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Paeds Casesophthalmology

Paeds Cases · ophthalmology

Ophthalmic manifestations of systemic disease: Case

Clinical long case of a four-year-old girl with the oligoarticular juvenile idiopathic arthritis and a positive antinuclear antibody, covering the silent sight-threatening uveitis and the ACR screening schedule, the stepwise management with the methotrexate and the adalimumab, and the contrast with the diabetic retinopathy screening, the neurofibromatosis optic pathway glioma and the metabolic eye signs.

paediatric ophthalmology long case4 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A four-year-old girl is reviewed in the paediatric rheumatology clinic with a six-week history of a swollen right knee. She is well in herself, with normal growth and development, and her eye examination on inspection is normal, with no redness and no complaint about her vision. The right knee is warm and swollen with a reduced range of movement, and the rest of the examination is unremarkable. The investigation returns the oligoarticular juvenile idiopathic arthritis with a positive antinuclear antibody. The examiner asks how you frame the ophthalmic risk, how you confirm and screen for the uveitis, how you manage it stepwise, and how your approach changes for the child with the type one diabetes and the neurofibromatosis optic pathway glioma.

Framing the case

This four-year-old girl has the oligoarticular juvenile idiopathic arthritis with a positive antinuclear antibody, and the framework that organises the case is the silent sight-threatening uveitis and the ACR screening schedule. The swollen knee is the visible problem, but the danger sits behind the iris in the anterior chamber, where the low-grade inflammation may scar the eye while the child plays. The first priority is the slit-lamp screening by the ophthalmology service at the every-three-months interval, because the uveitis produces no eye symptom and the slit-lamp is the only detection. [1]

Confirming and screening for the uveitis

The uveitis is screened with the slit-lamp examination by the ophthalmologist, and the slit-lamp detects the cells and the flare of the anterior chamber inflammation, the keratic precipitates on the corneal endothelium, the posterior synechiae between the iris and the lens, and the band keratopathy of the chronic disease. The intraocular pressure is measured, because the uveitis and the steroid both raise the pressure and the secondary glaucoma is the complication. This girl carries the four high-risk factors, the oligoarticular disease, the positive antinuclear antibody, the onset under seven years and the short duration, and the ACR guideline schedules the slit-lamp every three months. [1]

The stepwise management

The treatment of the chronic uveitis builds in the stepwise fashion. The topical corticosteroid, the prednisolone acetate one percent, is the first line for the acute inflammation, and the cycloplegic prevents the posterior synechiae. The systemic treatment is added when the topical steroid fails to control the inflammation or when the steroid-induced glaucoma and cataract threaten the sight, and the ACR guideline strongly recommends the methotrexate, at ten to fifteen milligrams per square metre once weekly, as the first-line steroid-sparing agent, with the folate supplementation and the full blood count and the liver function monitoring. The adalimumab, the anti-tumour-necrosis-factor monoclonal antibody, is the standard second-line biologic when the methotrexate fails. The co-management with the rheumatology and the ophthalmology continues through the childhood, because the uveitis may flare in the quiet joint. [1]

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References6ShowHide
  1. [1]Angeles-Han ST, Ringold S, Beukelman T, et al 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis. Arthritis Care Res, 2019.PMID 31021540
  2. [2]Herskin CW, Olsen BS, Madsen M, et al Screening for retinopathy in children with type 1 diabetes in Denmark. Pediatr Diabetes, 2020.PMID 31618523
  3. [4]Gutmann DH, Ferner RE, Listernick RH, et al Neurofibromatosis type 1. Nat Rev Dis Primers, 2017.PMID 28230061
  4. [6]Higueros E, Roe E, Granell E, et al Sturge-Weber Syndrome: A Review. Actas Dermo-Sifiliograficas, 2017.PMID 28126187
  5. [7]Yawn BP, Buchanan GR, Afenyi-Annan AN, et al Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA, 2014.PMID 25203083
  6. [9]Milewicz DM, Braverman AC, De Backer J, et al Marfan syndrome. Nat Rev Dis Primers, 2021.PMID 34475413
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