Case Presentation JBN

1. Case PresentationJBN Juan G. Santiago, MD Department of Ophthalmology University of Puerto Rico

2. Chief Complaint • “No veo la cara de las personas por el ojo derecho”

3. Present History • JBN is a 16 y/o male w/o hx of SI, NKDA that refers right eye decreased vision since 3 months ago. While playing baseball patient noted that when he closes his left eye, he can’t see people’s faces with his right eye. No worsening or improvement of vision since he first noted it. No associated history of recent illness, contact with animals, trauma, travel or use of medications. No eye redness, burning, itching, secretions, flashlights, photophobia, diplopia.

4. History • Eye history: None • Surgeries: None • Systemic history: None • Family history: None

5. SLE

6. DFE Right Eye Left Eye

7. Differential Diagnosis • Papilledema • Non-idiopathic etiologies • *Idiopathic Intracranial Hypertension • Neuroretinitis • Viral neuroretinits • Cat scratch neuroretinitis • Noninfectious Neuroretinitis • Leber’s Idiopathic Stellate Neuroretinitis • Optic Disc Drusen

8. Papilledema • Swelling of the optic nerve disc 2ry to raised ICP. • Abnormal neuroimaging and CSF composition. • Usually bilateral, may be asymmetrical. • Intracranial mass until proven otherwise.

9. Papilledema • Findings: • Disc edema, flame shaped hemorrhages • Transient visual obscurations • Diplopia (CN 6 palsies) • Normal color vision, acuity and pupils • Enlarged blind spot on VF • Headache, nausea, vomits • Deterioration of consciousness

10. Papilledema • Causes • Obstruction of the ventricles • Congenital vs Acquired • Space-occupying intracranial lesions • Mass / Hemorrhage • Impairment of CSF absorption • Meningitis / SAH / Trauma • Diffuse cerebral edema • Trauma • Severe systemic hypertension • Hypersecretion of CSF • Choroid plexus tumor

11. Papilledema • Diagnosis • Check blood pressure • Brain CT/MRI • Lumbar puncture (if no mass) • Measure opening pressure

12. Idiopathic Intracranial Hypertension • Papilledema with normal neuroimaging and CSF • 90% female; associated with obesity.

13. Idiopathic Intracranial Hypertension • Findings: • Headache / Nausea / Vomiting • Optic disc edema (asymmetric) • Transient visual obscurations • Photopsias • VF defects • May have visual loss, +RAPD, diplopia

14. Idiopathic Intracranial Hypertension • Associations • Endocrine disorders • Dural sinus thrombosis • Use of steroids • Birth control pills • Vitamin A

15. Diagnosis Normal brain MRI/CT Elevated CSF pressure with normal composition Treatment No vision loss: Headache treatment Weight loss Mild vision loss: Acetazolamide Weight loss Advanced VF loss: ON fenestration Serial lumbar punctures Lumbar-peritoneal shunt Idiopathic Intracranial Hypertension

16. Neuroretinitis • Optic disc edema and macular star formation • Usually unilateral • 50% have viral prodrome

17. Neuroretinitis • Symptoms • Decreased vision • Colors appear washed out • Retrobulbar pain • Pain associated with eye movement • Headache

18. Neuroretinitis • Findings • Optic disc swelling • Peripapillary NFL hemorrhages • Serous RD • Iritis, vitritis, and/or scleritis (rare) • Vascular occlusions may develop

19. Noninfectious Cerebral AV malformations Elevated ICP Malignant HTN ION PAN Leber’s Idiopathic Stellate Neuroretinits Infectious Viral (influenza A, mumps, cocksackie, EBV) Cat scratch disease (Bartonella) Syphilis Lyme disease TB Parasites (Toxocara, Toxoplasma, DUSN) Neuroretinitis

20. Neuroretinitis Leber’s Idiopathic Stellate Neuroretinitis • 3rd decade • Male = Female • >70% unilateral • Findings • Decreased vision, RAPD (75%), cecocentral scotoma, vitritis, optic disc edema, macular star, exudative peripapillary RD

21. Neuroretinitis • Diagnosis • ESR, VDRL, FTA-ABS, Lyme titer, Bartonella serology, Toxoplasma and Toxocara titers, PPD • FA: diffuse leakage from disc, peripapillary capillary staining; 10% have disc leakage on fellow eye; no leakage in macula

22. Neuroretinitis • Treatment • Treat underlying disease • Observe idiopathic form • Most >20/50 after 3 months • 3-5% permanent severe visual loss

23. Optic Disc Drusen • Composed of hyaline-like calcific material within the substance of the optic nerve head • 0.3% of the population • 75% bilateral • No sex predilection

24. Optic Disc Drusen • Findings • Disc margins may show irregular outline • Bumpy nodular chunky appearance (pseudopapilledema) • VF defect • Transient visual obscurations • + RAPD (unilateral OD drusen) • Calcify with age

25. Optic Disc Drusen

26. Optic Disc Drusen • Diagnosis • B-scan ultrasound: Detect calcific deposits • CT scan • Children with papilledema and “negative” CT • Calcifications of the optic discs • FA • Exposed drusen: Autofluorescence and late hyperfluorescence due to staining • Buried drusen: Not prominent

27. Optic Disc Drusen

28. Optic Disc Drusen

29. Optic Disc Drusen

30. Optic Disc Drusen • Complications • Peripheral visual field loss • Idiopathic central visual loss • Prepapillary or peripapillary hemorrhages • Ischemic Optic Neuropathy • Retinal Vascular Occlusions • Transient Visual Loss • Peripapillary Subretinal Neovascularization • Peripapillary Central Serous Choroidopathy

31. Optic Disc Drusen • Peripapillary Subretinal Neovascularization • Temporary vs Permanent visual loss • Simulate • Neuroretinitis • Juxtapapillary mass lesion • Hemorrhages • From CNV  visual symptoms • Absence CNV no symptoms or resolved w/o sequelae

32. Optic Disc Drusen

33. Labs CBC – WNL CMP – WNL PT/PTT – WNL U/A – WNL Head and Orbit CT Official report: “Negative study” Optic disc calcifications Lumbar puncture No elevated opening pressure Normal CSF Composition CSF Micro - Negative Case: Ancillary Studies

34. FA

35. Diagnosis • Optic Disc Drusen Associated to Peripapillary Subretinal Neovascularization !!!

36. Literature Review • Bilateral CNV associated with optic nerve drusen treated with photodynamic therapy with verteporfin. Silva R, et al. Eur J Ophthalmol. 2004 Sep-Oct;14(5):434-7 • 10 y/o girl with juxtapapillary CNV OD and juxtapapillary and juxtafoveal CNV OS associated with OND underwent PDT with verteporfin in both eyes • VA increased from 20/160 to 20/25 OD and from 20/1000 to 20/25 OS after 2 sessions of PDT and 2 years of f/up.

37. Literature Review • Laser photocoagulation for CNVM associated with OD drusen. Delyfer MN, et al. Acta Ophthalmol Scand. 2004 Apr;82(2):236-8. • CNV complications of OD drusen were diagnosed in 36 y/o woman and 14 y/o girl. In both cases VA was 20/100 in the affected eye. • VA improved progressively after photocoagulation, reaching 20/20 10 months after treatment in one case and 20/30 in the other.

38. Literature Review • Submacular surgery for choroidal neovascularization secondary to optic nerve drusen. Sullu Y, et al. Am J Ophthalmol. 2003 Aug;136(2):367-70. • 9 y/o girl • Subfoveal CNV is an uncommon complication of OND and surgical excision may be useful in selected cases.

39. Literature Review • Surgical removal of peripapillary choroidal neovascularization associated with optic nerve drusen. Mateo C, et al. Retina. 2004 Oct;24(5):739-45. • Retrospective study was carried out on four eyes (four patients) undergoing vitrectomy, retinotomy, and removal of CNV associated with optic disk drusen. • VA improved in all cases after a follow-up ranging from 12 to 42 months