2020–2021 BCSC Basic and Clinical Science Course™
12 Retina and Vitreous
Part II: Disorders of the Retina and Vitreous
Chapter 09: Choroidal Disease
Isolated choroidal hemangiomas are reddish-orange, well-circumscribed tumors of varying thickness that can affect the macula either directly or through subretinal fluid (Fig 9-16). Circumscribed hemangiomas transilluminate readily and exhibit highly echographic patterns on ultrasonography. During dye-based angiography, hemangiomas show very early filling of large vessels. Sturge-Weber syndrome (encephalofacial cavernous hemangiomatosis) causes a diffuse hemangioma that may present first as glaucoma or amblyopia in children. The areas corresponding to the hemangioma have a typical tomato ketchup appearance, and the underlying choroidal markings are not visible. The choroidal hemangiomas in Sturge-Weber syndrome are sometimes overlooked because they are diffuse and may blend imperceptibly into adjacent normal choroid. An ipsilateral facial nevus flammeus (port-wine stain) is also typically present in patients with this syndrome.
Figure 9-16 Choroidal hemangioma. A, Fundus photograph of the typical reddish-orange elevation of a circumscribed choroidal hemangioma. B, Soon after ICG injection, the vascular composition of the hemangioma is revealed. C, Hyperfluorescence of the tumor occurs in the middle phase of the angiogram study from a combination of dye within and leakage from the vessels of the hemangioma. D, In the late phase of the study, the dye “washes out” of the lesion, leaving hyperfluorescent staining in the adjacent tissues.
(Used with permission from Spaide RF, Goldbaum M, Wong DW, Tang KC, Iida T. Serous detachment of the retina. Retina. 2003;23: 820–846.)
The alterations caused by choroidal hemangiomas include cystic retinal edema, hard exudate, neurosensory detachment, and calcification and ossification in the choroid. Hemangiomas have been treated with laser photocoagulation, cryopexy, external-beam and plaque radiation, and PDT. The success rate for treatment of diffuse hemangiomas with visual acuity improvement is poor. PDT with verteporfin may have the lowest risk of treatment-related morbidity.
Beardsley RM, McCannel CA, McCannel TA. Recurrent leakage after Visudyne photodynamic therapy for the treatment of circumscribed choroidal hemangioma. Ophthalmic Surg Lasers Imaging Retina. 2013;44(3):248–251.
Blasi MA, Tiberti AC, Scupola A, et al. Photodynamic therapy with verteporfin for symptomatic circumscribed choroidal hemangioma: five-year outcomes. Ophthalmology. 2010; 117(8):1630–1637.
Madreperla SA, Hungerford JL, Plowman PN, Laganowski HC, Gregory PT. Choroidal hemangiomas: visual and anatomic results of treatment by photocoagulation or radiation therapy. Ophthalmology. 1997;104(11):1773–1778.
Tsipursky MS, Golchet PR, Jampol LM. Photodynamic therapy of choroidal hemangioma in Sturge-Weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas. Surv Ophthalmol. 2011;56(1):68–85.
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.