The most striking result of our study is the very small number of patients whose treatment results could be evaluated objectively. Despite the use of corticosteroids in the treatment of haemangiomas for more than three decades and the high risk of morbidity leading to permanent visual loss, studies with measurable outcomes in this setting are lacking. In those case series which were evaluated, we found insufficient evidence to demonstrate benefits of one corticosteroid over another in patients with periorbital haemangioma of infancy. Similarly, we found insufficient evidence for increased side effects of one steroid over another. We found weak evidence that intralesional steroid injections may result in reduced refractive error.
The five cases involving topical steroids generally demonstrated worse spherical equivalent refractive error after treatment, with no clear trend in the astigmatic component alone. The 23 cases involving intralesional injection demonstrated a trend towards reduced astigmatism and reduced spherical equivalent refractive error with treatment. Although one might argue that steroids speeded up involution, the improvement in refractive error cannot be attributed to steroid treatment rather than the natural course of development after spontaneous haemangioma involution. The Motwani series included four patients who were managed conservatively (without steroids, not included in table 3). The authors concluded that visual outcomes were similar in treated and untreated patients, but selection of patients was non-randomised.
Despite reports of successful steroid treatment of periorbital HOI dating back to the 1970s, the evidence for relative efficacy and safety of oral, topical, and intralesional steroids remains scant. Several of the largest studies produced in our literature search were not included in this review because they did not measure objective ophthalmological data before and after treatment. The largest single study meeting criteria, by Morrell and Willshaw, contained only 13 patients with measured objective end points both before and after treatment.19
The complications of untreated periorbital HOI have been well documented, including astigmatism, myopia, amblyopia, and strabismus. Several articles have reported on the beneficial effect of corticosteroids in preventing or reducing occlusion of the visual axis. However, a substantial proportion of these studies and case series has failed to document refractive error or visual acuity before and after treatment. Several studies developed customised grading systems in order to stratify patient responses to steroids. In most cases, the grading systems were either subjective or based on data such as change in lesional size, which may or may not cause an objective improvement in visual outcome. Corticosteroids may facilitate haemangioma involution and thereby decrease volume or surface area of the lesion. However, changes in volume or surface area are only relevant to the extent that the haemangioma impinges on the visual axis at some point and involution results in clearing of the axis. Oral steroid treatment has been show to produce a response in cutaneous haemangiomas as measured by surface area, but the goals of ophthalmological treatment differ, at least in part, from the goals of dermatological treatment of HOI.8
Because of the limited numbers of cases meeting our inclusion criteria and the variation in dosage and administration of corticosteroids given, the results are not conducive to a statistical meta-analysis. Since there were no studies comparing different modalities of steroid therapy and no studies of individual modalities large enough to estimate efficacy, no conclusions can be drawn regarding which modality is most efficacious. The studies reviewed indicate that intralesional injections appear efficacious insofar as they may reduce astigmatism and spherical equivalents of refractive error; the efficacy of topical steroids is less clear. However, greater numbers of patients in each group are needed to project an estimate of overall efficacy. A larger study might also clarify how much of observed reduction in refractive error is the result of correction with ageing or the natural course of haemangioma involution as opposed to steroid treatment effects.
Similarly, there are insufficient data to estimate the incidence of corticosteroid side effects for patients treated with steroids for periorbital HOI or complications of intralesional injections in particular. Numerous case reports have established the need for caution in giving prolonged courses of steroids and the need for precautions to avoid vascular occlusion when injecting intralesional steroids, but the incidence of such complications remains unknown.
A useful determination of relative efficacy and side effects of different steroid modalities for treatment of periorbital HOI could be achieved through a prospective, longitudinal multicentre trial. Objective measurements such as visual acuity, astigmatism, or strabismus must be measured both before and after treatment, with follow up more than 6 months after completion of treatment and concurrent measurements in the unaffected eye. We would recommend checking cycloplegic refractions monthly until stabilisation of haemangioma growth, with subsequent bimonthly and then quarterly checks for 3 years. Visual acuity can be measured by preferential looking, and standardised protocols of occlusion therapy can be used for treatment of amblyopia. Steroid preparations and dosages must also be standardised.
In an era of evidence based medicine, corticosteroid treatment of periorbital HOI is still based primarily on recommendations made decades ago as well as the personal preferences of the treating paediatric ophthalmologist. The need for objective data to guide future practices is evident, and establishing the relative efficacy and safety of oral, topical and intralesional steroids through a randomised prospective study would greatly benefit those children with periorbital HOI requiring ophthalmological intervention.