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
- optic atrophy and visual deterioration
Radiology is usually only required when the diagnosis is unclear, and this is most frequent in lesions with a deep component.
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.
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.
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.
Differential considerations include
Other vascular malformations of the orbit
- 1. Sweet C, R Silbergleit and B Mehta. “Primary intraosseous hemangioma of the orbit: CT and MR appearance.” AJNR Am J Neuroradiol 18, no. 2 (February 1, 1997): 379-381. [Link].
- 2. Weir Jamie and Alison D. Murray. Mosby's atlas and text of clinical imaging. Elsevier Health Sciences, 1998. ISBN: 0723425558, 9780723425557.
- 3. Shields Carol L. Eyelid, Conjunctival, and Orbital Tumors. Lippincott Williams & Wilkins, 2007. ISBN: 0781775787, 9780781775786.
- 4. Smoker WR, Gentry LR, Yee NK et-al. Vascular lesions of the orbit: more than meets the eye. Radiographics. 28 (1): 185-204. doi:10.1148/rg.281075040 - Pubmed citation
- 5. Dubois J, Garel L, David M et-al. Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J Roentgenol. 2002;178 (6): 1541-5. AJR Am J Roentgenol (citation) - Pubmed citation
- 6. Maguiness SM, Frieden IJ. Management of difficult infantile haemangiomas. Arch. Dis. Child. 2012;97 (3): 266-71. doi:10.1136/archdischild-2011-300851 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Strawberry haemangioma of orbit||✓|
|Infantile haemangioma, orbital||✓|