Customize your JAMA Network experience by selecting one or more topics from the list below.
The diffuse choroidal hemangioma is an uncommon congenital vascular tumor associated with the Sturge-Weber syndrome.1 Visual loss is often secondary to nonrhegmatogenous retinal detachments, cystoid degeneration, photoreceptor loss, and fibrous transformation of the proliferated retinal pigment epithelium.1 The use of low-dose external beam irradiation has been reported as a possible treatment for nonrhegmatogenous retinal detachments associated with diffuse choroidal hemangioma.2 We describe a patient with Sturge-Weber syndrome, bilateral diffuse choroidal hemangiomas, and nonrhegmatogenous serous retinal detachments treated with low-dose external beam irradiation.
Report of a Case
An 8-year-old girl with bilateral facial nevus flammeus was examined at the Bascom Palmer Eye Institute, Miami, Fla, for progressive visual loss in her left eye of 8 days' duration. Six months previously, she had developed an exudative detachment in her right eye with decreased visual acuity to the 20/400 level.
Examination revealed a facial angioma involving the right forehead and eyelid, cheek, and nose and crossing to the nose and upper eyelid of the left side. Visual acuity measured 20/400 OD and could not be improved with refraction. Visual acuity was 20/80 OS and improved to 20/60 with +2.50+0.50×70°. The anterior segments and intraocular pressures were normal. Funduscopic examination of the right eye revealed a diffuse reddish hue and massive choroidal thickening that was most prominent in the posterior pole. A retinal fold was present through the papillomacular bundle with hyperplastic and metaplastic changes in the retinal pigment epithelium surrounding the optic nerve and in the macula. A serous detachment of the retina was present inferiorly with a pocket of fluid overlying the macula. In the left eye, an even greater degree of choroidal thickening was associated with a bullous detachment of the inferior retina and a shallow detachment of the macular region. No significant retinal pigment epithelial changes were noted ophthalmoscopically.
B-scan ultrasonography measured the choroidal thickness as 8 mm in the macula of the right eye and 6 mm in the left eye. A simultaneous A-scan revealed the tumor to be of high reflectivity. Fluorescein angiography showed rapid filling and marked extravasation of fluorescein dye from the retinal and choroidal vessels. Staining of the peripapillary retinal pigment epithelial hyperplastic changes was noted in the right eye, while in the left eye there was a C-shaped area of fluorescein staining at the level of the retinal pigment epithelium extending temporally from the inferior portion of the macula.
Because of the extensive diffuse nature of the choroidal hemangiomas, low-dose external beam radiation therapy was recommended. The patient underwent an uncomplicated course of external beam radiation therapy to both eyes to a total dose of 20 Gy (10 fractions of 200 Gy over 10 days).
Follow-up 3 months after radiation therapy revealed complete resolution of the exudative detachments in each eye. The choroidal thickening appeared decreased with moderate retinal pigment epithelial mottling of the posterior pole in the right eye. Visual acuity had improved to 20/50 OS and remained stable in the right eye. B-scan ultrasonography revealed a choroidal thickening of 3.0 mm OU (Figure 1).
A, Horizontal axial B-scan echogram of the right eye shows a diffuse, highly elevated choroidal lesion with bullous retinal detachment. B, Horizontal axial B-scan echogram of the left eye shows a diffuse, highly elevated choroidal lesion with very shallow retinal detachment. C and D, Follow-up B-scan echograms of the right (C) and left (D) eyes show markedly decreased elevation of choroidal hemangiomas with resolution of retinal detachments.
At 11 months after radiation therapy, visual acuity measured 20/300 OD and 20/40+2 OS. Intraocular pressures were normal. The visual field in the left eye was full with enlargement of the blind spot temporally, while in the right eye there was superior constriction. There was no evidence of irradiation complications. There was no recurrence of serous exudation in either eye. The choroid measured less than 2 mm in each eye by ultrasonographic examination.
Twenty months after radiation therapy, visual acuity was 20/200 OD and 20/30+1 OS. Lenses remained clear and there was no evidence of recurrence of the choroidal thickening or serous exudation.
To our knowledge, this is only the second case of bilateral diffuse choroidal hemangiomas associated with bullous serous retinal detachments to be treated by low-dose external beam irradiation. Among the 4 patients desribed by Scott et al2 treated with low-dose ocular irradiation for diffuse choroidal hemangioma was 1 patient who had evidence of bilateral disease. However, he had been previously treated on multiple occasions with xenon arc photocoagulation prior to undergoing radiation therapy when his second eye became affected.
Since the initial report of Scott et al, 2 additional reports have described successful treatment of both unilateral diffuse choroidal hemangiomas associated with the Sturge-Weber syndrome and circumscribed choroidal hemangiomas with the use of lens-sparing low-dose external beam irradiation.3,4 Schilling et al3 treated 15 unilateral cases of diffuse choroidal hemangiomas with complete resolution of subretinal fluid in all cases, tumor mass shrinkage in 5 cases, and the improvement of vision in 7 eyes. Madreperla et al4 report their experience with 5 unilateral cases in which subretinal fluid was resorbed in all patients by 1 year after radiation therapy. They report improvement of the intraocular pressure after irradiation in 1 patient who had an elevated intraocular pressure.4
The occurence of bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus is rare.5 The treatment of eyes with diffuse choroidal hemangioma that develop bullous nonrhegmatogenous detachments is very difficult. These lesions generally do not respond well to the modalities commonly used to treat circumscribed choroidal hemangiomas.6 Another less common form of treatment that appears effective in the treatment of choroidal hemangiomas associated with exudative detachment is brachytherapy.4,7 Zografos et al7 report the successful treatment of 2 diffuse choroidal hemangiomas in which a 15-mm radioactive plaque was placed "in the presumed center of the tumor." Although the authors report successful resolution of associated serous detachment in each case, they conclude that this technique cannot be recommended in patients with diffuse choroidal hemangiomas.7
The treatment of exudative detachments secondary to diffuse choroidal hemangiomas with low-dose fractionated ocular irradiation appears promising. In this case, such treatment was safe and effective for the short term. The potential complications include cataract formation, keratitis sicca, radiation papillopathy, and radiation retinopathy.8 With follow-up of 20 months, our patient showed an impressive resolution of the massive thickening of the choroid (8 mm to 3 mm), resolution of the associated serous detachments without evidence of recurrence, and an improvement of the vision in both eyes. We can only speculate whether earlier treatment of the right eye may have resulted in preservation of better vision in that eye. At this time she shows no complications attributable to the radiation therapy, although longer term follow-up may reveal such complications.
Reprints: Justin L.Gottlieb, MD, Department of Ophthalmology, University of Wisconsin, 2870 University Ave, Suite 206, Madison, WI 53705 (e-mail: Gottlieb@eyesee.ophth.wisc.edu).
This research was supported in part by an American Cancer Society award, Tampa, Fla (Dr Murray), Fight for Sight, Schaumburg, Ill (Dr Murray), and a Stanley Glaser Award, Miami, Fla (Dr Murray).
Gottlieb JL, Murray TG, Gass JDM. Low-Dose External Beam Irradiation for Bilateral Diffuse Choroidal Hemangioma. Arch Ophthalmol. 1998;116(6):815–817. doi:
Coronavirus Resource Center