Therapeutic modalities for circumscribed choroidal hemangioma. Rev Cubana Oftalmol [online]. , vol, suppl.1, pp. ISSN Keywords: Circumscribed Choroidal Hemangioma; Photodynamic Therapy; de los pacientes con hemangioma coroideo tratados con terapia fotodinámica. Photographer: JEFFERSON R SOUSA – Study Center and Ophthalmological Research Dr. Andre M V Gomes, Institute Dr. Suel Abujamra São.
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Choroidal hemangioma is a rare congenital ocular tumor that can present as either circumscribed or diffuse. Circumscribed choroidal hemangioma CCH typically manifests as a red-orange mass within the posterior pole and appears similar to other ocular conditions, such as choroidal melanoma and choroidal metastasis.
Proper diagnosis is crucial and is aided by the use of ancillary testing. CCH itself is benign but can cause secondary complications such as subretinal fluid accumulation and subsequent retinal detachment. If these conditions should arise, several treatment options are available. En caso de producirse dichas situaciones, existen diversas opciones de tratamiento.
Choroidal hemangioma is a benign vascular tumor. Diffuse choroidal hemangiomas involve more than one quadrant of the choroid and are typically associated with systemic conditions such as Sturge—Weber syndrome. CCH may appear similar to other choroidal tumors, some of which are malignant, such as choroidal melanoma and choroidal metastasis. Treatment options include laser photocoagulation, thermotherapy, radiotherapy, photodynamic therapy PDTand anti-vascular endothelial growth factor VEGF therapy.
A year-old Caucasian male presented to clinic with coroiceo of blurred vision at distance that was worse in his left eye.
His medical history included hypertension, hyperlipidemia, sleep apnea, and post-traumatic stress disorder. His ocular history was unremarkable. Pupils were equal, round, and reactive to light OU, and there was no afferent pupillary defect. Confrontation fields were full, and extra-ocular muscles had full range of motion.
Amsler grid was unremarkable OD and revealed left-sided metamorphopsia OS. Slit lamp examination was unremarkable OU, and intraocular pressures were within normal limits. Dilated fundus exam of the left eye Fig.
An associated 3—4 DD central serous retinal detachment was also noted. Several ancillary tests were performed. OCT revealed a large choroidal mass with overlying subretinal fluid. Fundus photos were also taken. Fundus photo shows a circumscribed choroidal hemangioma superior temporal to the optic nerve in the left eye. The patient was diagnosed with CCH and referred to a retinal specialist. Fluorescein angiography was performed, and it showed hyperflourescence in all stages. The lesion was treated with argon laser photocoagulation over the entire mass.
Dilated fundus exam revealed an improvement in subretinal fluid, but a serous detachment was still present. FA was repeated as well as another session of argon laser photocoagulation. Over the next 15 years, the patient was seen every three to six months depending on the status of the condition.
After three treatments of argon laser photocoagulation, all of the subretinal fluid resolved. The CCH did not shrink in size.
Pigment later developed over the lesion. The subretinal fluid eventually returned 11 years after the last treatment session, but it did not coroiveo the patient’s vision. Therefore, observation was chosen as the treatment at that time. No changes in the amount of subretinal fluid or BVA have been noted at any follow-up visits since.
CCH is a rare vascular tumor. CCH is a very uncommon tumor that is likely to go undiagnosed unless the patient becomes symptomatic. Unlike diffuse hemwngioma hemangiomas, which are associated with phakomatoses such as Sturge—Weber syndrome, CCH has no correlation with systemic bemangioma. The specific cause of the blood vessel he,angioma is unknown.
In other words, it shows slow to no progression in size over time. CCH presents clinically as an orange-red mass in the posterior pole, usually within one to three disk diameters of the macula. The mass itself is not pigmented but overlying pigment can develop over time.
Hemajgioma is a slowly progressive tumor. It can appear to increase in size secondary to subretinal fluid accumulation. Presenting symptoms can range from no symptoms to significant vision loss. In a patient study by Shields et al. Other symptoms can include hemangimoa, floaters, flashes of light, and rarely ocular pain. Many of these symptoms are caused by subretinal fluid accumulation. This occurs secondary to a breakdown of the blood-retinal barrier, allowing fluid leakage that leads to a serous retinal detachment.
Clinically, signs include an orange-red dome-shaped lesion with indistinct borders. Other associated clinical signs can include dilated episcleral vessels, neovascularization of the iris, drusen, RPE hyperplasia, dilated retinal arteries and veins, and retinoschisis. Several posterior segment lesions can resemble CCH. These include central serous chorioretinopathy, choroidal melanoma, choroidal metastasis, posterior nodular scleritis, and non-specific retinal detachment.
The most concerning of these are choroidal melanoma and choroidal metastasis because of their risk of ocular and systemic morbidity and mortality.
Patients with central serous chorioretinopathy are typically middle-aged adults who present with complaints of decreased vision.
Examination reveals an elevated area in the macula, usually with associated subretinal fluid. It is typically the same color as the surrounding retina. This condition is benign and often resolves without treatment, although recurrence is possible. Posterior nodular scleritis can present as a tumor-like mass in the posterior pole. This can be distinguished from CCH because other signs and symptoms of ocular inflammation, such as vitreous cells or anterior scleritis, are usually present.
Choroidal metastases often present as elevated, plateau-shaped lesions. They can be found anywhere in the posterior segment of the eye, including the posterior pole, and are typically a creamy yellow color. Unlike CCH, they can be multifocal or bilateral. Choroidal melanoma is typically a large mushroom-shaped, pigmented lesion that can be found anywhere in the posterior segment of the eye.
The majority of melanomas are darkly pigmented with either a black or gray-green appearance, but amelanotic melanomas also exist. It is important to be able to differentiate between different types of ocular tumors in order to provide appropriate treatment. As mentioned previously, some conditions such as choroidal melanoma require correct diagnosis and prompt treatment to reduce the risk of ocular and systemic morbidity and mortality. Since CCH can mimic several other ocular conditions, additional testing is required for proper diagnosis.
Ultrasonography can be used to measure the internal reflectivity of an ocular mass. High internal reflectivity on A-scan, hemangiomaa acoustically solid on B-scan, indicates that the mass is composed of a variety of different cell types, which causes more disruption of the sound waves. Normal choroidal tissue is made of several different cell types.
Low internal reflectivity on A-scan, or acoustic hollowness on B-scan, is a sign that very similar cells are located within the lesion.
CCH is characterized by high internal reflectivity since it is composed of blood vessels and other cells that are similar to the normal choroidal structure. On the other hand, choroidal metastasis shows similar findings to CCH and is not as easily distinguished from CCH with ultrasonography.
FA allows viewing of the retinal blood vessels and is often used to locate areas of leakage. CCH shows a characteristic pattern on Coroiveo which includes hmeangioma early lacy hyperflourescence that increases through all stages. In contrast, choroidal melanoma and choroidal metastasis show slower and less intense hyperflourescence.
In contrast, choroidal melanoma and metastasis demonstrate slower and less intense filling. MRI can be used to locate lesions within the orbit. Both are hyperintense on T1-weighted images. On T2-weighted images, both are isointense compared to the vitreous and tend to disappear on the image.
If all other testing is inconclusive, a biopsy of the lesion can be performed. This is a more invasive hemmangioma and therefore carries greater risks. It is usually reserved for cases where the diagnosis coroifeo be made with less involved methods. OCT is a newer technology which shows high definition imaging hemngioma the posterior segment of the eye. Clinically, it is used most often to monitor for complications of CCH such as macular edema and serous retinal detachment.
Intraocular Vascular Tumors – EyeWiki
It can also be used to evaluate the efficacy of treatment. CCH is often an asymptomatic lesion. In this case, close observation is the only treatment warranted. Although the lesion itself is benign, it can lead to sight-threatening complications, including macular edema, serous retinal detachment, or clroideo glaucoma.
The most common symptom in patients with CCH is blurred vision secondary to macular edema. If complications do arise, there are several treatment options available. The goal of any treatment is resolution of the macular edema. In the s and s, the traditional treatment for CCH was xenon arc photocoagulation. This was performed over the entire surface of the tumor.
It produced hemanbioma resolution of subretinal fluid but often left residual scarring that could limit the patient’s visual recovery.
Argon laser can also cause scarring of the choroid and retina, however, which can lead to decreased visual acuity. Re-treatments are often necessary as subretinal fluid tends to recur. Photocoagulation can sometimes lead to significant, if not complete, resolution of subretinal fluid but typically has a little to no effect on the tumor size.