Ординатура / Офтальмология / Английские материалы / Pediatric Ophthalmology for Primary Care 3rd edition_Wright, Farzavandi_2008

Chapter 14

Ocular Inflammation

and Uveitis

This chapter covers ocular inflammation including uveitis, episcleritis and scleritis, endophthalmitis, acute retinal necrosis syndrome, and ocular mani festations of acquired immunodeficiency syndrome (AIDS).

Uveitis

Uveitis is a term for intraocular inflammation involving the uveal tract (iris, ciliary body, and choroid). Ophthalmologists classify uveitis according to the location—anterior uveitis, affecting anterior chamber and iris; inter mediate uveitis, affecting the ciliary body area and anterior vitreous; poste rior uveitis, affecting the choroid and retina; or panuveitis, affecting

the entire uveal tract. Normally, the vitreous and the aqueous fluid are devoid of cells. In patients with uveitis, leukocytes circulate in the aqueous and also can be found in the vitreous. In addition, the protein content of the aqueous humor increases with inflammation. Clinically, the ophthalmologist uses a slitlamp to identify cells and flare (representing protein) circulating in the anterior chamber. Cells can only be seen by high powered magni fication, and flare is the light scatter in the protein laden aqueous humor. White cells can precipitate on the back of the cornea and are termed keratic precipitates (Figure 14 1). The term iritis refers to anterior inflammation, and iridocyclitis describes intermediate intraocular inflammation. Ophthal mologists often send patients with uveitis to the pediatrician for evaluation of systemic disease. Table 14 1 lists the more common diseases associated with pediatric uveitis.

Figure 14 1.

A photograph of a patient with chronic uveitis and keratic precipitates on the posterior surface of the cornea. Note the multiple white circular lesions.

Juvenile Rheumatoid Arthritis

Juvenile rheumatoid arthritis (JRA) represents approximately 70% of all pediatric arthritis and can be divided into 3 types.

•Still disease

•Polyarticular JRA

•Pauciarticular JRA

Still Disease

Still disease (systemic JRA) accounts for approximately 20% of all JRA cases; however, it is rarely associated with uveitis. Patients with this form of JRA present with fever, salmon colored rash, lymphadenopathy, and hepato splenomegaly. Less than 6% will develop uveitis.

Polyarticular Juvenile Rheumatoid Arthritis

Polyarticular JRA involves more than 4 joints. This accounts for approxi mately 40% of all patients with JRA. Most patients are females, and approxi mately 30% will be negative for the rheumatoid factor. Uveitis may occur, although uncommon, and is present in about 15% of patients with polyar ticular JRA.

Pauciarticular Juvenile Rheumatoid Arthritis

Pauciarticular JRA involves 4 joints or fewer, and 2 forms of this type of JRA have been described. Pauciarticular type 1 (early onset) is predominantly seen in girls younger than 5 years and is associated with a high incidence

of uveitis (25% of patients). Most patients are antinuclear antibody positive (ANA+). Pauciarticular type 2 (late onset) is usually seen in older boys and is often associated with recurrent anterior uveitis. Approximately 75% will test positive for HLA B27. Many of the boys with late onset pauciarticular JRA will develop ankylosing spondylitis.

The ocular inflammation associated with JRA does not parallel the joint inflammation. The uveitis seen in patients with JRA usually occurs within 7 years of the onset of the arthritis; however, late onset uveitis may occur up to 20 years after JRA is diagnosed. Because uveitis associated with JRA is asymptomatic until severe ocular damage occurs, children with JRA should be screened early for uveitis. Patients with JRA who have the highest risk of developing uveitis are female, have early onset pauciarticular JRA, and are

ANA+. The early detection of ocular inflammation is critical to decreasing the visual loss that can occur with JRA uveitis. Table 14 2 lists a recom mended screening schedule for ocular examinations.

Complications of JRA uveitis include keratic precipitates (white cells on the posterior surface of the cornea), posterior synechiae (iris adherence to the lens), cataracts, glaucoma, cyclitic membrane (scarring in the area of the ciliary body), and blindness. Treatment is based on reducing ocular inflammation to prevent these ophthalmologic complications. Topical cor ticosteroids are used to reduce anterior chamber inflammation and may be required as frequently as a drop every hour in severe cases. Corticosteroids should be quickly tapered when inflammation is controlled, as corticoste roids can cause cataracts and glaucoma. The use of topical mydriatics to keep the pupil mobile and avoid pupillary scarring to the lens (posterior

synechiae) is also advocated. Most patients with JRA uveitis are treated with chronic topical corticosteroids and many experience serious side effects (cataracts and glaucoma). Systemic methotrexate may be used in those chil dren with severe uveitis to reduce the topical corticosteroid dose necessary to control inflammation.

Another complication of JRA is band keratopathy, representing calcium deposits within the surface corneal epithelium (Figure 14 2). Once severe pathology occurs, such as glaucoma, cataracts, and band keratopathy, the prognosis for good vision is poor (Figure 14 3). Approximately one third of cases with glaucoma progress to no light perception vision.

Table 14-2. Screening Schedule for Ocular Examinations in Patients With Juvenile Rheumatoid Arthritis

Figure 14 2.

Band keratopathy in a child with juvenile rheumatoid arthritis. Note the white band opacity extending horizontally across the cornea. Band keratopathy represents calcium deposits within the corneal epithelium.

Figure 14 3.

Patient with advanced uveitis associated with juvenile rheumatoid arthritis. Note the peripheral band keratopathy at the 3- and 9-o’clock position, the white pupil indicating a dense cataract, and a relatively small cornea indicating end-stage severe ocular disease (pre-phthisis bulbi).

Juvenile Reiter Syndrome

Juvenile Reiter syndrome is a rare entity consisting of the classic triad of arthritis, urethritis, and conjunctivitis (rarely uveitis). Children typically develop this syndrome after an episode of salmonella or shigella enterocolitis.

Juvenile Psoriatic Arthritis

Juvenile psoriatic arthritis is associated with skin changes, nail pitting, and joint involvement. It is seen more frequently in girls and may be associated with a chronic anterior uveitis.

Juvenile Inflammatory Bowel Disease

Juvenile inflammatory bowel disease is an enteropathic arthropathy and is associated with rheumatologic manifestations such as ulcerative colitis and Crohn disease. Patients with inflammatory bowel disease and HLA B27 haplotype have a higher incidence of sacroiliac joint disease and uveitis. Uveitis is a common ocular complication and occurs in approximately 11% of patients diagnosed with HLA B27–positive inflammatory bowel disease. Other ocular manifestations include conjunctivitis, corneal infil trates, episcleritis, scleritis, and optic neuritis. The ocular inflammation tends to parallel the intestinal inflammation. Some have suggested that a colectomy, resulting in improvement of the ulcerative colitis, also improves the ocular disease.

As with JRA related uveitis, patients should be treated with topical corticosteroids and mydriatics based on the degree of inflammation. Uveitis associated with spondyloarthropathies runs a course of recurrent bouts of acute inflammation. If treated early, the prognosis is usually quite favorable.

Sarcoidosis

Sarcoidosis is a granulomatous inflammatory disease most commonly seen in adults between the ages of 20 and 50 years but occasionally also seen in children. There are 2 distinct groups of pediatric sarcoidosis patients—one group with early onset of 5 years and younger, and the second group with later onset of 8 to 15 years of age. Pulmonary involvement is seen in almost 100% of patients in the older age group, even though the patients may

not have symptoms. Lymphadenopathy, hepatosplenomegaly, and ocular

before treatment is started. The diagnosis can be confirmed by a biopsy of the skin, lymph node, and conjunctival nodule. Blind biopsy of the conjunc tiva is usually not productive.

Treatment of systemic sarcoidosis consists of using systemic corticoste roids to treat the systemic inflammation. Ocular inflammation is also con trolled through the use of corticosteroids. These are given topically or orally, along with a mydriatic agent, to keep the pupil mobile. Because of the high incidence of ocular involvement, children with sarcoidosis should have regu lar ocular examinations.

Ocular Toxocariasis

The dog roundworm, Toxocara canis, may be found in the dirt of parks and playgrounds and is present in up to 80% of puppies. This is the most common nematode that causes ocular infections in the United States. In the dog, T canis has a complete life cycle. In humans, however, the cycle is

incomplete. Typically, a child ingests soil that contains toxocara ova. The ova hatch in the small intestine and the larvae pass through the intestinal wall to spread to various organs including the liver, lung, brain, and eye. This dis semination of the larvae is called visceral larva migrans (VLM).

Visceral larva migrans presents as a cough, fever, malaise, loss of appe tite, and in some cases, seizures. The peripheral blood has generalized leuko cytosis with prominent eosinophilia. The VLM syndrome most commonly occurs in children between 6 months and 3 years of age, often with a history of pica and proximity to puppies. Ocular infestation involves the choroid and retina and may result in severe inflammation with secondary scarring and even blindness. The larvae incite a granulomatous response that will not interfere with vision if it is confined to the periphery. Toxocara granulomas within the foveal area, however, can result in decreased vision and even legal blindness (Figure 14 6). In some cases, severe inflammatory reaction occurs, resulting in endophthalmitis and possible loss of the eye.

Toxocara ocular disease is one of the causes of leukocoria in infants and children. The diagnosis is made by the characteristic retinal lesions seen on ophthalmoscopy and by ocular ultrasound, in addition to obtaining serum enzyme linked immunosorbent assay (ELISA) titers for antibodies to toxocara. Treatment is observation for small peripheral lesions; how ever, larger lesions close to the macula and cases with severe inflammation