Infantile hemangiomas (IH) are the most common benign tumors of childhood. Timely diagnosis and management of higher-risk IH is key in avoiding permanent disfigurement, visual impairment, and life-threatening airway compromise. Here, we identify and critically appraise existing clinical practice guidelines (CPGs) for IH diagnosis and management. A systematic search of MEDLINE, SCOPUS, and EMBASE was conducted until August 2021. Four independent reviewers assessed each CPG utilizing the Appraisal of Guidelines for Research and Evaluation, 2nd edition (AGREE II). An scaled domain score of ≥60% demonstrated adequacy in a given domain. Intraclass correlation coefficients (ICC) assessed agreement and scoring consistency between the reviewers. Eight CPGs were eligible and included for critical appraisal. Only one CPG was classified as 'high quality', with the remaining seven guidelines being 'average' (n = 3) or 'low' (n = 4) quality. Six guidelines (75.0%) were conducted via nonsystematic literature searches. The 'Applicability' (40.4%±14.0) and 'Rigor of development' (46.9%±17.3) domains achieved the lowest scores, while the highest average scores were in 'Scope and purpose' (76.7%±11.3) and 'Editorial independence' (90.8%±13.0). We found high consistency between the four independent reviewers, with 'very good' (n = 5) or 'good' (n = 1) interrater reliability in all six AGREE II domains. Based on the AGREE II instrument, there is only one available high-quality consensus statement on the diagnosis and management of IH. Low scores in 'Rigor of development' and 'Applicability' suggest notable weaknesses in the development process and reporting quality of existing IH CPGs. Future guidelines should be backed by systematic literature searches and focus on guideline clinical translation.
Keywords: AGREE II; Infantile hemangioma; clinical practice guideline; consensus statement; propranolol.