First Author: J.Nobre-Cardoso PORTUGAL
Co Author(s): A. Cardoso A. Miranda N. Marques S. Barros B. Beltrán N. Campos 0 0 0 0 0 0 0 0 0
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Childhood sarcoidosis is a rare granulomatous disorder of unknown etiology with multisystemic manifestations. Ocular involvement is common in childhood sarcoidosis and may be present in 29% of cases, with granulomatous anterior uveitis being the commonest of manifestations. Posterior uveitis is rare and a prompt treatment is required to preserve visual function.
Hospital Garcia de Orta, E.P.E. (Almada/Portugal)
We present a case of a 10 year old boy of African origin without any relevant medical history that presented to the emergency room with a bilateral red eye, photophobia and decreased visual acuity.
The ophthalmologic examination revealed a visual acuity of 20/30 on the right and 20/60 on the left eye, normal IOP and an anterior segment with a granulomatous uveitis with mutton-fat keratic precipitates and Busacca nodules. Fundoscopic examination revealed multiple hypopigmented lesions throughout the retina that presented a window-defect on fluorescein angiography in both eyes. No signs of vitritis, vasculitis or active chorioretinitis were detected. The patient was treated with topical corticosteroids with rapid improvement of the anterior uveitis and visual acuity. Ancillary-tests were negative for infectious diseases but a slightly high angiotensin converting enzyme and lysozyme were detected. Extra-ocular evaluation detected an erythematous rash of the lower limbs but no other extra-ocular involvement. Four months later, the patient presented again to the emergency-room due to a month long decrease of visual acuity of the left eye with metamorphopsias. No anterior segment inflammation was present, but fundoscopy revealed a white granulomatous lesion adjacent to the optic disc with subretinal hemorrhage and edema. Optical-coherence-tomography confirmed these findings. Systemic corticosteroids were immediately started and, after three months, immunomodulatory therapy with adalimumab. During follow up, visual acuity improved to 20/20 with disappearance of the subretinal hemorrhage and edema but the granulomatous retinal lesion remained.
Sarcoidosis is a disease with many ocular manifestations. It is a rare cause of childhood uveitis and its diagnosis is hampered by the difficulty to correctly evaluate ancillary tests. In case of posterior uveitis, a prompt diagnosis with early immunosuppressive therapy is needed so that the visual prognosis is preserved. This case exemplifies a case of ocular sarcoidosis that was difficult to diagnose but prompt treatment with imunosupressives therapy when the patient presented with severe posterior uveitis allowed a good visual prognosis.