An early tabulation of complications in pediatric uveitis, from 1965, found cataract in 40% (26/65).² The cataract risk has been reported to be highest for panuveitis followed by anterior, intermediate, and posterior uveitis (risk ratio, 1.06, 1.00 reference category, 0.99, and 0.27, respectively, P = 0.007).³ In a study of 51 juvenile rheumatoid arthritis (JRA)-associated iridocyclitis patients,⁴ cataract was noted in 46%. Of note, the term juvenile idiopathic arthritis (JIA) is preferred over JRA.⁵,⁶,⁷,⁸,⁹ Herein, we use the term JIA preferentially even if the article quoted used the older JRA term in the published manuscript. Foster and Barrett¹⁰ reported that even with aggressive therapy to control inflammation, 18% of patients with JIA-associated anterior uveitis developed cataracts. Narayana et al.¹¹ studied the patterns of uveitis in children at a referral eye care center and reported that approximately 25% (8/31) developed cataracts. de Boer et al.¹² reported cataract in 35% (43/123) of children with uveitis. In eyes with Behçet disease, a 47% incidence of cataract in the involved eye has been reported.¹³ Cataract was reported in 53% of 148 pediatric patients from the uveitis service of a university referral center.³ On the basis of the number of events and total follow-up in the cohort, cataracts would be predicted to occur at a rate of 0.16 events per patient-year of follow-up.³ It was further noted that cataract was diagnosed in 11%, 15%, 24%, 39%, 44%, 56%, and 68% of patients after 3, 6, 12, 24, 36, 60, and 120 months, respectively, of the diagnosis of uveitis. Kalinina Ayuso et al.¹⁴ reported that cataract was present at initial presentation in 3% (1/35) of the eyes with intermediate uveitis, while during follow-up, cataract surgery was required in 26% (9/35) of the eyes. The mean time to the development of cataract was 2.6 years. Cataract surgery was required more frequently if the age of uveitis onset was younger than 7 years as compared to older than 7 years (44% versus 11%). Overall, cataract or cataract surgery has been reported in 18% to 68% of pediatric patients with uveitis.

Tugal-Tutkun et al.¹⁵ reviewed the records of 130 consecutive children with uveitis and noted that the cataract was most common in JIA-associated uveitis with an incidence of 71%, followed by 50% in pars planitis, 34% in idiopathic uveitis, and 28.5% in the remaining types of uveitis. BenEzra and Cohen¹⁶ noted that of 17 children with uveitis, eight (all girls) had chronic uveitis associated with JIA, one girl had Toxocara, and in eight children (five boys and three girls) no systemic manifestations or definite cause was detected. The children with JIA-associated uveitis had an earlier and more severe ocular disease, leading to the need for cataract surgery when 3 to 8 years old (mean age, 5 years).¹⁶ Children with idiopathic uveitis had a more chronic and less severe type of ocular disease, leading to the need for surgery when older (mean age, 12.8 years).¹⁶ Paikos et al.¹⁷ noted that five of seven pediatric uveitis patients with cataract had JIA and the other two had chronic anterior uveitis of unknown etiology. Quinones et al.¹⁸ noted 21/34 children had JIA, seven had pars planitis, and six had other conditions (herpes zoster virus uveitis, idiopathic anterior uveitis, idiopathic
panuveitis, or sarcoid panuveitis). Terrada et al.¹⁹ reported that uveitis was JIA associated in 9/16 children (56%); idiopathic in four (25%); and associated with Behçet disease, sarcoidosis, and varicella zoster in one patient each.

As seen above, JIA-associated uveitis is the most common cause of cataract in pediatric uveitis. It has also been observed that children with JIA-associated uveitis are younger, demonstrate an active intraocular inflammation for an extended period after surgery, and tend to develop secondary membranes postoperatively, necessitating additional surgical interventions.¹⁶ The relative risk for the development of band keratopathy, cataract, and posterior synechia was highest for JIA.³ Children in whom the diagnosis of uveitis was the initial manifestation of JIA had a significantly shorter mean uveitis to cataract interval than did children in whom arthritis preceded uveitis (3.5 versus 6.6 years).²⁰ Nemet et al.²¹ reported that children with JIA-associated uveitis were seen and underwent cataract surgery at an earlier age, and had a lower preoperative visual acuity and more severe uveitic complications when first seen as well as after surgery, than those with non-JIA-associated uveitis.

Terrada et al.¹⁹ noted that 21/22 eyes with uveitic cataract had moderate to severe posterior subcapsular cataract (PSC) and one eye had a total cataract. Although uveitis is usually a bilateral disease, the severity of inflammation and cataract is often asymmetrical. The percentage of cases with cataract that were bilateral at diagnosis was 41%.³ Lundvall and Zetterstrom²² reported four patients who underwent unilateral surgery and three who had bilateral surgery. Nemet et al.²¹ reported that only 1/18 patient required bilateral cataract surgery during follow-up. The median time from involvement of the first eye to involvement of the second eye cataract was 81 months in cases of bilateral pediatric uveitis.³

As mentioned previously, recurrent long-standing inflammation and corticosteroid use are the most common causes of cataract in eyes with pediatric uveitis. Sallam et al.²³ reported on the use of intravitreal triamcinolone acetonide for treating pediatric CME secondary to noninfectious uveitis and concluded that steroid-induced cataract occurred in 6/199 phakic eyes (55%). Thorne et al.²⁴ (while reporting a series of JIA-associated uveitis) reported that topical corticosteroid use was associated with an increased risk of cataract formation independent of active uveitis or the presence of posterior synechia. The incidence of new-onset cataract was 0.04/eye-year. There was a dose-dependent increase in the rate of cataract development among eyes receiving topical corticosteroids.

The incidence of cataract was 0.01/eye-year for eyes treated with ≤3 drops daily and 0.16/eye-year (P = 0.0006) for eyes treated with >3 drops daily. Among eyes receiving ≤2 drops daily, the incidence of cataract was 0/eye-year. The presence of posterior synechia, active uveitis, and use of topical corticosteroids at presentation were significantly associated with cataract development after controlling for confounding variables. As stated above, the use of topical corticosteroids was associated with cataract formation independent of uveitis activity. Using longitudinal data analysis and controlling for the duration of uveitis, the presence and degree of active uveitis, and the concomitant use of other forms of corticosteroids in a time-updated fashion, treatment with ≤3 drops daily of topical corticosteroid was associated with an 87% lower risk of cataract formation compared with eyes treated with >3 drops daily (relative risk, 0.13; P = 0.02).

BenEzra and Cohen¹⁶ reported that four of the nine eyes in children with JIA and cataract had a high intraocular pressure (IOP), and all had corneal involvement (mostly band keratopathy), extensive posterior synechia, a poor fixation pattern, and strabismus. Although band keratopathy can occur at any age, it is a hallmark of childhood uveitis. If it is present in a child with cataracts who is not a candidate for IOL implantation, it may preclude successful aphakic contact lens wear. Lundvall and Zetterstrom²² reported preoperative glaucoma in 3/10 eyes. Petric and colleagues noted glaucoma in 3/7 eyes before cataract surgery. All three eyes underwent combined cataract and trabeculectomy with mitomycin C.²⁵ Terrada et al.¹⁹ noted band keratopathy in 9/16 cases (41%) and posterior synechia in 18 cases (82%). Trabeculectomy was performed in 1/22 (4.5%) eyes before cataract surgery.¹⁹ Quinones et al.¹⁸ noted that 17/41 (42%) eyes had intraoperative posterior segment pathology—vitreous hemorrhage (1), vitreous debris or inflammation (5), fibrovascular membrane at the pars plana or retina (7), optic disc pallor/atrophy (1), papillitis (5), epiretinal membrane formation (4), CME (2), macular pseudohole (1), foveal scarring (1), and retinal vasculitis (6). Sijssens et al.²⁰ reported that the presence of adherent posterior synechia at the time of the diagnosis of uveitis is strongly associated with the early development of cataract requiring surgery in JIA-associated uveitis. More intensive screening in the early phase of JIA may result in less frequent development of posterior synechia and therefore less early development of cataract.

Cataract extraction and other elective surgeries should be deferred until inflammation is at its best level of control for at least 3 to 4 months consecutively.²⁶ Some parents
may request that surgery be scheduled to coincide with summer vacations or holidays, to minimize the child’s time away from school.²⁶ Such accommodations should be made only if inflammation is controlled at that point in time. Deferral of surgery may require that children have special educational arrangements, such as low vision aids or tutors, until surgery can be performed. The medical advantages of deferral should be weighed against the risks of amblyopia in very young patients with unilateral cataracts.²⁶

It may not be possible to completely inhibit all uveitis activity. However, every effort should be made to reduce inflammation prior to surgery. Inflammation needs to be in remission, for example, 10 cells or less in the slit-lamp high-power field in the anterior chamber (or 1+), or the flare value obtained with laser photometry under maximal topical and/or systemic therapy must be at the minimum before cataract surgery can be planned. Low IOP, cells in the vitreous body, and thickening of the choroid may also represent signs of inflammation.

Treatment with methotrexate in the first year after the diagnosis of uveitis is associated with a delay in cataract surgery.²⁰ The presence of posterior synechia may be an indication for early treatment with methotrexate because this drug has been associated with a delay in the development of visually significant cataracts requiring surgery.

Anti-inflammatory medication is commonly instituted prior to surgical intervention. Usually, the application of topical corticosteroids, such as prednisolone acetate 1% or dexamethasone 0.1%, five times daily for 1 week in addition to the individual treatment regimen is generally sufficient. A systemic corticosteroid application is indicated in selected cases with previous or current CME, with intermediate or posterior uveitis or with known attacks of severe inflammation after previous intraocular surgery. Although the optimal preoperative dosages are not well defined, prednisone 1 mg/kg body weight given for 3 days or intravenous methyl prednisone injections on the day before surgery may be effective. Patients with severe chronic uveitis exhibiting vision threatening complications should receive immunosuppressants in advance of surgery. This should be comanaged with a pediatric rheumatologist. The value of topical and/or systemic nonsteroidal anti-inflammatory drugs (NSAIDs) for the preoperative management of uveitic inflammation is not well known. IOP should be stabilized before surgery. However, miotic drugs before surgery are discouraged because of their tendency to disrupt the blood-aqueous barrier resulting in an increased rate of postoperative fibrin formation and because of the small pupil size that may make surgery more difficult and that may increase the bleeding rate. Since many of the uveitis patients are under long-term immunosuppressive treatment, additional measures to prevent postcataract surgery infections should also be considered. A reactivation rate of 36% for ocular toxoplasmosis within 4 months of cataract surgery has been reported, and thus, prophylactic treatment with antiparasitic drugs may be considered for patients with ocular toxoplasmosis. The frequency and intensity of uveitic recurrences prior to cataract surgery help predict the likely postoperative course.

The current trend is to perform lens aspiration with posterior capsulectomy plus anterior vitrectomy (with or without IOL implantation).¹⁷,¹⁸,¹⁹,²⁰

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