MANAGEMENT OF PAPILLOPHLEBITIS ASSOCIATED CYSTOID MACULAR EDEMA WITH INTRAVITREAL TRIAMCINOLONE ACETONIDE: A CASE REPORT Yo-Chen Chang, MD, Wen-Chuan Wu, MD
The authors report a 50-year-old man with papillophlebitis and associated cystoid macular edema in his left eye treated with intravitreal triamcinolone acetonide. His best-corrected visual acuity in the left eye rapidly improved from 20/100 preoperatively to 20/40 7 days postoperation. However, in his left eye, cystoid macular edema recurred 6 months later. Then he accepted a second intravitreal injection of triamcinolone acetonide.
From the Department of Ophthalmology, Kaohsiung Medical University, Hsiaokang Hospital, Kaohsiung, Taiwan.
eye showed hyperemic disk edema, venous engorgement and tortuosity, and moderate flame-shaped hemorrhage in the posterior pole (Figure 1A). No abnormalities were found in the right eye. Fluorescein angiography (FA) of the left eye showed cystic spaces in the macula, marked fluorescence of the disk, and no obvious retinal ischemia or neovascularization (Figure 1C). Systemic and hematologic examination including complete blood count, erythrocyte sedimentation rate, C-reactive protein, clotting screen, serum cholesterol and triglycerides, chest x-ray, and EKG were negative for systemic vasculitic disease. The patient was observed for 2 months, however, no angiographic or clinical improvement was observed. Then he was treated with an intravitreal injection of triamcinolone acetonide (40 mg/mL Kenacort-A; Bristol-Myers Squibb Co.). Topical 0.5% proparacaine hydrochloride (Alcaine; Alcon) was used for anesthesia. An injection of 4 mg (0.1 mL) triamcinolone acetonide was administered through the temporal upper pars plana, 4 mm from the corneal limbus. One week post injection, BCVA had improved to 20/40 and the IOP was 16 mmHg in the left eye. Funduscopic examination showed a dramatic reduction in retinal venous tortuosity and dilatation (Figure 1B). Fluorescein angiography showed almost complete resolution of optic disk edema and CME (Figure 1D). The BCVA at 3 months postinjection was 20/30 and the IOP was 16 mmHg in the left eye. Funduscopic examination showed very mild retinal venous dilatation and tortuosity. He accepted regular follow-up with intervals of 4 weeks and the fundus condition in his left eye was stable. However, 6 months after the injection, he had blurred vision in his left eye. The BCVA dropped from 20/30 to 20/100. Ocular coherence tomography (OCT-3, Carl Zeiss Meditec, Inc., Dublin, CA) showed recurrent CME in his left eye (Figure 2A). Then his left eye was treated again with an intravitreal injection of triamcinolone acetonide (4 mg). Two weeks post injection, BCVA had improved to 20/25 and the IOP was 17 mmHg in the left eye. OCT showed a dramatic resolution of CME in his left eye (Figure 2B). The BCVA of his left eye 1 month
P
apillophlebitis, first nominated by Lonn and Hoyt in 1966,1 is an uncommon ophthalmologic condition of uncertain pathogenesis. Most patients are healthy adults and complain of visual blurring, photopsia, or transient visual obscurations. Fundus examination demonstrates marked retinal venous engorgement in association with hyperemic disk edema and a variable amount of retinal hemorrhage. Fluorescein angiography typically shows marked venous staining, leakage and tortuosity, late staining, and leakage from the optic disk. Despite the potential for visual loss in affected eyes, there is no proven treatment for papillophlebitis and associated cystoid macular edema (CME).2 We report a patient with papillophlebitis and associated CME treated with intravitreal triamcinolone acetonide. Case Report A 50-year-old otherwise healthy man was referred to our department with a 1-week history of acute blurred vision in his left eye. His medical and ophthalmic history was unremarkable. On examination, best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/100 in the left. Intraocular pressures (IOP) were 16 and 15 mmHg, respectively. Anterior segment examination showed no evidence of neovascularization. Dilated funduscopy of the left Reprint requests: Wen-Chuan Wu, MD, Department of Ophthalmology, Kaohsiung Medical University, No.100, Zihyou 1st Rd, Kaohsiung, Taiwan, 807; e-mail: [email protected]
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INTRAVITREAL TRIAMCINOLONE FOR PAPILLOPHLEBITIS ASSOCIATED CYSTOID MACULAR EDEMA
15
Fig. 1. A, Pretreatment funduscopy of the left eye shows congested disk edema, tortuosity and dilatation of retinal vein, flame-shaped hemorrhage, and macular edema. B, One week after intravitreal injection of triamcinolone acetonide, there was a significant reduction in venous dilatation and tortuosity, disk edema, and flame-shape hemorrhage. C, Pretreatment fluorescein angiography showed diffuse leakage around the macula and the optic disk. There was no significant retinal ischemia or neovascularization. D, One week after the operation, fluorescein angiography showed minimal leakage around the macula and marked resolution of the disk edema.
postinjection was 20/20 and the IOP was 16 mmHg in the left eye. There was no recurrence of CME noted.
Discussion We describe the rapid anatomic and visual improvement of a patient with papillophlebitis treated with intravitreal triamcinolone acetonide. The improvement was noted by the first week after treatment and remained stable for as long as 6 months. A syndrome of unilateral disk edema, retinal venous engorgement, and a variable amount of retinal hemorrhage in a healthy adult has been termed papillophlebitis.1 This syndrome is basically a subtype of central retinal vein occlusion. On the basis of the
presumed central retinal vein inflammation at the disk in the pathogenesis of papillophlebitis,3 corticosteroids, both systemic and periocular injections, have been used in many of the reported cases,2 but their efficacy is unproven. The triamcinolone acetonide used in this study is an inexpensive and widely available corticosteroid. Intravitreal use was shown to be nontoxic in animal studies.4 Although the mechanism of triamcinolone acetonide inducing resolution of papillophlebitis and associated CME is not well understood, it is probably due to the anti-inflammation character and the ability to reduce the breakdown of the blood-retina barrier.5 According to the literature, the prognosis of papillophlebitis is usually good, but
Fig. 2. A, Preoperative ocular coherence tomography (OCT) of the left eye demonstrates a marked increase in foveal thickness (462 m) and retinal cystic changes consistent with cystoid macular edema. Best-corrected visual acuity was 20/100. B, Two weeks after treatment with intravitreal triamcinolone acetonide, OCT showed marked improvement in cystoid macular edema and restoration of a normal foveal contour (foveal thickness is 160 m). Best-corrected visual acuity was 20/25.
16
RETINAL CASES & BRIEF REPORTSℜ
one-third of cases have final acuity of 20/200 or worse. Most of them were due to CME or macular pigmentary change.2 Intravitreal triamcinolone acetonide has been shown to be safe and effective in treating CME caused by uveitis, diabetic maculopathy, central retinal vein occlusion, and after cataract surgery. However, the effects may be transient. In our case, CME recurred 6 months after first intravitreal triamcinolone acetonide injection. This tendency of CME to recur a few months after intravitreal injection of triamcinolone acetonide has also been described in uveitic CME and in retinal vein occlusion associated CME.6 It has been shown that 4 mg of triamcinolone acetonide injected into the vitreous cavity lasts for approximately 3 months after injection,7 so it is possible that recurrence of macular edema occurs when the intravitreal triamcinolone acetonide is absorbed. The results of the present case report suggest that the intravitreal injection of triamcinolone acetonide may be a therapeutic option to increase visual acuity in patients with papillophlebitis; however, the effects of
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intravitreal triamcinolone acetonide may be transient and repeated intravitreal injection might be necessary. References 1. 2. 3. 4.
5.
6.
7.
Lonn LI, Hoyt WF. Papillophlebitis, a cause of protracted benign optic disc edema. EENT Monthly 1966;45:62–68. Andrew COF, Howard S, Richard M, et al. Central retinal vein occlusion in young adults. Retina 1992;12:3–11. Hayreh SS. Optic disc vasculitis. Br J Ophthalmol 1972;56: 652–676. Mc Cuen BW, Bessler M, Tano Y, et al. The lack of toxicity of intravitreally administered triamcinolone acetonide. Am J Ophthalmol 1981;91:785–789. Wilson CA, Berkowitz BA, Sato Y, et al. Treatment with intravitreal steroid reduces blood-retinal barrier breakdown due to retinal photocoagulation. Arch Ophthalmol 1992;110: 1155–1159. Antcliff RJ, Spalton DJ, Stanford MR, et al. Intravitreal triamcinolone for uveitic cystoid macular edema: an optical coherence tomography study. Ophthalmology 2001;108:765– 772. Beer PM, Bakri SJ, Singh RJ, et al. Intraocular concentration and pharmacokinetics of triamcinolone acetonide after a single intravitreal injection. Ophthalmology 2003;110:681–686.
The authors report a 50-year-old man with papillophlebitis and associated cystoid macular edema in his left eye treated with intravitreal triamcinolone acetonide. His best-corrected visual acuity in the left eye rapidly improved from 20/100 preoperatively to 20/40 7 days postoperation. However, in his left eye, cystoid macular edema recurred 6 months later. Then he accepted a second intravitreal injection of triamcinolone acetonide.
From the Department of Ophthalmology, Kaohsiung Medical University, Hsiaokang Hospital, Kaohsiung, Taiwan.
eye showed hyperemic disk edema, venous engorgement and tortuosity, and moderate flame-shaped hemorrhage in the posterior pole (Figure 1A). No abnormalities were found in the right eye. Fluorescein angiography (FA) of the left eye showed cystic spaces in the macula, marked fluorescence of the disk, and no obvious retinal ischemia or neovascularization (Figure 1C). Systemic and hematologic examination including complete blood count, erythrocyte sedimentation rate, C-reactive protein, clotting screen, serum cholesterol and triglycerides, chest x-ray, and EKG were negative for systemic vasculitic disease. The patient was observed for 2 months, however, no angiographic or clinical improvement was observed. Then he was treated with an intravitreal injection of triamcinolone acetonide (40 mg/mL Kenacort-A; Bristol-Myers Squibb Co.). Topical 0.5% proparacaine hydrochloride (Alcaine; Alcon) was used for anesthesia. An injection of 4 mg (0.1 mL) triamcinolone acetonide was administered through the temporal upper pars plana, 4 mm from the corneal limbus. One week post injection, BCVA had improved to 20/40 and the IOP was 16 mmHg in the left eye. Funduscopic examination showed a dramatic reduction in retinal venous tortuosity and dilatation (Figure 1B). Fluorescein angiography showed almost complete resolution of optic disk edema and CME (Figure 1D). The BCVA at 3 months postinjection was 20/30 and the IOP was 16 mmHg in the left eye. Funduscopic examination showed very mild retinal venous dilatation and tortuosity. He accepted regular follow-up with intervals of 4 weeks and the fundus condition in his left eye was stable. However, 6 months after the injection, he had blurred vision in his left eye. The BCVA dropped from 20/30 to 20/100. Ocular coherence tomography (OCT-3, Carl Zeiss Meditec, Inc., Dublin, CA) showed recurrent CME in his left eye (Figure 2A). Then his left eye was treated again with an intravitreal injection of triamcinolone acetonide (4 mg). Two weeks post injection, BCVA had improved to 20/25 and the IOP was 17 mmHg in the left eye. OCT showed a dramatic resolution of CME in his left eye (Figure 2B). The BCVA of his left eye 1 month
P
apillophlebitis, first nominated by Lonn and Hoyt in 1966,1 is an uncommon ophthalmologic condition of uncertain pathogenesis. Most patients are healthy adults and complain of visual blurring, photopsia, or transient visual obscurations. Fundus examination demonstrates marked retinal venous engorgement in association with hyperemic disk edema and a variable amount of retinal hemorrhage. Fluorescein angiography typically shows marked venous staining, leakage and tortuosity, late staining, and leakage from the optic disk. Despite the potential for visual loss in affected eyes, there is no proven treatment for papillophlebitis and associated cystoid macular edema (CME).2 We report a patient with papillophlebitis and associated CME treated with intravitreal triamcinolone acetonide. Case Report A 50-year-old otherwise healthy man was referred to our department with a 1-week history of acute blurred vision in his left eye. His medical and ophthalmic history was unremarkable. On examination, best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/100 in the left. Intraocular pressures (IOP) were 16 and 15 mmHg, respectively. Anterior segment examination showed no evidence of neovascularization. Dilated funduscopy of the left Reprint requests: Wen-Chuan Wu, MD, Department of Ophthalmology, Kaohsiung Medical University, No.100, Zihyou 1st Rd, Kaohsiung, Taiwan, 807; e-mail: [email protected]
14
INTRAVITREAL TRIAMCINOLONE FOR PAPILLOPHLEBITIS ASSOCIATED CYSTOID MACULAR EDEMA
15
Fig. 1. A, Pretreatment funduscopy of the left eye shows congested disk edema, tortuosity and dilatation of retinal vein, flame-shaped hemorrhage, and macular edema. B, One week after intravitreal injection of triamcinolone acetonide, there was a significant reduction in venous dilatation and tortuosity, disk edema, and flame-shape hemorrhage. C, Pretreatment fluorescein angiography showed diffuse leakage around the macula and the optic disk. There was no significant retinal ischemia or neovascularization. D, One week after the operation, fluorescein angiography showed minimal leakage around the macula and marked resolution of the disk edema.
postinjection was 20/20 and the IOP was 16 mmHg in the left eye. There was no recurrence of CME noted.
Discussion We describe the rapid anatomic and visual improvement of a patient with papillophlebitis treated with intravitreal triamcinolone acetonide. The improvement was noted by the first week after treatment and remained stable for as long as 6 months. A syndrome of unilateral disk edema, retinal venous engorgement, and a variable amount of retinal hemorrhage in a healthy adult has been termed papillophlebitis.1 This syndrome is basically a subtype of central retinal vein occlusion. On the basis of the
presumed central retinal vein inflammation at the disk in the pathogenesis of papillophlebitis,3 corticosteroids, both systemic and periocular injections, have been used in many of the reported cases,2 but their efficacy is unproven. The triamcinolone acetonide used in this study is an inexpensive and widely available corticosteroid. Intravitreal use was shown to be nontoxic in animal studies.4 Although the mechanism of triamcinolone acetonide inducing resolution of papillophlebitis and associated CME is not well understood, it is probably due to the anti-inflammation character and the ability to reduce the breakdown of the blood-retina barrier.5 According to the literature, the prognosis of papillophlebitis is usually good, but
Fig. 2. A, Preoperative ocular coherence tomography (OCT) of the left eye demonstrates a marked increase in foveal thickness (462 m) and retinal cystic changes consistent with cystoid macular edema. Best-corrected visual acuity was 20/100. B, Two weeks after treatment with intravitreal triamcinolone acetonide, OCT showed marked improvement in cystoid macular edema and restoration of a normal foveal contour (foveal thickness is 160 m). Best-corrected visual acuity was 20/25.
16
RETINAL CASES & BRIEF REPORTSℜ
one-third of cases have final acuity of 20/200 or worse. Most of them were due to CME or macular pigmentary change.2 Intravitreal triamcinolone acetonide has been shown to be safe and effective in treating CME caused by uveitis, diabetic maculopathy, central retinal vein occlusion, and after cataract surgery. However, the effects may be transient. In our case, CME recurred 6 months after first intravitreal triamcinolone acetonide injection. This tendency of CME to recur a few months after intravitreal injection of triamcinolone acetonide has also been described in uveitic CME and in retinal vein occlusion associated CME.6 It has been shown that 4 mg of triamcinolone acetonide injected into the vitreous cavity lasts for approximately 3 months after injection,7 so it is possible that recurrence of macular edema occurs when the intravitreal triamcinolone acetonide is absorbed. The results of the present case report suggest that the intravitreal injection of triamcinolone acetonide may be a therapeutic option to increase visual acuity in patients with papillophlebitis; however, the effects of
●
2007
●
VOLUME 1
●
NUMBER 1
intravitreal triamcinolone acetonide may be transient and repeated intravitreal injection might be necessary. References 1. 2. 3. 4.
5.
6.
7.
Lonn LI, Hoyt WF. Papillophlebitis, a cause of protracted benign optic disc edema. EENT Monthly 1966;45:62–68. Andrew COF, Howard S, Richard M, et al. Central retinal vein occlusion in young adults. Retina 1992;12:3–11. Hayreh SS. Optic disc vasculitis. Br J Ophthalmol 1972;56: 652–676. Mc Cuen BW, Bessler M, Tano Y, et al. The lack of toxicity of intravitreally administered triamcinolone acetonide. Am J Ophthalmol 1981;91:785–789. Wilson CA, Berkowitz BA, Sato Y, et al. Treatment with intravitreal steroid reduces blood-retinal barrier breakdown due to retinal photocoagulation. Arch Ophthalmol 1992;110: 1155–1159. Antcliff RJ, Spalton DJ, Stanford MR, et al. Intravitreal triamcinolone for uveitic cystoid macular edema: an optical coherence tomography study. Ophthalmology 2001;108:765– 772. Beer PM, Bakri SJ, Singh RJ, et al. Intraocular concentration and pharmacokinetics of triamcinolone acetonide after a single intravitreal injection. Ophthalmology 2003;110:681–686.
