Abstract: Most uveitics enjoy good vision despite potentially sight-threatening complications including cataract development. In those patients who develop cataracts, successful surgery stems from educated patient selection, careful surgical technique, and aggressive preoperative and postoperative control of inflammation. While commonly accepted in the adult patient population, recent investigations reflect the increased tolerance for primary intraocular lens placement in the pediatric cohort. The role of absolute control of inflammation continues with greater focus on immunomodulatory therapies. However, these agents bear their own side effect and complication profiles. Cataract extraction with intraocular lens implantation in the setting of meticulous control of inflammation can optimize visual outcome in adults and children with uveitis.
Cataract surgery in the setting of uveitis may pose a daunting challenge to the ocular surgeon. Cataract development in uveitics results from chronic inflammation and as a consequence of long-term corticosteroid treatment. The uveitic cataract presents unique challenges given the commonly encountered consequences of chronic inflammation which include limited visualization from band keratopathy and a miotic pupil and intraoperative difficulty stemming from posterior synechiae, pupillary membranes, and intraoperative bleeding from abnormal fragile iris vessels. The postoperative period may too be complicated by marked postoperative inflammatory response, particularly in children. With improvements in surgical instruments and techniques, cataract surgery in uveitics is more common and more commonly successful. Cataract extraction and intraocular lens placement in adults with a history of uveitis is regarded as relatively safe and effective; however, controversy continues to surround the matter in the pediatric cohort. Regardless of age, the importance of preoperative and postoperative inflammation control is paramount. The role of immunomodulatory therapy in the control of uveitis continues to grow, and with it, the potential to improve surgical outcomes in pediatric cases. As a result, aggressive control of inflammation with careful surgical planning broadens the population served by cataract extraction and may reduce potential complications, ultimately providing improved visual outcomes.
The etiologic cause and type of uveitis can influence not only disease course, but treatment response and rate of associated complications. A specific diagnosis may guide the clinical approach as well as surgical technique; thus, a comprehensive ocular and systemic history along with appropriate laboratory analysis is essential, including investigations for possible infectious and autoimmune etiologies (Foster and Rashid, 2003). For example, a diagnosis of juvenile idiopathic arthritis (JIA)-associated uveitis carries with it the burden of an increased rate of cataract development and often more complicated post-extraction course when compared to patients with other idiopathic diseases (BenEzra and Cohen, 2000; Skarin et al., 2009). Given the insidious nature of JIA-associated uveitis, clinicians regularly evaluate children not only for evidence of inflammation but also for development of reversible visual impairment from cataracts (Foster and Barrett, 1993). In contrast, patients with Fuchs’ heterochromic iridocyclitis associated cataracts enjoy good visual outcomes and experience fewer complications following cataract extraction than patients with other forms of uveitis (Avramides et al., 1997). A specific etiology may lend insight toward intraoperative surgical expectations and prognosis, particularly whether an intraocular lens will likely be tolerated. The ophthalmologist’s approach to cataract surgery and post-operative management must ultimately go beyond the diagnosis and reflect the patient’s individual clinical presentation.
Indications for Surgery
Cataract surgery is indicated in patients with uveitis in four settings (Rojas and Foster, 1996): 1) active inflammation secondary to leakage of lens proteins (i.e., phacoantigenic uveitis); 2) visually significant cataract with preoperatively controlled inflammation and expected good visual prognosis; 3) cataract impairing proper assessment and treatment of possible fundus pathology; and 4) cataract impeding posterior segment visualization in a patient requiring posterior segment surgery (e.g., pars plana vitrectomy).
Setting the Stage for Surgery — Control of Inflammation
The common theme in the treatment of uveitis, and particularly in preparation for cataract surgery, is meticulous control of inflammation. It is generally accepted that successful surgery requires a “quiet” eye devoid of active inflammation for a period of three months or greater. Corticosteroids, once viewed as primary treatment for uveitis, are now more commonly reserved for acute treatment of inflammation. Employment of corticosteroids in the treatment of uveitis may lead to elevation of intraocular pressure and may accelerate progression toward a vision-limiting cataract. Furthermore, long-term treatment of inflammatory conditions with systemic steroids has been shown to reduce bone mineral density in adults and can lead to growth retardation in children (van Staa, 2006; Lopes et al., 2008). The consequences of prolonged corticosteroid usage are well known and are of substantial concern. Thus, immunomodulatory agents have more recently gained favor; however, no consistent guidelines are yet established. While clinicians vary in their proposed regimens to accomplish the goal of a quiet eye, many continue to employ preoperative supplemental corticosteroids, either orally or via local injection, to ensure meticulous control of inflammation.
Antimetabolites have been shown to be effective in controlling intraocular inflammation and in the achievement of drug-free remission; however, they are not without potential side effects (Cervantes-Casteneda et al., 2009). A recent retrospective multi-center study examined the overall and specific cancer mortality in relation to patients with non-infectious ocular inflammation seen between 1979 and 2005 (Kempen et al., 2009). When comparing patients treated with immunosuppressive drugs to those not exposed to such agents, overall mortality and cancer-specific mortality were not statistically dissimilar, except in those patients treated with tumor necrosis factor inhibitors. In these individuals, mortality and cancer mortality were both significantly increased. Although not statistically significant, alkylating agents were also associated with an increase in cancer mortality. Long term experience with these agents and further investigations will ultimately establish the appropriate role of immunomodulatory therapy in the long-term control of inflammation as well as their importance perioperatively.
The goal of most cataract surgery is to improve vision. In children, there is the additional aim to prevent amblyopia. Cataract surgery in the setting of uveitis can be challenging, secondary to the presence of a miotic pupil, pupillary membrane, posterior synechiae, and band keratopathy. Visual outcomes in patients with uveitic cataracts reflect both surgical technique and concomitant disease, including commonly encountered posterior segment limitations of optic atrophy, epiretinal membrane formation, and cystoid macular edema (CME). Despite these challenges, adult uveitic cataracts are safely treated with small incision cataract extraction and intraocular lens implantation with the majority experiencing improved vision; on the other hand, controversy surrounds the proper approach to treatment of uveitic cataracts in the pediatric population (Estafanous et al., 2001).
Historically, cataract surgery in pediatric patients with uveitis is associated with higher rates of complication secondary to technical difficulty and increased postoperative inflammation. Cataract extraction is often performed by phacoemulsification; however, some cases warrant a pars plana approach, particularly if concomitant posterior disease is present. BenEzra and Cohen (2000) compared outcomes from cataract surgery performed through the limbus in JIA-associated uveitis patients and non-JIA-associated uveitis patients. They found that JIA-associated uveitis patients tended to be younger, with persistent intraocular inflammation post surgery, and with a greater tendency for secondary membrane development. The younger patients in their study did not tolerate contact lens correction which led to discontinuation of aphakic correction and subsequent development of low vision and strabismus. While an intraocular lens may provide ideal visual rehabilitation, its mere presence may lead to persistent postoperative inflammation and decreased vision eventually requiring explantation.
Advancement in surgical techniques and devices has popularized primary placement of an intraocular lens in the pediatric population. Cassidy et al. (2001) established aspiration and primary lens implantation to be safe and effective in children with no prior history of uveitis. A recent retrospective, multicenter analysis by Nemet et al. (2007) found no significant difference in postoperative course or complications when comparing non-JIA associated uveitis patients and JIA-associated uveitis patients. Similarly, our group at the Massachusetts Eye Research and Surgery Institution, in a retrospective study examining visual outcomes and postoperative inflammation in a carefully selected pediatric uveitic population, found that placement of an intraocular lens did not significantly raise the level of postoperative inflammation (Quinones et al., 2009). In addition, Lam et al. (2003) reported favorable visual outcomes after cataract surgery with primary posterior chamber lens implantation in children with JIA-associated uveitis. Each of these studies stressed the importance of aggressive perioperative control of intraocular inflammation and concluded that primary intraocular lens placement is not contraindicated in the pediatric cohort (Nemet et al., 2007; Quinones et al., 2009; Lam et al., 2003). Adults and children alike demonstrate visual improvements after phacoemulsification and primary lens implantation. The same holds true for most patients with uveitis (Kawaguchi et al., 2007). Estafanous et al. (2001) described improved visual acuity in 95% of eyes with a history of uveitis undergoing cataract surgery; the majority of these individuals were corrected to 20/40 or better. In the pediatric cohort, we similarly described improved postoperative acuity in 92% of patients undergoing the same procedure (Quinones et al., 2009). Tailoring surgery to the patient’s needs identifies those patients who are at risk of postoperative complications and helps guide appropriate surgical technique and perioperative treatment.
Intraocular Lens Considerations
Several studies have investigated which intraocular lens is optimal in patients with a history of uveitis. In adults, acrylic, silicone, and hydrogel lenses have all been shown to be relatively safe choices with favorable visual outcome (Rauz et al., 2000). A prospective randomized study by Alio et al. (2002) found a slight advantage with acrylic lenses; patients receiving acrylic lenses demonstrated reduced measures of postoperative intraocular inflammation and reduced rates of posterior capsular opacification six months after surgery. Of note, the study by Alio et al. excluded those patients with a history of JIA. Roesel et al. (2008), in a prospective randomized study in patients with non-infectious uveitis, compared two acrylic lenses, one hydrophobic and one hydrophilic, and found similar visual outcomes and capsular opacification development between the two lenses. Advancements in lens materials and designs continue to offer potential improved surgical outcomes.
Commonly, posterior synechiae, capsular opacification, CME, and persistent inflammation can follow cataract surgery in uveitic patients. The main vision limiting factor in many patients after surgery is recalcitrant CME. Using ocular coherence tomography, Belair et al. (2009) compared the incidence of CME post cataract extraction with lens implant in adult patients with a history of uveitis and those without. While CME was expectedly higher in patients with a history of ocular inflammation, there was a statistically significant reduction in rate of macular edema in patients preoperatively treated with oral corticosteroids. The rate of CME was also significantly lower in those patients whose uveitis was controlled for greater than three months prior to surgery. In addition, we found that post cataract surgery visual acuity was better in those children treated with immunomodulatory therapy (Quinones et al., 2009). Although not statistically significant, it reinforces the importance of perioperative control of inflammation and its influence on postoperative outcomes. Furthermore, it reflects the role of immunomodulators in the adequate control of ocular inflammation and in the goal to reduce exposure to systemic corticosteroids.
Cataract surgery in a patient with uveitis requires thorough diagnostic investigation, diligent perioperative control of inflammation, and meticulous surgical technique. Corticosteroids and immunomodulatory agents add to the physician’s armamentarium to effectively achieve zero-tolerance of inflammation; however, their usage is not without potential complication. As a result, further investigations in both systemic and localized drug delivery are needed to identify their appropriate role in the long-term treatment of ocular inflammatory disease, both in the preoperative and postoperative venue. Likewise, improvements in intraocular design, surgical instrumentation, and implantation techniques will continue to influence cataract surgery techniques and outcomes in patients with uveitis.
Declaration of Interest
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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[Discovery Medicine, 9(44):51-54, January 2010.]