What is the appropriate management for a child with suspected febrile vasculitis affecting the eyes, presenting with fever and eye symptoms?

Fluorescein Angiography (FVA) in Ophthalmology

Fluorescein angiography (FVA) is not a standard term in ophthalmology; you likely mean fundus fluorescein angiography (FFA or FA), which is a diagnostic imaging technique used to evaluate retinal and choroidal vasculature by injecting fluorescein dye intravenously and photographing its passage through ocular blood vessels.

Primary Clinical Applications

Retinal Vascular Disease Assessment

• FFA is essential for diagnosing and monitoring retinal vasculitis, particularly in pediatric populations where it reveals vascular leakage, occlusion, and inflammatory changes that are not visible on clinical examination alone 1.
• In pediatric idiopathic uveitis, FFA demonstrates retinal vasculitis in approximately 80% of cases, making it a critical diagnostic tool for identifying posterior segment involvement 1.
• The presence of retinal vasculitis on FFA predicts worse treatment outcomes, with only 76.3% achieving inflammation control after one year compared to 85.1% without vasculitis 1.

Retinal and Ophthalmic Artery Occlusions

• FFA helps differentiate between various types of arterial occlusions (central retinal artery occlusion, branch retinal artery occlusion, and ophthalmic artery occlusion) by demonstrating delayed or absent arterial filling patterns 2.
• The technique identifies areas of retinal non-perfusion and helps guide management decisions, though it does not change the urgent need for systemic stroke workup in acute presentations 2.

Specific Disease Contexts

Kawasaki Disease Ocular Complications

• While Kawasaki disease primarily causes anterior segment findings (bilateral nonexudative conjunctivitis, anterior uveitis), posterior segment involvement including retinitis, retinal detachment, and choroidal detachment can occur rarely 2, 3.
• FFA may be indicated when posterior segment complications are suspected, though most ocular manifestations in Kawasaki disease resolve within 2-8 weeks without requiring advanced imaging 4.
• Ophthalmologic consultation should be obtained early in suspected Kawasaki disease cases, particularly incomplete presentations, as 45% of children lack complete diagnostic criteria at admission and eye findings can facilitate earlier diagnosis 5.

Pediatric Uveitis Evaluation

• FFA is the gold standard for detecting retinal vasculitis in children with uveitis, revealing inflammation that may not be apparent on clinical examination 1.
• The test should be performed in pediatric patients with idiopathic uveitis to assess for posterior segment involvement, as this finding significantly impacts prognosis and treatment intensity 1.

Technical Considerations and Limitations

When FFA is NOT Indicated

• FFA is not routinely necessary for typical anterior segment conditions like simple conjunctivitis, even in the context of systemic diseases like Kawasaki disease 2.
• In acute retinal artery occlusions, FFA does not change immediate management, which focuses on urgent stroke evaluation and treatment of underlying causes like giant cell arteritis 2.

Important Clinical Pitfalls

• Do not delay treatment for Kawasaki disease to obtain FFA; diagnosis is clinical, and IVIG therapy should be administered within 10 days of fever onset to prevent coronary artery aneurysms 2.
• In children with fever and eye symptoms, consider incomplete Kawasaki disease even without complete diagnostic criteria, as delayed diagnosis increases risk of coronary complications from 5% to 24% 6, 5.
• Optic disc swelling in Kawasaki disease can persist for up to 6 months despite systemic treatment, requiring prolonged ophthalmologic monitoring 7.

Diagnostic Algorithm for Pediatric Fever with Eye Involvement

Immediate Assessment Required

• Check for Kawasaki disease criteria: fever ≥5 days plus bilateral nonexudative conjunctivitis, oral changes (strawberry tongue), polymorphous rash, extremity changes, and cervical lymphadenopathy 6.
• If ≥2 principal features present with fever ≥5 days, immediately measure ESR and CRP 6.
• If ESR ≥40 mm/hr or CRP ≥3 mg/dL, obtain echocardiography, complete blood count, comprehensive metabolic panel, and urinalysis—do not wait for FFA 6.

Role of Ophthalmology Consultation

• Obtain ophthalmology consultation for visual acuity assessment, slit-lamp examination for anterior uveitis, and fundoscopic examination for posterior segment involvement 8, 5.
• FFA should be reserved for cases with suspected posterior segment complications (retinitis, vasculitis, optic disc swelling) that persist beyond typical resolution timeframes 7, 3.

Red Flags Requiring Immediate Ophthalmology Referral

• Vision loss or decreased visual acuity, moderate-to-severe pain, corneal involvement, severe purulent discharge, or lack of response to initial therapy all mandate urgent ophthalmology evaluation 8.