A capillary hemangioma is usually present at birth and may enlarge slowly. Isolated capillary and cavernous hemangiomas of the bulbar conjunctiva are rare and are more likely to represent extension from adjacent structures. The palpebral conjunctiva is frequently involved with a capillary hemangioma of the eyelid. The presence of diffuse hemangiomatosis of the palpebral conjunctiva or conjunctival fornix indicates an orbital capillary hemangioma. A cavernous hemangioma of the orbit may present initially under the conjunctiva.
Table 12-4 Vascular Tumors of the Eyelid and Conjunctiva*
Nevus flammeus, a congenital lesion described as a port-wine stain, may occur alone or as part of Sturge-Weber syndrome, associated with vascular hamartomas, secondary glaucoma, and/or leptomeningeal angiomatosis. Some cases result from a mutation in the gene coding for the vascular endothelial protein receptor for angiopoietin 1, which controls the assembly of perivascular smooth muscle.
Ataxia-telangiectasia (also called Louis-Bar syndrome) is a syndrome of epibulbar telangiectasis, cerebellar abnormalities, and immune alterations. In this autosomal recessive disease, the epibulbar and interpalpebral telangiectasia of the arteries lacks an associated lymphatic component. The epibulbar vascular lesions of ataxia-telangiectasia can grow with the patient and the eyeball, but episodes of hemorrhage or swelling do not occur. See BCSC Section 6, Pediatric Ophthalmology and Strabismus, for additional discussion of ataxia-telangiectasia, including illustrations.
Inflammatory vascular tumors
Inflammatory conjunctival lesions often show vascular proliferation. Pyogenic granuloma, a common type of reactive hemangioma, is misnamed because it is not suppurative and does not contain giant cells. The lesion may occur over a chalazion or when minor trauma or surgery stimulates exuberant healing tissue with fibroblasts (granulation tissue) and proliferating capillaries that grow in a radiating pattern. This rapidly growing lesion is red, pedunculated, and smooth (Fig 12-13); it bleeds easily and stains with fluorescein dye. Topical or intralesional corticosteroids may be curative. Excision with cauterization to the base and generous postoperative topical corticosteroids may minimize recurrences.
Figure 12-13 Pyogenic granuloma (in association with a chronically inflamed chalazion).
(Reproduced with permission from Mannis MJ, Holland EJ, eds. Cornea. Vol 1. 4th ed. Philadelphia: Elsevier; 2017:326.)
Subconjunctival granulomas may form around parasitic and mycotic infectious foci. They have also occurred with connective tissue diseases such as rheumatoid arthritis. Sarcoid nodules appear as tan-yellow elevations that can resemble follicles. Juvenile xanthogranuloma is a histiocytic disorder that can present as a conjunctival mass. A fibrous histiocytoma, composed of fibroblasts and histiocytes with lipid vacuoles, arises, in rare cases, on the conjunctiva or limbus. Nodular fasciitis is a very rare benign tumor of fibrovascular tissue in the eyelid or under the conjunctiva; it may originate at the insertion site of a rectus muscle. Necrobiotic xanthogranuloma is a very rare tumor that may affect the anterior orbit and eyelids. The lesion can present as subconjunctival or subdermal nodular fibrovascular tissue. Biopsy is essential to establish the diagnosis because this tumor is often associated with paraproteinemias, multiple myeloma, or lymphoma.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.