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By
Mutahir Shah
Resident M Phil VS
Pakistan Institute of Community
Ophthalmology
Optic Atrophy and Neuroretinitis
Optic Atrophy:-
 Introduction
 Optic atrophy refers to the late stage changes that
take place in the optic nerve resulting from axonal
degeneration in the pathway between the retina and
the lateral geniculate body, manifesting with
disturbance in visual function and in the appearance
of the optic nerve head.
 It can be classified in several ways,
 including by whether axonal death is initiated in the retina
(anterograde)
 or more centrally (retrograde), and by cause.
 Optic ‘atrophy’ is not true atrophy, a term that strictly
refers to involutional change secondary to lack of use.
Primary Optic Atrophy
 Primary optic atrophy
 Primary optic atrophy occurs without antecedent
swelling of the optic nerve head.
 It may be caused by lesions affecting the visual
pathways at any point from the retrolaminar portion of
the optic nerve to the lateral geniculate body.
 Lesions anterior to the optic chiasm result in
unilateral optic atrophy, whereas those involving the
chiasm and optic tract will cause bilateral changes.
• Signs
 Flat white disc with clearly delineated margins (Fig.
19.7A).
 Reduction in the number of small blood vessels on
the disc surface.
 Attenuation of peripapillary blood vessels and
thinning of the retinal nerve fibre layer (RNFL).
 The atrophy may be diffuse or sectoral depending on
the cause and level of the lesion.
 Temporal pallor of the optic nerve head may indicate
atrophy of fibres of the papillomacular bundle, and is
classically seen following demyelinating optic neuritis.
 Band atrophy is a similar phenomenon caused by
involvement of the fibres entering the optic disc
nasally and temporally; it occurs in lesions of the optic
chiasm or tract and gives nasal as well as temporal
Optic atrophy. (A) Primary due to compression; (B) primary due to nutritional
neuropathy – note predominantly temporal pallor;
• Important causes
 Optic neuritis.
 Compression by tumours and aneurysms.
 Hereditary optic neuropathies.
 Toxic and nutritional optic neuropathies; these
may give temporal pallor, particularly in
early/milder cases when the papillomacular fibres
are preferentially affected (Fig. 19.7B).
 ○ Trauma.
Secondary optic atrophy
 Secondary optic atrophy is preceded by long-standing
swelling of the optic nerve head.
 • Signs vary according to the cause and its
course.
 Slightly or moderately raised white or greyish disc
with poorly delineated margins due to gliosis (Fig. C).
 Obscuration of the lamina cribrosa.
 Reduction in the number of small blood vessels on
the disc surface.
 Peripapillary circumferential retinochoroidal folds,
especially temporal to the disc (Paton lines –C),
sheathing of arterioles and venous tortuosity may be
secondary due to chronic papilloedema – note prominent Paton lines
Causes
 Include
 chronic papilloedema,
 anterior ischaemic optic neuropathy
 papillitis.
 Intraocular inflammatory causes of marked disc
swelling are sometimes considered to cause
secondary rather than consecutive atrophy
Consecutive optic atrophy
 Consecutive optic atrophy is caused by disease of
the inner retina or its blood supply.
 The cause is usually obvious on fundus
examination,
 e.g. extensive retinal photocoagulation, retinitis
pigmentosa or prior central retinal artery occlusion.
 The disc appears waxy, with reasonably preserved
architecture
consecutive due to vasculitis
Neuroretinitis
 Neuroretinitis refers to the combination of optic
neuritis and signs of retinal, usually macular,
inflammation.
 Cat-scratch fever is responsible for 60% of cases.
 About 25% of cases are idiopathic (Leber
idiopathic stellate neuroretinitis).
 Other notable causes include syphilis, Lyme
disease, mumps and leptospirosis.
Diagnosis
 Symptoms.
 Painless unilateral visual impairment, usually gradually
worsening over about a week.
 Signs
 VA is impaired to a variable degree.
 Signs of optic nerve dysfunction are usually mild or
absent, as visual loss is largely due to macular
involvement.
 Papillitis associated with peripapillary and macular
oedema (Fig. 19.12A).
 A macular star (Fig. 19.12B) typically appears as disc
swelling settles; the macular star resolves with a return to
normal or near-normal visual acuity over 6–12 months.
 Venous engorgement and splinter haemorrhages may be
present in severe case.
 Fellow eye involvement occasionally develops.
Progression of neuroretinitis. (A) Severe papillitis; (B) later stage in a
different patient showing macular star
Investigation and Treatment
 Optical coherence tomography (OCT)
demonstrates sub- and intraretinal fluid to a
variable extent.
 Fluorescein angiography (FA) shows diffuse
leakage from superficial disc vessels.
 Blood tests may include serology for Bartonella
and other causes according to clinical suspicion
 Treatment
 This is specific to the cause, and often consists of
antibiotics. Recurrent idiopathic cases may
require treatment with steroids and/or other
immunosuppressants.
Optic atrophy and neuroretinitis

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Optic atrophy and neuroretinitis

  • 1. By Mutahir Shah Resident M Phil VS Pakistan Institute of Community Ophthalmology Optic Atrophy and Neuroretinitis
  • 2. Optic Atrophy:-  Introduction  Optic atrophy refers to the late stage changes that take place in the optic nerve resulting from axonal degeneration in the pathway between the retina and the lateral geniculate body, manifesting with disturbance in visual function and in the appearance of the optic nerve head.  It can be classified in several ways,  including by whether axonal death is initiated in the retina (anterograde)  or more centrally (retrograde), and by cause.  Optic ‘atrophy’ is not true atrophy, a term that strictly refers to involutional change secondary to lack of use.
  • 3. Primary Optic Atrophy  Primary optic atrophy  Primary optic atrophy occurs without antecedent swelling of the optic nerve head.  It may be caused by lesions affecting the visual pathways at any point from the retrolaminar portion of the optic nerve to the lateral geniculate body.  Lesions anterior to the optic chiasm result in unilateral optic atrophy, whereas those involving the chiasm and optic tract will cause bilateral changes.
  • 4. • Signs  Flat white disc with clearly delineated margins (Fig. 19.7A).  Reduction in the number of small blood vessels on the disc surface.  Attenuation of peripapillary blood vessels and thinning of the retinal nerve fibre layer (RNFL).  The atrophy may be diffuse or sectoral depending on the cause and level of the lesion.  Temporal pallor of the optic nerve head may indicate atrophy of fibres of the papillomacular bundle, and is classically seen following demyelinating optic neuritis.  Band atrophy is a similar phenomenon caused by involvement of the fibres entering the optic disc nasally and temporally; it occurs in lesions of the optic chiasm or tract and gives nasal as well as temporal
  • 5. Optic atrophy. (A) Primary due to compression; (B) primary due to nutritional neuropathy – note predominantly temporal pallor;
  • 6. • Important causes  Optic neuritis.  Compression by tumours and aneurysms.  Hereditary optic neuropathies.  Toxic and nutritional optic neuropathies; these may give temporal pallor, particularly in early/milder cases when the papillomacular fibres are preferentially affected (Fig. 19.7B).  ○ Trauma.
  • 7. Secondary optic atrophy  Secondary optic atrophy is preceded by long-standing swelling of the optic nerve head.  • Signs vary according to the cause and its course.  Slightly or moderately raised white or greyish disc with poorly delineated margins due to gliosis (Fig. C).  Obscuration of the lamina cribrosa.  Reduction in the number of small blood vessels on the disc surface.  Peripapillary circumferential retinochoroidal folds, especially temporal to the disc (Paton lines –C), sheathing of arterioles and venous tortuosity may be
  • 8. secondary due to chronic papilloedema – note prominent Paton lines
  • 9. Causes  Include  chronic papilloedema,  anterior ischaemic optic neuropathy  papillitis.  Intraocular inflammatory causes of marked disc swelling are sometimes considered to cause secondary rather than consecutive atrophy
  • 10. Consecutive optic atrophy  Consecutive optic atrophy is caused by disease of the inner retina or its blood supply.  The cause is usually obvious on fundus examination,  e.g. extensive retinal photocoagulation, retinitis pigmentosa or prior central retinal artery occlusion.  The disc appears waxy, with reasonably preserved architecture consecutive due to vasculitis
  • 11. Neuroretinitis  Neuroretinitis refers to the combination of optic neuritis and signs of retinal, usually macular, inflammation.  Cat-scratch fever is responsible for 60% of cases.  About 25% of cases are idiopathic (Leber idiopathic stellate neuroretinitis).  Other notable causes include syphilis, Lyme disease, mumps and leptospirosis.
  • 12. Diagnosis  Symptoms.  Painless unilateral visual impairment, usually gradually worsening over about a week.  Signs  VA is impaired to a variable degree.  Signs of optic nerve dysfunction are usually mild or absent, as visual loss is largely due to macular involvement.  Papillitis associated with peripapillary and macular oedema (Fig. 19.12A).  A macular star (Fig. 19.12B) typically appears as disc swelling settles; the macular star resolves with a return to normal or near-normal visual acuity over 6–12 months.  Venous engorgement and splinter haemorrhages may be present in severe case.  Fellow eye involvement occasionally develops.
  • 13. Progression of neuroretinitis. (A) Severe papillitis; (B) later stage in a different patient showing macular star
  • 14. Investigation and Treatment  Optical coherence tomography (OCT) demonstrates sub- and intraretinal fluid to a variable extent.  Fluorescein angiography (FA) shows diffuse leakage from superficial disc vessels.  Blood tests may include serology for Bartonella and other causes according to clinical suspicion  Treatment  This is specific to the cause, and often consists of antibiotics. Recurrent idiopathic cases may require treatment with steroids and/or other immunosuppressants.