SURVEY OF OPHTHALMOLOGY

MAJOR Cataract

VOLUME 35. NUMBER 2. SEPTEMBER-OCTOBER

1990

REVIEW Extraction

PHILIP L. HOOPER,

in Uveitis

M.D., NARSING

Patients

A. RAO, M.D., AND RONALD

E. SMITH, M.D.

Abstract. Cataracts are known to develop at an accelerated rate in many forms of uveitis. Until recently, cataract surgery in such eyes was regarded as a hazardous procedure that yielded unpredictable and often discouraging results. Recent evidence from a number of reports suggests that newer surgical techniques and careful medical management allow a significant number of patients with uveitis to undergo cataract extraction successfully. Intraocular lens implantation using in-the-bag posterior chamber lens technique has been successful in selected cases. Careful patient selection, coupled with the use of an appropriate surgical technique, appear to be of major importance. Herein we review the current literature on cataract extraction in uveitis and provide guidelines for patient and technique selection based on the type of inflammation present. The management ofcommon surgical problems and complications is discussed, as is the role of the intraocular lens. Specific surgical techniques useful in the management of eyes with cataract and uveitis are discussed. (Sure Ophthalmol 35:120-144, 1990)

Key words. cataract juvenile rheumatoid

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Fuchs’ heterochromic cyclitis intraocular lens arthritis lensectomy pars planitis uveitis

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Cataracts are a common complication of many forms of uveitis, with incidence rates approaching 50% in uveitis associated with juvenile rheumatoid arthritis,30S52*74 pars planitis,‘j5 and Fuchs’ syn&.ome.19.2~>33,3" Cataract development in other forms of uveitis correlates best with the location and chronicity of the inflammation and with the use of corticosteroid therapy. Syndromes that are recurrent, even if they produce low grade inflammation, induce cataracts more frequently than do acute entities of short duration. Pathologically, lens abnormalities secondary to inflammation are seen initially in the anterior subcapsular epithelium, equatorial zone, or in the posterior subcapsular region.6g Changes in the anterior epithelium are commonly seen in association with posterior synechiae and consist of localized areas of cellular necrosis surrounded by a zone of hyperplastic lens epithelium. These areas appear clinically as focal opacities that directly underlie the adherent zone of the synechiae, and are therefore seldom appreciated clinically. More diffuse anterior subcapsular cataracts may be seen in association with severe inflammation in the anterior chamber. This form of cataract is seen most commonly in association with uveitis secondary to atopic dermatitis or chemical burns. Histopathologically, a multilayered

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iridocyclitis vitrectomy

fibrillar plaque is seen between the anterior capsule and the lens epithelium. This plaque has been found to be composed of metaplastic lens epithelial cells.6g Inflammation within the posterior chamber leads initially to changes in the equatorial region of the lens. These changes consist of a posterior migration of lenticular epithelium and a disruption of subjacent cortical fibers. The changes in the cortical fibers begin initially with loss of the tight junctional complexes between cells, which is followed by cellular lysis, liquefaction and the appearance of a clinical opacity. Posterior subcapsular opacities are seen when the posteriorly migrating epithelial cells undergo degeneration and hyperplasia, often associated with the development of abnormal hypertrophied “bladder” cells. The posterior subcapsular cataracts that develop in association with steroid treatment, aside from a tendency to be more localized, appear similar histopathologically.24 The precise mechanism by which intraocular inflammation produces lenticular opacities is unknown. Lenticular changes secondary to synechiae likely result from focal hypoxia and the build-up of toxic metabolites as a result of the local alteration of aqueous flow. Damage to lens fiber membranes by inflammatory by-products such as phospholipase A 120

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and other lysosomal enzymes has been shown in experimental models of uveitis.60 Other inflammatory products, such as oxygen free radicals, have also been shown to potentiate inflammation and lens damage in animal models.36 Deposition of immune complexes on the lens capsule during an inflammatory episode may also result in lens damage by activating complement and intensifying the inflammatory activity adjacent to the lens.14 The mechanism by which steroids produce posterior subcapsular cataract is also unknown. Alterations in electrolyte balance and cellular metabolism are seen following steroid administration, and may play a role in cataract development. Clinical studies have shown that the tendency to develop cataracts increases with the dose and duration of steroid treatment.73 It is likely, therefore, that cataract development in most uveitis patients is the end result of multiple factors related to the inflammation itself, the development of synechiae, and the use of steroids. The optimum management of an inflamed eye should minimize inflammation using the lowest possible longterm dose of steroids. The high incidence of cataract formation in many forms of uveitis attests to the difficulties inherent in achieving this ideal in clinical practice.

I. Historical Perspective Until the advent of corticosteroids in the early 196Os, ocular inflammation was difficult and often impossible to control, and articles discussing the results of cataract extraction in inflamed eyes reported a high incidence of severe complications.‘1*63 Intraoperative complications included intraocular hemorrhage, choroidal detachment, and vitreous loss. Postoperatively, a severe exacerbation of inflammation often ensued, resulting in pupillary membrane formation and glaucoma, or ciliary body detachment and phthisis. In many cases, the complications resulted in marked reduction of vision, or even loss of the eye.74 More recent publications have reported a marked decrease in the incidence of intraoperative complications during cataract extraction in uveitic eyes. Postoperative complications also appear to be occurring less frequently. While the precise reason for this improvement remains speculative, a major reason would appear to be an increased ability to control inflammation perioperatively. Credit must also be given to the rapid evolution in microsurgical techniques that has occurred in recent years. Despite an improvement in the overall incidence of complications, disappointing results continue to occur with appreciable frequency. Although treatment for each patient must be individualized,

121 knowledge of the likely response of an inflamed eye to cataract surgery should allow the surgeon to better manage the patient intraoperatively and during the perioperative period. This knowledge can best be attained by reviewing the current literature on cataract extraction in uveitis.

II. Review of Current Literature Several factors must be kept in mind when assessing the current literature on cataract extraction in patients with uveitis. Inflammatory sequellae may develop at relatively predictable rates in eyes with a given inflammatory syndrome. These rates do, however, vary markedly among syndromes. It is therefore important to consider each syndrome separately when assessing the results and complications encountered following cataract extraction. The majority of patients with indentifiable uveitis syndromes undergoing cataract extraction have uveitis associated with juvenile rheumatoid arthritis, Fuchs’ heterochromic iridocyclitis or pars planitis. Only in these syndromes is sufficient information available on both the natural history of uveitis and the result following cataract extraction to allow any assessment of surgical prognosis. Even within these groups, the patient population undergoing cataract extraction each year at any given medical center is too small for meaningful evaluation. As a result, large series have been accumulated over many years. But during this time, many changes have occurred in surgical technique and perioperative management, which make direct comparisons between published studies difficult. Eyes with idiopathic uveitis (i.e., no uveitis syndrome identification is possible) have such varied clinical courses that they must be further stratified based on the duration, location, and type of inflammation before even limited generalization regarding surgical prognosis can be made. Thus, although this group is the largest in many series, much more information is required before surgical results can adequately be assessed. Inflammatory activity in most patients continues following surgery. Phthisis or glaucoma, which result in significant visual loss in inflamed eyes, often do not develop for months or even years after the onset of inflammation, so short follow-up periods may not reveal surgically induced alterations in the incidence rates of these complications. Even the best retrospective studies contain potential sources of error. A large-scale prospective study could potentially solve these problems. Unfortunately, the large number of centers and time required make the cost prohibitive for such a study that would benefit a relatively small group of

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patients. In the sections that follow, we will review the current literature on cataract extraction in patients with uveitis. Based on the literature and our own experience, we suggest guidelines that should minimize the risk of complications and discuss approaches for dealing with those that do occur. Implantation of intraocular lenses in patients with uveitis and cataract is also discussed. A. CATARACT EXTRACTION IN FUCHS’ HETEROCHROMIC IRIDOCYCLITIS 1. Literature Review By far the largest volume of information regarding cataract surgery in uveitis patients concerns those with Fuchs’ iridocyclitis. The uveitis associated with Fuchs’ heterochromic iridocyclitis tends to be low grade and chronic; posterior synechiae rarely form and patients are often not aware of the disorder until complications develop or inflammation is discovered during a routine eye examination.lg If patients are symptomatic, the two most common symptoms at time of presentation are cataract and vitreous floaters.35 The reported incidence of cataract formation in Fuchs’ heterochromic syndrome ranges from 15% to 75%, with the vast majority of series reporting an incidence of around 50%.‘g827*33,35 Most cataracts developing in Fuchs’ heterochromic iridocyclitis are of the posterior subcapsular type, with the remainder being cortical or mixed.lg Patients develop a cataract after inflammation has been present in the eye over a number of years, with the majority of patients having had uveitis for greater than 15 years at the time of cataract surgery. As a result, most patients are more than 40 years old at the time cataract surgery is required. Some controversy has existed in the literature regarding the results following cataract extraction in Fuchs’ syndrome. Prior to 1967, a number of authors reported that cataract extraction in Fuchs’ syndrome was uncomplicated or associated with transient hyphema. Other authors even found that these patients tolerated surgery better than did patients with primary cataract.1g*23,33 Unfortunately, reports from this period provide little information on the length of follow-up, type of surgery, and management of inflammation pre- and postoperatively. In 1967, Ward and Hart” reported a series of nine patients with Fuchs’ syndrome in whom significant complications developed following cataract extraction. This series was selected only to show that complications can occur, and no incidence figures were provided. Six of the nine patients underwent intracapsular cataract extraction; three un-

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derwent extracapsular cataract extraction, two following capsular rupture during intracapsular extraction. Complications encountered following surgery included hyphemaj vitreous hemorrhage, intractable glaucoma and persistent vitreous haze. Shortly following this series, Norn4* published a retrospective series of 19 patients who underwent cataract extraction over the 35-year period from 1930 to 1965. These patients were followed-up for 18 months to 35 years after surgery. Six patients underwent intracapsular cataract extraction and the remainder underwent extracapsular cataract extraction, four as a result of capsular rupture during intracapsular cataract extraction. The complications encountered perioperatively included one patient who had severe inflammation and development of a pupillary membrane following surgery that was complicated by vitreous loss. Postoperative hyphema developed in three (16%). No vitreous hemorrhages were seen, and the hemorrhages that did occur in the anterior chamber resolved without significant sequellae in all patients. Five patients (26%) developed synechiae or capsular opacity, but only one patient developed severe inflammation postoperatively. Glaucoma developed postoperatively in six (32%) patients; however, in only two (11%) patients did it begin immediately after operation. Vision at the time of last follow-up was better than 20/40 in 12 patients (63%) and worse in seven patients (37%). The decreased vision was secondary to glaucoma in three patients, due to amblyopia in two patients and due to an unspecified cause in two patients. Since the Norn series (published in 1968), five additional reports that discuss cataract extraction in Fuchs’ syndrome have been published. Most of these report a significantly lower incidence of complications. In 1974, Smith and O’ConnoP reviewed patients with Fuchs’ syndrome who underwent cataract extraction between 1955 and 197 1. This series of 29 patients was composed of 23 who underwent intracapsular surgery and six who underwent extracapsular cataract extraction, two as a result of capsular rupture. The patients were followed-up for from one month to 16 years, with a mean followup of four years. No more specific follow-up information was given. Postoperative complications included one patient with vitreous loss who developed glaucoma and retinal detachment. One patient developed a hyphema that resolved without sequellae, three had transient cornea1 striae, and one had transient elevation of intraocular pressure. No patients developed a severe increase in inflammation postoperatively. Glaucoma eventually developed in three patients (10%). Vision was 20/40 or

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better in 25 (86%) of the 29 patients. Decreased vision was caused by vitreous debris in most patients. The remainder of patients with decreased vision developed this as a result of glaucoma, retinal detachment, macular degeneration or posterior capsular opacity. As part of a review on the findings in and prognosis of Fuchs’ syndrome, Liesegang35 described the results following cataract extraction in 17 patients. No information was provided regarding the type of operation performed or the length of follow-up. Seven patients (41%) developed hyphema following surgery, four (24%) developed glaucoma, three (18%) extensive synechiae, and two (12%) vitreous loss. Three patients (18%) developed pupillary membrane, five (29%) developed cornea1 edema, two (12%) cystoid macular edema, and one substantial vitritis. Insufficient information was given to determine the number of patients without complication. Visual acuity at the time of last follow-up (duration unspecified) was 20/40 or better in eight patients (47%), six patients (35%) having less than 20/200 vision. Five eyes (29%) developed poor vision as a result of glaucoma, and in one eye poor vision was the result of glaucoma and pupillary membrane formation. In 1983, Jain et a12’ described the results of cataract extraction in 2 1 patients with Fuchs’ syndrome: 17 had planned extracapsular surgery, one had planned intracapsular surgery, and three had unplanned extracapsular cataract extraction. In this series also, follow-up was unspecified and no indication was given as to the actual distribution of complications among the operated eyes. Complications encountered included hyphema in live eyes (24%), vitreous loss in one eye, and “phacoanaphylactic uveitis” in two eyes. Sixteen eyes (76%) had vision of 20/40 or better at an unspecified time following operation. In 1982, Mills and Rosen44 described the surgical results in eight patients with Fuchs’ heterochromic uveitis who underwent intracapsular cataract extraction followed by iris supported intraocular lens insertion. Follow-up varied from 16 months to two complications included hyyears. Postoperative phema in one patient and elevated intraocular pressure in four patients (50%). Only one patient developed a permanent elevation in intraocular pressure. One patient eventually developed a lentitular membrane, dense vitreous haze and hand motion vision. The remainder of the patients had vision of 20/30 or better. In 1989, Gee and Tabbara” reported on 16 patients who underwent cataract extraction. All patients had extracapsular cataract extraction and 11 also had a posterior chamber intraocular lens in-

123 serted. An unspecified number had phacoemulsification performed. Follow-up ranged from six to 48 months, with individual breakdown provided. Two patients (13%) developed transient hyphema during operation and one patient developed raised intraocular pressure. No patient developed a significant increase in inflammation postoperatively. Vision in all patients was 20/40 or better. Other published series also have reported a similar rate of visual rehabilitation with few complications.5 2. Summary and Recommendations In summary, the recurring complications seen following cataract surgery in the Fuchs’ cyclitis syndrome, in order of reported incidence, were hyphema, vitreous hemorrhage, glaucoma, and progressive vitreous opacification. Other complications such as retinal detachment, extensive synechiae formation and cornea1 edema were reported by some authors, but were not seen in other series. As in noninflamed eyes, vitreous loss during surgery was associated with an increase in the incidence of persistent inflammation, glaucoma and retinal detachment. The reported incidence of intraoperative and postoperative hemorrhage varied from 3.6% to 76%. The origin of these hemorrhages is obscure. Although abnormal vessels are seen on the iris and in the angle in patients with Fuchs’ heterochromic iridocyclitis, their presence appears to be a relatively poor predictor of the development of intraocular hemorrhage following surgery. The hyphemas that developed in eyes as a result of otherwise uncomplicated cataract extraction on the whole did not result in serious sequellae. Although recurrent postoperative hemorrhages were described occasionally, only one series reported the development of total hyphema or vitreous hemorrhage following uncomplicated cataract extraction. The more recent articles reported the lowest incidence of hyphema, which may reflect the use of microsurgical techniques and a switch to extracapsular surgery. The most visually significant reported complication of cataract extraction in Fuchs’ eyes appeared to be the development of glaucoma. Permanent elevation of intraocular pressure was reported to develop at some time following operation in 3% to 35% of these eyes. However, permanent intraocular pressure elevation has been reported to develop in approximately 1 5%-25%34,35 of unoperated eyes with Fuchs’ heterochromic iridocyclitis. Therefore, reported incidence rates following surgery may actually reflect only the natural history of the disease. Review of the literature reveals that, initially, control of intraocular pressure can be achieved by medication alone, but that eventually up to 70% of

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patients will require filtering surgery.95 In one series, this was successful in 60% of patients.“’ In those series that reported a significant number of patients with vision of 20/40 or less postoperatively, a major reason for visual loss was progressive vitreous opacification. The progression of vitreous opacification did not appear to be altered following surgery. This process appeared to be poorly responsive to steroid therapy. Patients with Fuchs’ heterochromic iridocyclitis do not require more than periocular steroids in addition to frequent topical steroid drops to control inflammation resulting from uncomplicated cataract extraction. An increase in inflammation can be seen in the immediate postoperative period, but this usually decreases to preoperative levels with time. Cataract surgery in patients with Fuchs’ heterochromic iridocyclitis is indicated when there is significant cataract-induced visual decrease. While most patients have had visual acuities of less than 20/200 at the time of surgery, earlier surgery can be justified on the basis of vocational or avocational need. Aside from the use of a careful microsurgical technique that avoids excessive manipulation of the iris, few special precautions appear to be needed to ensure successful results from surgery. Pupillary mobility should be maintained in the postoperative period, and intraocular pressure should be closely monitored. While no definitive studies address the issue of the preoperative use of corticosteroids, it is probably a good practice to use topical corticosteroids for a few weeks prior to surgery to achieve minimal or no evidence of anterior chamber reaction at the time of surgery. Postoperative corticosteroids are also recommended. Intraocular lens implantation in these patients will be discussed later in this review. B. CATARACT EXTRACTION IN UVEITIS ASSOCIATED WITH JUVENILE RHEUMATOID ARTHRITIS 1. Literature

Review

Another insidious form of intraocular inflammation is seen in uveitis associated with juvenile rheumatoid arthritis (JRA). Like inflammation associated with Fuchs’ syndrome, the chronic nongranulomatous anterior segment inflammation associated with this syndrome is often asymptomatic until complications supervene. The complications developing from this syndrome are more severe, however. Commonly seen complications include band keratopathy, extensive posterior synechiae, and hypotony or glaucoma, in addition to cataract. Rehabilitation of the 40-60% of these eyes

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that develop cataract is considerably more difficult than in eyes with Fuchs’ heterochromic iridocyclitis.28,3’,74Most patients with this syndrome develop intraocular inflammation in the first decade of life. As a result, cataracts develop at an earlier age with this form of uveitis than with other forms of ocular inflammation. Young eyes respond to surgery with considerably more inflammation than do eyes of older patients. As a result, even in noninflamed eyes, techniques that are successful in adults do not always work well in patients in this age group. In addition to making surgery more difficult, the young age at which media opacities develop makes the development of amblyopia a distinct possibility in many patients. Postoperatively, therefore, these patients may require treatment for amblyopia in addition to the usual anti-inflammatory medications. A number of factors combine to make cataract surgery more hazardous in JRA patients. A marked tendency to form synechiae is seen, with 43 to 61% of patients eventually developing this complication.74 Although glaucoma is common in this syndrome, with a reported incidence of about 25% in most studies, some eyes are hypotonous by the time cataract extraction is contemplated.32~6”~74 In addition to making surgery technically more difftcult, hypotony is associated with an increased risk of postoperative choroidal effusion, macular edema, and phthisis. Fewer reports describing the results of cataract extraction in eyes with JR&associated uveitis exist than are available for eyes with Fuchs’ syndrome. The reports again span several decades and encompass a variety of surgical techniques. In a 1974 review of 76 children with JRA-associated uveitis, SmileyGS reported the results of cataract extraction that had been performed on 18 eyes. Limited information was provided regarding the method of surgery. Some eyes had intracapsular cataract extraction, some were needled, and some aspirated. Follow-up was reported to be from 2-5 years. The complications that developed following surgery were not specifically enumerated, but only three eyes (17%) retained 20/40 or better vision during follow-up. Two of these eyes were described as inflamed, one of which had secondary glaucoma. Twelve eyes (66%) were totally blind live years after surgery. Perioperative control of inflammation was not discussed aside from a brief mention that subconjunctival steroids were not helpful. A 1975 review by Key and Kimura3’ provided results on 23 JRA patients who underwent cataract extraction. Results from this series were much more encouraging than those from the earlier report. In 20 eyes the cataract was removed by needling and

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aspiration or an unspecified extracapsular procedure, while three had intracapsular cataract extraction. No information regarding the duration of follow-up was provided. Aside from a brief reference to persistent inflammation and macular degeneration, the complications encountered were not specified. Three eyes (13%) attained 20/20 vision, nine (39%) had 20/40 to 20/100 vision, and 11 (48%) retained less than 20/100 vision. Periocular and systemic steroids were used perioperatively, but no information was provided regarding the dosages used. The surgical results following phacoemulsification alone in eleven eyes were presented by Praeger and colleagues55 in 1976. An intensive regimen of perioperative steroids was used, which included intramuscular steroids preoperatively, followed by intravenous Solu-Cortef @ intraoperatively and for four days postoperatively. Oral steroid was begun in a dose of 60 to 75 mg every three hours on day live and reduced by 2.5 mg every three days if inflammation was under control. In addition, 80 mg of Depo-Medrol@ was given subconjunctivally and topical steroid drops and ointment were applied every two hours around the clock. Conventional surgical technique was used with the posterior capsule scraped and left intact. Patients were followedup for approximately six months to three years. Complications encountered included transient glaucoma, which developed eight months postoperatively in two eyes (18%). Significant opacification of the posterior capsule occurred, but no patients required capsulotomy. No patients developed severe postoperative inflammation. The visual result following operation was 20/20 in three eyes (27%), 20125 in five eyes (45%) and 20130 in three eyes (27%). The largest series of patients with juvenile rheumatoid arthritis who had cataract extraction was reported by Kanski and Shun-Shin.‘” This 1984 report totalled 162 eyes, 61 of which had the cataract removed by needling and aspiration, and 101 of which had lensectomy and limited anterior vitrectomy. The only complication for which information was provided was phthisis. In the needled group, 15 eyes (25%) were phthisical, and in the group that had lensectomy-vitrectomy, only two eyes (3%) became phthisical after follow-up of between six months and eight years (no further breakdown of data was provided). Vision was hand motion or less in 15 lensectomized eyes (15%), 30 (30%) had vision of 20/400 to count fingers, and 56 (56%) had vision better than 20/60. In eyes that were operated on by other methods, 29 eyes (48%) had vision of hand motion or less, 19 eyes (3 1%) had vision of 20/400 to count lingers, and only 13 eyes (21%) attained

125 20160 or better vision. No information was given regarding the average duration of follow-up in these groups. The techniques used to suppress inflammation perioperatively were not specified. A recent report by Foster et ali8 reviewed their results following extracapsular cataract extraction and intraocular lens implantation in patients with uveitis. A comparison group of eleven patients who had extracapsular cataract extraction alone included six patients with JRA-associated uveitis. In this group, 67% of the patients achieved 20/40 or better vision postoperatively. Breakdown on the JKA patients alone was not provided. Follow-up in this group varied between one and seven years. Mild recurrence of inflammation was seen in two patients, synechiae to the posterior capsule were seen in two patients and one patient developed posterior capsule opacification. Intensive preoperative and postoperative corticosteroid treatment was used and is recommended by Foster and colleagues. A number of authors have discussed the results of lensectomy combined with a complete vitrectomy in small numbers of patients with cataract secondary to the uveitis of juvenile rheumatoid arthritis. A 1979 report by Diamond and Kaplan” described two patients with JR4 who underwent pars plana lensectomy-vitrectomy. The visual result was 20/25 in one patient and 20/70 in the other. No increase in inflammation was seen following surgery. However, these patients received oral, periocular and intravitreal steroids. Follow-up in this series ranged from six to 38 months, but was not stated for the two patients with JRA. Algvere and colleagues’ in 198 1 reported a group of four eyes of patients with JRA that underwent vitrectomy and lensectomy or pupillary membrane removal. Two patients were followed-up for two months, one for six months and one for one year following surgery. Patients received oral prednisone preoperatively and subtenon steroids at completion of surgery. Vision was unchanged in one patient, improved to 20/200 in two patients, and to 20/100 in one patient. Inflammation was not appreciably changed following operation and no complications occurred. At our institution, Nobe and associates4’ reported similar results in two eyes followed for seven and 15 months, respectively, following surgery. Both eyes showed improvement of vision with acuity limited to 20/50 by macular edema and amblyopia in one eye and to 20/80 by macular edema and maculopathy in the other. Petrilli and colleagues5* included two patients with uveitis associated with JRA in their 1986 review of the results of lensectomy-vitrectomy. Patient follow-up for the total group was greater than 12

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ET AL

1

Results of Caturact Surgery in Various Uveitic Entities Results of Surgery

Uveitis Syndrome Fuchs’ heterochromic docyclitis

iri-

Uveitis associated with juvenile rheumatoid arthritis

Pars planitis

Lens-induced inflammation (e.g., phacoanaphylaxis) Other forms

Recent series report vision 20140 or better in over 85% of patients; series in which IOL was used did not report higher incidence of complications. Visual results following surgery depended on macular status; recent series reported 60-75% eyes with 20/40 or better vision. Visual results following surgery dependent on macular status; approximately 50% of eyes achieved 20/40 or better vision. Visual results good following prompt surgery to remove all lens material Fair results in inactive or controlled nongranulomatous inflammation; results of surgery on eyes with granulomatous inflammation variable

months, but no specific information regarding follow-up was provided. One eye attained 20130 vision following surgery; the other eye was amblyopic and vision improved only to counting fingers. No specific information was given regarding the course of inflammation following surgery. Two other series included single patients with juvenile rheumatoid arthritis who had cataract extraction.**” No untoward complications from surgery were encountered. Vision was limited in one eye by band keratopathy and in the other by macular edema. A series of 10 eyes has been compiled by Flynn et a1.16These patients all underwent pars plana lensectomy and vitrectomy using standard techniques. Patients were all given triamcinolone 20 mg and dexamethasone 2 mg subconjunctivally at the completion of surgery. Complications encountered following surgery included hypotony, which was present in four eyes (40%) at from one to six weeks postoperatively. One of these eyes developed choroidal detachments, which ultimately resolved. One patient developed a vitreous hemorrhage and one a

Complications Encountered

Use of Intraocular

Lens

Intraocular hemorrhages (3.676%); glaucoma (3-35%); progressive vit-

Good results recently re-

Glaucoma (20%); hypotony (incidence of transient or permanent hypotony up to 40%); chronic macular edema was major cause of permanent visual loss. CME significant complication after surgery (half of patients); can develop post-op, but often preexisting; glaucoma reported in 10% CME and glaucoma, if chronic

Not reported

ported

reous opacihcation

Synechiae; glaucoma; CME

Reported in small number of patients; limited follow-up

Lens implantation as primary procedure avoided except in phacolytic glaucoma In nongranulomatous uveitis, results with short-term follow-up fair if inflammation absolutely controlled pre-op; few patients with granulomatous uveitis reported

Iibrinoid reaction and pupillary membrane. These complications resolved without sequellae. Two eyes (20%) eventually developed glaucoma during follow-up. Three eyes (30%) had macular edema visible at the time of surgery; this resolved in one eye and persisted in two others. Two other eyes developed mild chronic macular edema. Eyes were followed-up for six months to five years following surgery. All eyes showed improved vision after surgery, with seven eyes (70%) maintaining better than 20/40 vision. The remaining eyes had vision of 20/50,20/60 and 20/70 as a result ofglaucoma, maculopathy and persistent macular edema, respectively. 2. Summary and Recommendations The surgical results of cataract extraction in patients with uveitis associated with JRA are not as encouraging as are those in patients with Fuchs’ syndrome. Problems that limit postoperative vision include glaucoma, hypotony, and macular edema. In the larger series, glaucoma developed in approximately 20% of operated eyes; it was a late com-

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plication, which occurred months to years postoperatively. As a result, series with short follow-up periods may not note the true incidence of this complication. Incidence rates for postoperative glaucoma parallel those in unoperated eyes. The delayed onset and parallel incidence rates suggest that, similar to the situation in Fuchs’ syndrome, risk for the development of glaucoma is unchanged by surgery. Hypotony is a more serious complication and was usually seen in the immediate postoperative period. The development of hypotony in these eyes may reflect an occult ciliary body detachment, persistent cyclitic membrane, or hyposecretion secondary to postoperative inflammation. Unless a cyclitic membrane was present, hypotony usually resolved over a 6-8 week period. As will be discussed subsequently, chronic macular edema is often seen in these eyes preoperatively or is discovered at the time of operation. Most series report this to be the major cause of persistent decreased visual acuity. The addition of a limited vitrectomy in combination with lens removal resulted in a decrease in the incidence of phthisis from 25% to 2% in the series of Kanski and Shun-Shin.‘* A similar low incidence of severe complications was reported in other series where lensectomy was combined with a complete vitrectomy. In addition, the steroid regimen required to control inflammation in these eyes appeared to be less rigorous than that required following other surgical techniques. However, the poor results following other techniques of cataract extraction may have reflected the inadequacies of older regimens of inflammation control or the lack of microsurgical technique, as Praeger and colleagues have suggested.“5 The reports by Foster’7.‘8 add additional credence to this point of view. At present, we would recommend attempting extracapsular surgery alone only on eyes with no vitritis or vitreous opacities and with normal intraocular pressure. If vitritis or vitreous opacities are present, with hypotony or evidence of cyclitic membrane formation, combining anterior segment surgery with a pars plana vitrectomy would seem to be a According to Foster and colsafer approach. leaguesI eyes with moderate vitritis without hypotony or cyclitic membrane may respond favorably to extracapsular cataract extraction alone, so long as the vitritis has been controlled absolutely for at least three months. Such eyes may show a significant increase in vitritis postoperatively, and should therefore be closely monitored. If inflammatory debris begins to organize in the vitreous cavity, vitrectomy should be done. Treatment with systemic, topical, and periocular steroids is recommended during the perioperative

127 period for all eyes with uveitis associated with JRA that undergo cataract extraction. Surgery should be delayed until the anterior chamber is free of inflammatory cells (“flare” will persist). C. CATARACT EXTRACTION PARS PLANITIS 1. Literature

IN

Review

Inflammation in eyes with pars planitis is limited primarily to the posterior segment, although mild iridocyclitis is present in some cases. This contrasts markedly with the inflammation seen in Fuchs’ syndrome, or JRA-associated uveitis, which is located primarily anterior to the lens. As the anterior chamber is largely free of inflammation in pars planitis, synechiae seldom develop and the incidence of glaucoma is Iow.~~ The chronic inflammation of the vitreous and ciliary body seen with pars planitis often persists with a remittent course over many years and results in exudate formation within the vitreous and along the inferior pars plana. As a result of this chronic inflammation in close proximity to the lens, cataracts eventually develop in 40% of patients with pars planitis. 65 Lens opacities first develop as a diffuse haze in the posterior subcapsular region. A large percentage of the cataracts remain at this stage; only half of the cataracts in one large series eventually became visually significant.G5 There is little published data available that assesses the results of intracapsular versus extracapsular surgery alone in patients with pars planitis. In a 1976 review of the complications encountered in 100 patients with pars planitis, Smith et al@’ reported on 20 eyes that underwent cataract extraction. Intracapsular or extracapsular technique was used, and patients were followed for up to four years following surgery. No information was provided regarding the regimen used to control inflammation perioperatively. Two eyes (10%) developed glauoma postoperatively, but no other major complications were noted. In the group as a whole, 13 patients (65%) achieved vision better than 20/40. No breakdown was provided by type of surgery or preoperative vision. Foster,18 in a 1989 review, included four eyes with pars planitis that underwent extracapsular cataract extraction followed by posterior chamber lens implantation. These eyes were followed-up for between 18 months and three years. Postoperative vision improved an average of seven Snellen lihes, two eyes achieving 20140 or better and two restricted to 20/70. Poor vision was felt to be the result of macular edema. Other authors, citing the poor results that follow conventional surgery in eyes with active inflamma-

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tion, have chosen to combine vitrectomy with lens extraction. In Diamond and Kaplan’s9 1978 series on lensectomy-vitrectomy, four eyes had uveitis diagnosed as pars planitis. Inflammation had been present from 4-19 years preoperatively and postoperative follow-up averaged approximately one year in all patients. Patients received oral prednisone and topical steroids for four days preoperatively, and surgery was carried out only if less than 1 + cell and flare was seen in the anterior chamber preoperativeiy. Intravitreal steroids were given at the completion of surgery and oral and topical steroids were continued for five days postoperatively at preoperative levels, then tapered and discontinued. No postoperative complications were encountered. The postoperative vision was 20140 or better in three eyes; the remaining eye had vision of 20/70 as a result of retinal edema. In a series of 19 eyes that underwent lensectomyvitrectomy for uveitic cataract, reported by Tutein Nolthenius and Deutman” in 1983, eight eyes were diagnosed as having pars planitis. Eyes were “mostly quiet” at the time of surgery. No information was given regarding preoperative steroid use in these patients. Surgery was performed via the pars plana and an encircling band was placed anterior to the equator in all cases. Peripheral cryoablation was also performed. Subtenon injection of steroids was done at the completion of surgery. Patients were followed-up for 6-19 months postoperatively, with most patients having longer than one year of followup. Four eyes were noted to have macular edema following surgery and two eyes contained epiretinal membranes. No complications of surgery were encountered in any patient. Vision of 20140 or better was achieved in four eyes (50%) and 20/100 or better in three more (38%). In 1985, Girard and associates** reported on the surgical results in six eyes with pars planitis that underwent pars plana lensectomy-vitrectomy. Their patients had uveitis for from 3-13 years preoperatively and were followed-up for l-7 years after operation. No specific preoperative steroid regimen was used and no details were given regarding the amount of inflammation present at the time of operation. All patients received steroids subconjunctivally at the completion of surgery. Oral and topical steroids were gradually tapered following surgery. Four patients (67%) had vision better than 20140 after surgery; one patient had vision of 201200 secondary to previous chorioretinitis, and one patient had vision reduced to 20/100 by macular edema. No complications occurred in any patient. A 1988 report by Mieler et a143 on the use of vitrectomy in the management of pars planitis de-

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scribed eight eyes that had cataracts removed as a primary or secondary procedure. Six eyes had pars plana lensectomy combined with vitrectomy and two underwent extracapsular surgery following vitrectomy. Eyes were followed-up for 6-90 months postoperatively, with an average follow-up of 22 months. Vision in four of the eight eyes improved to 20/40 or better. Two of the remaining eyes had vision of 20/100 postoperatively and vision in the others was 20/80 and 20/50, respectively. In three of the four eyes with poorer than 20/40 vision after surgery, acuity was limited by cystoid macular edema. Two eyes developed vitreous hemorrhage postoperatively. In one eye, this required a repeat vitrectomy. No other complications were seen. 2. Summary and Recommendations Macular edema was the major complication encountered following surgery in pars planitis patients and was also the major cause of poor vision. This complication was seen to some degree in almost half of the eyes that underwent cataract extraction and was responsible for 80% of the eyes with less than 20/40 vision. Few other complications were seen and inflammation appeared to remain under control following surgery. The incidence of glaucoma in these eyes after surgery averaged around 1O%, which closely parallels the natural rate of glaucoma in this syndrome.65 Most patients had improved vision after their cataracts were removed. Approximately 50% of eyes achieved 20/40 or better vision postoperatively. However, the ultimate vision depended upon the macular status. The best management of macular edema and the optimal timing for surgery remain unknown in these patients. Following surgery, vision in a sizable number of eyes has been limited by macular edema or macular pathology, which in most cases has been said to exist at the time of cataract extraction. It is tempting to speculate that more aggressive, early treatment of inflammation and macular edema might have resulted in improved acuity following cataract extraction. Whether lensectomy-vitrectomy alters the course of inflammation and, more specifically, the course of macular edema after surgery remains a controversial issue. As will be discussed subsequently, there is some evidence to suggest that surgery has a favorable influence on vitreous inflammation and macular edema in patients undergoing lensectomyvitrectomy.‘0~2’~z2The number of patients with pars planitis is too small and follow-up is too varied to draw any conclusions for this group of patients alone. Data on the results of conventional surgery in pars planitis patients is so limited that no conclusion

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can be drawn from the literature regarding the efficacy of extracapsular surgery. By the time a cataract develops, most patients have significant amounts of vitreous debris, which may well compromise the final outcome of cataract extraction alone. Our own experience suggests that eyes with pars planitis but minimal inflammatory activity appear to tolerate anterior segment surgery alone without significant complication. Most patients, however, have marked vitritis, and/or vitreous opacities with macular edema, and therefore benefit from vitrectomy, as well. D. CATARACT LENS-INDUCED

EXTRACTION

IN

INFLAMMATION

1. Literature Review

Lens-induced inflammatory syndromes differ from those considered previously because in these conditions the inflammation itself occurs as a result of an immune or other inflammatory response to a damaged cataract lens. Three clinical syndromes can be seen as a result of these inflammatory responses. The first, often termed phacoanaphylactic endophthalmitis orphacoantigenic uveitis, appears as a granulomatous uveitis that is often severe. Inflammation usually begins 1-14 days following lens capsule rupture, but can be delayed for months or years after the initiating trauma. 5 Pathologically, a zonal granulomatous reaction to lens material is seen, along with extensive infiltration of iris, ciliary body and adjacent choroid by lymphocytes, plasma cells, epithelioid cells and giant cells.5 Experimental evidence suggests that the disease occurs as a result of localized immune complex deposition on the exposed lens materia1.37,57*62 The second lens-induced syndrome was initially termedphacototic uveitis by Irvine and Irvine.“j This is the form of lens-induced uveitis that is most often misdiagnosed as chronic nonspecific uveitis when it occurs following extracapsular cataract extraction. It presents as a nongranulomatous uveitis, often associated with a fibrinous exudate, that tends to be persistent and to respond incompletely to steroids. Pathologically, the tissues of the anterior uveal tract are seen to be infiltrated largely by lymphocytes and plasma cells. 57 The epithelioid and giant cells seen in phacoanaphylaxis are absent. However, recent data suggest that the underlying pathologic mechanism in the two entities is the same,57 and that they represent varying degrees of immune response to lenticular antigens. The third lens-induced syndrome, phacolytic glaucoma, usually occurs in the setting of a hypermature cataract. This syndrome produces minimal obvious inflammation or keratic percipitates; however, large cells and white clumps may be seen in the

129 anterior chamber and in the angle.*“34 Cytology reveals these cells to be macrophages, containing lens protein. Few lymphocytes can be seen. Glaucoma appears to occur as a result of mechanical obstruction of the trabecular meshwork by macrophages and high molecular weight complexes of lens protein.‘* The diagnosis of a lens-induced inflammatory syndrome is made initially by a high index of suspicion. With few exceptions, phacoanaphylaxis has been reported to occur only after ocular trauma or surgery, or in association with sympathetic ophthalmia.5,7,5g More severe forms of this uveitis may be confused with bacterial or fungal endophthalmitis. The presence of granulomatous keratic percipitates is more suggestive of phacoanaphylaxis than of infection, but these are not always seen. Patients with phacoanaphylaxis tend also to have less pain and better vision than do patients with infectious endophthalmitis. In most situations, unless the diagnosis is clear-cut, it is best to assume that infection is present, obtain appropriate cultures and begin treatment with antibiotics.‘j* If cultures remain negative after three days, and there is no clinical response to antibiotics, topical and systemic steroid treatment can be started. Treatment with steroids usually only moderates an intense phacoanaphylactic inflammatory response, and surgical lens removal should be carried out as soon as the diagnosis is made and the eye is as quiet as possible. The more mild nongranulomatous lens-induced uveitis is often treated for an extended period with steroids before the diagnosis is suspected. In many of the reported cases, surgery was carried out only when medical management failed to adequately control the inflammation; thus, many mild cases may remain undiagnosed. Recently, chronic intraocular infection with anaerobic organisms has been shown by several authors to mimic this chronic mild form of lens-induced uveitis.‘4~4”2,50,“4~64The most frequently isolated anaerobe has been Propionibacterium acnes, which is gram positive and usually sensitive to penicillin and clindamycin.‘3,50 In light of this, all cultures obtained during the work-up of a patient with chronic post-surgical uveitis should be done both aerobically and anaerobically. While a detailed discussion of chronic diffuse or localized postoperative endophthalmitis is beyond the scope of this article, it is important that the ophthalmologist recognize that such infections can mimic otherwise clinically typical lens-induced uveitis. Phacolytic glaucoma is seen usually in association with a hypermature cataract and is an uncommon finding after trauma or surgery.12 Intraocular pressures are commonly very high and the cornea is often cloudy at the time of presentation. Control of intraocular pressure can be accomplished using

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beta blockers, carbonic anhydrase inhibitors and hyperosmotic agents. Once the intraocular pressure is controlled, the eye often appears only minimally inflamed, in spite of the presence of large aggregates of cells in the anterior chamber. Anterior chamber paracentesis reveals the macrophages that are characteristic of this syndrome.‘” Although initial medical management to control intraocular pressure and reduce inflammation is critical in stabilizing eyes with any of these lensinduced syndromes, once the diagnosis has been made, definitive treatment is lens extraction. The goal of surgery is to remove as much as possible of the inciting lens material. Techniques used to achieve this goal have varied. Earlier articles report success using irrigation alone to wash out remaining cortical material when phacoanaphylaxis developed following extracapsular cataract extraction. In a 1965 article, Riise5’ described two patients who, following extracapsular cataract extraction, developed severe inflammation that could be only partially controlled with steroids. Irrigation of cortical remnants was not possible. Following removal of the entire lens capsule, along with the remaining cortical material contained within it, the inflammation subsided and steroid treatment could be withdrawn. No pathologic cotifirmation of lens-induced inflammation was provided. In 1976, Smith and Weiner” described a patient who developed severe inflammation three months after phacoemulsificatidn. Irrigation and aspiration of the remaining cortical material cleared the inflammation; however, extensive posterior synechiae and an opaque capsule limited final vision. Pathologic examination was consistent with phacoanaphylaxis. A 1987 report by Abrahams’ described two patients who developed granulomatous uveitis six months and one year, respectively, after extracapsular cataract extraction and lens implantation. Both patients were treated by first removing the intraocular lens after creation of a sector iridectomy and synechiolysis. Alpha chymotrypsin was instilled and the entire lens capsule removed. An anterior vitrectomy was performed and the eye closed. Inflammation subsided following surgery and vision returned to 20125 in one patient and to 20140 in the other. Pathologic examination confirmed the diagnosis of phacoanaphylaxis. Definitive treatment of phacolytic glaucoma is lens removal, generally by intracapsular technique. Most authors have reported good success both in control of intraocular pressure and in recovery of vision following this procedure.12 A recent retrospective report by Lane and colleagues34 describes five patients with phacolytic glaucoma who were treated by extracapsular cata-

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ract extraction and intraocular lens insertion. Patients were followed for five months to three years after surgery, with most having approximately six months of follow-up. Following surgery, the intraocular pressure in all patients was 20 mm Hg or less without medical therapy, and vision was 20/50 or better. No surgical complications occurred. Gross and Pearce25 also described successful extracapsular surgery in such a patient. 2. Summary Lens-induced uveitis should be suspected in any patient who develops persistent inflammation after surgery or ocular trauma, particularly if the inflammation is granulomatous. If suspected, bacterial and fungal infection should be ruled out by appropriate cultures. P. acne~ is emerging as an important pathogen in selected cases of chronic postoperative inflammation.3*49’334M2,54.” The surgical management of eyes with lens-induced uveitis has varied. Because these eyes show a marked tendency to form synechiae and cyclitic membranes, a technique that combines vitrectomy with complete removal of lens remnants is most likely to be successful. Extracapsular techniques should also be considered if complete cortex removal is possible. Intracapsular extraction techniques are successful in curing phacolytic glaucoma. Recent reports suggest that careful extracapsular surgery also can be performed. This technique, which maintains the barrier of an intact posterior capsule and allows intraocular lens implantation, is probably now the treatment of choice considering the availability of microsurgical techniques. E. CATARACT EXTRACTION IN IDIOPATHIC AND OTHER FORMS OF WEITIS 1. Literature

Review

As mentioned previously, cataract formation due to uveitis is correlated with the duration and location of the inflammatory activity in an eye. Aside from entities that produce only choroiditis, all forms of uveitis are capable of producing lens opacities. The largest number of uveitic cataracts will be seen in association with so-called idiopathic nongranulomatous uveitis. This form of uveitis can be further subdivided into two groups on the basis of HLA B27 antigen positivity. Patients who are HLA B27 positive may subsequently be discovered to have a spondyloarthropathy or inflammatory bowel disease. Granulomatous uveitis is seen less frequently and so, numerically, is associated with fewer cataracts. The incidence of cataract formation in many of

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these entities is, however, higher than that seen with nongranulomatous uveitis. This group includes patients with uveitis associated with sarcoidosis, toxoplasmosis, Vogt-Koyanagi-Harada syndrome and sympathetic ophthalmia. Many eyes in this group will have chronic inflammation or recent inflammatory activity. This inflammatory activity often produces synechiae, glaucoma, and vitreous opacities in addition to cataract. These complications, in turn, increase their risk of untoward events following cataract surgery. Little information has been published in the literature that allows assessment of intra- or extracapsular cataract surgery alone on these eyes. For the purposes of discussion, we will subdivide this large group of patients into three smaller groups. The first group consists of eyes with intermittent “nongranulomatous” anterior uveitis that is inactive at the time of cataract surgery. This group of patients has been the subject of few formal reports. Duke-Elder” and Smith and Nozik6’j both reported that these patients do well following conventional surgery as long as inflammation has been absent for at least two to three months preoperatively. Most of this evidence is anecdotal, however. Foster et al’* described 16 patients with this type of inflammation; nine had idiopathic inflammation, five had inflammation associated with ankylosing spondylitis, and two had inflammation associated with inflammatory bowel disease. All patients had fewer than O-2 anterior chamber cells/O.2 mm of high slit beam for at least three months preceding surgery. This low level of inflammation was achieved by various regimens of therapy that were tailored to the individual patient. Cataracts were removed by extracapsular techniques, including phacoemulsilication, and 14/16 eyes had posterior chamber intraocular lenses implanted. Vision was improved in all cases, with most eyes achieving 20/40 or better visual acuity. Few complications were noted; the most serious appeared to be the development of posterior synechiae, and in 6/14 eyes (43%), macular pathology was seen postoperatively. The second group consists of patients with nongranulomatous inflammation that is active at the time of surgery. These patients are felt to be poor surgical candidates based largely on the poor results seen in early studies of cataract extraction in juvenile rheumatoid arthritis. No study has been reported that systematically assesses the response to conventional cataract sureerv in these oatients. Patients with granulomatous uveitis make up the third group of patients. In most conditions that produce granulomatous uveitis, a panuveitis develops which results in a significant vitritis. Even when minimally inflamed, these eyes can respond to sur-

131 gery in an unpredictable manner. In 1983 Reynard and Minckler5’ reported the results of cataract extraction in six sympathizing eyes of patients with sympathetic ophthalmia. All eyes showed minimal inflammation at the time of surgery. Two eyes underwent intracapsular cataract extraction, three extracapsular cataract extraction, and one cataract was needled. Following surgery, all of these eyes required steroid treatment during follow-up to control recurrences of inflammation. Uncontrolled inflammation led to the formation of cyclitic membranes or phthisis in three eyes in spite of corticosteroid therapy. Two eyes (33%) achieved visual acuity of better than 20140 during follow-up period, which ranged from one to 23 years. The three eyes with severe postoperative inflammation retained only light perception vision; one eye, with chronic inflammation and macular edema, retained 20/100 vision. The report by Foster et al’” included 10 patients with granulomatous uveitis. These patients were managed in a fashion similar to their patients with nongranulomatous inflammation. Vision improved in all patients; however, 3/7 (43%) patients for whom detailed data were provided were noted to have macular pathology postoperatively. 2. Summary and Recommendations In summary, it appears that patients with nongranulomatous inflammation who have had minimal or no inflammatory activity for at least two to three months prior to surgery respond well to carefully performed extracapsular cataract extraction. Postoperative exacerbations of inflammation can usually be controlled by increasing the anti-inflammatory medication used preoperatively. Eyes with active nongranulomatous inflammation should have surgery postponed until the inflammation has been controlled for 8-12 weeks. The cataract can then usually be removed by extracapsular extraction with careful removal of all cortical remnants. Corticosteroid treatment should be continued. Although the report of Foster et al”’ is encouraging, the number of reported cases of extracapsular cataract extraction in eyes with granulomatous uveitis remains too small to draw meaningful conclusions about the predictability of the outcome. Achieving control of inflammation in these eyes during the perioperative period remains difftcult. F. ROLE OF LENSECTOMY VITRECTOMY

COMBINED

WITH

1. Literature Review Reports of combined lens extraction with vitrectomy in inflamed eyes first appeared in the literature in 1978.’ The development of this technique was spurred by dissatisfaction with the unpredict-

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able results that often followed cataract extraction in eyes with many forms of uveitis. Posterior segment complications following surgery account for a majority of uveitic eyes with poor vision after cataract extraction. The potential for some of these complications to develop should be decreased following vitrectomy. For example, inflammatory membranes use the vitreous framework as a scaffold. By removing this support, such membranes should occur less frequently, and, without vitreous connections, those that do form should produce less traction on the retina and ciliary body, thereby decreasing the incidence of retinal detachment and hypotony. Following episodes of ocular inflammation the vitreous has been shown to contain clones of cells that will respond to the antigenic stimulus that incited the original inflammation.6’ An eye containing these cells is therefore “primed” to respond in an accelerated fashion to any subsequent challenge with that or a similar antigen. By removing these cells, vitrectomy theoretically may favorably modify the course of intraocular inflammation following subsequent antigen exposure. Pilot studies in rabbit eyes using a protein-induced model of uveitis demonstrated that a combined procedure of lens removal and vitrectomy could be accomplished in uveitic eyes without producing unacceptable sequellae.50~3’ A number of studies that report the results following the use of this technique on human patients have appeared in the literature. All studies are composed of patients who have uveitis due to a variety of causes, whose eyes have been inflamed for various periods of time, and who have been followedup for various durations, all of which must be kept in mind as the results of these studies are reviewed. In 1978, Diamond and Kaplan9 reported results following lensectomy-vitrectomy on a group of 15 eyes from patients with various types of uveitis. In four patients the uveitis was diagnosed as pars planitis, and in one each the diagnosis was BehGet’s disease, ankylosing spondylitis, atopic dermatitis and Reiter’s syndrome. The remaining patients had idiopathic iridocyclitis. All patients had vision less than or equal to 20/200 prior to surgery and all patients were given topical and systemic prednisone for four days preceding surgery. Inflammation had been inactive for at least four weeks prior to surgery and all but one patient had less than 1 + cell and flare in the anterior chamber preoperatively. Lensectomy and vitrectomy were performed through a scleral incision and all patients received 40 u.g of dexamethasone intravitreally at the completion of surgery. Oral steroids were tapered postoperatively and were discontinued in all patients by

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four weeks after surgery. Patients were followed-up for 4-27 months, with half of the eyes being followed-up for approximately one year. The complications encountered consisted of retinal tears which developed four months postoperatively in one patient and macular edema which developed in another patient nine months postoperatively. In eight eyes (53%), vision improved to 20125 or better following surgery. Vision was 20170 in two eyes, 20/100 in two eyes, 20/200 in one eye, and 201400 in the remaining two eyes at the time of last follow-up. All eyes were visually improved following surgery. Macular pathology was present in all eyes with poor vision, with six eyes having macular edema and another eye having macular pucker. In all but one of these eyes, the changes were present at the time of surgery. No patient developed a severe exacerbation of inflammation following surgery. A subsequent report by the same authors was published in 1979.” This series was composed of 25 eyes: seven from patients with idiopathic iridocyclitis, eight from patients with pars planitis, three from patients with adult rheumatoid arthritis, two from patients with juvenile rheumatoid arthritis, and one each from patients with ankylosing spondylitis, atopic dermatitis, Behcet’s disease, Reiter’s syndrome, and toxoplasmosis. As in the first group, all patients had vision less than 20/200 preoperatively. No more than 2 + cell and flare were present in the anterior chamber prior to operation. The medications used before, during, and after surgery were the same as were used in the patients described in the first report. Retinal detachments developed in three patients (12%) during or following surgery; all were reattached successfully. One of these eyes developed transient choroidal ischemia after scleral buckling surgery. Another eye was noted to have extensive tractional retinal detachment at the time of surgery. Following repair, this eye also developed choroidal ischemia and macular edema, which persisted. Ten eyes (40%) were hypotonous prior to surgery; this resolved in all but one eye following surgery. Macular edema was present in 14 eyes (56%) following surgery, 12 (86%) of which had this change at the time of surgery. Some gradual resolution of macular edema was noted during follow-up in four eyes (29%). Preretinal membranes were seen in two eyes and one eye had macular degeneration. The postoperative vision was improved in all eyes; eleven eyes (44%) had vision 20/25 or better, four (16%) had 20/70 vision, six (24%) had 20/100 vision and the remaining four (16%) had vision of 20/200 or worse. in 1980, FitzgeraldI reported her results following vitreous surgery in four patients with toxoplas-

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mic retinochoroiditis. In three patients, the lens was also removed. All patients received steroids preoperatively, and two received two-drug therapy for toxoplasmosis. Vitreous surgery was by the pars plana route in all patients; however, one patient had the lens removed using limbal phacoemulsification. Complete information on postoperative drug therapy was not available. One patient developed an increase in inflammation nine days after surgery while on atropine only. This responded well to steroids and antitoxoplasma treatment. Another patient developed a retinal detachment in the postoperative period. The final acuity was improved in all patients, with two patients who had vision of 20140 and 20160, respectively. One patient had counting fingers vision secondary to macular toxoplasma scars. In 1981, Algvere and associates’ described their results following vitrectomy in 14 eyes with chronic uveitis. Three of these patients, two with JRA and one with pars planitis, also had lensectomy. All of these patients did well following surgery. A detailed analysis of the results on these patients has been presented earlier in this review. At our institution, Nobe et a14’ described the results following lensectomy-vitrectomy in 12 uveitic eyes. Seven patients had idiopathic uveitis (type unspecified), two had JRA-associated uveitis and one each had sarcoidosis, toxoplasmosis and Behset’s disease. Preoperative vision in all patients was 20/400 or less. All patients received topical, periocular, or systemic steroids preoperatively in doses that were based on the amount of inflammation seen. All patients had less than 1 + cell and flare in the anterior chamber at the time of surgery. Surgery was performed through the pars plana. A sweep was introduced through a limbal incision to break synechiae if these were present. Patients were followedup for one to 35 months after surgery, with half being followed-up one year or more. Inflammation increased transiently following surgery in seven eyes (SS%), but responded to treatment in all instances. Intraocular pressure ranged from 7 to 22 mm Hg preoperatively; the postoperative range was from 10 to 28 mm Hg, and two eyes required medication for glaucoma. In the immediate postoperative period (less than two weeks), three eyes (25%) developed choroidal effusions. One of these eyes also had vitreous hemorrhage and recurrent hyphema that required reoperation, which was successful. In the other eyes, the effusions resolved spontaneously. Another eye had cornea1 edema and hyphema, which spontaneously resolved, and tractional retinal detachment developed in one eye five months postoperatively. Vision improved in most eyes: two eyes (17%)

133 achieved 20125 vision, four (33%) had 20150 to 20180 and six (50%) had only 20/100 to 201200. Macular edema accounted for decreased vision in seven eyes (58%). Other macular pathology was the cause of decreased vision in the remainder. A report in 1983 by Tutein Nolthenius and Deutman” described 17 eyes that underwent lensectomy-vitrectomy for uveitic cataract. Six other eyes in this series did not undergo lensectomy and will not be considered here. Pars planitis was present in eight eyes and uveitis associated with JRA in two; the results of surgery on these eyes have already been discussed. The remaining eyes in the series were those of three patients with idiopathic iridocyclitis and four who had idiopathic posterior uveitis. Two of the patients with posterior uveitis and one of the patients with iridocyclitis also had a retinal detachment preoperatively. No specific information was given regarding steroid use preoperatively; however, all patients were said to have had inflammation “mostly suppressed” prior to surgery. Patients were operated on via the pars plana. “Most” eyes also had an encircling element placed, and those with retinal detachment also were insufflated with SF6. A subtenon injection of steroid was given to all patients upon completion of surgery. Patients were followed-up for 6-23 months postoperatively; four were followed-up for one year or more. Surgery for retinal detachment was successful in all eyes and no other eyes developed retinal detachment during follow-up. No significant increase in inflammation was seen following surgery. “Many” patients had steroid tapered and discontinued during follow-up. Two eyes were hypotonous prior to surgery. In both eyes, this resolved following vitrectomy. No eye developed increased intraocular pressure during follow-up. Vision in all patients improved, with those having iridocyclitis achieving the best visual acuity. One eye achieved 20125, another 20/40, and the third 20/50 vision during follow-up. Decreased vision was secondary to macular edema in the second eye, and to macular degeneration after retinal detachment in the third. Vision in eyes with posterior uveitis ranged between 20/70 and 4/200. This was limited by macular disease in three eyes and by amblyopia in one. In the same year, Dangel et al’ described their results following lensectomy-vitrectomy in eight eyes with complicated cataract. Three eyes were from patients with sarcoidosis, two each from patients with ankylosing spondylitis and idiopathic uveitis, and one was from a patient with JRA-associated uveitis. Vision was worse than 201200 in all patients. No information was provided regarding preoperative steroid use in most patients, and the amount of inflammatory activity at the time of sur-

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gery was not discussed. All surgery was by a limbal approach, but the type of vitrectomy performed varied from partial to complete, depending on the amount of vitreous debris that was present. All patients received subtenon steroids at completion of surgery, and all were treated with topical steroids following the procedure; no significant increase in inflammation was seen. No complications were encountered. Patients were followed-up for 6-22 months postoperatively. At the time of last followup, three eyes (38%) had 20140 vision, one had 20/100, and the rest (50%) saw 20/200 or worse. Decreased vision was secondary to macular pathology in all cases. In this series, four eyes (50%) showed a decrease in vision during follow-up from the best vision achieved shortly after surgery. The series of Girard et al*’ has been discussed in part in the section on pars planitis. This series consisted of 2 1 eyes that underwent lensectomy-vitrectomy. Nine eyes in this group had idiopathic panuveitis; tuberculous panuveitis was diagnosed in two eyes and sympathetic ophthalmia, toxoplasmosis, Fuchs’ syndrome and Cogan’s syndrome accounted for one eye each. The patients all were receiving systemic and/or local steroids prior to surgery. The amount of inflammation present prior to surgery in each eye was not specified. All patients received subtenon steroids at completion of surgery and topical steroids were used postoperatively. In patients who were using oral steroids preoperatively, these were continued postoperatively. One eye developed glaucoma and one hypotony during follow-up, which ranged from l-6 years. In all patients, steroids could eventually be stopped. In one eye with tuberculous uveitis, vision worsened postoperatively; in another eye the vision was unchanged. In the remaining eyes, visual acuity improved. At an unspecified time following surgery, a visual acuity of 20140 or better was achieved in nine eyes (43%). Vision was 20/100 or worse in four eyes (19%) (two secondary to retinal detachment, and one each secondary to macular scar and macular edema). The most recent report is that of Petrilli and colleagues5’ published in 1986. This report included 39 eyes, two of which have been discussed in the section on juvenile rheumatoid arthritis. The remaining eyes consisted of 10 with toxoplasmosis, eight with Vogt-Koyanagi-Harada syndrome, three each with uveitis secondary to rubella, tuberculosis, and BehGet’s disease, two each with Fuchs’ syndrome and ankylosing spondylitis, and one each with Reiter’s syndrome, sympathetic ophthalmia and phacoanaphylaxis; three eyes had idiopathic iridocyclitis. Inflammation producing 2-3 + cells in the anterior chamber was present in 12 eyes prior to surgery. No information is given regarding ste-

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roid use preoperatively, and patients did not receive periocular steroids at completion of surgery. All patients received 40 mg prednisone orally daily and used steroid drops four times daily postoperatively, but the duration of steroid use was not specified. All patients were followed for more than one year, but specific details were not given. No eye was said to have increased inflammation postoperatively; however, one eye with toxoplasmosis did develop a sterile hypopyon during follow-up. The complications that occurred included transient ocular hypertension in an unspecified number of eyes and a retinal detachment in one eye. The final visual acuity was 20/40 or better in nine eyes (23%) and 20/80 or better in eight more (2 1%). The remaining eyes all had vision of 20/100 or less, which was secondary to macular lesions in most eyes. The vision worsened following surgery in three eyes (8%); this was secondary to progression of macular changes in two eyes and was iatrogenic in one eye. 2. Summary and Recommendations As can be seen, a significant volume of information on lensectomy-vitrectomy in uveitis patients is accumulating in the literature. Few complications appear to occur following surgery, the most significant being macular edema, retinal detachment and glaucoma. The major cause of visual decrease in all series was macular pathology, usually macular edema. The reported incidence of macular edema varied from approximately 15-50%. What is difftcult to discern from the literature is how much of this edema existed at the time of surgery and how much developed de nom following surgery. Dense media opacities precluded the detailed fundus examination necessary to detect macular edema in most patients preoperatively. All authors have reported that at least some of the macular changes were visible at the time of surgery. One author confirmed the presence of macular edema in his patients with fluorescein angiography during the second postoperative week.” However, this does not prove that the macular edema existed prior to surgery, as a high percentage of noninflamed eyes show angiographic macular edema during this time period after surgery.“3 Some authors reported that macular edema improved in a small number of patients,‘0*‘5,22 while other series included patients in whom it appeared to worsen.47 Most macular edema did not change significantly during follow-up. In one series, the likelihood of macular edema and poor vision postoperatively appeared to correlate with the type of uveitis present in the eye.” Eyes that were inflamed as a result of local, rather than systemic, disease

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IN UVEITIS

PATIENTS

appeared to have the better prognosis, regardless of the duration of the uveitis preoperatively. Other authors did not specifically address this issue; however, analysis of their data does not appear to support this contention. The incidence of postoperative retinal detachment was much lower than the incidence of macular edema in these series. In all but two cases, detachments were rhegmatogenous. Aside from unsuspected retinal detachments that were noted at the time of surgery, most patients who developed this complication had tears that were noted at the time of surgery or in the early postoperative period. All detachments occurring in the postoperative period were successfully treated. All patients in the series of Tutein Nolthenius and Deutman71 had encircling elements placed routinely at th[e time of surgery. This practice was begun after two of the first three patients they operated on developed retinal detachments. The experience in other studies, however, suggests that this procedure is not necessary in the majority of patients. One series reported a significant number of patients who developed a transient increase in intraocular pressure postoperatively, which resolved spontaneously over time. 47 In the group as a whole, permanent intraocular pressure rise was noted in only three patients who had not had glaucoma preoperatively. The variation in diagnoses and followup among patients does not allow a definitive statement to be made about any surgically induced change in the natural incidence of this condition. Hypotony from various causes was noted preoperatively in some eyes in most of the series. Diamond and Kaplan9 reported normalization of intraocular pressure in nine of ten eyes following surgery. Other series have also confirmed that low intraocular pressure may normalize postoperatively. Only three patients from the entire group developed new hypotony following surgery, and in two of these eyes, intraocular pressure subsequently rose to normal levels. In the group as a whole, only three eyes had vision worse after surgery than before. Although vision improved in a majority of eyes, the stability of this visual improvement is open to question in some studies because of short and varied follow-up periods. In one study, half of the eyes showed a decrease in vision between the best postoperative acuity and acuity at the time of last follow-up.8 Conversely, other authors have reported gradual improvement in vision in some patients during follow-up? Overall, patients appeared to require less medication to control inflammation postoperatively. In the series of Girard et al,22 all patients had steroids

discontinued postoperatively. Twelve of 16 eyes in this series had required systemic steroids to control inflammation preoperatively. Recurrences in the series of Nobe et alg7 could be managed by topical steroid alone. Diamond and Kaplan” reported that in 10 eyes that experienced a recurrence, the inflammation in seven eyes abated faster in the vitrectomized eye. Because of the variation in patient follow-up, an assessment of the effect of surgery on the frequency of recurrence of inflammation or the chronicity of postoperative uveitis cannot be made. A posterior surgical approach and complete, rather than anterior, vitrectomy was used in all but one series. Nobe et al47 mention that they chose this approach because they experienced a significant increase in inflammation following surgery in earlier patients who had only an anterior vitrectomy. Although a pars plana approach facilitates removal of the posterior vitreous, Dangel et al’ state that, because these patients have a posterior vitreous detachment, most of the vitreous can be removed via a limbal approach. Most eyes in these series had extremely poor vision at the time of surgery. Whether the visual results would have been improved if patients were operated on earlier and if macular edema were treated more aggressively cannot be assessed, Many authors provided little information about the preoperative inflammatory activity in their patients. In those papers that discuss this aspect, patients were operated an only when minimal activity was present. Petrilli et a15* did not experience an increase in complications in the 30% of patients in their series who had active inflammation at the time of surgery. The absolute number of patients in this group was too small, however, to allow any conclusion to be drawn about the need for tight control of inflammation in the perioperative period. Pars plana vitrectomy, combined with lensectomy, appears to be the procedure of choice in forms of uveitis with significant vitritis and/or vitreous opacities, which cannot be cleared with medical therapy. Most studies have been in patients with idiopathic uveitis, but this technique is important also in patients with juvenile rheumatoid arthritis and uveitic cataracts and in pars planitis patients (see earlier discussion). (The “therapeutic” effect of vitrectomy without lensectomy in cases of chronic vitritis opacities with cystoid macular edema has been suggested by laboratory3’13’ and clinical studAn analysis of this technique is beyond ies. g~‘“~4’~22 the scope of this review, which focuses on cataract management and not therapeutic vitrectomy in uveitis patients.) Although an occasional patient will develop increased inflammation following the com-

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35 (2) September-October

bined procedure, in most instances the inflammation lessens and is more easily controlled after operation.g~‘0~2’~“2 The complications that do develop after the combined procedure can be controlled and managed successfully in most patients. However, aside from the syndromes of pars planitis or uveitis associated with juvenile rheumatoid arthritis, there is too little data available to reliably predict the response to surgery of an eye with a given form of inflammation. In light of this fact, cataract surgery on eyes that fall into this group should be performed only when vision is significantly reduced. As most authors stress, care must be taken to minimize inflammatory activity preoperatively with the use of steroids or, if necessary, immunosuppressive agents. Surgery should be attempted only when inflammation is under control.

III. Suggested Guidelines for the Management of a Patient with Uveitis and a Cataract Based on the foregoing literature review and our own experience, we suggest the following guidelines for the management of the inflamed eye with a cataract. A. ASSESSMENT OF THE POTENTIAL VISUAL IMPROVEMENT FOLLOWING SURGERY

FOR

Determining to what degree visual reduction in a uveitis patient is due to lenticular opacities can be difficult. By the time a cataract develops, patients often have restricted pupillary mobility secondary to synechiae formation, have vitreous opacities, and may well have macular alterations secondary to cystoid macular edema. Fluorescein angiography may be helpful in assessing the macular status; however, there is only rough correlation between leakage seen angiographically and visual acuity.4g At the time cataract surgery is contemplated, media opacities usually prevent this procedure from providing useful information about macula status. Macular acuity analyzers, such as the potential acuity meter or the laser interferometer, can be helpful in the setting where macular pathology coexists with cataract. Unfortunately, macular edema is known to give unreliable readings in such instances. The laser interferometer is less affected by vitreous opacities and poor pupillary dilation; however, it may provide an overly pessimistic estimate of potential acuity in the setting of uveitis.51 The potential acuity meter may provide an overly optimistic estimate of acuity, and its use can be restricted by the inability to dilate the pupil well in many of these patients. Entoptic phenomenon can be used to provide a rough estimate of macular function

HOOPER

1990

ET AL

when other methods fail. Ultrasonography may provide much useful information in patients in whom the posterior segment cannot be assessed directly. The presence of retinal detachments, significant choroidal thickening, and vitreous and cyclitic membranes should be looked for specifically during the examination, as their presence will alter the subsequent surgical approach. Visually significant macular changes may be present in spite of a normal ultrasound examination. Only if significant abnormalities are found is this examination helpful in assessing an eye’s visual potential. B. TIMING

OF SURGERY

As the type of inflammation present in an eye is the major factor that correlates with the incidence of complications following surgery, all surgical guidelines must take this into account. As discussed previously, eyes with inactive uveitis and Fuchs’ heterochromic iridocyclitis do well following conventional cataract extraction. (It is probably wise to treat Fuchs’ heterochromic iridocyclitis patients with corticosteroids if significant iridocyclitis is present.) Surgery in these patients can be predicated largely on their macular status and visual demands. On the other hand, eyes that continue to have vitreous debris and very low-grade vitreous cellular reactions despite corticosteroids and other immunosuppressive therapy appear to do best if vitrectomy is combined with cataract extraction. As the surgery is more difficult and the risks are higher, these patients should have significant visual reduction to justify surgery. An exception can be made in patients who have chronic uveitis with cyclitic membranes, hypotony, retinal detachment or vitreous hemorrhage. Many of these patients seem to do better if vitrectomy is performed and the pre-existing posterior segment complications are corrected at the time of surgery. Controversy exists regarding the role of vitrectomy with or without lensectomy in patients who have intractable low-grade intraocular inflammation. Some evidence suggests that surgery on these patients may actually make the inflammation easier to manage.” These patients should be observed and treated intensively with corticosteroids and possibly other immunosuppressive agents over an extended period (as much as three months) before surgery is performed. C. CONTROL OF INFLAMMATION PREOPERATIVELY Preoperatively, the degree of inflammatory control required to achieve successful cataract extraction varies with the uveitis syndrome.

CATARACT

EXTRACTION

IN UYEITIS

PATIENTS TABLE

137 2

Selection of Patients and Surgical Procedure Recommended in Vatious Uveitis Entities Uveitis Syndrome Fuchs’ iridocyclitis Uveitis associated with JRA Chronic nongranulomatous ology or idiopathic) Chronic granulomatous or idiopathic)

(any eti-

(any etiology

Pars planitis

Lens-induced

inflammation

Inactive uveitis, any type (no inflammation, off medication)

Surgery Recommended

101, Use

Extracapsular extraction/phacoemulsification Lensectomylvitrectomy if vitreous opacities present ECCE/phaco if vitreous clear; lensectomy/vitrectomy if vitreous opacities clinically significant Lensectomy/vitrectomy if vitreous opacities clinically significant; otherwise, ECCE Lensectomy/vitrectomy or ECCE dependent on level of vitreous involvement; save peripheral capsule for later secondary IOL ECCE/phaco to remove all cortex

Consider if uveitis inactive at time of surgery No

Extracapsular phaco

extraction;

Patients with Fuchs’ iridocyclitis always have some inflammation in the eye, yet this does not seem to change significantly following surgery. If the diagnosis of Fuchs’ iridocyclitis is certain, a regimen that employs preoperative topical steroids, subtenon steroids at the completion of surgery, and topical steroids in the postoperative period should be adequate to keep postoperative inflammation at the preoperative level for that particular eye. When in doubt, use more corticosteroids to reduce the anterior chamber reaction. In other forms of active chronic uveitis, the answer is less certain. A review of the literature reveals a wide variety of methods to control intra- and postoperative inflammation in these eyes. Techniques generally have in common the use of periocular depot steroids at the completion of surgery and topical steroid administration in the postoperative period. There is no firm consensus, however, on the use of systemic steroids pre- and postoperatively. Adequate control of the inflammation is, however, vital to prevent the formation of pupillary membrane and anterior synechiae following surgery. We therefore would suggest that patients who have required systemic or periocular steroids in the recent past receive these perioperatively. Prednisone, 60 to 80 mg daily, is used preoperatively for one to several weeks. Topical steroids are also indicated. Treatment must be adequate to reduce cellular reaction in the anterior chamber to less than 1 + , preferably to zero, with little to no active vitritis (pigmented cells and debris in the vitreous cavity may be difficult to distinguish from inflammatory cellular reaction). If steroids alone are insufficient to achieve this level of control, surgery should be

ECCE/

Possible if no tendency to form synechiae and if uveitis absolutely controlled Not recommended

Not recommended unless uveitis absolutely controlled

Not recommended as primary procedure Consider unless history of frequent uveitis recurrences

postponed or immunosuppressive agents added.“~‘* Following surgery, steroids should be maintained at their preoperative level for one week to ten days prior to beginning tapering. D. TYPE

OF SURGERY

The choice of the surgical approach in any given case depends to a large extent upon the etiology and location of the inflammation and the experience of the surgeon. Cataracts in eyes with uveitis that has been inactive for several months can be approached anteriorly using routine surgical techniques. Patients with Fuchs’ iridocyclitis also do well with conventional techniques. Extracapsular surgery can be used in most instances. The decision as to whether extracapsular surgery should be by phacoemulsification or nucleus expression and cortex aspiration depends largely on the preference of the surgeon and the degree of pupillary dilatation, which may be limited by posterior synechiae. The small wound necessary for phacoemulsihcation may be beneficial in patients who are young and on high doses of steroids. Atraumatic phacoemulsification can be difficult if the pupil is poorly dilated. Excessive manipulation and traumatization of the iris should be avoided. Intracapsular surgery is rarely indicated. If used at all, it is reserved for patients in whom a phacolytic component of the inflammation is suspected. In many of these eyes the lens capsule is exceedingly friable and may rupture during removal. A careful extracapsular technique, coupled with an anterior vitrectomy, if necessary, may be utilized in these instances.

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Eyes with chronic intractable low-grade vitritis and/or opacities and debris and with resistant anterior segment inflammation pose the most difficult challenge. If anterior segment inflammation can be controlled preoperatively, and only a minimal vitritis exists, it is reasonable to attempt cataract extraction alone. If inflammation has been difftcult to control despite steroids and even immunosuppressives, if significant vitritis is present, or if chronic macular edema is present, a combined procedure is suggested. A complete vitrectomy should be done in such eyes. Some authors have reported this can be accomplished through an anterior approach since the posterior vitreous is often completely detached in chronically inflamed eyes.8 Most surgeons, however, prefer conventional pars plana techniques, as this approach allows better visibility and facilitates the use of endophotocoagulation or endodiathermy if retinal tears, fibrovascular membranes, or other retinal complications are discovered.9,‘0.2’ When a combined procedure is chosen, lens removal can be done equally well by a limbal or pars plana approach prior to the vitrectomy. Using a pars plana approach, satisfactory and safe removal of the cataractous lens and the vitreous can be achieved in some cases with the automated vitrectomy instrument alone when the cataract is ~~soft_“21,22 Ultrasonic fragmentation may be needed in other cases. If a limbal approach to the cataract is planned, the three quadrantic pars plana incisions are created prior to beginning the cataract extraction. The superior ports are plugged and the infusion line secured to the inferotemporal port. A limbal incision for the cataract extraction is then created in the usual manner. Use of the limbal approach allows part of the posterior capsule to be preserved, which may facilitate secondary implantation of an intraocular lens at a later date if the inflammation resolves. We prefer this combined approach, i.e., an extracapsular cataract extraction (phacoemulsification if possible) followed by a pars plana vitrectomy. An opening should be made in the posterior capsule at the close of the vitrectomy since there is rapid opacification of the posterior capsule and a single cavity effect may provide for easier exit of inflammatory by-products as well as for enhanced drug penetration.

IV. Specific Techniques Applicable to Cataract Surgery In Uveitic Eyes Several common “roadblocks” may be encountered when performing cataract surgery on patients with uveitis:

HOOPER

1990 A. MANAGEMENT

ET AL

OF SYNECHIAE

Many uveitis patients develop posterior synechiae and, as a result, a pupil that dilates poorly. In an effort to pharmacologically dilate a stubborn pupil, a trial of 1% atropine and Neo-Synephrine@ 2.5% four times a day may be used. This can be followed, if necessary, by the use of dilating agents such as Neo-Synephrine applied by pledget for persisting synechiae. If dilation sufftcient for adequate visualization of the lens (approximately 5 to 6 mm) cannot be achieved, a superior sector iridotomy or iridectomy can be performed at the time of surgery. The synechiae are then lysed with an iris sweep while the chamber is maintained by a viscoelastic substance. Injection (via a fine cannula) of a viscoelastic material between the iris and the lens is another method to achieve synecheolysis, in this case by means of hydraulic dissection. Following this injection, a dilute solution of epinephrine (1: 100,000) is injected into the anterior chamber and the pupil dilated. This technique avoids extensive iris manipulation, which may provoke postoperative inflammation. Unfortunately, this procedure works poorly on eyes in which the iris is fibrotic as a result of chronic inflammation, and it may not provide sufficient opening to express a large nucleus or to perform intracapsular surgery. Sphincterotomies can provide additional pupillary opening in these instances, but an irregular pupil will result. Laser pupiloplasty is not recommended, as this often produces inflammation in eyes with uveitis. B. TREATMENT

OF RUBEOSIS

Chronic intraocular inflammation may result in rubeosis iridis. This is more commonly seen in uveitis associated with Behcet’s disease, sarcoidosis, or Herpes zoster, but may be seen in any condition in which the inflammation is severe enough and chronic enough to produce ocular ischemia. Iris vascular anomalies and aberrant vessels at the angle have also been described in Fuchs’ syndrome, even in the absence of demonstrable retinal ischemia. The line vessels resulting from these conditions are often subtle, but can be a source of intraoperative and postoperative bleeding. Vessels in the angle, at the side of the limbal incision, can be ablated preoperatively with an argon laser. Significant neovascularization requires a search for other causes, such as diabetes or vein occlusion. An appropriate course of photocoagulation should be provided if a noninflammatory cause for retinal ishemia is found. If no secondary causes are found, the eye should receive intensive treatment for the inflammation in an effort to induce regression of the new vessels. Surgery should be postponed in such cases.

CATARACT

EXTRACTXON

IN UVEITIS PATIENTS

During surgery on an eye with a small number of new vessels, care should be taken to minimize trauma to the iris and to the new vessels. If bleeding occurs that does not respond to elevation of the infusion source to raise intraocular pressure, epinephrine (1: 100,000) may be added to the infusion. Extensive cautery should be avoided as this may produce synechiae, as well as increase postoperative inflammation. A small amount of oozing should not be a cause for concern, as this usually resolves at the completion of surgery or in the immediate postoperative period. Uveitis patients must be questioned closely regarding the use of aspirin-containing drugs, since many of these patients will have an associated rheumatologic disorder, and many over the counter analgesics contain acetylsalicylic acid. Use of these drugs should be discontinued at least two weeks prior to surgery to minimize the risk of platelet dysfunction during surgery. C. MANAGEMENT HYPERTENSION

OF OCULAR

Elevation of intraocular pressure and glaucoma are common complications of JRA-associated uveitis and with Fuchs’ heterochromic iridocyclitis, but are also seen in other forms of ocular inflammation that involve the anterior segment. The glaucoma results often from the combination of progressive angle closure and inflammatory damage to the trabecular meshwork. Eyes in which elevated intraocular pressure has been detected preoperatively should receive a careful gonioscopic examination to determine the location and extent of peripheral anterior synechiae. The optic disc should be evaluated for pathologic changes and the visual field plotted. If inflammation is active, topical steroids should be used to lessen the anterior ocular

pressure

with an open tapered

chamber

inffammation.

is elevated angle,

steroid

and discontinued

If the intra-

in an uninflamed treatment

should

eye be

for six to eight weeks to

see if the intraocular pressure elevation is due to the steroids. Elevation of intraocular pressure in eyes with uveitis that are to undergo cataract surgery should be normalized for two to three weeks prior to the operation. This can usually be achieved in eyes with open angles by using beta blockers and, if necessary, carbonic anhydrase inhibitors. Cholinergic agonists should generally be avoided in eyes with anterior segment inflammation as they disrupt the blood-aqueous barrier. The small immobile pupil that results from these medications also increases the incidence and severity of posterior synechiae. Eyes with significant angle closure may also re-

139

TABLE 3 Manazemxnt

of Uveitis-Associated Complications

(1) Posterior synechiae:

Vigorous dilation with au-opine 1% and Neo-Syneph-

rine@ 2.5% preoperatively Peripheral iridectomy followed by “hydraulic dissection” of iris with viscoelastic substances Sector iridotomy/iridectomy followed by mechanical synechiolysis (2) Rubeosis: Control inflammation with steroids. Search for other causes of ocular ischemia and treat appropriately iffound (e.g., panretinal photocoagulation, other). Fine new iris vessels in Fuchs’ heterochromic cyclitis do not require treatment. Avoid use of drugs containing acetylsalicylic acid for several days preoperatively. (3) Ocular hypertension/glaucoma: Determine if angle is open or closed (gonioscopy). Control active inflammation, consider tapering steroids if angle is open and inflammation controlled. Consider “steroid responder” possibility. Avoid cholinergic agonists (miotics). Avoid laser procedures if inflammation is active. (4) Hypotony: Determine if secondary to cyclitic membrane, or ciliary body detachment (ultrasound, other tests). Treat active inflammation vigorously with steroids. Lensectomy/vitrectomy for progressive and persistent hypotony. _

spond to medical management if some functional angle remains and if aqueous secretion is decreased as a result of ciliary body inflammation or scarring. If pupillary block is suspected on the basis of extensive posterior synechiae and peripheral bowing of the iris, an iridectomy should be performed. In uninflamed eyes, this can be accomplished using the YAG laser, but in eyes with active inflammation and flare this is often unsuccessful and results in a significant exacerbation of ocular inflammation. Surgical iridectomy should be performed in these cases. Eyes with closed angles and a normal intraocular pressure should be suspected of harboring a ciliary body detachment or cyclitic membrane. Eyes with open or closed angles in which intraocular pressure cannot be controlled preoperatively need to be considered for a filtering procedure prior to, or in conjunction with, cataract extraction. Elevated intraocular pressure in the immediate postoperative period in an eye with normal preoperative tension should be treated with beta blockers and carbonic anhydrase inhibitors, along with intensive steroid treatment to control inflammation. Gonioscopy should be done to rule out angle closure. In most eyes with open angles, intraocular pressure will return to normal as inflammation lessens. If angle closure is present, its cause should be ascer-

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35 (2) September-October

tained, although this is most frequently seen in association with pupillary block resulting from fibrin and inflammatory debris. Increased steroid treatment and use of strong mydriatics usually restore aqueous flow in this situation. Persistent elevation in intraocular pressure, even after inflammation has been controlled, that is unresponsive to medical management requires filtration surgery. D. MANAGEMENT OF HYPOTONY CYCLITIC MEMBRANES

AND

Hypotony is a common complication in eyes with longstanding, severe uveitis of any type. This hypotony usually results from cyclitic membrane formation, ciliary body detachment or severe ciliary body inflammation with shut-down of aqueous production. Although hypotony can result from inflammatory destruction of the ciliary body itself, this is relatively rare. Hypotony that persists after inflammation has been controlled should be investigated and a cause found prior to proceeding with cataract surgery. Transillumination of the eye may help distinguish between cyclitic membrane/ciliary body detachment and atrophy of the ciliary body itself. While direct observation is usually the best way to detect these complications, the presence of synechiae or significant media opacities make visualization impossible in many patients. In these patients, careful ultrasonography of the ciliary body region may allow a proper diagnosis to be made. Pupillary membranes after cataract extraction in uveitis patients have been safely operated on using endosurgical vitrectomy techniques.56 If a cyclitic membrane is detected in an hypotonous eye, a growing consensus suggests that the appropriate technique of management is to combine cataract extraction (lensectomy) with a pars plana vitrectomy and removal or lysis of traction bands and the pupillary component of any cyclitic membranes. E. IRIDECTOMY Some evidence exists that iris surgery increases the tendency for persistent flare during the postoperative period. We therefore avoid using an iridectomy if there is a low risk of pupillary block. However, in patients who have had cataract surgery that left the posterior capsule intact, a peripheral iridectomy should be performed if any tendency to synechiae formation was seen in the past. If a vitrectomy has been performed and the posterior capsule removed, or a capsulotomy performed, use of an iridectomy is optional and probably unnecessary. In addition, all patients who have intraocular lenses implanted should probably have an iridectomy.

1990

HOOPER

ET AL

V. Intraocular Lens Implantation in Eyes with Uveitis Intraocular lens implantation has become the preferred method of optical rehabilitation after cataract extraction. To date, the complication rates associated with posterior chamber intraocular lenses have been low in adult patients who have undergone cataract surgery.3V4V”Intraocular lenses offer significant advantages to the patient because they provide excellent vision and are maintenance free. Lens implantation in patients with uveitis, however, is a subject that has generated much controversy. Many uveitis patients at the time of cataract surgery are young and have good manual dexterity, and adapt well to contact lens use. Some patients, however, do not tolerate contact lenses well. Furthermore, patients who require frequent use of topical steroids postoperatively may have an increased risk for cornea1 infection when a contact lens is present. Many valid concerns have been raised about lens implantation in any inflamed eye. The first issue concerns the effect that lens implantation itself has on the inflammatory process within the eye. Pathologic studies have shown that intraocular lens haptics can erode into the ciliary body.3*4*6,38,3Y This source of continued irritation could exacerbate preexisting inflammation. Because of these and other findings, there is general agreement that anterior chamber lenses are contraindicated in uveitis patients, especially in eyes that have a history of chronic or recurrent intraocular inflammation of any type. In order to avoid any contact between haptics and inflamed or damaged intraocular structures, such as iris or ciliary body, “in-the-bag” posterior chamber lenses are recommended for all cases in which intraocular lenses are used in the primary surgical procedure.‘8s20 “In the bag” placement of the intraocular lens would appear to decrease the risks associated with lens implantation. While one earlier pathologic study has shown that there is a poor correlation between presumed location of the intraocular lens haptic and its actual location,38 modern cataract surgical techniques now seem to ensure more reliable placement of the haptics. At least, in-the-bag intraocular lens placement should be the goal. Even if the intraocular lens does not alter the course of inflammation within the eye, its presence may make several complications of the inflammation more likely to occur or more difficult to treat. Posterior synechiae developing in the presence of an intraocular lens may lead to pupilllary block and angle closure. A peripheral iridectomy done at the time of surgery will decrease this risk, but the syne-

CATARACT

EXTRACTION

IN UVEITIS

PATIENTS

chiae themselves may be impossible to prevent. In this situation the lens itself may act as a scaffold for pupillary membrane formation. Progressive decentration of the intraocular lens can also occur. Prevention of such decentration can be achieved to some extent by the use of in-the-bag fixation techniques. Anterior chamber lenses in an inflamed eye have the additional disadvantage of producing fibrosis in the angle. This may combine with trabecular damage from inflammation to produce an intractable elevation in intraocular pressure. Retina1 tears resulting from contracture of the vitreous base are seen with increased frequency in many uveitis syndromes. Such tears may be more difficult to visualize and treat when an intraocular lens is present. Progressive deposition of pigment and debris on the intraocular lens may be seen if inflammation continues postoperatively. This can result in decreased visual acuity and may necessitate increased amounts of steroids. The biomaterials (PMMA or polypropylene) used in intraocular lens haptics of most posterior chamber lenses are themselves not totally inert.” If the intraocular lens is positioned in-the-bag, and away from the iris and ciliary body, the nature of this material is probably not important. However, some authors feel strongly that PMMA haptics should be used for the “theoretical advantage” of avoiding complement activation in the aqueous by polypropylene haptics.18 Such activation by polypropylene has been shown to occur;“’ others, however, have demonstrated a similar effect using PMMA.” Because of these potentially serious complications, eyes with chronic, active inflammation that cannot be absolutely controlled medically and that form synechiae readily should not be considered for lens implantation. Patients with Fuchs’ heterochromic iridocyclitis, despite mild chronic “uveitis,” appear to be an exception to this rule. In 1982, Mills and Rosen44 reported their results following intracapsular cataract extraction with implantation of an iris supported intraocular lens in eight patients with Fuchs’ heterochromic iridocyclitis (the details of this report have been presented earlier in this review), Only one patient developed significant complications during the two-year follow-up. This patient developed elevated intraocular pressure and dense vitritis; a lenticular membrane formed and hand motion vision resulted. In 1980, Mooney and 0’Connor4” described the results of lens implantation in eleven eyes with Fuchs’ iridocyclitis. Ten of these patients had extracapsular surgery with implantation of an iridocapsular lens. An intracapsular procedure and iris supported lens were used in one patient. Specific

141 details of the surgical technique used and length of follow-up were not provided. The complications that developed after surgery consisted of cystoid macular edema in one eye and opacity of the posterior capsule, which required needling, in another. All but two patients had vision of 20/30 or better postoperatively. One patient had vision restricted by myopic degeneration, the other by vitreous haze. No complications due to the lenses themselves were seen. A more recent report reviews Fuchs’ heterochromic iridocyclitis patients who underwent extracapsular surgery with “in the bag” posterior chamber lens implantation.“’ This series was composed of eleven patients who were followed-up for 6-48 months following surgery. The details of the surgical technique used and the methods employed to control inflammation have already been presented. The patients all tolerated the lenses well and no complications related to the lenses were observed during follow-up. Other series also show a similar low incidence to intraocular lens-related complications in Fuchs’ iridocyclitis patients. Based upon this growing experience, it would seem reasonable to consider posterior chamber lens in-the-bag implantation in eyes with cataract due to Fuchs’ heterochromic iridocyclitis if the diagnosis is certain and no synechiae are present or are likely to occur. We do not recommend anterior chamber lenses in this or any other uveitis syndrome. The use of posterior chamber lens implants in other forms of uveitis that have been controlled for a period of time preoperatively is more controversial. The series of patients compiled by Foster et al’” contained 32 eyes that received posterior chamber lens implants after undergoing extracapsular cataract extraction. These patients all had inflammation controlled for a minimum of three months prior to surgery by a variety of regimens, depending on the type of underlying inflammation. Eyes were followed for an average of 25 months (range, 3-35 months) after surgery. A variety of types of inflammation were included, consisting of nine eyes with idiopathic anterior uveitis, four each in patients with ankylosing spondylitis and pars planitis, three patients with sarcoidosis and two each with Fuchs’ iridocyclitis and inflammatory bowel disease, herpes, birdshot retinopathy, and Behcet’s disease. All patients had improved vision postoperatively, with 87% achieving vision of 20/40 or better. Macular pathology accounted for failure of most of the remaining eyes to achieve this level of vision. Three eyes developed synechiae and two developed posterior capsule opacification postoperatively. Other permanent complications encountered included one eye with elevated intraocular pressure, one

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with hyphema and two with “flare uveitis.” Kaplanzg mentions a series of 13 eyes that underwent cataract extraction followed by posterior chamber lens implantation. This series consisted of three patients each with Fuchs’ iridocyclitis and idiopathic panuveitis, two with idiopathic iridocyclitis, and one each with sarcoidosis, pars planitis, and toxoplasmosis. The length of follow-up was not specified. Detailed data were not provided regarding preoperative and postoperative vision, but vision in all patients improved. One patient developed increased inflammation postoperatively and another developed synechiae and pupillary block. Secondary opacification of the posterior capsule developed in two patients. Both series contain a mixture of patients followed for various amounts of time postoperatively. The actual experience with lens implantation in many of the less common forms of uveitis remains extremely small. Thus, although these results, along with anecdotal experience, are encouraging, care must be taken to weigh the benefits of lens implantation against the longterm potential for complications in patients who often have 30-40 year life expectancies at the time of surgery. Patients in whom ocular inflammation is difficult to control or who sporadically have significant (3 + cell or greater) recurrences of inflammation in the anterior segment should be considered for lens implantation only in exceptional circumstances. Anterior chamber lenses are contraindicated, while in-the-bag posterior chamber lenses are the intraocular lenses of choice. Conversely, when inflammation can be controlled for several months prior to surgery, lens implantation should be strongly considered for the second eye in patients who would otherwise be rendered bilaterally aphakic, or in patients who have vocational or avocational needs that are poorly served by contact lens wear. If doubt about the status of a given eye exists at the time of surgery, the safest procedure would appear to be initial cataract removal alone, followed by secondary posterior chamber lens implantation at a later date, when the eye is totally quiescent and the uveitis “burnedout.” VI.

Summary

Modern experience with cataract extraction in uveitis suggests that, in many instances, cataracts can be removed from uveitic eyes with relative safety. Preoperative management and careful surgical planning are, however, critical in achieving a successful surgical result. Factors that must be considered include the type of uveitis, its duration, recent activity and associated complications. Adequate preoperative control of inflammation appears to be

HOOPER

1990

ET AL

the most important factor upon which surgical SUCcess is predicated. Eyes that undergo cataract surgery alone should have anterior segment and posterior segment inflammation controlled for 8-l 2 weeks prior to surgery. The only exceptions to this rule are patients with Fuchs’ heterochromic iridocyclitis or lens-induced inflammation. Eyes with significant vitritis and/or post-inflammatory vitreous opacities should be considered for lens extraction combined with vitrectomy. Vitrectomy should also be done in hypotonous eyes in which there is a strong suspicion that the hypotony is secondary to cyclitic membrane formation or tractional ciliary body detachment. If inflammation has been adequately and absolutely controlled preoperatively, it should not be much more difficult to control in the postoperative period following uncomplicated surgery. Significant exacerbation of inflammation in the postoperative period in such eyes may be due to infection or secondary to retained lens material. Steroid unresponsiveness or progression of inflammation during intensive steroid treatment is an indication to proceed with paracentesis and vitreous biopsy to rule out the presence of an infectious organism, as in any case of postoperative inflammation. The main cause for a poor visual result following successful modern cataract surgery in patients with uveitis is the presence of macular edema or its sequellae. Although acute macular edema may respond to steroids, the chronic macular edema changes often discovered at the time of surgery may not. Although fluorescein angiography and laser interferometry are helpful in predicting the likelihood of improvement following surgery in these eyes, a significant margin for error exists; better tests are needed since macular edema may give false positive test results using some macular testing techniques. Lens implantation in uveitis cases remains controversial. Posterior chamber lens implants in patients with Fuchs’ heterochromic iridocyclitis appear to be well tolerated. In other forms of uveitis, a decision should be made on an individual basis depending upon the activity of the uveitis, the individual’s age, occupation and dexterity, along with the status of the fellow eye. Eyes that show a marked tendency for synechiae formation are poor candidates for intraocular lenses. Anterior chamber lenses should be avoided. Posterior chamber lenses placed in-the-bag are recommended ifan implant is used

at all.” Acknowledgment

The authors wish to thank Ann Dawson for editing this manuscript and Ann Guild and Linda Keith for assistance in its preparation.

CATARACT

EXTRACTION

IN UVEITIS PATIENTS

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subcapsular cataracts and glaucoma associated with longterm bra1 corticosteroid therapy in patients with rheumatoid arthritis and related conditions. Br 1 Ofihthulmol 53:361-372, 1969 74. Wolf MD, Lichter PR, Ragsdale CG: Prognostic factors in the uveitis of juvenile rheumatoid arthritis. Ophthalmology 94:1242-1248, 1987 I

1

Outline I. Historical perspective II. Review of current literature A. Cataract extraction in Fuchs heterochromic iridocyclitis B. Cataract extraction in uveitis associated with juvenile rheumatoid arthritis C. Cataract extraction in pars planitis D. Cataract extraction in lens-induced inflammation E. Cataract extraction in idiopathic and other forms of uveitis F. Role of lensectomy combined with vitrectomy III. Suggested guidelines for the management of a patient with uveitis and cataract A. Assessment of the potential for visual improvement following surgery B. Timing of surgery C. Control of inflammation preoperatively D. Type of surgery IV. Specific techniques applicable to cataract surgery in uveitic eyes A. Management of synechiae B. Treatment of rubeosis C. Management of ocular hypertension D. Management of hypotony and cyclitic membranes E. Iridectomy V. Intraocular lens implantation in eyes with uveitis VI. Summary

This work was supported in part by a grant from Research to Prevent Blindness, Inc., New York, and by the Ontario Ministry of Health. Reprint address: Ronald E. Smith, M.D., Doheny Eye Institute, 1355 San Pablo Street, Los Angeles, CA 90033.