choroidal hemangioma

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.
  • Epidemiology

    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.

    Clinical presentation

    Mostly asymptomatic and incidentally diagnosed with routine ophthalmic examinations:

    • CCH presents with symptoms in 3-6 decades of life 
    • DCH presents with symptoms in 1-2 decades of life 

    Patients also may present with metamorphopsia and reduced visual acuity.

    Pathology

    No known genetic predisposition for CCH. SW syndrome is entirely sporadic and is associated with DCH.

    Radiographic features

    Angiography
    • 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)
    Ultrasound
    • 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.

    Differential diagnosis 

    Practical points

    • 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