The outlook is getting brighter for most people with circumscribed choroidal hemangiomas. As recently as the early 1970s these benign vascular tumors were often misdiagnosed as malignant and managed with enucleation.
Photodynamic therapy with verteporfin (Visudyne), however, has effectively revolutionized the treatment of circumscribed choroidal hemangiomas, according to James J. Augsburger, MD, professor and chairman of ophthalmology at the University of Cincinnati. The procedure can flatten or eliminate these once difficult-to-manage tumors while preserving or improving vision in most patients. Moreover, it’s an in-office procedure requiring only local anesthesia.
“It’s the treatment of choice for circumscribed choroidal hemangiomas,” said Arun D. Singh, MD, associate professor of ophthalmology and director of ophthalmic oncology at The Cleveland Clinic. PDT offers site-specific tumor destruction without damaging the overlying retina or retinal vasculature. Dr. Singh cautioned, however, that PDT should not be used to treat diffuse choroidal hemangiomas, such as those found in Sturge-Weber syndrome (encephalotrigeminal angiomatosis), because it can cause complications.
Signs, Symptoms and Diagnosis
“Typically it’s diagnosed in patients in their 30s to early 50s with some recently detected progressive visual blurring in the eye,” said Dr. Augsburger, who is also director of ocular oncology and the diagnostic ultrasonography service at the University of Cincinnati Medical Center. “Sometimes the initial finding is a unilateral hyperopic shift.”
Through the ophthalmoscope, circumscribed choroidal hemangiomas appear as orange or reddish-orange masses with indistinct margins that meld with the surrounding choroidal tissue. Some have areas of increased pigmentation similar to what is seen with choroidal melanomas.
The tumors typically are located in the posterior pole and tend to be 6 mm or less in thickness. Intrinsic vessels or feeder vessels are not apparent. Most circumscribed choroidal hemangiomas are asymptomatic and go undetected unless visual symptoms develop. However, occasionally a very thin circumscribed choroidal hemangioma will be detected on a routine eye exam.
Symptomatic cases usually involve an exudative retinal detachment related to accumulated subretinal fluid. Visual symptoms include reduced vision, photopsia and metamorphopsia.
Dx clue: early, intense hyperfluorescence. The differential diagnosis for circumscribed choroidal hemangioma includes amelanotic choroidal melanoma, choroidal osteoma, metastasis to the choroid, choroidal granuloma, posterior scleritis and atypical serous retinopathy.
The diagnosis is established with ultrasonography and angiography. According to Dr. Singh, B-scan ultrasonography reveals a smooth, dome-shaped mass with high internal reflectivity on A-scan.1 On angiography, these masses demonstrate very early hyperfluorescence. In the choroidal filling phase, fluorescein angiography shows a tumor with a delicate lacelike network of intrinsic vessels.
Indocyanine green dye angiography, however, provides the best view of the intrinsic vascular pattern. Hyperfluorescence intensifies rapidly, revealing the pattern within 30 seconds of dye injection. It peaks at 3 to 4 minutes and in the late phase—20-plus minutes postinjection—creates a washed-out appearance as the dye dissipates. Optical coherence tomography can be useful in assessing overlying retinal changes.
Treat Me With PDT
|Left eye fundus with circumscribed choroidal hemangioma. |
When and How to Treat
“Not all patients with circumscribed choroidal hemangioma need to be treated,” said Dr. Singh. He noted that if the hemangioma does not threaten vision—or if the patient’s vision is completely gone—nothing will be gained by treating the tumor. The decision to treat depends on the severity of symptoms, degree of vision loss and the potential for recovery of vision.
Dr. Augsburger agreed. “If there is no subretinal fluid, deterioration of vision or thickness in the macula, there’s pretty good evidence that you can just leave them alone.” He informs these patients they have a benign tumor and advises them to return promptly if they develop any changes in vision.
Among patients who do require treatment, Dr. Augsburger said the ideal candidate is someone with a recent onset of visual symptoms and whose tumor does not lie directly beneath the fovea. The ideal tumor is less than 7 to 8 mm in diameter and less than 3 to 4 mm in thickness. “Larger ones are more likely to have retinal degeneration overlying the tumor and perhaps more extensive subretinal fluid that has been there for a longer period of time,” he added.
Tapping into PDT. The successful use of PDT to treat macular degeneration spurred its off-label application to symptomatic circumscribed choroidal hemangiomas. Dr. Augsburger was not convinced that the macular degeneration data could be extrapolated to these hemangiomas. “These are relatively mature blood vessels that have been enlarging slowly,” he noted. “With macular degeneration, it’s clearly new vessels that are growing into an area not normally occupied by blood vessels. They are different in caliber and in characteristics. I thought that if PDT worked for new choroidal vessels, it probably would not work in choroidal hemangiomas.”
Despite those early doubts, Dr. Augsburger has been impressed with PDT’s efficacy and durability. The results in terms of tumor regression have been excellent, he said, especially with relatively thinner lesions. “Many become virtually no thicker than the normal choroid. Some of the thicker tumors don’t flatten out completely, but those still have very good outcomes. The subretinal fluid disappears in almost all cases, and it does so in a matter of weeks. Most patients do very well visually unless their vision was really bad before we treated them.” Occasionally a patient will require a second treatment. But third treatments are rare.
The Rocky Road to PDT
PHOTOCOAGULATION. Very early on, circumscribed choroidal hemangiomas were treated with solar photocoagulators. These crude devices were perched on hospital rooftops, where they captured sunlight through the lens and directed it into the eye. They could be used only on clear days and soon gave way to xenon arc photocoagulators. These could destroy the tumor, but they often destroyed vision as well.
LASERS. A variety of laser procedures followed, including one that used a light polka dot or scatter pattern over the surface. This was intended to eliminate the retinal fluid but not destroy the tumor. But the tumor continued to age, and a large percentage of patients developed a blister of subretinal fluid.
RADIOTHERAPY. Low-dose radiation delivered by linear accelerator, radioactive plaque, or proton beam has been used to shrink hemangiomas and eliminate subretinal fluid. Radiotherapy is especially helpful for thicker tumors. The main drawback is that when tumors extend into the macula or are located close to the optic disc, even relatively low doses of radiation can sometimes damage those structures.
Large, single spot. Dr. Singh uses the standard TAP—Treatment of Age-Related Macular Degeneration with Photodynamic Therapy—study protocol when treating circumscribed choroidal hemangiomas. Verteporfin 6 mg/m2 is given as an intravenous infusion over 10 minutes. Five minutes after the infusion is complete the tumor is treated with a 690-nm diode laser at an intensity of 600 mW/cm2 for 83 seconds (50 J/cm2).
“One should use only a single large spot instead of multiple overlapping spots,” Dr. Singh said. “If you use multiple overlapping spots, you’re going to have problems with excessive uptake in certain parts of the hemangioma. That is going to destroy the retinal pigment epithelium and consequently the patient’s vision. You want to avoid the fovea. If the hemangioma comes up to the fovea, then you want to place your beam eccentric so that you’re not treating the fovea directly. Otherwise the retinal pigment epithelium will atrophy in that area and vision will drop.” Spot size is limited by the device used and cannot be expanded. “The spot size goes only so far, and the hemangioma may be bigger than that,” Dr. Singh said. “But that’s OK. You don’t have to treat the whole hemangioma. As long as the fluid disappears and the fovea becomes near normal in appearance, you can stop your treatment.”
1 Singh A. D. and P. K. Kaiser. Clinical Ophthalmic Oncology
(Philadelphia: Saunders-Elsevier, 2007).
___________________________ Drs. Augsburger and Singh report no related financial interests.