Monitoring infantile hemangiomas: What’s the score?
By Howard B. Pride, MD, FAAD
Geisinger Medical Center, Department of Dermatology
Aug. 11, 2021
Vol. 3, No. 32
Infantile hemangiomas (IH) are common vascular tumors that generally run a self-limited course of rapid growth in the first 2-3 months of life, slow growth for another 4-6 months, and then total or near-total involution over the next 4-5 years (1). Despite this harmless progression, some hemangiomas need aggressive therapy and evaluation. While a recent, comprehensive clinical practice guideline was recently published in Pediatrics (2), primary care providers are understandably confused as to which infants to refer and which to simply monitor. A standardized referral instrument would be a valuable tool for primary care providers and an enhancement of patient care, providing timely referral for potentially significant IHs and avoiding unnecessary referrals.
This article by Léauté-Labrèze et al (3) represents the combined efforts of IH experts from 8 European countries. In stage 1 of this multicenter, cross-sectional, observational study, a group of experts from the IH European Task Force developed the 12-question IH Referral Score (IHReS) screening tool, a quick and easy-to-use questionnaire. Referral to a specialist is considered mandatory if any of the questions in part 1 are answered yes; these include complications or risk of complications; location on the central face or ears, breast (in girls), or midline/lumbrosacral; a size of 4 cm or greater, or more than 5 hemangiomas. If not, part 2 includes a series of six parameters related to location, size of the biggest hemangioma, and current age of the child and the growth, with a scoring system to determine whether referral is warranted. A set of 42 IH reference images was then developed.
In stage 2, a second group of international experts independently classified the set of 42 reference images as needing or not needing referral. This was considered the gold standard for stage 3 where expert and nonexpert physicians did a gestalt evaluation of the 42 images and then employed the IHReS screening tool to make referral decisions. The process was repeated 2 weeks later to assess intrarater reliability. The IHReS tool had a sensitivity of 96.6% and a specificity of 55% when the threshold of ≥ 4 points was used for questions 7-12. The positive predictive value (positive score leads to appropriate referral) was 40.5% and the negative predictive value (negative score leads to appropriate non-referral) was 98.3%, an indication that an appropriate referral will rarely be missed, but there may be some over-referral. Interrater and intrarater reliability was very good with the use of the tool.
I very much welcome this innovation. I have surveyed a handful of my primary care pediatric colleagues and the response had been overwhelmingly positive regarding the desire to use such a tool. We plan to incorporate the IHReS screening tool into a pediatric dermatology quality improvement project.
Our expert’s viewpoint
Patrick McMahon, MD, FAAD
Cooper Medical School of Rowan University
Historically, when systemic steroids were the mainstay of treatment for life-threatening or visually obstructive hemangiomas, the clinical decision to initiate treatment included weighing the risks of long-term use of corticosteroids which, in turn, limited treatment to only the most severe hemangiomas. While appropriate referral of infants with at-risk hemangiomas to dermatology expertise has always been important, since 2008, when Léauté-Labrèze and colleagues discovered that oral beta-blocker therapy could dramatically limit growth of proliferating hemangiomas, early referral has become even more essential for a wider range of lesions. (4) Now topical and oral beta-blocker therapies have the potential to treat hemangiomas that may previously have been untreated medically, only to leave behind redundant saggy skin, sometimes requiring surgical excision. Sadly, there were generations of children who went through early childhood with disfiguring residual hemangiomas who would now certainly qualify for referral to dermatology and initiation of preventative treatments. The onus is upon the primary care providers to know which neonates with hemangiomas should be monitored or referred for treatment. This is why educating primary care providers on appropriate referral to dermatologic expertise is essential so that dermatologists can intervene if and when required.
This is not to say that every patient who meets requirements for referral will benefit from treatment. Certainly, being monitored clinically with active non-intervention and education is still going to be an important option for dermatologists and families. However, early referral will allow treatments to be initiated preventatively when needed, ideally before proliferation occurs. Several articles have demonstrated that rapid proliferation of hemangiomas occurs approximately between 4 and 8 weeks of life. By age 3 months, 80% of the growth of superficial hemangiomas has been achieved. (5, 6) The collective goal is to have the conversation about the risks and benefits of topical and oral beta-blocker therapy before 5 weeks of age, potentially employing treatment to prevent complications such as ulceration, infection, functional impairments, scarring, and stretching of the skin.
I applaud the work done by our colleagues who developed this newly validated tool, the Infantile Hemangioma Referral Score, and hope that it can be widely used to optimize referrals for infants with at-risk hemangiomas. (3) To swiftly disseminate this tool, providers may want to employ the power of the electronic medical records used by primary care physicians and create digital questionnaires that will make this process even easier. There may also be utility in implementing forms of teledermatology, such as store-and-forward, provider-to-provider portals, for efficient triage or even full teleconsultations. Working together, primary providers and dermatologists can ensure expedited care for the new generation of babies with worrisome hemangiomas, minimizing complications and providing optimal long-term outcomes.
Chang LC, et al. Growth characteristics of infantile hemangiomas; implications for management. Pediatrics 2008;122:360-7
Krowchuk DP, et al. Clinical practice guideline for the management of infantile hemangiomas. Pediatrics 2019;143:e20183475
Léauté-Labrèze C, et al. The infantile hemangioma referral score: a validated tool for physicians. Pediatrics 2020;145:e20191628.
Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008 Jun 12;358(24):2649-51.
Maguiness SM, Frieden IJ. Management of difficult infantile haemangiomas. Arch Dis Child. 2012 Mar;97(3):266-71.
Chang LC, Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Nopper AJ, Frieden IJ; Hemangioma Investigator Group. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008 Aug;122(2):360-7.
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