Diagnosis and Management of Infantile Hemangiomas

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Key points

  • Infantile hemangiomas (IH) show great heterogeneity in size, morphology, growth, residua remaining after involution, and in degree of response to therapy.

  • Propranolol is now preferred first-line therapy, given that its efficacy, tolerability, and safety are superior to that of oral corticosteroids.

  • The period of most rapid proliferation is complete by 8 weeks after birth, suggesting that referral to specialists should occur early, within the first month of life for concerning IH.

  • More infants are

Diagnosis

Proper diagnosis of IH hinges on a solid understanding of the classification structure for vascular anomalies, and the most comprehensive and widely accepted classification schema is put forth by the International Society for the Study of Vascular Anomalies (ISSVA). The classification rests primarily on distinguishing vascular tumors, of which IH are the most common, from vascular malformations (Fig. 1, Table 1). In all but a few cases, the diagnosis of IH can be determined by good history

Epidemiology

The incidence of IH has remained unclear, because they are not often present at the time of hospital discharge after birth (when most registries for birth defects are completed.) Current evidence from a prospective study that followed nearly 600 pregnant women and their infants until the infants were 9 months of age suggests an overall incidence of IH of 4.5%, and up to 9.8% in premature infants.7 Although the cause of IH is not yet fully known, there are several important risk factors for

Natural history

The growth cycle of IH is unique among vascular tumors and malformations, showing proliferation and preprogrammed involution. IH start with a nascent phase as either an area of pallor, a telangiectatic patch, or something mistaken for an ecchymosis or birth trauma.16 They may present anywhere on the body surface but are present most commonly on the head and neck, a finding which is also true of vascular malformations.17 Typically, most begin to proliferate to a noticeable degree at between 2

Complications

Most IH follow a benign course without the need for intervention aside from anticipatory guidance, but evidence shows that approximately 12% are complex and warrant referral for specialist evaluation; of those seen at tertiary care centers, nearly a quarter developed complications, ranging from ulceration to obstruction of the eye, airway, or auditory canal to cardiac compromise, in a study based on a cohort of more than 1000 infants.9 The same study documented that segmental IH are 11 times

Multifocal IH

Infants can present with multiple, often small, focal IH; clinically, some infants present with monomorphic small superficial His, sometimes numbering in the hundreds, whereas others present with multiple focal IH of various sizes and appearance. This phenomenon has been referred to as benign neonatal hemangiomatosis when it occurs without visceral involvement and as diffuse or disseminated neonatal hemangiomatosis (DNH) when it is accompanied by symptomatic IH internally, in the liver,

Complications associated with segmental IH

PHACE (OMIM 606519), a neurocutaneous syndrome of skin, brain, eye, and ventral body anomalies, is the most well-documented segmental IH syndrome occurring in the setting of segmental facial IH (Fig. 12), although a few cases have been reported with nonfacial IH and systemic complications relevant to PHACE.40, 41, 42, 43 Diagnostic criteria were published in 2009 and help to risk stratify patients into definite, possible, or probable PHACE syndrome, based on organ involvement at presentation.44

Therapy

Although there have been impressive and fundamental changes in our understanding of the heterogeneous presentations and pathogenesis of IH in the last decade, clearly the most dramatic changes have occurred in the realm of therapy, with the complete shift toward β-blockers as the standard of care. An important simultaneous but perhaps less obvious effect of the use of propranolol, which is widely accepted to be a safer and more well-tolerated drug than oral corticosteroids (OCS), is that

Summary

Improved insight into the natural history, associated complications, and response to propranolol has helped to advance IH research and practice. In many cases, the decision to treat is made obvious by associated complications. For most patients whose indication for therapy is risk of disfigurement, it is a fundamentally important concept to decide at which stage within the growth cycle of the IH to intervene with close monitoring, topical, systemic, adjunctive (laser or intralesional) therapy,

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