Choroidal hemangioma

Choroidal hemangiomasBenign vascular haematomatous tumours of the choroid. 

Present in 2 forms based on the extent of choroidal involvement:

1. Circumscribed choroidal hemangioma (CCH): Solitary tumour with no systemic associations
2. Diffuse choroidal hemangioma (DCH): Usually in association with Sturge-Weber (SW) syndrome 

Epidemiology

• Rare

  • exact incidence worldwide not known

  • 200 diagoses of CCH made from 1974 - 2000 in Wills Eye Institute 

  • approximately half of patients with SW have DCH

• Likely congenital

• Nearly all reported cases are benighhn vascular harmatomas thatin Caucasians 

• No gender preference 

Clinical presentation

• 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 
• Metamorphopsia 
• Reduced visual acuity 

Aetiology

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

Radiographic features

• Angiography - diagnostic 
  • 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 (tumour 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 Tomorgaphy (OCT):
  • detection of retinal changes such as fluid changes, underlying geographical atrophy etc
  • useful to evaluate treatment response and long-term monitoring 

Treatment 

• Observation if asymptomatic 

• End-point of treatment is resolution of fluid, and not complete regression of tumour

• 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

• DCH need to be worked up for SW and managed accordingly

Prognosis

• Prognosis for CCH generally improving with PDT, however, very much dependent on location of lesion. 

• Long delay between 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 

• Choroidal metastases 
• Amelanotic choroidal melanoma 
• Choroidal osteoma 
• Central serous chorioretinopathy
• Retinal detachment 
• Age-related macular degeneration 
• Retinoblastoma 
• Inflammatory conditions

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 diffuseobserved without treatment
• CCH should be considered in the differential diagnosis of circumscribed.

  Terminology

  Epidemiology

  patients diagnosed with central serous chorioretinopathy
Clinical presentation

Pathology

Radiographic features

Radiology report

Treatment and prognosis

History and etymology

Differential diagnosis

Practical points