Macular Star.pptx

How to approach and manage the case of optic disc edema with macular star.
Different investigations to choose for OEDMS, and how to rule out masqueraders

1. What’s behind that STAR? - Dr. Mohd Azaz Quraishi

2. Wish Vs.

3. Facts 1 1916- Leber : “Stellate Retinopathy” considering it as retinal pathology. 1977- Don Gass : challenged this definition. He showed that the disk swelling developed either before or coincident with the macular star and noted no retinal vascular leakage on fluorescein angiography. Termed it “Neuroretinitis”

4. Concept of Terminologies 1 IDIOPATHIC INFECTIOUS/ INFLAMMATORY

5. General Pathology 2 The radial arrangement of the hard exudates arises from the anatomy of the OPL (Henle’s layer) of the retina.

6. OEDMS Idiopathic Age of onset: 6-50 yr, Mean: 20-40yr Gender: M=F B/L: 5-33% Pain: occasional Viral prodrome: Approx 50% Presenting VA: variable (6/6 to PL)

7. Dyschromatopsia: Often prominent, may be more severely affected. Field defect: Central/ Arcuate /Altitudinal RAPD: + nt , if B/L – nt Vitreous cells: common (90%) Macular star: + nt , but take up to 1-2 wks to develop Hence pt with disc edema with normal macula must be re-examined within 2 weeks

8. Disc Edema: earliest sign Diffuse; rarely segmental Tends to resolve during 2 weeks to 2 months period Optic atrophy in some cases can be seen

9. Neuroretinitis Focal inflammation of optic nerve and adjacent neural retina. Infectious/idiopathic Immunocompromised , HIV, various medications, health care workers etc are high risk populations.

10. Infectious causes:

11. Inflammatory causes: VKH syndrome Polyarteritis nodosa Behcet’s disease Sarcoidosis Idiopathic retinal vasculitis , aneurysms & neuroretinitis (IRVAN) Tubulointerstitial nephritis and uveitis (TINU) Inflammatory bowel disease, etc.

12. IMAGING

13. Irregular pattern of exudates in recurrence of Neuroretinitis Classic macular star pattern FUNDUS PHOTOGRAPH

14. FFA 3 - Fluorescein angiography may reveal disc edema and leakage and blockage of fluorescence in areas of hard exudates. - Occasionally , staining may be found in the seemingly uninvolved contra lateral eye.

15. OCT 3 - Showing thickening in different layers of retina.  - Small arrow shows location of OPL (Henle's layer), where exudates are deposited.   - The large arrow shows subretinal fluid causing local neurosensory retinal detachment.

16. MRI 4 Often not required for diagnosis A spectrum of neuroimaging findings : Normal optic nerve Intraocular optic disc enhancement at the nerve-globe junction (below) Optic nerve sheath enhancement (optic perineuritis) Optic nerve and optic sheath enhancement Fig.: Thickening or enhancement of the prelaminar optic disc, orbital optic nerve, its sheath, periorbita , or neighboring meninges .

17. MASQUERADERS 5

18. 1. Hypertensive retinopathy Differentiating features: History, B/L, AV changes, CWS, Flame shaped hemorrhages HTN retinopathy Grade 4 Neuroretinitis: Typical picture

19. 2. Papilloedema 6 Frisén grade zero: normal optic disc. Grade1: Minimal edema. "C" shaped greyish halo surrounding the disc (arrow) with spared temporal disc margin . G rade 2 : Marginal edema. Circumferential Halo. Elevation of nasal border . Grade 3: Moderate edema. Circumferential halo. All borders become elevated (cup not included). One or more segment(s) of blood vessels leaving the disc becomes obscured (arrow ). Grade 4: e dema becomes evident. Circumferential halo. All borders elevated (including the cup). One or more major vessels on the disc becomes obscured (arrow). G rade 5 : all of grade 4 features plus partial or total obscuration of all vessels on and leaving the disc

20. 3. Idiopathic Intracranial Hypertension (IIH) 6 Differentiating features: 90% affected young female Family history + ve Headache (~84% pts)- B/L, worse in morning, lying position, intensify with coughing/straining Transient visual obscuration Sixth cranial nerve palsy + nt

21. 4. NA-AION Typical sign of AION: Superior and inferior segments of the disc margin are obscured due to edema. Differentiating features: - History: age, vascular risk factors + nt Macular star very rare, if + nt oftne incomplete Absence of vitreous cells. Typical optic disc edema

22. 5. Diabetic papillopathy It is diagnosis of exclusion Minimal visual loss Optic disc edema No signs of raised ICT U/L or B/L RAPD/ Dyschromatopsa – nt Dilated vessels remains in disc substance, unlike NVD which proliferate into vitreous

23. Rare Ones… 7, 8 Toxic ( procarbazine , bis-chloroethylnitrosourea ) BRVO. Disc & Juxtapapillary tumors ( angioma , melanoma etc). AV malformations.

24. Approach to ODEMS 9

25. Red Flags 5 Clinical features suggesting possible neuroretinitis : ► Pre-existing known inflammatory or infective disease ► History of recent exposure to animals (especially cats), or overseas travel ► History of preceding influenza-like illness or systemic symptoms ► Lack of ocular pain in association with visual loss ► Lack of RAPD in association with a central scotoma

26. MANAGEMENT

27. Investigations 4 Lab Tests and Imaging: Bartonella henselae serology- Indirect fluorescence assay (IFA) ( IgG )- - < 1:64: the patient does not have current Bartonella infection - 1:64-1:256: possible Bartonella infection - 1:256: strongly suggests active or recent infection. Positive IgM indicates acute infection, but can be positive several years after CSF is preferable for serology in Neuroretinitis

28. Toxoplasma titres FTA-ABS or TPHA for syphilis Toxocara titre HIV test Chest X-ray/ Mantoux MRI- if nothing conclusive and neurological symptoms + nt

29. Treatment 8 Most of the cases are self limiting within few months If symptoms like fever, lymphadenopathy and visual dysfunction + nt , antimicrobial therapy must be started CSD: - Doxycycline /Ciprofloxacin/ Cotrimoxazole etc, with or without steroids have been tried. Azithromycin is safe to use during pregnancy and in children.

30. Toxoplasmosis: Pyrimethamine /Sulfadiazine/ Clindamycin with steroid can be used Recurrent idiopathic neuroretinitis : Immunosuppressive therapy with Azathioprine is used, for long term remission. Alternately, high dose IV/oral corticosteroids , with fast tapering, can be used

31. References Brazis P W, Lee A G, Optic disc edema with macular star, Mayo Clin Proc 1996; 71:1162-1166. Yap SM,  Saeed  M, Logan P, et al, Bartonella neuroretinitis (cat-scratch disease), Practical Neurology 2020;20:505-506 eyewiki.aao.org/ Neuroretinitis#General_Pathology pressbooks.pub/ casebasedneuroophthalmology /chapter/ neuroretinitis / Lueck , C. J. (2020). Neuroretinitis: a tricky mimic. Practical Neurology, practneurol–2020–002629. Reier L, Fowler J B, Arshad M, et al. (May 11, 2022) Optic Disc Edema and Elevated Intracranial Pressure (ICP): A Comprehensive Review of Papilledema . Cureus 14(5): e24915 Mc. Lennan , R., & Taylor, H. R. (1978). Optic neuroretinitis in association with BCNU and procarbazine therapy. Medical and Pediatric Oncology, 4(1), 43–48 Purvin V, Sundaram S, Kawasaki A. Neuroretinitis: review of the literature and new observations. J Neuro-Ophthalmol 2011;31:58–68 Abdelhakim , A., & Rasool , N. (2018).  Neuroretinitis. Current Opinion in Ophthalmology, 1.

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