Previous classification of these tumours have included Haik’s which divided them into superficial strawberry naevi (25%), bluish subcutaneous (68%), and deep orbital (7%).4 Rootman described them slightly differently: superficial strawberry naevus (40%), combined subcutaneous and deep orbital (30%), and combined superficial strawberry and subcutaneous (30%).5
The ultrasound classification we present here is simple, easy to perform (even from static images, 88% in this series, and dynamic scanning is used in practice), and does not need sedation or a general anaesthetic unlike other imaging modalities such as CT or magnetic resonance imaging.
Both A and B scan ultrasound findings have previously been described as characterised by variable internal reflectivity.6 Areas of low reflectivity are thought to correspond to solid hypercellular regions of endothelial proliferation, areas of moderate reflectivity to ectatic vascular channels, and areas of high reflectivity to fibrous septae separating tumour lobules. Computed tomography (CT imaging) often reveals a soft tissue mass in the anterior orbit or as an extraconal mass with finger like posterior projections such as those seen in five of our cases using ultrasound.1 The CT tumour image enhances with iodinated contrast medium, and major feeding vessels supplying the tumour may be depicted. Using magnetic resonance (MR) imaging the tumour is of intermediate intensity on T1 weighted studies (hypointense to orbital fat and isointense to muscle).7 The lesion is hyperintense on T2 weighted studies caused by slow blood flow within the tumour mass; major feeding vessels appear as black serpigenous structures caused by the “flow void” phenomenon (rapidly moving blood in large vessels outpaces the stimulation-reception parameters of the MR sequence).1 Although CT and MR imaging may both yield more detail in regard to the anatomical relations of the tumour, both would require a general anaesthetic or sedation in this age group whereas ultrasound does not because it is quick, painless, and requires minimal co-operation from the child. Surgical findings, although only available in four cases, confirmed the ultrasound classification in all four providing some support for its validity. In one case neuroimaging was performed as well and this confirmed the ultrasound findings (fig 3).
| Figure 3(A) Clinical photograph of a deep periocular haemangioma. (B) A vertical ultrasound scan of the case shown (A). Again the left of the picture corresponds to the superior part and the right to the (more ...) |
At our unit we use a management protocol for children with periocular haemangiomas which considers age, anatomical extent, location in the skin, and visual effect. The location of the haemangioma in the skin is defined as superficial (usually bright red), deep (usually bluish), or mixed (see figs 2–4). A deep skin lesion may be purely preseptal, whereas one with a superficial component may have orbital extension (see figs 2 and 4).
The ultrasound classification defines the anatomical extent of the lesion more accurately than previously described clinical classifications which tend to confuse orbital extent and location in skin.4,5 Its importance lies in the fact that it may help define appropriate treatment—for example, a preseptal intralesional steroid injection is unlikely to be effective when the tumour is deep and intraconal.
We believe that ultrasound anatomical classification is an important first step in determining appropriate treatment of periocular haemangiomas. We present the first such classification to the best of our knowledge.