Capillary haemangiomas of the orbit, also known as strawberry haemangiomas, on account of its colouring, or orbital infantile haemangiomas, are the most common orbital tumours of infancy, and unlike orbital cavernous haemangiomas, they are neoplasms rather than vascular malformations.
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Clinical presentation
Usually present as a periorbital subcutaneous mass. 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
Pathology
Capillary haemangiomas are usually located anterior to the globe, in the eyelid, and are present at birth or shortly thereafter 3. They may involve the extra-ocular muscles and lacrimal glands and may extend intracranially through the optic canal or superior orbital fissure 4. Only rarely are they associated with systemic haemangiomas or with other pathology such as PHACES syndrome.
Radiographic features
Radiology is usually only required when the diagnosis is unclear, and this is most frequent in lesions with a deep component.
Ultrasound
On ultrasound, capillary haemangioma characteristically is hyperechoic and compressible lesions 4 with high peak intra-tumoural on arterial shift. Ultrasound is mostly useful for smaller, limited lesions. At some institutions, other criteria such as >5-6 vessels/cm2 and a maximum Doppler systolic flow of >2 kHz are used 5.
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 patient age and clinical appearance.
MRI
The MRI appearance of capillary haemangioma is usually slightly hypointense on T1, iso- to hyperintense on T2 with multiple serpiginous flow voids 4. Enhancement is homogeneous with gadolinium with marked enhancement of intratumoural vessels. Its lobulated appearance with thin septa is characteristic.
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-tumoural laser therapy has also been used for larger lesions and, in recalcitrant cases, interferon or vincristine can be considered 6.
Differential diagnosis
Differential considerations include:
- other vascular malformations of the orbit
- orbital tumours