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Ординатура / Офтальмология / Английские материалы / Illustrated Tutorials in Ophthalmology Kanski, Bolton 2001

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Leber hereditary optic neuropathy

Maternal mitochondrial DNA mutations

Presents

Typically in males - third decade

Occasionally in females - any age

Initially unilateral visual loss

Fellow eye involved within 2 months

Bilateral optic atrophy

Signs

Disc hyperaemia and dilated capillaries (telangiectatic microangiopathy)

Vascular tortuosity

Swelling of peripapillary nerve fibre layer

Subsequent bilateral optic atrophy

PAPILLOEDEMA

1.Introduction

Circulation of cerebrospinal fluid

Causes of raised intracranial pressure

Hydrocephalus

2.Classification of papilloedema

Early

Established (acute)

Longstanding (chronic)

Atrophic (secondary optic atrophy)

Circulation of cerebrospinal fluid

a

(a) Subarachnoid space

b

(b) Lateral ventricle

c

d (c) Third ventricle

e

(d) Aqueduct

(e) Fourth ventricle

Causes of Raised Intracranial Pressure

1.Space-occupying lesions

2.Blockage of ventricular system

3.Obstruction of CSF absorption

4.Benign intracranial hypertension (pseudotumour cerebri)

5.Diffuse cerebral oedema

6. Hypersecretion of CSF

Hydrocephalus

Dilated cerebral ventricles

Communicating - obstruction to CSF flow in basilar cisterns or cerebral subarachnoid space

Non-communicating - obstruction to CSF flow in ventricular

system or at exit of foramina of fourth ventricle

Early papilloedema

VA - normal

Mild disc hyperaemia

Indistinct disc margins - initially nasal

Mild venous engorgement

Normal optic cup

Spontaneous venous pulsation - absent (also absent in 20% of normals)

Established papilloedema (acute)

VA - usually normal

Severe disc elevation and hyperaemia

Very indistinct disc margins

Obscuration of small vessels on disc

Marked venous engorgement

Reduced or absent optic cup

Haemorrhages + cotton-wool spots

Macular star

Longstanding papilloedema (chronic)

VA - variable

Marked disc elevation but less hyperaemia

Disc margins - indistinct

Variable venous engorgement

Absent optic cup

Atrophic papilloedema (secondary optic atrophy)

VA - severely decreased

Mild disc elevation

Indistinct disc margins

Disc pallor with few crossing vessels

Absent optic cup

CONGENITAL OPTIC NERVE ANOMALIES

1.Without systemic associations

Tilted optic disc

Optic disc drusen

Optic disc pit

Myelinated nerve fibres

2.With systemic associations

Optic disc coloboma

Morning glory anomaly

Optic nerve hypoplasia

Aicardi syndrome

Megalopapilla

Peripapillary staphyloma

Optic disc dysplasia

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