Table 19.1  The Standardization of Uveitis Nomenclature

(SUN) Working Group Classification of Uveitis.

The SUN Working Group Anatomical Classification of

Uveitis

Adapted from: Standardization of nomenclature for reporting clinical data: results of the First International Workshop. Am J Ophthalmol. 2005;140:509–516

literature. Classically, fluorescein angiogram reveals abnormal retinal capillary permeability, with late pooling of dye in a perifoveal petaloid pattern.

Although perifoveal capillaries have been demonstrated to be the source of edema in CME, the precise pathophysiology has not been completely elucidated. Inflammatory mediators such as free radicals, prostaglandins, and others (serotonin, bradykinin, histamine, substance P, and leukotrienes) may be important ­factors liberated by anterior or posterior segment inflammation [5, 6]. Diffusion to the posterior pole may cause disruption of the blood-retinal barrier located at the endothelium of retinal vessels. This theory is supported by evidence that cyclooxygenase inhibitors reduce the incidence of angiographic CME [7]. However, this finding has only been demonstrated conclusively in pseudophakic CME. Pathologically, swelling and degeneration of Muller cells is present in early CME, suggesting that dysfunction of this cell type may contribute to extracellular fluid accumulation in the retina [8].

The incidence of CME associated with various uveitides is not well documented, although certain entities such as pars planitis are associated with a higher incidence of CME [9].

19.3  Social and Family Impact

The diseases discussed in this chapter can have a profound social impact on the child and his/her family support system. As with any pediatric disease, pediatric uveitis is met with immediate parental concern, and often emotional stress, over the current and future well-being of their child. The potential sight-threaten- ing complications of these diseases can have a dramatic effect on the development and education of children. Many pediatric uveitides require frequent visits to the ophthalmologist and other physicians, which can place a financial strain on the family; additionally, it may be difficult for many families to schedule visits that they can attend with their child. Many conditions also require the frequent administration of drops, which can place a burden on the child’s caregiver. Chronic systemic administration of corticosteroids places a child in the prepubescent age group at risk for growth retardation. Alternatively, systemic immunomodulators are being employed in more clinical situations, but there can be parental concern over their potential reproductive side-effects as well [10].

The remainder of the chapter will discuss in more detail the most common uveitides in the pediatric population that can affect the retina. We will first divide the entities by etiology (infectious, non-infectious), and then discuss each disease based on its SUN anatomical location.

19.4  Noninfectious

19.4.1  Juvenile Rheumatoid Arthritis

19.4.1.1  Historical Context

Chronic juvenile arthritis associated-uveitis is the most common cause of chronic intraocular inflammation in children [1]. The association between chronic arthritis