The refined Hurley classification: the inter‐rater and intrarater reliability and face validity
Authors: L.M. Prens, A. Rondags, R.J. Volkering, I.C. Janse, K. Politiek, Y.S. Zuidema, I. Turcan, J. Hasselt, H.H. Zee, B. Horváth
Journal: British Journal of Dermatology (2019)

SFH who presented to our outpatient department between 2005 and 2019 underwent cervical and cerebral MRI (cMRI) with MRA for the evaluation of CVA anomalies.Infantile haemangioma with the following characteristics were considered segmental: (i) positive criteria: long plaque-type haemangioma covering (part of) a cutaneous territory and (ii) negative criteria: round or oval form, or pedunculation.CVA anomalies were stratified according to Metry et al. 7 and Hess et al. 4 as low risk (dysgenetic or persistence of embryonal vessels, anomalous course and/or origin) or high risk (severe stenosis or undetectable blood vessels).Mean age at presentation was 4Á6 months (range: 1 week to 5 years).SFH diameter was < 5 cm in 31 of 58 patients (mean 3Á1 cm, range 0Á8-4Á9 cm) and ≥ 5 cm in 27 of 58 patients (mean 8Á6 cm, range 5Á7-12Á5 cm).cMRI with MRA was performed at a mean age of 7 months (range 4 days to 13 years).The anatomical distribution of SFH was assigned to embryonic placodes, as described previously. 8Results of MRA studies (performed for various reasons, mainly seizures, developmental delay and asphyxia) of 50 age-matched children were evaluated for comparison.Prevalence of CVA anomalies in children with small and large SFH, respectively, was compared with the v²test.Correlation between size of SFH and prevalence of CVA was assessed with Mann-Whitney U-test, Kruskal-Wallis test and/or Pearson correlation coefficient, as appropriate.In both groups, placode segments 2 and 3 were the most commonly affected.Associated anomalies (cerebellar hypoplasia, sternal clefts, cardiac anomalies) were present in 5 children with SFH < 5 cm and in 10 children with SFH ≥ 5 cm.CVA anomalies were detected in 21 of 58 children with SFH (36%).All were ipsilateral to the SFH.Of the children with SFH < 5 cm, 6 of 31 (19%) had CVA (Fig. 1a-d) compared with 15 of 27 (56%) children with SFH ≥ 5 cm (Fig. 1e) (P < 0Á01, v² test).Of 21 CVA anomalies, 19 involved the circle of Willis (90%, Fig. 1f).SFH diameter was significantly larger in children with CVA than in those without (P < 0Á01, Mann-Whitney U-test).Prevalence of CVA anomalies and size of SFH were positively correlated (r = 0Á47, Pearson correlation coefficient; P <0Á001).In 17 of 21 patients with CVA anomalies (81%), we observed long-segment narrowing or hypoplasia of the major vessels, while minor anomalies were found in 6 of 21 (29%).There was a weak positive correlation between the extent/severity of CVA anomalies and size of SFH (P < 0Á1, Kruskal-Wallis test).On follow-up, none of our patients had evidence of cerebral vasculopathy or stroke.CVA anomalies were detected in 5 of 50 controls (10%).To our knowledge, this is the first study to address the correlation between SFH size and likelihood of associated cerebrovascular anomalies.Our findings challenge current recommendations that cMRI should only be performed in children with SFH ≥ 5 cm, 6 irrespective of age.This threshold appears arbitrary, particularly as all haemangiomas (including SFH) grow with age.Our results suggest that the risk of associated CVA anomalies is proportional to the diameter of SFH without a clear size 'threshold'.In view of the potential risk of stroke and the prevalence of CVA anomalies even in children with smaller SFH, MRA may be indicated irrespective of SFH diameter, but there is yet no general consensus.Although propranolol is generally well tolerated in children with PHACE, 7 caution is advised (i.e., lower starting dose, slow increase, three instead of two doses) in using systemic beta blockers in children with CVA anomalies at higher risk of stroke. 3,7