Capillary hemangioma of the orbit
Capillary hemangiomas of the orbit, also known as strawberry hemangiomas, on account of its coloring, or orbital infantile hemangiomas, are the most common orbital tumors of infancy, and unlike orbital cavernous hemangiomas, they are neoplasms rather than vascular malformations.
Usually present as a periorbital subcutaneous mass. In a minority of cases the tumor is deep to the skin overlying the orbit and has a more variable presentation, including 1:
- palpable lump
- optic atrophy and visual deterioration
Capillary hemangiomas 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 hemangiomas or with other pathology such as PHACES syndrome.
Radiology is usually only required when the diagnosis is unclear, and this is most frequent in lesions with a deep component.
On ultrasound, capillary hemangioma characteristically is hyperechoic and compressible lesions 4 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 used 5.
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.
The MRI appearance of capillary hemangioma is usually slightly hypointense on T1, iso- to hyperintense on T2 with multiple serpiginous flow voids 4. 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 considered 6.
Differential considerations include:
- 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