Capillary hemangioma of the orbit

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. 

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 thereafter³. They may involve theextra-ocular muscles andlacrimal glands and may extendsextend intracranially through theoptic canal orsuperior orbital fissure⁴. Only rarely are they associated with systemic haemangiomas or with other pathology such asPHACES 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 lesions⁴ with high peak intra-tumoral 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 used⁵.

CT

The CT appearance is that of a strongly enhancing lobulated mass. The enhancement is typically homogeneous. On imaging alone, it is hard 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 hypointense on T1, iso- to hyperintense on T2 with multiple serpiginous flow voids⁴. Enhancement is homogenous with gadolinium with marked enhancement of intratumoral 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-tumoral laser therapy has also been used for larger lesions and, in recalcitrant cases, interferon or vincristine can be considered⁶.

Differential diagnosis

Differential considerations include:

• other vascular malformations of the orbit
  • cavernous haemangioma of the orbit
  • orbital venous varix
  • orbital lymphangioma
• orbital tumours
  • retinoblastoma
  • orbital metastases
  • rhabdomyosarcoma
  • chloroma