Bike WRF, Manthey KF, Nussenblatt RB (eds): Intermediate Uveitis. Dei Ophthalmol. Basel, Karger, 1992, vol 23, pp 204-211
Cataract Extraction in Intermediate Uveitis David J. Forster, Narsing A. Rao, Ronald E. Smith Doheny Eye Institute and Department of Ophthalmology, USC School of Medicine, Los Angeles, Calif., USA
Intermediate uveitis (pars planitis) is a relatively common entity among children and young adults, and consists of vitreous cells and `snowball opacities', a characteristic `snowbank' or exudate over the pars plana region, and minimal anterior chamber reaction [1-4]. The disease may follow a benign course, often requiring no treatment, or may follow a chronic or relapsing course, with frequent exacerbations and requiring intensive medical and/or surgical intervention. Complications associated with these long-standing cases include cystoid macular edema (CME) and subsequent macular degeneration, cataracts, band keratopathy, glaucoma, vitreous traction with secondary retinal detachment, vitreous hemorrhage and macular heterotopia. Of these, macular disease and cataracts are the most common sequelae.
Cataract formation was found to be the most common complication of intermediate uveitis in two large studies. In 1960, Brockhurst et al. [1] reported on 100 patients with `peripheral uveitis' followed for 1-8 years. Of this group, 36 patients developed cataracts, and in many cases their visual loss was attributed entirely to cataract formation. Smith et al. [2, 3] studied 100 patients with `chronic cyclitis' followed for a least 4 years. Of 182 eyes, 76 (42%) developed cataract; 34 of these were present on initial examination. Lens opacities accounted for 6% of cases of decreased visual acuity (20/40 or less) on initial examination, but were responsible for 19% of cases of decreased acuity at the time of final examination. Cataract formation appeared to be more severe in eyes with evidence
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of increased vitreous disease or those in which the disease had a prolonged course. As most cases of intermediate uveitis are characterized by inflammation in the vitreous cavity primarily, with less inflammation in the anterior chamber, it is reasonable to expect that most associated lens opacities will occur in the posterior cortical or subcapsular areas and, in fact, this seems to be the case. In the study by Brockhurst et al. [ 1 ], the cataracts were described as `posterior cortical', while in the series by Smith et al. [2] they were described as `complicated cataracts starting with posterior subcapsular involvement'. Although cataracts are recognized as a common complication of many forms of uveitis, the exact mechanism by which inflammation causes cataract formation is not completely understood. Inflammatory by-products such as phospholipase A and other lysosomal enzymes have been shown to produce damage to lens fiber membranes in experimental uveitis [5]. Oxygen free radicals produced by the inflammatory response have also been shown to cause damage to the lens in animal models [6]. Another potential mechanism is the deposition of immune complexes on the lens capsule during episodes of active inflammation [7]. An important point to remember in these patients is the role of corticosteroids in the development of lens opacities, primarily those of the posterior subcapsular type. Topical, periocular and systemic steroids have all been implicated in cataract formation, and this risk appears to be related to the dose and duration of treatment [8]. Accordingly, care must be taken to not overtreat these patients, especially in light of the chronic nature of the disease and the fact that in many patients the disease will follow a benign course. We generally institute treatment only after visual acuity has declined to 20/40, usually from CME, or if the patient complains of marked vitreous floaters [4].
One of the main issues regarding cataract surgery in patients with intermediate uveitis involves the decision to perform cataract extraction alone or to combine this procedure with vitrectomy. Also unresolved is the question of whether intraocular lenses should be implanted in these patients. Little data exist in the literature regarding the results of intracapsular versus extracapsular cataract extraction alone in patients with intermediate
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uveitis. In their 1976 review of complications of intermediate uveitis, Smith et al. [3] included 20 eyes that had undergone either intracapsular or extracapsular cataract extraction. While no information was given regarding the type or dose of anti-inflammatory therapy used in this group, 65% of these eyes had vision of 20/40 or better at the time of their last visit. In 1989, Foster et al. [9] reported their results of cataract surgery with posterior chamber intraocular lens implantation in 38 patients with uveitis. Included in their series were 4 eyes with intermediate uveitis. Preoperative visual acuity ranged from 20/200 to 5/200, while postoperative visual acuity ranged from 20/15 to 20/70 after a mean follow-up of 25 months. Three of the 4 eyes (1 with final acuity of 20/40 and 2 with final acuity of 20/70) had associated CME. The authors concluded that CME represents the major obstacle to visual recovery in patients with uveitis who undergo cataract extraction, and suggested that aggressive treatment of CME, including the use of immunosuppressive agents, is necessary during both the pre- and postoperative periods to improve the chance of success. Most other reports have dealt with the results of combined lens extraction and vitrectomy in patients with uveitis. In 1978, Diamond and Kaplan [10] reported on the results of combined lensectomy-vitrectomy in 15 eyes with uveitis and complicated cataracts, 6 of which had pars planitis. Inflammation had been present for 4-19 years preoperatively, and patients were followed for a mean of 14 months postoperatively. Patients were treated during the immediate pre- and postoperative period with topical and systemic steroids, and received intravitreal steroids at the time of surgery. In all patients except one, surgery was cancelled if slitlamp examination showed greater than 1+ cell and flare on the day of surgery. The exception was a patient who had undergone previous intracapsular surgery but who developed a severe inflammatory pupillary membrane, elevated intraocular pressure and persistent anterior chamber reaction in spite of intensive topical and systemic steroids. Postoperatively, visual acuity was 20/25 or better in 4 eyes, and 20/70 in both eyes of the above-mentioned patient, in whom decreased visual acuity was secondary to diffuse retinal edema. No other complications were reported. A report by Tutein Nolthenius and Deutman in 1983 [11] included 8 eyes with `posterior cyclitis' of 1-12 years duration. Lens fragmentation and aspiration, as well as vitrectomy, were performed through the pars plana, and eyes with pars planitis were treated with peripheral cryotherapy. Aside from the use of subconjunctival steroids at the time of surgery, no information was given regarding steroid therapy perioperatively. After a mean
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follow-up period of 13 months, visual acuity was 20/40 or better in half of the eyes, with 7 of 8 eyes (88%) having vision of 20/80 or better. Four eyes had evidence of CME, and 2 others had macular pucker. No surgical complications were reported in these eyes, and no eyes experienced a worsening of vision from preoperative levels. The results of pars plana lensectomy and vitrectomy by ultrasonic fragmentation were also reported by Girard et al. in 1985 [12]. Five eyes with pars planitis of 3-13 years duration were included, all of which were treated with local and systemic steroids (no further specification). Preoperative acuity ranged from 20/60 to 20/400, while postoperative acuity was 20/20-20/30 in 3 eyes, 20/100 in 1 eye and 20/200 in another. The latter 2 eyes had previous macular disease (CME or chorioretinitis). All 5 eyes had an improvement in vision, however. The patients were followed for an average of 2.8 years (range 1-7 years) after surgery with no steroid therapy and no exacerbations of the uveitis. The authors concluded that, in addition to improving vision in these patients, this combined technique resulted in remission of the ocular inflammatory disease as well. Finally, Mieler et al. [13] reported on a series of 12 eyes with peripheral uveitis that underwent vitreous surgery, 8 of which also had cataract surgery. Four eyes had initial pars plana lensectomy and vitrectomy, 2 had secondary lensectomy and 2 had secondary extracapsular surgery. Duration of disease preoperatively ranged from 1 to 72 months (mean 32 months), with postoperative follow-up of 6-90 months (mean 28 months). Vision improved in all eyes postoperatively, with 4 of the 8 eyes attaining vision of 20/40 or better. Vision in the other 4 eyes ranged from 20/50 to 20/100; 3 of these eyes had clinically significant CME. Postoperative complications included vitreous hemorrhage in 2 eyes and steroid-induced glaucoma in 1 eye. The authors found that eyes with active neovascularization of the vitreous base at the time of initial vitreous surgery developed more complications postoperatively, often requiring further surgery. They concluded that adequate control of active neovascularization with either preoperative cryotherapy or intraoperative photocoagulation may reduce the incidence of complications and improve the chances of success with surgery. In summary, the majority of patients had improvement of vision after cataract extraction, either alone or with vitrectomy. The number of patients in these series is too small to allow adequate comparison of cataract surgery alone with combined lensectomy/vitrectomy. However, in all patients the most common cause of vision less than 20/40 was CME, which may have been present preoperatively in a large percentage of cases. Therefore, ag-
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gressive treatment of CME both pre- and postoperatively is of prime importance in the visual rehabilitation of these patients.
Timing of Surgery and Perioperative Management Little information exists regarding the optimal timing of surgery in these patients. As in patients with other forms of uveitis, it is probably best to delay surgery until intraocular inflammation is minimal. Also, the evidence seems to point to the importance of adequate control of CME [9] and of neovascularization of the vitreous base [13] prior to performing surgery. Thus, intensive medical and/or surgical control of inflammation, neovascularization and CME is recommended. With or without a cataract, we advocate a four-step approach to therapy in these patients [4], similar to that described by Kaplan [14]. If vision falls to 20/40 or less from CME, or in the presence of marked vitreous floaters, we treat initially with corticosteroids, either by sub-Tenon injections or systemically. Step 2 is cryotherapy of the pars plana exudate and is used if there is a poor response to steroids. Step 3 entails pars plana vitrectomy, and step 4 is immunosuppressive therapy. The indications for and details of these therapeutic approaches are outlined elsewhere in this publication. After cataract surgery, the patient should remain on systemic steroid and/or immunosuppressive therapy if such agents were required preoperatively. These are continued for a week to 10 days and then tapered gradually as the inflammation and CME resolve.
There is no consensus regarding which type of surgery should be performed on patients with intermediate uveitis: cataract extraction alone or combined lensectomy/vitrectomy. As can be seen from the studies cited above, good results have been obtained with either procedure. Several factors may make the combined lensectomy/vitrectomy approach the preferred route in some of these patients. Since many of these patients have a significant amount of vitreous inflammation and/or debris, removal of vitreous opacities may improve vision. In addition, some authors believe that `debulking' the vitreous cavity may result in the removal of in-
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flammatory cells, mediators and/or immune complexes that may be linked to persistent inflammation and CME [10, 13, 15]. Therefore, in the subset of intermediate uveitis patients who have minimal vitreous opacities and minimal or no CME, standard extracapsular cataract extraction or phacoemulsification, if possible, seems to be the preferred method. This approach will probably be sufficient in many cases. In patients with significant vitreous opacities and CME, combined cataract extraction and vitrectomy is probably the preferred surgical approach. In the latter cases, while a pars plana lensectomy-vitrectomy approach may be performed, a limbal approach can also be used for extracapsular extraction or phacoemulsification. This approach allows partial preservation of the posterior capsule, which may facilitate the implantation of a posterior chamber intraocular lens at a later date should the pars planitis `burn out'. (Some authors consider primary intraocular lens implantation in such cases [9].) The limbal approach can be easily combined with a pars plana vitrectomy. After the cataract extraction is completed, a standard pars plana approach is undertaken. Because of the rapid capsular opacifi cation that occurs in many patients with uveitis, a primary posterior capsulectomy should probably be performed at the time of the vitrectomy. The issue of intraocular lens implantation in patients with intermediate uveitis remains controversial. Pathological studies have shown that intraocular lens haptics can erode into the ciliary body, possibly exacerbating intraocular inflammation [16, 17]. Also, polypropylene and polymethylmethacrylate have been shown to activate the complement pathway, even in eyes with no history of inflammation [18, 19], although this may be less so with polymethylmethacrylate haptics. Careful in-the-bag placement of haptics probably reduces the risk of inflammatory reaction to these substances. However, because of the chronic nature of the inflammation in many patients with intermediate uveitis, as well as the fact that most of these patients are relatively young and thus should be able to manage contact lenses well, we do not recommend intraocular lens implantation, at least initially. In patients who remain free of inflammation or CME for a prolonged period (i.e. 1-2 years), secondary posterior chamber lens implantation could be considered if the patient does not tolerate contact lenses. In summary, cataract formation is one of the most common complications of long-standing intermediate uveitis and occurs as a result of prolonged inflammation as well as from corticosteroid therapy. Good results have been obtained with cataract surgery alone (extracapsular) or in combination with pars plana vitrectomy. The combined approach is preferred by
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us in patients with significant vitreous debris and CME; cataract extraction alone is recommended in the subset of patients with minimal vitritis and little or no CME. The issue of posterior chamber lens implantation remains unresolved. As CME is the primary cause of reduced vision in these patients, aggressive pre- and postoperative treatment of this condition with corticosteroids and/or immunosuppressive agents is essential for a good visual result.
Acknowledgments Supported in part by NIH grant ΕΥ03040 from the National Eye Institute and by a grant from Research to Prevent Blindness, Inc, New York. The authors wish to express their appreciation to Anita Fernandez and Iτene Cumplido for typing the manuscript and to Ann Dawson for editorial assistance.
1 Brockhurst RJ, Schepens CL, Okamura ID: Uveitis. II. Peripheral uveitis: Clinical description, complications and differential diagnosis. Am J Ophthalmol 1960;49:12571266. 2 Smith RE, Godfrey WA, Kimura SJ: Chronic cyclitis. I. Course and visual prognosis. Trans Am Acad Ophthalmol Otolaryngol 1973;77:760-768. 3 Smith RE, Godfrey WA, Kimura SJ: Complications of chronic cyclitis. Am J Ophthalmol 1976;82:277-282. 4 Smith RE: Pars planitis; in Ryan SJ (ed): Retina. St. Louis, Mosby, 1989, vol 2, pp 637645. 5 Secchi AG: Cataracts in uveitis. Trans Ophthalmol Soc UK 1982;102:390-394. 6 Marak GE Jr, Rao NA, Scott JM, et al: Antioxidant modulation of phacoanaphylactic endophthalmitis. Ophthalmic Res 1985;17:297-301. 7 Fisher RF: The lens in uveitis. Trans Ophthalmol Soc UK 1981;101:317-320. 8 Williamson J, Paterson RWW, McGavin DDM, et al: Posterior subcapsular cataracts and glaucoma associated with long-term oral corticosteroid therapy in patients with rheumatoid arthritis and related conditions. Br J Ophthalmol 1969;53:361-372. 9 Foster CS, Fong LP, Singh G: Cataract surgery and intraocular lens implantation in patients with uveitis. Ophthalmology 1989;96:281-288. 10 Diamond JG, Kaplan HJ: Lensectomy and vitrectomy for complicated cataract secondary to uveitis. Arch Ophthalmol 1978;96:1798-1804. 11 Tutein Nolthenius PA, Deutman AF: Surgical treatment of the complications of chronic uveitis. Ophthalmologica 1983;186:11-16. 12 Girard LJ, Rodriguez J, Mailman ML, et al: Cataract and uveitis management by pars plana lensectomy and vitrectomy by ultrasonic fragmentation. Retina 1985;5:107114.
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References
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Ronald E. Smith, MD, Doheny Eye Institute, 1355 San Pablo Street, Los Angeles, CA 90033 (USA)
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13 Mieler WF, Will BR, Lewis H, et al: Vitrectomy in the management of peripheral uveitis. Ophthalmology 1988;95:859-864. 14 Kaplan HJ: Intermediate uveitis (pars planitis, chronic cyclitis): Α four-step approach to treatment; in Saari KM (ed): Uveitis Update. Amsterdam, Excerpta Medica, 1984, pp 169-172. 15 Algvere P, Alanko H, Dickhoff K, et al: Pars plana vitrectomy in the management of intraocular inflammation. Acta Ophthalmol 1981;59:727-736. 16 McDonnell PJ, Champion R, Green WR: Location and composition of haptics of posterior chamber intraocular lenses: Histopathologic study of postmortem eyes. Ophthalmology 1987;94:136-142. 17 Apple DJ, Mamalis N, Loftfield K, et al: Complications of intraocular lenses: A historical and histopathological review. Suri Ophthalmol 1984;29:1-54. 18 Mondino BJ, Nagata S, Glovsky MM: Activation of the alternative complement pathway by intraocular lenses. Invest Ophthalmol Vis Sci 1985;26:905-908. 19 Tuberville AW, Galin MA, Perez HD, et al: Complement activation by nylon- and polypropylene-looped prosthetic intraocular lenses. Invest Ophthalmol Vis Sci 1982;22: 727-733.
Cataract Extraction in Intermediate Uveitis David J. Forster, Narsing A. Rao, Ronald E. Smith Doheny Eye Institute and Department of Ophthalmology, USC School of Medicine, Los Angeles, Calif., USA
Intermediate uveitis (pars planitis) is a relatively common entity among children and young adults, and consists of vitreous cells and `snowball opacities', a characteristic `snowbank' or exudate over the pars plana region, and minimal anterior chamber reaction [1-4]. The disease may follow a benign course, often requiring no treatment, or may follow a chronic or relapsing course, with frequent exacerbations and requiring intensive medical and/or surgical intervention. Complications associated with these long-standing cases include cystoid macular edema (CME) and subsequent macular degeneration, cataracts, band keratopathy, glaucoma, vitreous traction with secondary retinal detachment, vitreous hemorrhage and macular heterotopia. Of these, macular disease and cataracts are the most common sequelae.
Cataract formation was found to be the most common complication of intermediate uveitis in two large studies. In 1960, Brockhurst et al. [1] reported on 100 patients with `peripheral uveitis' followed for 1-8 years. Of this group, 36 patients developed cataracts, and in many cases their visual loss was attributed entirely to cataract formation. Smith et al. [2, 3] studied 100 patients with `chronic cyclitis' followed for a least 4 years. Of 182 eyes, 76 (42%) developed cataract; 34 of these were present on initial examination. Lens opacities accounted for 6% of cases of decreased visual acuity (20/40 or less) on initial examination, but were responsible for 19% of cases of decreased acuity at the time of final examination. Cataract formation appeared to be more severe in eyes with evidence
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of increased vitreous disease or those in which the disease had a prolonged course. As most cases of intermediate uveitis are characterized by inflammation in the vitreous cavity primarily, with less inflammation in the anterior chamber, it is reasonable to expect that most associated lens opacities will occur in the posterior cortical or subcapsular areas and, in fact, this seems to be the case. In the study by Brockhurst et al. [ 1 ], the cataracts were described as `posterior cortical', while in the series by Smith et al. [2] they were described as `complicated cataracts starting with posterior subcapsular involvement'. Although cataracts are recognized as a common complication of many forms of uveitis, the exact mechanism by which inflammation causes cataract formation is not completely understood. Inflammatory by-products such as phospholipase A and other lysosomal enzymes have been shown to produce damage to lens fiber membranes in experimental uveitis [5]. Oxygen free radicals produced by the inflammatory response have also been shown to cause damage to the lens in animal models [6]. Another potential mechanism is the deposition of immune complexes on the lens capsule during episodes of active inflammation [7]. An important point to remember in these patients is the role of corticosteroids in the development of lens opacities, primarily those of the posterior subcapsular type. Topical, periocular and systemic steroids have all been implicated in cataract formation, and this risk appears to be related to the dose and duration of treatment [8]. Accordingly, care must be taken to not overtreat these patients, especially in light of the chronic nature of the disease and the fact that in many patients the disease will follow a benign course. We generally institute treatment only after visual acuity has declined to 20/40, usually from CME, or if the patient complains of marked vitreous floaters [4].
One of the main issues regarding cataract surgery in patients with intermediate uveitis involves the decision to perform cataract extraction alone or to combine this procedure with vitrectomy. Also unresolved is the question of whether intraocular lenses should be implanted in these patients. Little data exist in the literature regarding the results of intracapsular versus extracapsular cataract extraction alone in patients with intermediate
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uveitis. In their 1976 review of complications of intermediate uveitis, Smith et al. [3] included 20 eyes that had undergone either intracapsular or extracapsular cataract extraction. While no information was given regarding the type or dose of anti-inflammatory therapy used in this group, 65% of these eyes had vision of 20/40 or better at the time of their last visit. In 1989, Foster et al. [9] reported their results of cataract surgery with posterior chamber intraocular lens implantation in 38 patients with uveitis. Included in their series were 4 eyes with intermediate uveitis. Preoperative visual acuity ranged from 20/200 to 5/200, while postoperative visual acuity ranged from 20/15 to 20/70 after a mean follow-up of 25 months. Three of the 4 eyes (1 with final acuity of 20/40 and 2 with final acuity of 20/70) had associated CME. The authors concluded that CME represents the major obstacle to visual recovery in patients with uveitis who undergo cataract extraction, and suggested that aggressive treatment of CME, including the use of immunosuppressive agents, is necessary during both the pre- and postoperative periods to improve the chance of success. Most other reports have dealt with the results of combined lens extraction and vitrectomy in patients with uveitis. In 1978, Diamond and Kaplan [10] reported on the results of combined lensectomy-vitrectomy in 15 eyes with uveitis and complicated cataracts, 6 of which had pars planitis. Inflammation had been present for 4-19 years preoperatively, and patients were followed for a mean of 14 months postoperatively. Patients were treated during the immediate pre- and postoperative period with topical and systemic steroids, and received intravitreal steroids at the time of surgery. In all patients except one, surgery was cancelled if slitlamp examination showed greater than 1+ cell and flare on the day of surgery. The exception was a patient who had undergone previous intracapsular surgery but who developed a severe inflammatory pupillary membrane, elevated intraocular pressure and persistent anterior chamber reaction in spite of intensive topical and systemic steroids. Postoperatively, visual acuity was 20/25 or better in 4 eyes, and 20/70 in both eyes of the above-mentioned patient, in whom decreased visual acuity was secondary to diffuse retinal edema. No other complications were reported. A report by Tutein Nolthenius and Deutman in 1983 [11] included 8 eyes with `posterior cyclitis' of 1-12 years duration. Lens fragmentation and aspiration, as well as vitrectomy, were performed through the pars plana, and eyes with pars planitis were treated with peripheral cryotherapy. Aside from the use of subconjunctival steroids at the time of surgery, no information was given regarding steroid therapy perioperatively. After a mean
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follow-up period of 13 months, visual acuity was 20/40 or better in half of the eyes, with 7 of 8 eyes (88%) having vision of 20/80 or better. Four eyes had evidence of CME, and 2 others had macular pucker. No surgical complications were reported in these eyes, and no eyes experienced a worsening of vision from preoperative levels. The results of pars plana lensectomy and vitrectomy by ultrasonic fragmentation were also reported by Girard et al. in 1985 [12]. Five eyes with pars planitis of 3-13 years duration were included, all of which were treated with local and systemic steroids (no further specification). Preoperative acuity ranged from 20/60 to 20/400, while postoperative acuity was 20/20-20/30 in 3 eyes, 20/100 in 1 eye and 20/200 in another. The latter 2 eyes had previous macular disease (CME or chorioretinitis). All 5 eyes had an improvement in vision, however. The patients were followed for an average of 2.8 years (range 1-7 years) after surgery with no steroid therapy and no exacerbations of the uveitis. The authors concluded that, in addition to improving vision in these patients, this combined technique resulted in remission of the ocular inflammatory disease as well. Finally, Mieler et al. [13] reported on a series of 12 eyes with peripheral uveitis that underwent vitreous surgery, 8 of which also had cataract surgery. Four eyes had initial pars plana lensectomy and vitrectomy, 2 had secondary lensectomy and 2 had secondary extracapsular surgery. Duration of disease preoperatively ranged from 1 to 72 months (mean 32 months), with postoperative follow-up of 6-90 months (mean 28 months). Vision improved in all eyes postoperatively, with 4 of the 8 eyes attaining vision of 20/40 or better. Vision in the other 4 eyes ranged from 20/50 to 20/100; 3 of these eyes had clinically significant CME. Postoperative complications included vitreous hemorrhage in 2 eyes and steroid-induced glaucoma in 1 eye. The authors found that eyes with active neovascularization of the vitreous base at the time of initial vitreous surgery developed more complications postoperatively, often requiring further surgery. They concluded that adequate control of active neovascularization with either preoperative cryotherapy or intraoperative photocoagulation may reduce the incidence of complications and improve the chances of success with surgery. In summary, the majority of patients had improvement of vision after cataract extraction, either alone or with vitrectomy. The number of patients in these series is too small to allow adequate comparison of cataract surgery alone with combined lensectomy/vitrectomy. However, in all patients the most common cause of vision less than 20/40 was CME, which may have been present preoperatively in a large percentage of cases. Therefore, ag-
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gressive treatment of CME both pre- and postoperatively is of prime importance in the visual rehabilitation of these patients.
Timing of Surgery and Perioperative Management Little information exists regarding the optimal timing of surgery in these patients. As in patients with other forms of uveitis, it is probably best to delay surgery until intraocular inflammation is minimal. Also, the evidence seems to point to the importance of adequate control of CME [9] and of neovascularization of the vitreous base [13] prior to performing surgery. Thus, intensive medical and/or surgical control of inflammation, neovascularization and CME is recommended. With or without a cataract, we advocate a four-step approach to therapy in these patients [4], similar to that described by Kaplan [14]. If vision falls to 20/40 or less from CME, or in the presence of marked vitreous floaters, we treat initially with corticosteroids, either by sub-Tenon injections or systemically. Step 2 is cryotherapy of the pars plana exudate and is used if there is a poor response to steroids. Step 3 entails pars plana vitrectomy, and step 4 is immunosuppressive therapy. The indications for and details of these therapeutic approaches are outlined elsewhere in this publication. After cataract surgery, the patient should remain on systemic steroid and/or immunosuppressive therapy if such agents were required preoperatively. These are continued for a week to 10 days and then tapered gradually as the inflammation and CME resolve.
There is no consensus regarding which type of surgery should be performed on patients with intermediate uveitis: cataract extraction alone or combined lensectomy/vitrectomy. As can be seen from the studies cited above, good results have been obtained with either procedure. Several factors may make the combined lensectomy/vitrectomy approach the preferred route in some of these patients. Since many of these patients have a significant amount of vitreous inflammation and/or debris, removal of vitreous opacities may improve vision. In addition, some authors believe that `debulking' the vitreous cavity may result in the removal of in-
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flammatory cells, mediators and/or immune complexes that may be linked to persistent inflammation and CME [10, 13, 15]. Therefore, in the subset of intermediate uveitis patients who have minimal vitreous opacities and minimal or no CME, standard extracapsular cataract extraction or phacoemulsification, if possible, seems to be the preferred method. This approach will probably be sufficient in many cases. In patients with significant vitreous opacities and CME, combined cataract extraction and vitrectomy is probably the preferred surgical approach. In the latter cases, while a pars plana lensectomy-vitrectomy approach may be performed, a limbal approach can also be used for extracapsular extraction or phacoemulsification. This approach allows partial preservation of the posterior capsule, which may facilitate the implantation of a posterior chamber intraocular lens at a later date should the pars planitis `burn out'. (Some authors consider primary intraocular lens implantation in such cases [9].) The limbal approach can be easily combined with a pars plana vitrectomy. After the cataract extraction is completed, a standard pars plana approach is undertaken. Because of the rapid capsular opacifi cation that occurs in many patients with uveitis, a primary posterior capsulectomy should probably be performed at the time of the vitrectomy. The issue of intraocular lens implantation in patients with intermediate uveitis remains controversial. Pathological studies have shown that intraocular lens haptics can erode into the ciliary body, possibly exacerbating intraocular inflammation [16, 17]. Also, polypropylene and polymethylmethacrylate have been shown to activate the complement pathway, even in eyes with no history of inflammation [18, 19], although this may be less so with polymethylmethacrylate haptics. Careful in-the-bag placement of haptics probably reduces the risk of inflammatory reaction to these substances. However, because of the chronic nature of the inflammation in many patients with intermediate uveitis, as well as the fact that most of these patients are relatively young and thus should be able to manage contact lenses well, we do not recommend intraocular lens implantation, at least initially. In patients who remain free of inflammation or CME for a prolonged period (i.e. 1-2 years), secondary posterior chamber lens implantation could be considered if the patient does not tolerate contact lenses. In summary, cataract formation is one of the most common complications of long-standing intermediate uveitis and occurs as a result of prolonged inflammation as well as from corticosteroid therapy. Good results have been obtained with cataract surgery alone (extracapsular) or in combination with pars plana vitrectomy. The combined approach is preferred by
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us in patients with significant vitreous debris and CME; cataract extraction alone is recommended in the subset of patients with minimal vitritis and little or no CME. The issue of posterior chamber lens implantation remains unresolved. As CME is the primary cause of reduced vision in these patients, aggressive pre- and postoperative treatment of this condition with corticosteroids and/or immunosuppressive agents is essential for a good visual result.
Acknowledgments Supported in part by NIH grant ΕΥ03040 from the National Eye Institute and by a grant from Research to Prevent Blindness, Inc, New York. The authors wish to express their appreciation to Anita Fernandez and Iτene Cumplido for typing the manuscript and to Ann Dawson for editorial assistance.
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References
Cataract Extraction in Intermediate Uveitis
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Ronald E. Smith, MD, Doheny Eye Institute, 1355 San Pablo Street, Los Angeles, CA 90033 (USA)
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