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Capillary haemangioma of the orbit

Dr Maxime St-Amant and Dr Frank Gaillard et al.

Capillary haemangioma of the orbit, also known as strawberry haemangioma, on account of its colouring, is the most common orbital tumour of infancy, and unlike orbital cavernous haemangiomas is a neoplasm rather than a vascular malformation ³.

Demographics and clinical presentation

Capillary haemangiomas are usually located anterior to the globe, in the eyelid, and are present at birth or shortly thereafter ³. Only rarely are they associated with systemic haemangiomas or with other pathology such as PHACES syndrome.

In a minority of cases the tumour is deep to the skin overlying the orbit and has a more variable presentation, including ¹:

palpable lump
proptosis
diplopia
ptosis
optic atrophy and visual deterioration

Radiographic features

Radiology is usually only required when the diagnosis is unclear, and this is most frequent in lesions with a deep component.

CT

The CT appearance is that of a strongly enhancing lobulated mass. The enhancement is typically homogeneous. On imaging alone, it is difficult to differentiate these lesions from other vascular lesions of the orbit, thus relying on patients age and clinical appearance.

MRI

The MRI appearance of capillary haemangioma is usually slightly hypo-intense on T1, iso- to hyper-intense on T2 with multiple serpiginous flow foids⁴. Enhancement is homogenous with gadolinium with marked enhancement of intra-tumoral vessels. Its lobulated appearance with thin septa is characteristic.

Ultrasound

On ultrasound, capillary haemangioma characteristically are hyperechoic and compressible lesions⁴ with high peak intra-tumoral on arterial shift. Ultrasound is mostly useful for smaller, limited lesions. At some institutions, other criterias such as > 5-6 vessels/cm² and a maximum Doppler systolic flow of > 2 kHz are used⁵.

Treatment and prognosis

During the first 3 - 6 months of life these masses enlarge before gradually reducing in size from the age of 12 - 18 months. 30% have resolved by the age of 3 years and 75 -90% by the age of 7 years ³. Injection of corticosteroids or systemic administration is effective, but reserved for cases where vision is affected. Intra-tumoral laser therapy has also been used for larger lesions and, in recalcitrant cases, interferon or vincristine can be considered⁶.