This document describes the stages and clinical features of papilloedema, which is optic disc swelling caused by increased intracranial pressure. It outlines 4 stages: early, fully developed, chronic, and atrophic. Early stage shows obscured disc margins and mild hyperemia. The fully developed stage shows elevated optic discs up to 1-2mm with obliterated cups and hemorrhages. Chronic stage discs appear dome shaped with resolved hemorrhages. The atrophic stage results in optic nerve atrophy and visual field loss after 6-9 months if pressure is not reduced. Treatment requires reducing intracranial pressure through imaging, hospitalization, and possibly decompression surgery.

1. Christa Maria Joel
PAPILLOEDEMA

2. Clinical features
• General features of raised intracranial pressure
- Headache
- Nausea
- Projectile vomiting
- Diplopia
- Focal neurological deficit may be associated.

3. • Ocular features
- History of recurrent attacks of transient blackout of vision (amaurosis
fugax).
- Visual acuity and pupillary reactions remain fairly normal until the late
stages of diseases when optic atrophy sets in, so loss of vision is not a
common feature.
- Clinical features can be described under 4 stages: early, fully
developed, chronic and atrophic.

4. Early or incipient papilloedema
• Ophthalmoscopic features-
- Obscuration of disc margins: nasal margins are
involved first followed by superior, inferior and
temporal.
- Blurring of peripapillary nerve fibre layer.
- Absence of spontaneous venous pulsation at the
disc.
- Mild hyperemia of the disc.
- Splinter hemorrhages in the peripapillary region
may be present.
Symptoms- absent, visual acuity
normal.
Pupillary reactions- normal
Visual fields- fairly normal

5. Established or fully developed papilloedema
• Symptoms: History of transient visual obscurations in one or both eyes lasting
a few seconds after standing. Visual acuity: normal.
• Pupillary reaction- fairly normal.
• Ophthalmoscopic features-
- Apparent optic disc edema is seen as its forward elevation above the plane of
retina, usually upto 1-2 mm.
- Physiological cup of the optic disc is obliterated.
- Disc becomes markedly hyperaemic and blurring of the margin is present all
around.
- Multiple cotton wool spots and superficial hemorrhages may be seen near the
disc.

6. • Veins become tortuous and engorged.
• Paton’s lines- circumferential greyish white folds may develop due to
separation of nerve fibres by the edema.
• Rarely, hard exudates may radiate from the fovea in the form of an
incomplete scar. (macular star or macular fan)
• Visual fields show enlargement of blind spot.

7. Chronic or long standing or vintage papilloedema
• Symptoms: visual acuity variably reduced.
• Pupillary reactions- usually normal.
• Ophthalmoscopic features:
- Acute hemorrhages and cotton wool spots resolve and
peripapillary oedema is resorbed.
- optic disc gives appearance of the dome of a
champagne cork. The central cup remains obliterated.
Small drusen like crystalline deposits (corpora
amylacea) may appear on the disc surface.
- Visual fields: blind spot enlarged, and visual fields begin
to constrict.

8. Atrophic papilloedema
• Symptoms: develops after 6-9 months of chronic papilloedema, severly
impaired visual acuity.
• Pupillary reaction: light reflex impaired.
• Ophthalmoscopic features:
- Greyish white discolouration, pallor of the disc due to atrophy of the
neurons and associated gliosis.
- Prominence of the disc decreases in spite of persistent raised
intracranial pressure.
- Retinal arterioles narrowed, veins become less congested, whitish
sheathing develops around the vessels.

9. • Visual fields: concentric contraction of peripheral fields becomes
apparent as atrophy sets in.

10. Treatment and prognosis
• Neurological emergency, requires immediate hospitalisation.
• Urgent neuroimaging like CT scan or MRI with gadolinium
enhancement may reveal primary pathology.
• Unless causative disease is treatable or cerebral decompression is
done, course of papilloedema is chronic and visual prognosis is bad.

11. Differential diagnosis
• Pseudopapilloedema- non specific term used to describe elevation of
the disc similar to papilloedema in conditions such as optic disc drusen,
hypermetropia and persistent hyaloid tissue.
• Papillitis

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