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Paeds Vivasophthalmology

Paeds Vivas · ophthalmology

Ophthalmic manifestations of systemic disease: Viva

Branching clinical structured oral on the ophthalmic manifestations of systemic disease in children, covering the silent uveitis of juvenile idiopathic arthritis and the ACR screening schedule, the diabetic and the sickle retinopathy, the neurocutaneous phakomatoses from the optic pathway glioma to the Sturge-Weber glaucoma, the metabolic signs of the Marfan ectopia lentis and the Wilson Kayser-Fleischer ring, and the vitamin A deficiency.

branching clinical structured oral5 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A four-year-old girl presents with a swollen right knee for six weeks and a positive antinuclear antibody, with a normal eye examination and no eye symptom. The examiner asks how you frame the ophthalmic risk, what the screening schedule is and why, how you manage the chronic uveitis stepwise, and how your approach changes for the child with the type one diabetes, the neurofibromatosis optic pathway glioma and the Marfan ectopia lentis.

This is a branching oral built to probe the reasoning that holds the silent uveitis and the screening schedule at the centre, and to expose the candidate who has memorised the headline without the safety-critical corners. The questions escalate from the framing of the ophthalmic risk to the screening schedule, the stepwise treatment, and the contrast with the diabetes, the neurofibromatosis and the metabolic eye signs, with deliberate probes into the red flags and the global child health. [1]

Opening question: framing the ophthalmic risk

The examiner opens with the swollen knee and the positive antinuclear antibody and asks: what is the ophthalmic risk in this child, and what is your first step? [1]

A strong answer names the chronic anterior uveitis of the juvenile idiopathic arthritis as the sight-threatening silent inflammation, and states that the first step is the slit-lamp screening by the ophthalmology service at the high-risk interval. [1]

Model answer. This child carries the highest risk of the chronic anterior uveitis of any child in the clinic, because the oligoarticular antinuclear-antibody-positive disease in the young child is the profile that carries the uveitis in the greatest proportion, yet the uveitis is silent and produces no eye symptom. The first step is the slit-lamp screening by the ophthalmology service at the every-three-months interval, because the slit-lamp is the only detection of the cells and the flare before they scar the anterior chamber. [1]

Probe one: the screening schedule

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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. [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
  5. [8]Bollani L, Auriti C, Achille C, et al Congenital Toxoplasmosis: The State of the Art. Front Pediatr, 2022.PMID 35874584
  6. [9]Milewicz DM, Braverman AC, De Backer J, et al Marfan syndrome. Nat Rev Dis Primers, 2021.PMID 34475413
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