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Ophthalmology – Choroidal Hemangioma
Choroidal hemangioma is a benign vascular tumor of the choroid composed of abnormal blood vessels. It exists in two main forms: circumscribed choroidal hemangioma (CCH), which is a localized solitary lesion without systemic associations, and diffuse choroidal hemangioma (DCH), which is typically associated with Sturge–Weber syndrome. While both forms are benign, they can significantly affect vision due to associated retinal changes.
Epidemiologically, CCH is rare, and DCH is even rarer. CCH typically presents in adulthood, often between the third and sixth decades of life, whereas DCH tends to present earlier, often in childhood or adolescence. There are no known genetic risk factors for CCH, while DCH occurs as part of Sturge–Weber syndrome, a sporadic neurocutaneous disorder characterized by vascular malformations involving the skin, brain, and eye.
The pathophysiology involves proliferation of thin-walled vascular channels within the choroid. These vascular spaces can leak fluid, leading to accumulation of subretinal fluid and serous retinal detachment. Chronic leakage may result in retinal pigment epithelium (RPE) changes, photoreceptor damage, and eventual visual impairment.
Clinically, patients often present with painless visual loss or metamorphopsia. On examination, circumscribed choroidal hemangioma appears as a unilateral, round or oval, orange-red elevated lesion located posterior to the equator. It is frequently associated with serous retinal detachment and overlying retinal changes such as edema or RPE alterations. Diffuse choroidal hemangioma, in contrast, presents as widespread thickening of the choroid, giving a characteristic “tomato ketchup” appearance of the fundus. In these cases, other ocular and systemic features of Sturge–Weber syndrome—such as episcleral vessel dilation and glaucoma—are often present.
Diagnostic evaluation includes multimodal imaging. Fluorescein angiography typically shows early hyperfluorescence with progressive leakage. Indocyanine green angiography demonstrates early intense hyperfluorescence followed by a characteristic “washout” pattern in later phases. Ultrasonography reveals a dome-shaped lesion with high internal reflectivity, similar to normal choroidal tissue. Optical coherence tomography is useful in detecting associated retinal changes such as subretinal fluid or macular edema and is particularly helpful for monitoring treatment response.
Management depends on symptoms and the risk to vision. Asymptomatic lesions without vision-threatening complications can be observed. Treatment is indicated when there is subretinal fluid or involvement of the macula. Photodynamic therapy (PDT) with verteporfin is the treatment of choice for circumscribed lesions and is also effective in diffuse cases. Other options include external beam radiotherapy and plaque radiotherapy, particularly in cases not amenable to PDT. Laser photocoagulation and transpupillary thermotherapy are less commonly used due to higher risks of tissue damage.
Prognosis depends largely on the duration and extent of retinal involvement. Early treatment of vision-threatening lesions improves outcomes, while delayed treatment may result in permanent photoreceptor and RPE damage. Complications include persistent serous retinal detachment and, in advanced cases, neovascular glaucoma, which can lead to severe vision loss and may require enucleation. Regular follow-up is essential to monitor for recurrence or progression.
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