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. - Dr. Mohd Azaz Quraishi

2. Vs.

3.  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. Macular
Star with
disc
edema
OEDMS= optic disc
edema with macular star
Neuroretinitis
IDIOPATHIC
INFECTIOUS/
INFLAMMATORY

5.  The radial arrangement of the hard exudates
arises from the anatomy of the OPL (Henle’s
layer) of the retina.
Inflammation of Optic Disc vasculature
Exudation of fluid from the
peripapillary retina through the ELM
Penetrates OPL, accumulates behind
the macula
Subsequent resorption leaves behind
hard exudates in a star-shaped pattern

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

10. Bacterial
•2/3rd of cases: Cat scratch d/s (Bartonela henselae, B.
grahamii etc )
•M. tb/ Salmonella species
•Rocky mountain spotted figure
•Rickettsia typhi
•Treponema, Leptospira
Viral
•Mumps, Measles, rubella
•Influenza virus
•Varicella zoster
•HSV; EBV; CMV

11. Fungal
•Histoplasmosis
•Coccidiodomycosis
Parasites/
Protozoa
•Toxoplasma gondii
•Toxocara canis

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

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

15. 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.

16. 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.

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

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

20. (A)Frisén grade zero: normal optic disc.
(B) Grade1: Minimal edema. "C" shaped
greyish halo surrounding the disc (arrow)
with spared temporal disc margin.
(C) Grade 2: Marginal edema. Circumferential
Halo. Elevation of nasal border.
(D) 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).
(E) Grade 4: edema becomes evident.
Circumferential halo. All borders elevated
(including the cup). One or more major
vessels on the disc becomes obscured
(arrow).
(F) Grade 5: all of grade 4 features plus partial
or total obscuration of all vessels on and
leaving the disc

21.  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

22. 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

23. -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

24.  Toxic ( procarbazine,
bis-chloroethylnitrosourea)
 BRVO.
 Disc & Juxtapapillary tumors (angioma,
melanoma etc).
 AV malformations.

26. 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

28.  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

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

30.  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.

31.  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

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