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Ординатура / Офтальмология / Учебные материалы / Section 6 Pediatric Ophthalmology and Strabismus 2015-2016.pdf
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endophthalmitis. There is often little external evidence of inflammation. Patients may present with leukocoria, strabismus, or decreased vision. These are also common presenting symptoms of retinoblastoma, which must be differentiated from ocular toxocariasis. Although enzyme-linked immunosorbent assay (ELISA) titers for Toxocara species have a high sensitivity and specificity and are useful in diagnosing this disease, a positive laboratory finding does not eliminate the possibility of retinoblastoma, as elevated titers may be found in a significant percentage of the general pediatric population.

Figure 24-3 Toxocariasis, right eye. A, Distortion of posterior pole vessels. B, Peripheral granuloma.

Treatment includes observation of peripheral lesions, periocular or systemic steroids for posterior lesions and endophthalmitis, or surgical intervention to address retinal traction, cataract, glaucoma, or cyclitic membranes. Systemic antihelmintics are not useful in treating ocular toxocariasis, as the organism producing the inflammation is already dead.

Woodhall D, Starr MC, Montgomery SP, et al. Ocular toxocariasis: epidemiologic, anatomic, and therapeutic variations based on a survey of ophthalmic subspecialists. Ophthalmology. 2012;119(6):1211–1217.

Panuveitis

Sarcoidosis

Sarcoidosis may present in 2 distinct forms in children. In young patients (<5 years), lung disease is rare and sarcoidosis is more often characterized by the triad of uveitis, arthritis, and rash. Early-onset sarcoidosis is considered a pediatric granulomatous arthritis and is phenotypically and genetically similar to Blau syndrome (see discussion in next section). Older children (8–15 years) have the pulmonary abnormalities and lymph node findings more commonly associated with the adult form of the disease and are at risk for uveitis. Although anterior uveitis (Fig 24-4) is the most common manifestation of ocular sarcoidosis in children, this disease can produce a panuveitis.

Figure 24-4 Keratic precipitates in sarcoidosis. (Courtesy of Ken K. Nischal, MD.)

Diagnosis and treatment in children is similar to that in adults, but serum angiotensin-converting enzyme (ACE) levels in healthy children are higher than they are in adults and thus can be misleading when used to diagnose pediatric sarcoidosis. If this disorder is suspected, children should undergo careful evaluation for systemic disease.

Shetty AK, Gedalia A. Childhood sarcoidosis: a rare but fascinating disorder. Pediatr Rheumatol Online J. 2008;6:16.

Familial Juvenile Systemic Granulomatosis

Familial juvenile systemic granulomatosis (Blau syndrome) is an autosomal dominant disease that may be identical to early-onset sarcoidosis; both are classified as pediatric granulomatous arthritides. Both diseases have mutations in the NOD2 gene on chromosome 16, but in Blau syndrome other family members are affected. Both diseases present with granulomatous arthritis, uveitis, and rash during childhood. Pulmonary involvement and adenopathy are absent in Blau syndrome. Chronic panuveitis associated with multifocal choroiditis is the most common ocular presentation, but uveitis may be limited to the anterior segment in some cases, and the disease may be misdiagnosed as JIA. Ocular complications, including cataract, glaucoma, band keratopathy, and vision loss are common.

Okafuji I, Nishikomori R, Kanazawa N, et al. Role of the NOD2 genotype in the clinical phenotype of Blau syndrome and early-onset sarcoidosis. Arthritis Rheum. 2009;60(1):242–250.

Vogt-Koyanagi-Harada Syndrome