Choroidal hemangiomas are benign vascular hamartomatous tumors of the choroid. They present in two forms based on the extent of choroidal involvement:
- Circumscribed choroidal hemangioma (CCH): solitary tumor with no systemic associations.
- Diffuse choroidal hemangioma (DCH): usually in association with Sturge-Weber syndrome.
The exact incidence worldwide not known but it is rare. 200 diagnoses of CCH made from 1974-2000 in Wills Eye Institute. Approximately half of patients with Sturge-Weber syndrome have DCH. Nearly all reported cases are in Caucasians and there is no gender preference.
Mostly asymptomatic and incidentally diagnosed with routine ophthalmic examinations:
- CCH presents with symptoms in 3rd-6th decades of life
- DCH presents with symptoms in 1st-2nd decades of life
Patients also may present with metamorphopsia and reduced visual acuity.
No known genetic predisposition for CCH. SW syndrome is entirely sporadic and is associated with DCH.
- Fundus fluorescein angiography (FFA): hyper-fluorescence in pre-arterial or early arterial phase, followed by late staining and leakage
- Indocyanine Green Angiography (ICGA): early intense hyper-fluorescence followed by dye washout in late frames (tumor hypo-fluorescence with rim of hyper-fluorescence)
- A-scan: high internal reflectivity
- B-scan: acoustic features similar to adjacent (normal) choroid. Choroidal thickening, especially in DCH
Optical coherence tomography
- detection of retinal changes such as fluid changes, underlying geographical atrophy etc
- useful to evaluate treatment response and long-term monitoring
Treatment and prognosis
Observation if asymptomatic. DCH need to be worked up for Sturge-Weber syndrome and managed accordingly. The end-point of treatment is resolution of fluid and not complete regression of tumor:
- photodynamic therapy (PDT) with verteporfin is the treatment of choice for CCH and has been used successfully for treatment of DCH
- plaque radiotherapy: second-line therapy failing PDT, or unsuitable for PDT
- laser photocoagulation: use limited to extrafoveal CCHs when PDT not possible; high rates of recurrent subretinal fluid despite initial success
- external beam radiotherapy: commonly used for management of DCH
Prognosis for CCH generally improving with PDT, however, very much dependent on the location of the lesion. A long delay between the onset of symptoms and treatment is associated with poorer visual outcome.
Complications include neovascular glaucoma secondary to chronic extensive retinal detachments, with some requiring enucleation.
- choroidal metastasis
- amelanotic choroidal melanoma
- choroidal osteoma
- central serous chorioretinopathy
- retinal detachment
- age-related macular degeneration
- inflammatory conditions
- prompt referral to specialist ophthalmology care
- always remind patients to report any new symptoms such as increased metamorphopsia, reduced visual acuity, photopsia or visual field defects
- PDT is the treatment of choice for CCH, but asymptomatic patients can be observed without treatment
- CCH should be considered in the differential diagnosis of patients diagnosed with central serous chorioretinopathy
- 1. Maguire, J., Murchison, A. and Jaeger, E. (2012). Wills Eye Institute 5-minute ophthalmology consult. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, pp.158-159.
- 2. Prognosis, C. (2018). Circumscribed Choroidal Hemangiomas: PDT Improves Prognosis. [online] American Academy of Ophthalmology. Available at: https://www.aao.org/eyenet/article/circumscribed-choroidal-hemangiomas-pdt-improves-p [Accessed 3 Jun. 2018].