Pediatric Uveitis

19.1  General Introduction

Uveitis in the pediatric population represents a distinct subset of diagnoses that provide unique diagnostic and therapeutic challenges to the ophthalmologist. About 5–10% of the total cases of uveitis seen in tertiary care centers occur in pediatric patients, with an annual occurrence rate of 6:100,000 [1]. A delay in diagnosis can occur because of the relative rarity of uveitis in children, as well as difficulty for the child to recognize and verbalize symptoms. More so than in the adult population therapeutic options in the pediatric population are limited by side effects. Therapeutic effect can also be limited by family and patient compliance in administering systemic medications and drops. Lastly, children offer the unique risk of amblyopia, a problem not encountered in adults with uveitis.

19.2  Classification

In children as in adults, uveitis may be classified according to a number of parameters including the clinical course of inflammation (acute, chronic, recurrent), characterization of the inflammation (granulomatous, nongranulomatous), etiology (infectious, immunologic, idiopathic), and anatomical location (anterior, intermediate, posterior). A need for standardization led to the formation of the Standardization of Uveitis Nomenclature (SUN) working group, which in 2005 developed an anatomical classification system, clinical descriptors, standardized grading systems, and

C. Hood and C.Y. Lowder (*)

Cleveland Clinic Cole Eye Institute, Cleveland, OH, USA e-mail: lowderc@ccf.org

terminology for following the activity of uveitis [2]. Although originally developed for adults, this system can be applied with great utility to the pediatric population. Specifically, it can assist in formulating a differential diagnosis based on the anatomical location of inflammation. This can allow for a tailored, cost-effec- tive uveitis evaluation for a given constellation of signs and symptoms (Table 19.1).

The retina can be affected by uveitis directly by intraocular inflammation (posterior uveitis, panuveitis) or indirectly in which the common end-point is cystoid macular edema (CME). Although posterior uveitis accounts for a greater proportion of uveitis cases in children than in adults, recent studies report that the majority of children with uveitis have inflammation that is restricted to the anterior segment [3, 4].

CME is a common cause of visual loss in eyes with uveitis, and is usually a sequela of long-standing intraocular inflammation; it is rarely seen when the inflammatory process lasts less than 6 weeks. The severity of CME may wax and wane with the severity of the intraocular inflammation, but it is often slow to respond and can remain even after active inflammation appears to have resolved.

CME is characterized by intraretinal edema contained in cystoid spaces around the fovea, and is often clinically evident stereoscopically by slit lamp biomicroscopic exam with a Hruby lens, contact lens, or a 78or 90-diopter lens. Optical coherence tomography (OCT) allows for the detection of edema that is below the clinical threshold, and has become the standard of care for the quantitative measurement of uveitic CME. Serial scans can be used to objectively evaluate response to treatment. OCT scans demonstrate fluid accumulation in cystic areas of low reflectivity in the outer plexiform layer of the neurosensory retina, which correlates with histopathology studies reported in the