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Part 2 book “Harley’s pediatric ophthalmology” has contents: Diseases of the retina and vitreous, congenital abnormalities of the optic disk, disorders of the lacrimal apparatus in infancy and childhood, pediatric eyelid disorders, systemic hamartomatoses, pediatric neuroophthalmology,…. And other contents.
13 Pediatric Uveitis Grace T Liu • Alex V Levin INTRODUCTION Uveitis in the pediatric population is a significant cause of ophthalmic morbidity Approximately 2% to 14% of patients seen in uveitis clinics are children (1–3) Unique to the pediatric age group in the management and timely diagnosis of uveitis is the threat of amblyopia In addition, the associated systemic disease profile is much different than that seen in adults Children more often (71%) have an associated systemic illness than adults (55%) (4) Although juvenile idiopathic arthritis (JIA) is the predominant cause of anterior uveitis in children (5,6), it is important to recognize that uveitis in a child can be due to a wide range of etiologies, including serious life-threatening masquerade syndromes such as retinoblastoma and leukemia Presenting signs and symptoms are often not recognized until advanced stages, and the disorder may even be entirely asymptomatic until irreversible ocular damage has been sustained (Fig 13.1) The child may be unable to verbalize his/her symptoms, and can often function normally with visual acuity well below 20/20 for activities of daily living, especially when the disease is unilateral and the child is younger The approach to pediatric uveitis requires the understanding that early recognition through screening, where appropriate, can be of utmost importance Table 13.1 summarizes the diagnostic approach to the child with pediatric uveitis To the contrary, a study from the US National Eye Institute found that idiopathic uveitis (28.8%) was the leading etiology in the United States, followed by JIA (20.9%), and pars planitis (17.1%) A retrospective study characterizing disease characteristics and visual outcome of 527 children in the United States with uveitis, found that 54% were female; 62% White, 15% Hispanic, 12% Black, 3% Asian, and 2% multiracial (9) The median age at diagnosis was 9.4 years CLASSIFICATION Although consortium-driven classification systems have been proposed, they may be difficult to use in the clinical setting The Standardization of Uveitis Nomenclature (SUN) criteria were developed for classification and description of uveitis by anatomic location (Table 13.2) Specific grading criteria, such as quantitative grading of inflammation, were also elaborated (10) Morphologic classification, according to cell type, for example granulomatous versus nongranulomatous, may be less useful in children A broader classification might separate etiologies into exogenous, representing any external injury or invasion of microorganisms from outside the globe, versus endogenous, resulting from factors that originate within the patient TREATMENT AND COMPLICATIONS Topical Medical Therapy EPIDEMIOLOGY The frequency and etiology of childhood uveitis is in part dependent on geography Widespread globalization may also affect the current distribution of the disease A meta-analysis of worldwide studies showed that 7% of patients with uveitis are children Parasitic anterior uveitis (49.3%) is the most common etiology globally, with idiopathic being the second most common (25.5%) (7) A group from Saudi Arabia reported idiopathic anterior non-granulomatous uveitis as the most common type of uveitis in children (26%) (8) A report from Israel found infectious diseases to be the primary etiology of uveitis in children and adolescents (31.2%) (1) Even with low-grade iritis, the goal is early, aggressive treatment to suppress inflammation maximally, in hopes of preventing the development of vision-threatening complications (11–14) When inflammation is more severe, topical corticosteroids may be indicated as frequently as every to hours Follow-up within to weeks after initiating treatment is critical to ensure improvement Perhaps the most common reason for recalcitrant and recurrent uveitis is the too rapid tapering of topical steroids Although a fairly rapid taper may be appropriate on the first episode, any indication of iritis recurrence during the taper should be met with a change to a slow taper It may take weeks, months, or even 283 (c) 2015 Wolters Kluwer All Rights Reserved 284 HARLEY’S PEDIATRIC OPHTHALMOLOGY FIGURE 13.1 Child with asymptomatic oligoarticular juvenile idiopathic arthritis who did not present until visual loss was noted by which time she had a dense white cataract, multiple posterior synechiae, and active anterior uveitis Note the absence of conjunctival inflammation years to accomplish a full taper in some children Although there are certainly risks of steroid-induced cataract or glaucoma, the risk of these complications from inadequately treated uveitis is even greater Our experience with high dose and chronic topical steroid use actually shows a reduction in such complications and better vision outcomes (15) Cycloplegic agents are also important, given the increased tendency of children to form synechiae Many different regimens have been suggested (16) We prefer a minimum of one dose of cyclopentolate 1% at bedtime It is also important to consider (and treat with glasses if needed) the blur induced by cycloplegia, especially in school-aged children A recent study specifically on the use of difluprednate in pediatric uveitis demonstrated its use to be effective for anterior segment inflammation and reduction of cystoid macular edema (CME) when used as an adjuvant to systemic immunomodulatory therapy, but it was not without risks (17) Glaucoma Table 13.1 EVALUATION AND TESTING FOR PEDIATRIC UVEITIS History of Present Illness Medical history: Systemic illnesses (e.g., sarcoidosis, JIA, AIDS, TB) Social history: Sexual history (where appropriate based on age) Birthplace Travel (e.g., Ohio River Valley), camping/environment (exposure to ticks) Review of Systems: General: fever, weight loss, malaise, night sweats, weight loss, lymphadenopathy Ear, nose, throat: hearing loss, tinnitus Cardiac: murmurs (e.g., mitral regurgitation in Kawasaki disease) Respiratory: shortness of breath, cough, history of “asthma” (e.g., could be sarcoid) Gastrointestinal: oral ulcers, diarrhea, bloody stools Genitourinary: dysuria, discharge, ulcers Musculoskeletal: lower back pain, arthralgias, joint stiffness, myalgia Dermatologic: rashes, desquamation, alopecia, vitiligo, tick and insect bites Neurologic: headaches, meningitis, paresthesias Laboratory Studies: First line: CBC, ESR, ANA, RF, serum calcium, FTA-ABS, RPR, HLA-B27 (if clinically appropriate based on age and presentation), Lyme titers (if endemic region), urinalysis, tuberculin skin test, chest radiograph Second line: Serology: EBV titers, HSV/HZV, toxoplasmosis, toxocara, brucellosis titers, BUN and Cr, serum lysozyme (continued) (c) 2015 Wolters Kluwer All Rights Reserved CHAPTER 13: PEDIATRIC UVEITIS 285 Table 13.1 (continued) Other radiographic studies, when indicated: Sacroiliac joint, gastrointestinal series Ultrasound—joints Ancillary tests, when indicated: Fluorescein angiography Vitreous tap Lumbar puncture Renal biopsy AIDS, acquired immunodeficiency syndrome; ANA, antinuclear antibody; BUN, blood urea nitrogen; CBC, complete blood count; Cr, creatinine; EBV, Epstein-Barr virus; ESR, erythrocyte sedimentation rate; FTA-ABS, fluorescent treponemal antibody absorption; HLA-B27, human leukocyte antigen-B27; HSV, herpes simplex virus; HZV: herpes zoster virus; JIA, juvenile idiopathic arthritis; RF, rheumatoid factor; RPR, rapid plasma reagin; TB, tuberculosis and cataract were both observed at significant rates, and the authors recommend close monitoring of pediatric patients on this medication Systemic Medical Therapy Methotrexate is usually the first-line systemic agent if topical therapy fails to control the iritis, or if chronic high frequency dosing of topical steroids is needed Absorption of oral methotrexate in children can be variable, and subcutaneous injection may be more effective GI upset is a common side effect of oral administration (18) Caution should be taken with use of chronic oral corticosteroids in children The risks of growth retardation, osteoporosis, adrenal suppression, gastrointestinal upset, emotional lability, and susceptibility to infection must be Table 13.2 THE SUN WORKING GROUP CLASSIFICATION OF UVEITIS (10) Type Primary Site of Inflammation Includes Anterior uveitis Anterior chamber Iritis Iridocyclitis Anterior cyclitis Intermediate uveitis Vitreous Pars planitis Posterior cyclitis Hyalitis Posterior uveitis Retina or choroid Focal, multifocal, or diffuse choroiditis Chorioretinitis Retinochoroiditis Retinitis Neuroretinitis Note: panuveitis includes inflammation in anterior chamber, vitreous, and retina or choroid SUN, Standardization of Uveitis Nomenclature (c) 2015 Wolters Kluwer All Rights Reserved 286 HARLEY’S PEDIATRIC OPHTHALMOLOGY considered Secondary glaucoma may occur more frequently in children than in adults (19,20) In general, oral corticosteroids are reserved for short course (