Choroidal hemangioma is a congenital, unilateral, benign tumor composed of endothelium-lined vascular channels. Two types exist:
- Circumscribed: no association with systemic disease.
- Diffuse: occurs in Sturge-Weber syndrome, characterized by facial portwine stain (cutaneous hemangioma or nevus flammeus), central nervous system hemangiomas and a high incidence of glaucoma (up to 70 per cent of cases).
Patients with choroidal hemangioma require regular review to enable timely detection and treatment of complications including cystic retinal degeneration and exudative retinal detachment.
Circumscribed choroidal hemangiomas are usually diagnosed between the second and fourth decade when vision is affected by retinal detachment or cystic retinal degeneration. Symptoms of macular involvement include blurred central vision, micropsia and metamorphopsia. Diffuse choroidal hemangiomas are usually diagnosed in infancy during the assessment of Sturge-Weber syndrome.
A circumscribed choroidal hemangioma typically appears as a slightly elevated, round, orange-red lesion on fundoscopy. Compression of surrounding melanocytes at the periphery may produce a surrounding ring of hyperpigmentation.
Diffuse choroidal hemangiomas almost invariably occur ipsilateral to the facial port-wine stain. They have poorly defined borders, and often occupy the majority of the fundus, which assumes a deep red colour. (This is typically most apparent upon comparison with the fellow eye.) In view of the increased risk of glaucoma in Sturge-Weber syndrome, the intraocular pressure must be measured routinely.comparison with the fellow eye.) In view of the increased risk of glaucoma in SturgeWeber syndrome, the intraocular pressure must be measured routinely.
Uncommon. Diffuse choroidal hemangioma occurs in about half of patients with Sturge-Weber syndrome.
Potential vision-threatening complications include retinal detachment, cystic retinal degeneration and glaucoma.
Choroidal melanoma, choroidal metastasis, choroidal nevus, choroidal osteoma, retinal capillary hemangioma, posterior scleritis, central serous retinopathy, VogtKoyanagi-Harada.
Neurofibromatosis, Tuberous sclerosis, Von Hippel Lindau syndrome, Age-related macular degeneration.
Ocular tests With fluorescein angiography, the lesion fluoresces simultaneously with the retinal vasculature and leaks fluorescein in the late phases.
Ultrasonography is useful to confirm the diagnosis (solid echogenic lesions with high internal reflectivity) and to assess overlying serous retinal detachment. Optical coherence tomography clearly delineates subretinal fluid.
Magnetic resonance imaging is used to diagnose central nervous system hemangiomas in patients with SturgeWeber syndrome.
Review Choroidal hemangiomas not involving the macula and with no surrounding subretinal fluid are reviewed at yearly intervals. This is supplemented
Circumscribed choroidal hemangioma.
by patient self-monitoring with an Amsler grid to facilitate early detection of retinal detachment near the macula.
Laser The decision to treat a choroidal hemangioma is influenced by the extent of visual loss and the potential for visual recovery. Laser therapy, radiotherapy, transpupillary thermotherapy and cryotherapy have been applied to areas of retinal detachment that threaten vision. More recently, photodynamic therapy using verteporfin has yielded promising results, particularly in circumscribed choroidal hemangiomas. The principal complication of photodynamic therapy is choroidal ischemia, which may cause sudden permanent vision loss within 24 hours of treatment; longer-term followup studies will reveal the efficacy and safety of this treatment modality. Historically, the long-term visual prognosis of choroidal hemangioma has been poor, even in adequately treated patients.
Referral Referral to a neurologist is often appropriate for patients with SturgeWeber syndrome.