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Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive amblyopia therapy with low-dose external beam irradiation
Visual Rehabilitation in a Child with Diffuse Choroidal Hemangioma by Using Aggressive Amblyopia Therapy with Low-Dose External Beam Irradiation Eric A. Packwood, MD, Susan A. Havertape, DBO, CO, Oscar A. Cruz, MD, and Eric S. Mann, MD, PhD
Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma.1-3 Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy.
CASE REPORT A 7-year-old boy with Sturge-Weber syndrome was examined at the Saint Louis University Eye Institute because of progressive visual loss in the right eye of 3 months’ duration. He had a visual acuity of 20/300 and best corrected visual acuity of 20/80 OD and 20/20 OS with a cycloplegic From the Department of Ophthalmology, Saint Louis University School of Medicine, St Louis, Missouri. Submitted December 10, 1999. Revision accepted March 3, 2000. Reprint requests: Eric S. Mann, MD, PhD, Saint Louis University Eye Institute, 1755 S Grand Blvd, St Louis, MO 63104 (e-mail: [email protected]). JAAPOS 2000;4:321-2. Copyright © 2000 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2000 $12.00 + 0 75/1/106958 doi:10.1067/mpa.2000.106958
Journal of AAPOS
refraction of +5.00 + 1.00 × 90 OD and plano + 0.50 × 180 OS. Tonometry revealed intraocular pressure of 20 mm Hg OD and 12 mm Hg OS. The patient had a nevus flammeus of the right side of the face. Optic nerve examination revealed cup-to-disc asymmetry between the eyes of 0.60 OD and 0.5 OS. Fundus examination of the right eye revealed a red, elevated, diffuse choroidal lesion with choroidal thickening within the macula and toward the nasal side to the optic nerve (Figure 1). Clinical and ultrasonographic features were consistent with a diffuse choroidal hemangioma. No associated exudative subretinal fluid was present. Glasses were prescribed for full-time wear and amblyopia therapy with patching of the left eye was begun, initially for 4 to 6 hours a day and then increased to all but 1 day a week. Administration of timolol gel-forming solution 0.5% (Timoptic-XE; Merck, West Point, Pa) OD once a day was also recommended. Vision improved to 20/40 during the following year. After stable 20/40 visual acuity was achieved, follow-up examination revealed an inferior exudative retinal detachment not involving the macula in the right eye (Figure 2), with vision decreasing to 20/50. Over the next 6 months, the exudative retinal detachment became extensive and bullous but limited inferiorly, with vision decreasing to 20/200 despite continued part-time patching of the left eye. With subretinal fluid now threatening the macula, low-dose external beam irradiation with a total dose of 1000 cGy, divided in 5 fractions of 200 cGy each for more than 10 days, was performed with use of a lens-sparing technique. Five months after radiotherapy, the exudative retinal detachment resolved and the tumor size decreased, with vision improving to 20/60. Vision improved to 20/30 with further aggressive amblyopia therapy during the next year. The patient experienced reversal of right optic nerve cupping, with a cup-to-disc of 0.5 and intraocular pressure less than 20 mm Hg while receiving Timoptic-XE 0.5% OD daily. Two and a half years after external beam radiation and amblyopia therapy, vision remains 20/30 OD with no evidence of ocular complications from radiation treatment. Patching was discontinued once the patient was 10 years old. Exudative retinal detachment or choroidal thickening has not recurred. October 2000
321
Journal of AAPOS Volume 4 Number 5 October 2000
322 Packwood et al
FIG 1. Fundus photograph of the right eye revealing diffuse choroidal hemangioma with hyperpigmentation of the retinal pigment epithelium nasal to the optic nerve but not in the macula.
FIG 2. B-scan echogram of the right eye shows a diffuse, highly elevated choroidal lesion with inferior bullous retinal detachment.
DISCUSSION
ing the macula unequivocally accounts for the visual loss, further investigation is needed to exclude a nonorganic component to the amblyopia. The clinician must be cautious not to assume that an amblyopic eye cannot be rehabilitated because of the coincidental presence of an organic abnormality.4
Previous reports of low-dose radiotherapy to eyes with diffuse choroidal hemangioma demonstrate resolution of serous retinal detachments, but vision often remains poor, limited to 20/200 or worse, in the majority of cases.1-3 Children in the amblyopia-prone years could be affected, suggesting that nonorganic amblyopia may contribute to visual loss. To our knowledge, this is the first case demonstrating visual rehabilitation from concurrent external beam radiation and amblyopia therapy to an eye with diffuse choroidal hemangioma in Sturge-Weber syndrome. Our patient clearly demonstrated anisometropia in part caused by the choroidal hemangioma, which created anisometropic amblyopia. Amblyopia is a potential element of visual loss with this clinical entity. This case emphasizes the importance of the team approach to treatment of vision disorders in children. Unless the organic abnormality involv-
References 1. Augsburger JA, Freire J, Brady LW. Radiation therapy for choroidal and retinal hemangiomas. Front Radiat Ther Oncol 1997; 30:265-80. 2. Madreperla SA, Hungerford JL, Plowman PN, Laganowski HC, Gregory PT. Choroidal hemangiomas: visual and anatomic results of treatment by photocoagulation or radiation. Ophthalmology 1997; 104:1773-9. 3. Scott TA, Augsburger JJ, Brady LW, Hernandez C, Woodleigh R. Retina 1991;11:389-93. 4. Kushner BJ. Functional amblyopia associated with organic ocular disease. Am J Ophthalmol 1981;91:39-45.
An Eye on the Arts – The Arts on the Eye
My father, Bud Wharton, was an eye doctor. We lived on the outskirts of Jackson proper, in a scrubby settlement called Pearl. Dad saw patients in the back room of the house, which had metal cabinets for his nested lenses that tinkled like glass wind chimes when you opened and shut the drawers. Up front, we ran a store. We had to, because in hard times everyone’s eyes get better or at least good enough. I don’t think Dad ever forgave me, later on, for becoming a Free Will Baptist. He failed to see why anyone would need more bluster and testimony about God’s Plan than what he found, for example, within the fine-veined world of an eyeball. —Barbara Kingsolver (from The Poisonwood Bible)
Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma.1-3 Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy.
CASE REPORT A 7-year-old boy with Sturge-Weber syndrome was examined at the Saint Louis University Eye Institute because of progressive visual loss in the right eye of 3 months’ duration. He had a visual acuity of 20/300 and best corrected visual acuity of 20/80 OD and 20/20 OS with a cycloplegic From the Department of Ophthalmology, Saint Louis University School of Medicine, St Louis, Missouri. Submitted December 10, 1999. Revision accepted March 3, 2000. Reprint requests: Eric S. Mann, MD, PhD, Saint Louis University Eye Institute, 1755 S Grand Blvd, St Louis, MO 63104 (e-mail: [email protected]). JAAPOS 2000;4:321-2. Copyright © 2000 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2000 $12.00 + 0 75/1/106958 doi:10.1067/mpa.2000.106958
Journal of AAPOS
refraction of +5.00 + 1.00 × 90 OD and plano + 0.50 × 180 OS. Tonometry revealed intraocular pressure of 20 mm Hg OD and 12 mm Hg OS. The patient had a nevus flammeus of the right side of the face. Optic nerve examination revealed cup-to-disc asymmetry between the eyes of 0.60 OD and 0.5 OS. Fundus examination of the right eye revealed a red, elevated, diffuse choroidal lesion with choroidal thickening within the macula and toward the nasal side to the optic nerve (Figure 1). Clinical and ultrasonographic features were consistent with a diffuse choroidal hemangioma. No associated exudative subretinal fluid was present. Glasses were prescribed for full-time wear and amblyopia therapy with patching of the left eye was begun, initially for 4 to 6 hours a day and then increased to all but 1 day a week. Administration of timolol gel-forming solution 0.5% (Timoptic-XE; Merck, West Point, Pa) OD once a day was also recommended. Vision improved to 20/40 during the following year. After stable 20/40 visual acuity was achieved, follow-up examination revealed an inferior exudative retinal detachment not involving the macula in the right eye (Figure 2), with vision decreasing to 20/50. Over the next 6 months, the exudative retinal detachment became extensive and bullous but limited inferiorly, with vision decreasing to 20/200 despite continued part-time patching of the left eye. With subretinal fluid now threatening the macula, low-dose external beam irradiation with a total dose of 1000 cGy, divided in 5 fractions of 200 cGy each for more than 10 days, was performed with use of a lens-sparing technique. Five months after radiotherapy, the exudative retinal detachment resolved and the tumor size decreased, with vision improving to 20/60. Vision improved to 20/30 with further aggressive amblyopia therapy during the next year. The patient experienced reversal of right optic nerve cupping, with a cup-to-disc of 0.5 and intraocular pressure less than 20 mm Hg while receiving Timoptic-XE 0.5% OD daily. Two and a half years after external beam radiation and amblyopia therapy, vision remains 20/30 OD with no evidence of ocular complications from radiation treatment. Patching was discontinued once the patient was 10 years old. Exudative retinal detachment or choroidal thickening has not recurred. October 2000
321
Journal of AAPOS Volume 4 Number 5 October 2000
322 Packwood et al
FIG 1. Fundus photograph of the right eye revealing diffuse choroidal hemangioma with hyperpigmentation of the retinal pigment epithelium nasal to the optic nerve but not in the macula.
FIG 2. B-scan echogram of the right eye shows a diffuse, highly elevated choroidal lesion with inferior bullous retinal detachment.
DISCUSSION
ing the macula unequivocally accounts for the visual loss, further investigation is needed to exclude a nonorganic component to the amblyopia. The clinician must be cautious not to assume that an amblyopic eye cannot be rehabilitated because of the coincidental presence of an organic abnormality.4
Previous reports of low-dose radiotherapy to eyes with diffuse choroidal hemangioma demonstrate resolution of serous retinal detachments, but vision often remains poor, limited to 20/200 or worse, in the majority of cases.1-3 Children in the amblyopia-prone years could be affected, suggesting that nonorganic amblyopia may contribute to visual loss. To our knowledge, this is the first case demonstrating visual rehabilitation from concurrent external beam radiation and amblyopia therapy to an eye with diffuse choroidal hemangioma in Sturge-Weber syndrome. Our patient clearly demonstrated anisometropia in part caused by the choroidal hemangioma, which created anisometropic amblyopia. Amblyopia is a potential element of visual loss with this clinical entity. This case emphasizes the importance of the team approach to treatment of vision disorders in children. Unless the organic abnormality involv-
References 1. Augsburger JA, Freire J, Brady LW. Radiation therapy for choroidal and retinal hemangiomas. Front Radiat Ther Oncol 1997; 30:265-80. 2. Madreperla SA, Hungerford JL, Plowman PN, Laganowski HC, Gregory PT. Choroidal hemangiomas: visual and anatomic results of treatment by photocoagulation or radiation. Ophthalmology 1997; 104:1773-9. 3. Scott TA, Augsburger JJ, Brady LW, Hernandez C, Woodleigh R. Retina 1991;11:389-93. 4. Kushner BJ. Functional amblyopia associated with organic ocular disease. Am J Ophthalmol 1981;91:39-45.
An Eye on the Arts – The Arts on the Eye
My father, Bud Wharton, was an eye doctor. We lived on the outskirts of Jackson proper, in a scrubby settlement called Pearl. Dad saw patients in the back room of the house, which had metal cabinets for his nested lenses that tinkled like glass wind chimes when you opened and shut the drawers. Up front, we ran a store. We had to, because in hard times everyone’s eyes get better or at least good enough. I don’t think Dad ever forgave me, later on, for becoming a Free Will Baptist. He failed to see why anyone would need more bluster and testimony about God’s Plan than what he found, for example, within the fine-veined world of an eyeball. —Barbara Kingsolver (from The Poisonwood Bible)