Treatment requires the use of periocular, systemic steroids, which can reduce inflammation, but may have ocular and systemic side effects.

Discontinuous treatment with Infliximab, single or associated, favours disease remission.

Untreated or poorly treated intermediate uveitis has reserved visual potential with irreversible vision loss. (6)

It is the inflammation of the choroid, retina, +/- optic nerve and represents 25-30% of uveitis, the most common etiology being: toxoplasmosis, toxocariasis, CMV, Lyme, non-infectious uveitis in systemic diseases: sarcoidosis, Behcet’s disease, VKH, idiopathic retinitis, tubulointerstitial nephritis. (9)

Sarcoidosis is a multisystemic disease of unknown etiology, present in the 8–15-year-old children (sporadically), characterized by the formation of granulomas at tissue level, ocularly associated with anterior chronic granulomatous uveitis, with “mutton fat” precipitates, with moderately severe inflammation of the vitreous, with intermediate uveitis. Children under 5 years old often have anterior uveitis, skin lesions, elbow-hand arthritis. Older children have adult-like uveitis. Intermediate uveitis can be present with moderate vitreous inflammation, snow ball, snow bank, exudates, choroidal granulomas (sarcoid), periphlebitis “headlight in the fog”. (11)

Ocular manifestations in sarcoidosis are present in 20-30% of cases (2,3)

The anterior pole in sarcoidosis may present:

• nongranulomatous anterior uveitis, unilateral, with sudden onset, with adenopathy and erythema nodosum
• granulomatous, chronic, bilateral uveitis, with “mutton fat” precipitates, associated with chronic sarcoidosis, with pulmonary fibrosis
• uveopapillitis, Heerdfort syndrome, bilateral, granulomatous panuveitis, parotid gland involvement, cranial nerve palsy, rash, fever
• other ocular manifestations: conjunctival granulomas: dry eye, scleral nodules, cataract, glaucoma, papilledema, optic neuritis, muscle paralysis, proptosis

The posterior pole in sarcoidosis shows:

• retinal periphlebitis, retinal vascular occlusions
• granulomas in the choroid, retina, papilla, preretinal (Landers sign)
• retinal neovascularisation, CME

The treatment of ocular manifestations in sarcoidosis is complex and adapted to the evolving clinical aspect.

• Uveitis treatment – topically -mydriatic, cyclopegic, steroid therapy
• periocularly–subtenon Triamcinolone, 40mg, every 4 weeks 
• systemically -oral steroid, prednisone 1mg/kg/day
• immunosuppressants – Methotrexate, Azathioprine, in recalcitrant forms, with chronic evolution and prolonged inflammation;

Ciclosporin in steroid-refractory forms.

• CME treatment – Epinephrine (drops), Acetazolamine, NSAIDs (orally, topically), topical, systemic, subtenon steroids (Metylprednisolone 80mg/ml, 0.5ml)
• medical-surgical treatment of neovascularisation, of glaucoma, differentiated according to the type of glaucoma (inflammatory, open-angle, post steroid, neovascular, closed-angle glaucoma).

Familial, juvenile, systemic granulomatosis – Blau syndrome. It is an autosomal dominant condition, characterized by glanulomatous polyarthritis, skin rash, granulomatous anterior uveitis. In young children, it can be considered infantile sarcoidosis.

Behcet’s disease is a chronic, recurrent, multisystemic, rare inflammatory disease in children, with immunological determinism (autoimmune) in the possible presence of an infectious trigger.

The ocular manifestation in Behcet’s disease is the anterior uveitis (iridocyclitis) with hypopyon, acute, recurrent, accompanied by serious sequelae and complications that cause vision loss. (19)

• posterior uveitis can be a determining factor for decreased vision, with progression to blindness, can be isolated or associated with: anterior uveitis (panuveitis), arterial or venous vasculitis, with vascular occlusions generating retinal ischemia and neovascularisation, retinal hemorrhages, inflammatory or papillary ischemic edema, uni/bilateral, macular edema, CME.

The evolution of ocular manifestations in Behcet’s disease is long (sometimes years), multiple, severe, recurrent, accompanied by complications.

The visual prognosis in Behcet’s disease is reserved by the evolution and recurrence of the disease, by complications and sequelae. (19)

Treatment:

• inflammation control, through topical, systemic anti-inflammatory therapy
• topical steroids associated with mydriatics and cycloplegics
• systemic corticosteroids, sometimes combined with immunosuppression therapy
• immunosuppressants - Azathioprine, 2-2.5 mg/kg/day, Cyclosporine 3-5 mg/kg/day (contraindicated in Behcet’s neurological determinations) - Cyclophosphamide, Methotrexate
• biological agents, antiTNF alpha: Infliximab 5m/kg/body, controls symptoms, minimizes recurrences, decreases corticosteroids, Adalimumab 40mg/2 weeks, in Infliximab intolerance
• Ophthalmological treatment of cataracts, ocular hypertension, vitrectomy if needed, laser photocoagulation, in ischemic areas
• Treatment of joint injuries: Colchicine, Azathioprine, Steroids (Methyl, Prednisone)
• Treatment of neurological lesions: Corticosteroids, Azathioprine, Cyclophosphamide

Vogt Koyanagi Harada syndrome (VKH) – multi-systemic, idiopathic, rare condition in children, where neurological manifestations are absent.

The ocular form presents anterior, granulomatous, recurrent, bilateral uveitis, with “mutton fat” precipitates, posterior synechiae, iris nodules, associated with posterior uveitis, papillary edema, Dalen-Fuchs nodules. Posterior uveitis is bilateral, suddenly associated with exudative retinal detachment, with a high decrease in vision. Exudative retinal detachment can be resorbed spontaneously or by systemic steroids. (2.3)

Long evolution, with complications: cataract 42%, secondary glaucoma 37%.

Treatment: intravenous Methylprednisolone 3 days, followed by oral Prednisone 1mg/kg/day.

Azatioprine, Mycophenolate mofetil, Methotrexate

Sympathetic ophthalmia is a granulomatous, bilateral panuveitis, rare in the child, in the second eye, when the first eye suffered a trauma in the region of the ciliary body; probably an autoimmune disease against the melanocytes of choroida, as a result of genetic predisposition. (1)

The clinical signs are serious, with anterior granulomatous uveitis, “mutton fat” corneal precipitates, posterior synechiae, iris nodules, associated with the posterior uveitis, hialitis, Dalen-Fuchs nodules, severe retinal detachment, papillary edema.

Treatment: corticosteroids, immunosuppressants, Azatioprin, Chlorambucil in recalcitrant forms; the recommended enucleation in the first weeks of trauma is currently controversial.

Toxoplasmosis is a parasitic disease produced by infestation with the toxoplasma gondii protozoan. Primary disease affects CNS and retina, having a predilection for nerve fibres.

Toxoplasmosis is the most common cause of pediatric posterior uveitis in the acquired form in 25% of cases. (1)

Acquired toxoplasmosis has multiple ocular manifestations, with anterior and posterior uveitis. The functional signs are related to the location of the outbreak.

Anterior uveitis is granulomatous, with “mutton fat” precipitates and the irido-crystalline synechiae.

Anterior uveitis in the acquired toxoplasmosis is associated with posterior uveitis, neuroretinitis, papillitis, possibly complicated by choroidal neovascularisation.

Treatment: Pyrimetamine, and Mycophenolate mofetil, (folic acid supplements), Clindamycin, Azithromycin, Trimotropin and Sulfamethoxazole to prevent recurrences.

Uveites in children are rare: 3%, with a variable prevalence up to 10

Anterior, intermediate, posterior uveitis, panuveitis, are rare in children and have infectious etiology (bacterial, viral, parasitic), non-infectious etiology, strictly ocular, which is idiopathic uveitis (Fuchs’, Coats’ disease), or associated in system diseases (AIJ, spondyloarthropathy, sarcoidosis, Behcet’s disease).

Pediatric uveitis is chronic, recurrent, with prolonged evolution and is accompanied by serious complications: cataract, glaucoma, band keratopathy, decreased, loss of vision.

JIA is the most common cause of uveitis in the child, with an incidence of up to 30%, before the age of 16, asymptomatic, with white eyes, associated with chronic, oligoarticular arthritis, ANA+, RF-. The patient with uveitis should be diagnosed early and treated according to the stage of the disease, by regular check-ups (scheduled at 3.6,12 months) to limit and avoid the unfavourable evolution leading to vision loss.

Uveitis in JIA is anterior, unilateral, non-granulomatous, with chronic and complicated evolution, requiring judiciary, corticosteroid therapy, controlled for side effects, locally and systemically.

The second-line therapy in JIA consists of immunosuppressants - Methotrexate, Azathioprine, Cyclophosphamide, Mycophenolate mofetil and AntiTNF alfa, Infliximab, Adalimumab.

The treatment of complications requires the surgical treatment of cataracts with phacoemulsification with/without IOL, medical, surgical treatment of glaucoma, retinal detachment.

Anterior uveitis in children is also present in ankylosing spondylitis, psoriatic arthritis, inflammatory bowel disease.

Intermediate uveitis is rare in children and is present in the idiopathic uveitis in children under 7 years old, having a prolonged evolution and a reserved visual prognosis.

Posterior uveitis in children is complicated by the decrease of vision and have multiple and varied etiopathogenesis. The etiology is infectious, bacterial, viral, parasitic, or non-infectious associated with systemic diseases in sarcoidosis, Behcet’s disease, VKH. It occurs sporadically, rarely in the child, with long evolution and complications.

Toxoplasmosis is the main cause of posterior uveitis in the child.

Pediatric uveitis has a chronic evolution, with complications and decrease/loss of vision, requiring aggressive, complex diagnosis and treatment with anti-inflammatory drugs, corticosteroids, immunosuppressants.

Prolonged monitoring of uveitis in the child is mandatory for improving the visual prognosis.

The authors did not report any potential conflicts of interest in research, authorship, and / or publication of this article. 

Funding: The authors have not received any financial support for the research, authorship and / or publication of this article.