VISUAL LOSS IN CHILDREN DUE TO chronic uveitis remains a great concern today. The pathological condition of uveitis is defined as inflammation of any of the structures of the uvea, including the iris, ciliary body or choroid. Anterior uveitis and iridocyclitis (inflammation of the iris and ciliary body), affect the front and intermediate zones of the eye, while posterior uveitis, affecting the cho­roid, is characterized by inflammation in the back portion of the eye.

 

Incidence

Although there is a lower incidence of uveitis in children, the rate of visual loss may, in fact, be worse than that of adults. Uveitis in the pediatric population is a serious concern for ophthalmologists and pediatricians, given the greater life span of children and potential long-term disability from vision loss.

The incidence of childhood uveitis per year is estimated to be 4.3 to 6.9/100,000 in North America and Eu­rope, while the incidence of adult uveitis is estimated to be 26.6 to 102/100,000. The prevalence of pediatric uveitis is about 30 cases in 100,000 versus 93 in 100,000 in adults.^1-5

Major changes in the pattern and anatomic distribution of uveitis have occurred since early studies were reported in the 1960s. No­tice­able changes oc­curred over the last decade, in­cluding the rise in the amount of panuveitis and the de­crease in posterior uveitis, happening presumably due to a decrease in tox­oplasmosis and toxocariasis in children in developing countries. Idi­o­pathic uveitis remains the most common diagnosis, despite the in­creas­ing sophistication of diagnostic techniques and, possibly, autoimmunity in the population.⁶

It is not easy to obtain reliable data. Although most data suggest that uveitis has increased, the cause of the increase is unknown. Hopefully, it is only a reflection of better surveillance and the fact that eye doctors are diagnosing uveitis more quickly.

 

Etiology

Ophthalmologists are familiar with the challenges in the diagnosis and treatment of pediatric uveitis. Child­ren can have the condition and not be aware of it because uveitis does not produce noticeable symptoms, such as light sensitivity or red eye. This often leads to late presentation to the ophthalmologist and frequently pathology is already established and a significant amount of visual acuity has been lost. Other challenges include obtaining a history and examining the children.

Numerous causes for pediatric uve­itis exist. They include the classical broad catego­ries of: trau­ma—intraocular foreign body (rare); cancer (rare), autoimmune disorders (by far the most common); and infectious origins (second most common).

Figure 1. Active uveitis, with posterior synechiae and with fibrin and cells on the anterior lens capsule.

With regard to autoimmune disorders, uveitis associated with juvenile idiopathic arthritis (JIA) is the most common. Other endogenous syndromes associated with uveitis in­clude: Kawasaki syndrome; chronic infantile neurological cutaneous and articular/neonatal-onset multisystem inflammatory disease syndrome; sarcoidosis; HLA-B27-associated uveitis; tubulonephritis and uveitis syndrome; psoriasis; enteropathic associated uve­itis; and familial juvenile systemic gran­ulomatosis.

 

Foster Study

Our recent study of a 269 children with uveitic syndromes found that uve­itis remains a serious cause of mor­bidity and visual loss in children. Time­ly referral to uveitis specialists may lead to improved visual outcomes in children with chronic uve­itis.⁶

Our retrospective cohort study analyzed demographics, anatomic data, diagnoses, systemic associations, and visual outcomes of pediatric patients referred to me from 1985 to 2003. After retrospectively reviewing the records of 1,242 patients with uveitis, 269 patients 16 years and younger were identified.

 

Results

Among the 269 children with uve­itis, 53.5 percent were girls, 82 percent were Caucasian, and 82 percent were born in the United States. The type of uveitis varied: anterior uveitis (56.9 percent of cases); intermediate (20.8 percent); panuveitis (16 percent); and posterior (6.3 percent). The most frequent type of inflammations were: nongranulomatous (77.6 percent) and noninfectious (85.7 percent). The process was bilateral in 74.4 percent of patients. Mean follow-up was 22 months, with mean age of 8 years at diagnosis. Mean duration of uveitis at the time of presentation to me was two years.

The time between the diagnosis of uveitis and referral ranged from one day to 5.6 years. The length of time between diagnosis of uveitis and the referral to a uveitis specialist strongly correlated with the complication rate and degree of visual impairment in our study. In other words, the longer the time before the patients were seen by the uveitis expert, the worse the visual outcomes.

No systemic associations were found in 58 percent of patients. JIA was responsible for 33 percent of cases, 8 percent of patients had other systemic associations, and 1 percent had tubulointerstitial nephritis uveitis syndrome.

 

Complications

Uveitis is a major cause of vision loss. The best available data indicate that 12 percent of children with JIA-associated uveitis eventually have profound loss of vision. However, it may take many years to get to that point. The child's vision loss doesn't appear so bad in the short term, and loss is incremental. However before the eye doctor may realize, the child is not seeing adequately and has developed a cataract or some even more serious source of vision loss, such as maculopathy, from chronic uveitis. The slow "dance" with the steroids can be quite seductive.

Figure 2. Inactive uveitis, quiet for three months, ready for cataract surgery. Posterior synechiae are present, as is a pupillary membrane.

Complications of pediatric uveitis include band keratopathy, glaucoma, phthisis, cataract formation, macular edema, and optic nerve problems. In our current study the rates of complications were lower. This is possibly due to the short mean follow-up period (22 months) compared to other similar studies; however, we believe that it is, more likely than not, a consequence of our more therapeutically aggressive approach to the problem, and our intolerance to endless steroid therapy.

Figure 3. This patient has a long history of recurrent uveitis, and the uveitis was successfully placed into remission with systemic immunomodulatory therapy. Cataract surgery was then performed, and it was uncomplicated. A posterior chamber lens implant was used, and this turned out to be a mistake in judgment, with lens intolerance by this eye. After one year of multiple YAG laser sessions for membrane and lens deposit removal, it is now clear that this lens implant should be removed and the patient left aphakic. The chronic inflammation and membrane formation has dragged the lens optic into the pupil and has produced intractable macular edema.

Uveitis and Referral

Eye doctors are becoming more and more aware of the importance of screening even very young children by slit-lamp examination. In addition, they are recognizing the importance of moving a child with JIA-associated uveitis along to a uveitis specialist very early in the course of events in­stead of repeatedly treating with steroid drops as was done frequently in the past.

Treatment for pediatric uveitis by the general ophthalmologist includes a general diagnostic work up and then administration of a substantial number of corticosteroid eye drops each day to quiet the eye. Drops then are ta­pered to full discontinuation. If the uveitis flares up again, I recommend that the eye doctor immediately make the re­fer­ral to the uveitis specialist. Some eye care physicians, however, may choose to refer the pediatric patient for the initial treatment. In general, the chronicity of uveitis is a tip-off that the patient should consult a uveitis specialist.

 

Early Detection

Uveitis can begin in infancy. Our study included large numbers of children between 2 and 7 years old. This age group faces another hurdle. If vi­sion is damaged and a cataract develops, then it is only a matter of time until the development of amblyopia.

If the child does not receive treatment promptly so he can obtain a good formed image on the retina of that eye that is affected, then he will be deprived of good formed images during the formative years when the neuroconnections are being made. If the uveitis and cataract problems are resolved later when the child is 11 or 12-years old, the child may still have a lifetime of poor vision because of the inevitable and irreversible lazy eye or amblyopia.

Pediatric Uveitis Screening and Legislation Mandatory early eye screenings for children can help thwart the silent progression of pediatric uveitis. The Academy of Ophthalmology, joined by American Association for Pediatric Ophthalmology and Strabismus (AAPOS) and several local ophthalmological societies in states that do not currently have children's vision requirements, are campaigning to support children's vision screening legislation. The group has agreed on model legislation that requires children entering kindergarten, first and third grades to be screened and any child who fails a screening to receive a comprehensive examination. The AAO and AAPOS believe periodic vision screening is an appropriate and effective tool for detecting vision and eye problems, such as pediatric uveitis, as eyes develop and before serious problems arise. According to AAO, vision screenings performed by pediatricians, family physicians, other trained health professionals and trained lay screeners are a cost-effective use of health-care dollars. In Mississippi (HB 884 and SB 2382), and Oklahoma (SB 1795), legislation has been introduced based on the model legislation. Pending legislation in California (AB 1892) seeks to strengthen the existing screening law and require follow-up examinations for children identified as needing treatment. On Feb. 2, 2006, Oklahoma State Sen. Susan Paddack, D-Ada, introduced the "Children's Vision for Excellence Act" (SB-1795), which also follows the model bill's requirements. The AAO and the Oklahoma Academy of Ophthalmology were driving forces behind the development of the legislation. Another bill, (HB 2382), similar in language and content to the Paddack bill, was introduced in the Oklahoma House of Representatives by Rep. Lance Cargill, R-Harrah. It has the support of the Oklahoma Association of Optometric Physicians. However, the early vision screening campaign is not without its detractors. According to the AAO, the effort faces a challenge from optometry, which has mounted a campaign to discredit screenings and to push for a costly comprehensive eye exam mandate for all children. On a related matter, the North Carolina Society of Eye Physicians and Surgeons (NCSEPS) is involved in a broad coalition effort to repeal a special provision in the 2005-2006 state budget requiring all incoming kindergartners to have eye examinations. The House Speaker, an optometrist, got the provision put in the state budget bill without hearing or debate at the end of the legislative session. An NCSEPS-backed pro-screening bill was introduced in response. To learn more about these issues contact your state representative or go to aao.org.

Future Implications

The key to earlier and earlier de­tection is legislation. Activists like Jean Ramsey, MD, of Massachusetts, have become in­volved with their state legislators and have effectively brought legislation to the forefront that mandates vision screening for young preschool children. Through their work, they have found the resources to pay for the devices used for vision screening and set up the appropriate training for screeners. It is a big challenge, but it is a challenge worth meeting (See sidebar, p. 38).

Figure 4. Uveitis, active, and not ready for cataract surgery. Note the posterior synechiae, the pupillary membrane and the ciliary flush superiorly.

Because of such early vision screenings, the child whose uveitis (or other visual problem) would have been detected at age 8 now can be detected at age 3, when something can be done before permanent damage sets in. This legislation is a boon to ophthalmologists and to the population at large. It is going to be one of two critical elements of driving down the prevalence of blindness caused by pediatric uveitis. The other element is earlier referral of chronic uveitis patients to a uveitis specialist who is prepared to use drugs other than steroids.

By working together and following through on these two critical elements we can best preserve the health and vision of young patients with chronic uveitis.

 

Dr. Foster is the founder and president of the Ocular Immunology and Uveitis Foundation at the Massachusetts Eye Research and Surgery Institute, in Cambridge, Mass. He is also a clinical professor of ophthalmology at Har­vard Medical School. For more information visit uveitis.org.

 

1. Gritz, DC. Wong IG Incidence and prevalence of uveitis in Northern California: The Northern California Epidemiology of Uveitis Study. Ophthalmology 2004;111:491-500.

2. Edelsten C, Reddy MA, Stanford MR, Graham, EM. Visual loss associated with pediatric uveitis in English primary and referral centers. AM J Ophthalmol 2003;135:676-80.

3. PaivÖnsalo-Hietanen T, Tuominen J, Saari KM. Uveitis in children: population-based study in Finland. Acta Ophthalmol Scand 2000;78-84-8.

4. Darrell, RW, Wagener HP, Kurland LT. Epidemiology of uveitis. Incidence and prevalence in a small urban community. Arch Ophthalmol 1962;68:502-14.

5. Kimura SJ, Hogan MJ, Thygeson P. Uveitis in children. Arch Ophthalmol 1954;51:80-8.

6. Kump L, Cervantes-Castañeda R, Androudi S, Foster CS. Analysis of Pediatric Uveitis at a Tertiary Referral Center. Ophthalmology 2005;112:1287-92.