FVA in Ophthalmology in Children: Clarification and Diagnostic Approach
Critical Clarification: "FVA" is Not a Recognized Medical Term
"FVA" does not exist as a recognized medical abbreviation or diagnosis in pediatric ophthalmology or pediatric medicine according to major medical societies. 1 Neither the American Heart Association, American Academy of Pediatrics, nor European guidelines recognize this term. 1
Most Likely Clinical Scenarios You May Be Encountering
Based on the context of "febrile vasculitis affecting the eyes" in children, you are likely dealing with one of these conditions:
1. Kawasaki Disease (Most Common Acute Febrile Vasculitis in Children)
If the child is under 5 years old with fever ≥5 days plus characteristic features, Kawasaki disease is the most likely diagnosis and requires urgent intervention. 1
Diagnostic Criteria:
- Fever ≥5 days plus at least 4 of the following: 1
- Bilateral non-exudative conjunctivitis (most common ocular finding)
- Oral changes (strawberry tongue, cracked lips)
- Polymorphous rash
- Extremity changes (edema, erythema, desquamation)
- Cervical lymphadenopathy
Urgent Management:
- Immediate echocardiography to assess for coronary artery involvement 1
- Treatment with IVIG 2 g/kg plus aspirin to prevent coronary artery aneurysms 1
- Risk of coronary complications increases significantly if treatment is delayed beyond 10 days of fever onset 1
Initial Workup:
- Complete blood count with differential, ESR, CRP, comprehensive metabolic panel 1
- Urinalysis and urine culture (catheterized specimen) to exclude UTI 1
- Blood cultures before antibiotics 1
2. Pediatric Idiopathic Uveitis with Retinal Vasculitis
If the child has intraocular inflammation with retinal vascular involvement, pediatric idiopathic uveitis with retinal vasculitis is a common entity requiring specialized ophthalmologic care. 2
Key Clinical Features:
- Almost 80% of children with pediatric idiopathic uveitis show manifestations of retinal vasculitis 2
- Typically shows anterior segment involvement but posterior segment disease is common 2
- Associated with lower probability of inflammation control (76.3% vs 85.1% without vasculitis) 2
- Worse visual prognosis after 5 years compared to those without retinal vasculitis 2
Diagnostic Approach:
- Fundus fluorescein angiography is essential to assess presence and extent of retinal vasculitis 2
- Complete ophthalmic examination (may require general anesthesia in young children) 3
- Exclude infections and masquerade syndromes (leukemia, retinoblastoma) before starting immunosuppressive therapy 3
Treatment Algorithm:
- Noninfectious anterior uveitis: topical corticosteroid and mydriatic therapy 3
- Vision-threatening posterior inflammation: corticosteroid-sparing immunosuppressive drugs 3
- Methotrexate is the most commonly used systemic immunosuppressive agent for pediatric uveitis - effective, well-tolerated, easy to administer, inexpensive 3
- Cyclosporine as alternative - low risk of renal toxicity at standard doses 3
- Avoid alkylating agents in children due to risks of secondary malignancy, sterility, and bone marrow suppression 3
- Systemic corticosteroids may cause growth retardation in children, hence preference for steroid-sparing agents 3
3. Primary Retinal Vasculitis
Primary retinal vasculitis (inflammation of retinal vasculature without infectious etiology or systemic disease) is rare but can cause severe permanent vision loss. 4
Diagnostic Workup Considerations:
- Extensive diagnostic evaluation is typically unrewarding in the absence of suggestive medical history 4
- Only 1 in 25 patients (4%) with suggestive review of systems had underlying systemic disease (systemic lupus erythematosus) 4
- False-positive diagnostic test results occur in 20.8% of patients without suggestive history 4
- Few diagnostic tests should be ordered in patients with retinal vasculitis in the absence of medical history suggestive of underlying systemic disease 4
4. Varicella-Associated Retinal Vasculopathy
Varicella zoster can cause vaso-occlusive pathologies, vasculitis, or optic neuritis leading to profound visual loss, even with antiviral therapy. 5
Clinical Presentation:
- Sudden painless vision loss 5
- Can present as cilioretinal artery occlusion 5
- OCT angiography shows loss of capillary network around perifoveal region 5
Mandatory Referral Criteria to Pediatric Ophthalmology
Children with the following conditions MUST be referred to a pediatric ophthalmologist: 6
- Children ≤7 years who are nonverbal or unable to read letters with suspected eye disease 6
- Known or suspected cataracts, glaucoma, or blindness 6
- Congenital or genetic ocular anomalies or infections 6
- Systemic syndromes with possible ocular involvement (juvenile idiopathic arthritis, diabetes mellitus) 6
- Strabismus or amblyopia or risk factors for these conditions 6
- Ocular or periocular inflammation not responding to initial therapy within 3 weeks 6
Critical Pitfalls to Avoid
- Never use the term "FVA" in medical documentation - it is not recognized and will cause confusion 1
- Do not delay treatment for Kawasaki disease beyond 10 days of fever onset - coronary complications increase significantly 1
- Do not start immunosuppressive therapy before excluding infections and masquerade syndromes (leukemia, retinoblastoma) 3
- Avoid systemic corticosteroids as first-line in children with posterior uveitis - growth retardation risk; use steroid-sparing agents 3
- Do not order extensive diagnostic workup for retinal vasculitis without suggestive clinical history - high false-positive rate (20.8%) 4