformation) is present at birth, does not grow (or grows very slowly over several years), and does not regress spontaneously. By contrast, a vascular tumour might or might not be present at birth, has a tendency to grow, and for infantile haemangioma has the ability to regress
Indications and timing
Because most infantile haemangiomas tend to regress spontaneously, treatment is only required for complicated cases. Indications for treatment include life-threatening infantile haemangioma (obstructive subglottic tumours, compression of neural structures, bleeding gastrointestinal tumours, large haemangiomas causing cardiac insufficiency or hepatic dysfunction), infantile haemangioma causing functional impairment (periocular haemangiomas causing [imminent] amblyopia, obstructive tumours of the
Conclusions
Despite the lack of satisfactory experimental models, knowledge of infantile haemangioma has made tremendous progress during the past decade. Risk factors are better identified, understanding of the pathophysiology has improved, and a wide variety of clinical presentations has been described. Additionally, the recognition that β blockers are an effective treatment for infantile haemangioma has opened up a new therapeutic area and given rise to research in the field of vascular biology.
Search strategy and selection criteria
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Cited by (346)
Exploration of the optimal time to discontinue propranolol treatment in infantile hemangiomas: A prospective study
2024, Journal of the American Academy of DermatologyApolipoprotein A-I Binding Protein Inhibits the Formation of Infantile Hemangioma through Cholesterol-Regulated Hypoxia-Inducible Factor 1α Activation
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2023, Biochemical PharmacologyIntralesional betamethasone versus oral propranolol for localized infantile hemangiomas of the lip
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