Juvenile rheumatoid arthritis

Juvenile rheumatoid arthritis is a particularly serious form of anterior uveitis that occurs in children. A chronic iridocyclitis results, usually affecting young girls (aged 0–4 years) who have pauciarticular joint involvement. Unlike most forms of acute iridocyclitis, there rarely is associated discomfort, redness, or photophobia. Because many of these patients do not have ocular symptoms, it is important that they be followed closely by an ophthalmologist to detect and treat any inflammatory disease and sequelae – specifically glaucoma, cataracts, and band keratopathy.

There are several ophthalmic prognostic factors that have been elucidated, the most important of which is whether there is uveitis at the time of presentation of the arthritis.With uveitis, one-quarter will

likely lose vision to 20/200; one-half develop cataracts; one-quarter develop band keratopathy; and one-quarter develop glaucoma.360,361

If no uveitis appears in the first 5 to 7 years after the onset of juvenile rheumatoid arthritis, ocular involvement is unlikely.361,362

Medical treatment of this glaucoma is similar to that for the uveitic glaucomas in adults: steroids, mydriatic cycloplegics, and topical and systemic antiglaucomatous medications.363 The glaucoma is frequently from secondary synechial angle closure. When

surgery is required, a trabeculodialysis procedure has been used, producing reasonably successful results in this difficult disease.364,365

Trabeculodialysis is a rare instance where a goniotomy-like incision is effective in a non-infantile eye. In this procedure, after a parallel incision is made across the trabecular meshwork with a gonio-knife, small perpendicular flap edges are made at the two ends of the incision plane, and a long flap is peeled with the knife-point along its length towards the iris root. Scleral indentation, described for a similar procedure called goniosynechialysis in the management of chronic angle-closure glaucoma, may also enhance visulization for trabeculodialysis.366 Surgical alternatives include trabeculectomy with antimetabolites or glaucoma implant-shunt surgery.

As with adults, children suffering from infectious entities that afflict the anterior segment, such as herpes or opportunistic infections in immune-compromised patients,107 require appropriate diagnosis, management of the underlying condition, and attention to any ocular manifestations.

Steroid glaucoma in children

As discussed in Chapter 18, intensive exposure to topical or oral corticosteroids can often induce ocular hypertension, or even

frank glaucoma in susceptible individuals, presumably on a genetic basis.367,368 This phenomenon has been well documented in chil-

dren as well: in 4-week studies of b.i.d. or q.i.d. use of dexamethasone or fluorometholone topical drops in children under 10 years

old, elevated peak and elevated net IOPs were seen after 10 days, correlating with the intensity of the regimen.369,370

With the alarming worldwide increase of childhood-onset bronchial asthma, the widespread use of nasal and inhalation

corticosteroids on IOP is potentially of concern. Although only rare anecdotal cases have been reported371,372, ophthalmic moni-

toring for children on long-term steroids is advisable. Interestingly, under the age of 40, the association between inhaled steroids and cataract formation is negligible.373

Another potential concern for steroid-related elevated IOPs in children is following the use of intravitreal triamcinolone for intract­ able uveitis or complications of diabetic retinopathy. In several series of adult patients treated for a variety of retinal diseases, elevated IOP

responses greater than 10 mmHg were seen in between 28–60% of eyes after a single injection, manifesting between 1–12 weeks.374–376

Presumably the mechanism was that of steroid-induced trabecular metabolic dysfunction, since actual debris (from the injection) was rarely seen in the angle.377 Pressures, though usually responsive to topical medications, surgically responded either to removal of the intravitreal drug with pars plana vitrectomy378 or standard trabeculectomy.379 Given the comparable response of pediatric eyes to topical steroids as seen in adult eyes, monitoring for this complication of a new therapeutic modality is also warranted.

Neovascular glaucoma

Neovascular glaucoma is the end-stage manifestation for many diseases­ in which the common pathway is vascular ischemia.380,381

Childhood glaucomas associated with this difficult form of angle closure include retinoblastoma, Eales disease, Coats disease,382 X- linked familial exudative retinopathy (Norries disease),383–385 and so forth.

Trauma

Both blunt and penetrating trauma may occur in children and produce glaucoma from a variety of causes. Any two of the four cardinal signs of anterior segment injury may herald post-concussion glaucoma: angle recession more than 180°; traumatic cataracts; iris injuries; or lens displacement.386 Management is the same as that for trauma-induced glaucoma in the adult, but children require life-long surveillance for late-onset angle-recession glaucoma.

Birth injuries, particularly those caused by forceps damage, can present a difficult problem in the differential diagnosis of congenital glaucoma. Trauma to the periorbital skin area can be helpful in making this diagnosis, particularly when the glaucoma is unilateral. Forceps injury typically will cause breaks in Descemet’s membrane, with corneal edema. The breaks tend to be multiple and vertically oriented but may occur in any direction. The corneal edema usually disappears over several weeks as the corneal endothelium heals. Ocular trauma associated with delivery can produce a transient or permanent glaucoma depending on the degree and area of damage.

Medical management of the IOP elevation is advised when birth injury is the suspected cause. Both the IOP and the corneal ­damage from trauma may improve over several weeks or months.

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