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Optic Disk Neovascularization in Juvenile Rheumatoid Arthritis
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AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Landers, Semple, and Morse). Infusion cannula in position showing its relationship to anterior structures. pupillary margin so that the flow of infusion fluid is downward into the vitreous cavity of the aphakic eye and, hence, away from the corneal endothelium (Fig. 2). The infusion cannula can be sewn onto the sclera in a manner similar to that of a standard pars plana infusion cannula. It is thus possible to approach the vitreous base with a minimal chance of the infusion cannula creating new retinal tears. We have used this infusion cannula successfully on several patients who had severe proliferative vitreoretinopathy and in trauma patients who have dense vitreous hemorrhage and preoperative ultrasonic evidence of a retinal detachment.
References 1. De Juan, E., Jr., and McCuen, B. W., II: Management of anterior vitreous traction in proliferative vitreoretinopathy. Retina 4:258,1989. 2. Aaberg, T. M.: Management of anterior and posterior proliferative vitreoretinopathy. XLV Edward Jackson memorial lecture. Am. J. Ophthalmol. 106:519,1988. 3. Charles,S.: Anterior loop traction. In Vitreous Microsurgery, ed. 2. Baltimore, Williams & Wilkins, 1987, p. 137.
Optic Disk Neovascularization in Juvenile Rheumatoid Arthritis H. Christopher Semple, M.D., Maurice B. Landers III, M.D., and Lawrence S. Morse, M.D.
Retinal Service, Department of Ophthalmology, University of California, Davis.
August, 1990
Inquiries to Maurice B. Landers III, M.D., Department of Ophthalmology, University of California, Davis, 1603 Alhambra Blvd., Sacramento, CA 95816. Juvenile rheumatoid arthritis is a chronic, progressive disease that occurs during childhood. The most common ocular manifestation of juvenile rheumatoid arthritis is iridocyclitis. Other ocular manifestations include cataract, secondary glaucoma, and band keratopathy, which are related to chronic iridocyclitis.' Patients with ocular involvement generally have the pauciarticular disease and lack the systemic manifestations, lymphadenopathy, and fever. The patients with ocular involvement have a negative rheumatoid factor, but 80% of the patients have a positive antinuclear antibody titer associated with the disease.! We report a patient with juvenile pauciarticular rheumatoid arthritis who developed optic disk neovascularization. A 4-year-old girl had juvenile rheumatoid arthritis. The patient's condition had been initially diagnosed by her pediatrician two years previously, when she had arthritis of her left wrist and right knee. An antinuclear antibody titer was performed and found to be positive. In the same year, she developed bilateral anterior uveitis, which required long-term topical corticosteroid therapy in both eyes to control the inflammation. The arthritis was controlled by oral prednisone. Minimal inflammation persisted over the next two years, and visual acuity remained 20/30 in each eye. At 31/2 years of age, the patient was referred to our institution in March 1989 when visual acuity began to deteriorate because of bilateral progressive cataracts in both eyes that were secondary to chronic inflammation and longterm topical corticosteroid therapy. Visual acuity was R.E.: 20/400 and L.E.: hand motions. The results of an anterior segment examination disclosed nasal band keratopathy in the right eye and a clear cornea in the left eye. There were trace cells and mild flare in the right eye and trace cells and no flare in the left eye. Extensive posterior synechiae were seen in the right eye. Intraocular pressure was R.E.: 11 mm Hg and L.E.: 18 mm Hg. A severe cortical cataract was noted in the right eye and a dense, white cataract was noted in the left eye. The patient had a red reflex in the right eye and no red reflex in the left eye. The patient's cataract was removed by using a vitrectomy instrument in the left eye on March 21, 1989, and in the right eye on June 6, 1989. The entire lens capsule was removed during
Vol. 110, No.2
211
Letters to the Journal
Fig. 1 (Semple, Landers, and Morse). Fundus photograph of optic nerve head neovascularization in the right eye, July 31, 1989.
both procedures. Seven weeks later, visual acuity was R.E.: 20/200 and L.E.: 20/60. The results of an examination at this time disclosed no inflammation in the left eye and trace cell and flare in the right eye. The retina, disk, and vessels were normal in the left eye. The retina was attached in the right eye, but florid optic nerve head neovascularization was noted (Fig. 1). A fluorescein angiogram confirmed the neovascularization of the disk (Fig. 2). Capillary nonperfusion was not detected, and cystoid macular edema was seen in the late phase of the angiogram. The patient was given 1 % prednisolone acetate every three hours and 1 % atropine twice daily in the right eye. A subtenon's injection of triamcinolone acetonide 20 mg was given in the right eye. Two months after subtenon's injection of triamcinolone acetonide, the optic disk neovascularization had regressed and visual acuity improved to R.E.: 20/80. A second subtenon's injection of triamcinolone ace tonide 20 mg was given, and two months later the neovascularization had completely involuted and best-corrected visual acuity was R.E.: 20/50. Iridocyclitis is the most common ocular complication of juvenile rheumatoid arthritis.P Although optic disk neovascularization is associated with chronic uveitis, most cases of uveitis with optic disk neovascularization, retinal neovascularization, or both have associated vasculitis and ischemia as the possible cause of the neovascularization. There are several pub-
Fig. 2 (Semple, Landers, and Morse). Transit phase of intravenous fluorescein angiogram of right eye, which confirms extensive neovascularization of the optic nerve head, August 1989.
lished reports associating optic disk neovascularization alone with posterior uveitis without associated capillary nonperfusion noted on fluorescein angiogram.t! Most of these cases were diagnosed as chronic cyclitis and none had documented juvenile rheumatoid arthritis. The treatment of the optic disk neovascularization in this patient was based on the resolution of the chronic inflammation. Neovascularization associated with posterior uveitis has been treated with either panretinal photocoagulation/ corticosteroid therapy, or both, although panretinal photocoagulation is probably of limited value in the absence of angiographic evidence of ischemia."
References 1. Chylack, L. T., Jr., Bienfang, D. C. c.. Bellows, A. R., and Stillman, J. S.: Ocular manifestations of juvenile rheumatoid arthritis. Am. J. Ophthalmol. 79:1026/ 1975. 2. Kanski, J. J.: Anterior uveitis in juvenile rheumatoid arthritis. Arch. Ophthalmol. 95:1794,1977. 3. Kelly, P. J., and Weiter, J. J.: Resolution of optic disk neovascularization associated with intraocular inflammation. Am. J. Ophthalmol. 90:545, 1980. 4. Shorb, S. R., Irvine, A. R., Kimura, S. J./ and Morris, B. W.: Optic disk neovascularization associ-
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ated with chronic uveitis. Am. J. Ophthalmol. 82:175, 1976. 5. Graham, E. M., Stanford, M. R., Shilling, J. S., and Sanders, M. D.: Neovascularization associated with posterior uveitis. Br. J. Ophthalmol. 71:826, 1987.
Complete Visual Recovery After Bacillus cereus Endophthalmitis in a Child Paul M. Beer, M.D., Irene H. Ludwig, M.D., and Andrew J. Packer, M.D.
Department of Ophthalmology, Mary Imogene Bassett Hospital (P.M.B., I.H.L.) and Hartford Hospital (A.J.P.). This study was presented at the annual meeting of the Vitreous Society, Orlando, Florida, December 9,1989. Inquiries to Paul M. Beer, M.D., Vitreoretinal Service, Department of Ophthalmology, Mary Imogene Bassett Hospital, One Atwell Rd., Cooperstown, NY 13326.
Bacillus cereus endophthalmitis destroys vision rapidly and may lead to loss of the globe.'> Diagnosis was made at an early stage in two patients in whom useful vision was preserved.v' We treated a 3Jh-year-old boy who developed Bacillus endophthalmitis and had complete visual recovery. A healthy boy was struck in his right eye by a manure fork and sustained a 6-mm horizontal corneal laceration, not involving the visual axis. The child was brought to the operating room within four hours of the injury. The corneal laceration was closed. No hypopyon was noted. A dense fibrinous membrane was peeled from the iris, which allowed excellent visualization of the fundus. Prominent retinal periphlebitis was noted in the superotemporal quadrant. The adjacent pars plana, contiguous with the limbal extent of the corneal laceration, was covered by white inflammatory material from the 1 o'clock to the 2 o'clock meridians. Two clumps of vitreal cells were seen overlying the inflamed pars plana. The remainder of the vitreous cavity was clear (Figure). Based on the finding of retinal periphlebitis, a diagnosis of endophthalmitis was suspected. A pars plana sclerotomy was made in the area of maximal involvement and 0.2 ml of solid
Figure (Beer, Ludwig, and Packer). Intraoperative fundus appearance. Striking periphlebitis (arrowhead) and snowbanking of pars plana with clumps of vitreous cells (arrow) was found in the quadrant contiguous with the corneal laceration.
vitreous was removed with the vitreous cutter and replaced with 1 mg of clindamycin and 200 JLg of intravitreal gentamicin. Subconjunctival gentamicin 20 mg and cefazolin 40 mg were administered. Vitreous cultures grew B. cereus and coagulase negative Staphylococcus aureus. Systemic ceftriaxone sodium and clindamycin were administered for 14 days. Clinically, the endophthalmitis resolved in the immediate postoperative period, as no hypopyon or vitreous clouding developed. Patching of the left eye was begun on the ninth postoperative day. A posterior subcapsular cataract developed and was removed 24 days after the original injury. Prompt refractive correction was provided by using a silicone contact lens and aphakic spectacle correction with patching while lens wear time was being accumulated. Full-time contact lens wear was achieved six weeks after the injury, and bifocal spectacles were then prescribed. Patching was tapered to one hour daily. Twelve months after injury, visual acuity measured 20/30 in each eye (illiterate E), 3D-second stereopsis was present, and ocular alignment was normal. The success in this patient was because of
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Landers, Semple, and Morse). Infusion cannula in position showing its relationship to anterior structures. pupillary margin so that the flow of infusion fluid is downward into the vitreous cavity of the aphakic eye and, hence, away from the corneal endothelium (Fig. 2). The infusion cannula can be sewn onto the sclera in a manner similar to that of a standard pars plana infusion cannula. It is thus possible to approach the vitreous base with a minimal chance of the infusion cannula creating new retinal tears. We have used this infusion cannula successfully on several patients who had severe proliferative vitreoretinopathy and in trauma patients who have dense vitreous hemorrhage and preoperative ultrasonic evidence of a retinal detachment.
References 1. De Juan, E., Jr., and McCuen, B. W., II: Management of anterior vitreous traction in proliferative vitreoretinopathy. Retina 4:258,1989. 2. Aaberg, T. M.: Management of anterior and posterior proliferative vitreoretinopathy. XLV Edward Jackson memorial lecture. Am. J. Ophthalmol. 106:519,1988. 3. Charles,S.: Anterior loop traction. In Vitreous Microsurgery, ed. 2. Baltimore, Williams & Wilkins, 1987, p. 137.
Optic Disk Neovascularization in Juvenile Rheumatoid Arthritis H. Christopher Semple, M.D., Maurice B. Landers III, M.D., and Lawrence S. Morse, M.D.
Retinal Service, Department of Ophthalmology, University of California, Davis.
August, 1990
Inquiries to Maurice B. Landers III, M.D., Department of Ophthalmology, University of California, Davis, 1603 Alhambra Blvd., Sacramento, CA 95816. Juvenile rheumatoid arthritis is a chronic, progressive disease that occurs during childhood. The most common ocular manifestation of juvenile rheumatoid arthritis is iridocyclitis. Other ocular manifestations include cataract, secondary glaucoma, and band keratopathy, which are related to chronic iridocyclitis.' Patients with ocular involvement generally have the pauciarticular disease and lack the systemic manifestations, lymphadenopathy, and fever. The patients with ocular involvement have a negative rheumatoid factor, but 80% of the patients have a positive antinuclear antibody titer associated with the disease.! We report a patient with juvenile pauciarticular rheumatoid arthritis who developed optic disk neovascularization. A 4-year-old girl had juvenile rheumatoid arthritis. The patient's condition had been initially diagnosed by her pediatrician two years previously, when she had arthritis of her left wrist and right knee. An antinuclear antibody titer was performed and found to be positive. In the same year, she developed bilateral anterior uveitis, which required long-term topical corticosteroid therapy in both eyes to control the inflammation. The arthritis was controlled by oral prednisone. Minimal inflammation persisted over the next two years, and visual acuity remained 20/30 in each eye. At 31/2 years of age, the patient was referred to our institution in March 1989 when visual acuity began to deteriorate because of bilateral progressive cataracts in both eyes that were secondary to chronic inflammation and longterm topical corticosteroid therapy. Visual acuity was R.E.: 20/400 and L.E.: hand motions. The results of an anterior segment examination disclosed nasal band keratopathy in the right eye and a clear cornea in the left eye. There were trace cells and mild flare in the right eye and trace cells and no flare in the left eye. Extensive posterior synechiae were seen in the right eye. Intraocular pressure was R.E.: 11 mm Hg and L.E.: 18 mm Hg. A severe cortical cataract was noted in the right eye and a dense, white cataract was noted in the left eye. The patient had a red reflex in the right eye and no red reflex in the left eye. The patient's cataract was removed by using a vitrectomy instrument in the left eye on March 21, 1989, and in the right eye on June 6, 1989. The entire lens capsule was removed during
Vol. 110, No.2
211
Letters to the Journal
Fig. 1 (Semple, Landers, and Morse). Fundus photograph of optic nerve head neovascularization in the right eye, July 31, 1989.
both procedures. Seven weeks later, visual acuity was R.E.: 20/200 and L.E.: 20/60. The results of an examination at this time disclosed no inflammation in the left eye and trace cell and flare in the right eye. The retina, disk, and vessels were normal in the left eye. The retina was attached in the right eye, but florid optic nerve head neovascularization was noted (Fig. 1). A fluorescein angiogram confirmed the neovascularization of the disk (Fig. 2). Capillary nonperfusion was not detected, and cystoid macular edema was seen in the late phase of the angiogram. The patient was given 1 % prednisolone acetate every three hours and 1 % atropine twice daily in the right eye. A subtenon's injection of triamcinolone acetonide 20 mg was given in the right eye. Two months after subtenon's injection of triamcinolone acetonide, the optic disk neovascularization had regressed and visual acuity improved to R.E.: 20/80. A second subtenon's injection of triamcinolone ace tonide 20 mg was given, and two months later the neovascularization had completely involuted and best-corrected visual acuity was R.E.: 20/50. Iridocyclitis is the most common ocular complication of juvenile rheumatoid arthritis.P Although optic disk neovascularization is associated with chronic uveitis, most cases of uveitis with optic disk neovascularization, retinal neovascularization, or both have associated vasculitis and ischemia as the possible cause of the neovascularization. There are several pub-
Fig. 2 (Semple, Landers, and Morse). Transit phase of intravenous fluorescein angiogram of right eye, which confirms extensive neovascularization of the optic nerve head, August 1989.
lished reports associating optic disk neovascularization alone with posterior uveitis without associated capillary nonperfusion noted on fluorescein angiogram.t! Most of these cases were diagnosed as chronic cyclitis and none had documented juvenile rheumatoid arthritis. The treatment of the optic disk neovascularization in this patient was based on the resolution of the chronic inflammation. Neovascularization associated with posterior uveitis has been treated with either panretinal photocoagulation/ corticosteroid therapy, or both, although panretinal photocoagulation is probably of limited value in the absence of angiographic evidence of ischemia."
References 1. Chylack, L. T., Jr., Bienfang, D. C. c.. Bellows, A. R., and Stillman, J. S.: Ocular manifestations of juvenile rheumatoid arthritis. Am. J. Ophthalmol. 79:1026/ 1975. 2. Kanski, J. J.: Anterior uveitis in juvenile rheumatoid arthritis. Arch. Ophthalmol. 95:1794,1977. 3. Kelly, P. J., and Weiter, J. J.: Resolution of optic disk neovascularization associated with intraocular inflammation. Am. J. Ophthalmol. 90:545, 1980. 4. Shorb, S. R., Irvine, A. R., Kimura, S. J./ and Morris, B. W.: Optic disk neovascularization associ-
212
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August, 1990
ated with chronic uveitis. Am. J. Ophthalmol. 82:175, 1976. 5. Graham, E. M., Stanford, M. R., Shilling, J. S., and Sanders, M. D.: Neovascularization associated with posterior uveitis. Br. J. Ophthalmol. 71:826, 1987.
Complete Visual Recovery After Bacillus cereus Endophthalmitis in a Child Paul M. Beer, M.D., Irene H. Ludwig, M.D., and Andrew J. Packer, M.D.
Department of Ophthalmology, Mary Imogene Bassett Hospital (P.M.B., I.H.L.) and Hartford Hospital (A.J.P.). This study was presented at the annual meeting of the Vitreous Society, Orlando, Florida, December 9,1989. Inquiries to Paul M. Beer, M.D., Vitreoretinal Service, Department of Ophthalmology, Mary Imogene Bassett Hospital, One Atwell Rd., Cooperstown, NY 13326.
Bacillus cereus endophthalmitis destroys vision rapidly and may lead to loss of the globe.'> Diagnosis was made at an early stage in two patients in whom useful vision was preserved.v' We treated a 3Jh-year-old boy who developed Bacillus endophthalmitis and had complete visual recovery. A healthy boy was struck in his right eye by a manure fork and sustained a 6-mm horizontal corneal laceration, not involving the visual axis. The child was brought to the operating room within four hours of the injury. The corneal laceration was closed. No hypopyon was noted. A dense fibrinous membrane was peeled from the iris, which allowed excellent visualization of the fundus. Prominent retinal periphlebitis was noted in the superotemporal quadrant. The adjacent pars plana, contiguous with the limbal extent of the corneal laceration, was covered by white inflammatory material from the 1 o'clock to the 2 o'clock meridians. Two clumps of vitreal cells were seen overlying the inflamed pars plana. The remainder of the vitreous cavity was clear (Figure). Based on the finding of retinal periphlebitis, a diagnosis of endophthalmitis was suspected. A pars plana sclerotomy was made in the area of maximal involvement and 0.2 ml of solid
Figure (Beer, Ludwig, and Packer). Intraoperative fundus appearance. Striking periphlebitis (arrowhead) and snowbanking of pars plana with clumps of vitreous cells (arrow) was found in the quadrant contiguous with the corneal laceration.
vitreous was removed with the vitreous cutter and replaced with 1 mg of clindamycin and 200 JLg of intravitreal gentamicin. Subconjunctival gentamicin 20 mg and cefazolin 40 mg were administered. Vitreous cultures grew B. cereus and coagulase negative Staphylococcus aureus. Systemic ceftriaxone sodium and clindamycin were administered for 14 days. Clinically, the endophthalmitis resolved in the immediate postoperative period, as no hypopyon or vitreous clouding developed. Patching of the left eye was begun on the ninth postoperative day. A posterior subcapsular cataract developed and was removed 24 days after the original injury. Prompt refractive correction was provided by using a silicone contact lens and aphakic spectacle correction with patching while lens wear time was being accumulated. Full-time contact lens wear was achieved six weeks after the injury, and bifocal spectacles were then prescribed. Patching was tapered to one hour daily. Twelve months after injury, visual acuity measured 20/30 in each eye (illiterate E), 3D-second stereopsis was present, and ocular alignment was normal. The success in this patient was because of