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Diagnosis and Management of Infantile Hemangioma

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Results<br />

We present results for Contextual Questions (CQ) followed by those for our network metaanalysis,<br />

which includes studies <strong>of</strong> beta-blockers <strong>and</strong> steroids. We then present results for each<br />

Key Question (comparative effectiveness questions).<br />

We identified 966 publications potentially relevant to the CQ in our database searches. We<br />

also flagged studies for potential relevance to CQ in our screening <strong>of</strong> studies for Key Questions.<br />

We included 68 studies in the narrative summary <strong>of</strong> information addressing CQ.<br />

CQ1. Natural History <strong>of</strong> Untreated IH <strong>and</strong> Adverse Outcomes<br />

<strong>of</strong> Untreated IH<br />

Natural History <strong>of</strong> IH<br />

IH have been estimated to occur in around 5 percent <strong>of</strong> neonates <strong>and</strong> infants. 2 IH may be<br />

classified into subtypes including localized, segmental, indeterminate, <strong>and</strong> multifocal. Several<br />

studies have shown most IH to be <strong>of</strong> the localized type, <strong>and</strong> regardless <strong>of</strong> type, most IH involute<br />

with time; 30-33 however, the presentation <strong>and</strong> course <strong>of</strong> IH in individual children are<br />

heterogeneous. 34 IH usually present within the first month <strong>of</strong> life <strong>and</strong> undergo rapid proliferation<br />

over the first several months <strong>of</strong> life. 35,36 One study found that IH reached 80 percent <strong>of</strong> their final<br />

size by 5 months <strong>of</strong> age. 9 Many experts recommend referral at an early age (as early as 4 to 8<br />

weeks <strong>of</strong> life) to subspecialists given this rapid proliferation. 9<br />

Segmental IH are more likely to have more prolonged growth, defined as after 9 months <strong>of</strong><br />

age. 9,37 Involution typically starts by 1 year <strong>of</strong> age, but the timing <strong>of</strong> involution varies<br />

markedly. 38,39 In one large retrospective review <strong>of</strong> 1109 referred patients (median age=8 months)<br />

conducted in the pre-propranolol era, 769 were returned to the care <strong>of</strong> their primary provider<br />

without subspecialty followup, <strong>and</strong> only 102 (9%) required intervention. 40<br />

Most lesions involute by age 5 to 7, 41,42 though timing varies, <strong>and</strong> disfigurement may<br />

remain. 22,38,43,44 The majority (80%) <strong>of</strong> lesions involuting after age 6 years in one series resulted<br />

in residual scarring or telangiectasia, compared with 38 percent involuting before age 6. 35 In<br />

studies <strong>of</strong> referred populations, residual lesions (e.g., telangiectasias, atrophy, fibr<strong>of</strong>atty tissue,<br />

hypopigmentation) were reported in 25 to 69 percent <strong>of</strong> untreated IH. 38,45 Lesions affecting<br />

visual cortex development may result in lasting deficits in vision even after resolution <strong>of</strong> the<br />

IH. 46<br />

Indications for Treatment<br />

The major indications for treatment <strong>of</strong> IH include risks <strong>of</strong> ulceration, disfigurement, <strong>and</strong><br />

functional impact. 39,47-52 While psychological impact on the child also plays a role in treatment<br />

decisions, data on the effects <strong>of</strong> IH on quality <strong>of</strong> life for the child suggest minimal impact. Such<br />

data are <strong>of</strong>ten limited by the necessity to parent-report in this young population. 22,53,54 Estimates<br />

<strong>of</strong> complications from IH vary but are generally noted to occur in approximately 30 percent <strong>of</strong><br />

the studied population. 31,32,55 One study found higher initial complication rates for patients<br />

referred to a surgical center, potentially due to the higher likelihood <strong>of</strong> more advanced lesions<br />

being referred. 56 Given that the literature typically includes children treated at referral centers, it<br />

is likely that the overall complication rate may be higher in study populations than in the general<br />

population.<br />

14

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