What’s the diagnosis?

1. What’s the diagnosis? Arijit Mitra Consutant Ophthalmogist - BMEC

2. Case 1 • 27 year female, British Iranian • Dentist, medical student • Bilateral fixed dilated – 24 hours • Difficulty in focussing – BE • VA – BE – 6/9 • PMH - NAD

4. Differential diagnosis • Pharmacological • Posterior synechia • Traumatic mydriasis • Bilateral Addie’s pupil • Bilateral afferent defects – End stage glaucoma, Optic neuropathies • Bilateral efferent/structural defects - midbrain strokes/trauma. Beware of bilateral third nerve palsy

5. Examination • Pupils change shape every hour !!! • Minimally reactive • No difference in light or dark • ? Vermiform movement of iris

6. Examination • Anterior segments - WNL • IOP – WNL • Fundus

7. OCT – 21/1/11

8. Working diagnosis • CSCR • But why is the pupil dilated? • Why is the pupil changing shape every few hours? • Referred to neuro-ophthalmology

9. Tadpole pupil - 1 • Segmental spasms of the iris dilator muscle produce intermittent pupillary distortion • 26 cases • 21 – F, 5 – M • Age - 24 to 48 years Thompson HS, Zackon DH, Czarnecki MD. Tadpole-shaped pupils caused by segmental spasm of the iris dilator muscle. Am J Ophthalmol 1983; 96: 467–477.

10. Tadpole pupil - 2 • Episodes are brief, lasting less than 5 min – 23 patients, less than 2 min – 18 patients, and less than 1 min – 8 patients • Can occur several times a day • 11 patients had Horner's syndrome, 4 had Adie's tonic pupil, and 11 had definite or probable migraine • The episodes eventually stopped, leaving no serious sequelae • Aetiology is unclear, although it is postulated that the cause is of benign neural origin

11. Over the next few days... • VA – 6/60 – BE • Refd to Moorfields Eye Hospital – confirmed Tadpole pupil • Fundus – more extensive central serous / exudative RD • Admitted

12. Case 2 • 30 year male, British Pakistani • Loss of vision – LE x 1/52 • VE – RE – 6/6, LE – 6/24 • PMH – NAD • Anterior segment and IOP - WNL

13. Case 2

14. Case 3 • 32 year female, British Pakistani • Loss of vision – BE x 1/52 • VE – RE – 6/12, LE – 6/12 • PMH – NAD • Anterior segment and IOP - WNL

15. Case 3 • Pharmacist • Grandma on Prednisolone • Self medicated with oral Prednisolone 40 mg x 2/7 • Felt her vision was improving

16. Case 3

17. Case 4 – Day 1 • 29 year male, Bangladeshi • Bilateral loss of vision – 3/52 • VA – RE – 6/24, LE – 6/18 • PMH - NAD

18. Examination • AC – 1+ cells • IOPS – WNL • Vitreous – 1+ cells

19. Case 4

20. Case 4 • Bloods – FBC, ESR, CRP, U&E, LFT, Glucose, HbA1c, Autoantibodies (ACE, ANCA, Anti ds-DNA), RF, Syphilis and Toxoplasma antibodies • Chest Xray • Mantoux test • OCT, FFA +/- ICG

21. Case 4 – Day 3 • Deaf • VA – BE – 6/60 • Anterior segments and IOP – unchanged • Fundus examination

22. Case 4

23. Investigations - Case - 1 • Full uveitis screen • MRI • Lumbar puncture • CSF – 100 % lymphocytic pleocytosis (with melanin laden cells)

24. Vogt-Koyanagi-Harada Syndrome

25. Treatment – Case 1 • IV Methylprednisolone – 1 gm /day x 3/7 • Oral Prednisolone • VA started improving • 5 days later – VA – 6/9 - BE

26. OCT – 21/1/11

27. OCT – 26/1/11

28. Case 4 – Pre-treatment

29. Case 4 – Post-treatment

30. Leopard - spots

31. MB - 1

32. Case 5 • 53 yo A-C medical secretary • Worsening vision and eye pain over 1-2/7 • Associated lethargy/tiredness • Preceding headache, neck pain and photophobia • RVA 6/12 (6/9) LVA 6/9(6/6)

39. MB – 2

40. Case 6 • 26yo Bangladeshi, 12/40 preg • 2/7 blurring vision • RVA 6/18 LVA 3/60(6/12) • No pain, headache, photophobia • No AC or Vit inflammation • Brother had TB 10 yrs ago

44. Treatment • TB ‘excluded’ • IV Methylprednisolone 500mg alt days x3 • Oral steroids delayed because of potential risk to foetus • 3 weeks post presentation – VA PL OU • Total RDs

46. Cyclosporin added (relative saftey in Pregnancy) and slowly increased

47. Outcome • Healthy baby • Continued to need immunosuppression • RVA 1/60 LVA 2/60

48. VKH