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3F: Neuro Final > Optic Neuropathies II > Flashcards

Optic Neuropathies II Flashcards

1
Q

Disorders of fetal vasculature

A

Benign disorders

  • peripapillary loops
  • bermeisters papilla
  • congential macrovessels
  • congential tortuosity

Detrimental disorders

  • PHPV
  • AV malformation (Wyburn mason)
  • cap hemangioma (VHL)
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2
Q

Peripapillary loops (PPL)

A
  • benign
  • rare
  • most PPL arise from the arterial supply

Complications

  • may predispose CRAO and vitreous hemorrhage
  • thick blood disorders and emboli may cause insult due to the twisting and kinking of the loops
  • in some cases, the vitreous is attached to the tissue surrounding the loop and it becomes a source of vitreous traction
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3
Q

Bergmeisters papilla

A

Benign

  • arises as a result of incompletely regression of the primary vitreous
  • consists of primitive epithelioid cells, glia, fibrous remnant tissue which supported the hyaloid artery system
  • solid white mass or veil of varying size and density extending in to the vitreous cavity from the nerve head
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4
Q

Congential retinal macrovessels

A
  • benign
  • congential macrovessels are normally formed vessels that develop preferentially around the macula
  • visual acuity is normal and the vessels remain competent throughout life
  • not known why
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5
Q

Congenital tortuosity of retinal vessles

A
  • frequently seen in small hyperopic discs and with developmental disorders including tilted discs and ROP
  • bilateral and tends to affect the arteries and veins
  • most cases are idiopathic
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6
Q

Familial retinal arteriolar tortuosity (FRAT)

A

Characterized by marked tortuosity of second and third order retinal arteries and venous system (the venous column is not involved)

Veins usually not invovled

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7
Q

PHPV

A

-pathologic entities resulting from abnormal persistence of the fetal fibrovascular primitive stroma i.e. hyaloid system of the eye, which should disappear by the time of birth

Complications
-persistence and hypertrophy of these vessels can result in PHPV in the anterior and/or posterior chambers. It can give rise to leukocoria, RD, and subretinal hemorrhage

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8
Q

Spectrum of anteiror PHPV

A
  • cataracts
  • mittendorf dot
  • retrolental fibrous membranes and stalks
  • ciliary body disruption
  • microcornea
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9
Q

Spectrum of posterior PHPV

A 
Bergmesites 
Vitreoretinal veils 
Persistent fibrous stalks from the posterior lens o the ONH
Vitreoretinal traction
Retinoschisis 
RD
Macular ectopia
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10
Q

AVM

A
  • failure to develop cap networks between arterial and venous circulations
  • WM sybndtome is an AVM with cutaneous and intracranial abnormalities
  • looks like a bag of intestines

Complications

  • mild forms are typically stable
  • moderate to severe forms may leak
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11
Q

WM syndrome consists of

A

Retinal AVM associated with cutaneous or intracranial vascular proliferation’s

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12
Q

Cap hemangioma

A

Vasoproliferative tumor
Egg shaped lesion
VHL disease includes ocular and intracranial findgins

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13
Q

Useful clue to Dx cap hemangioma

A

Exudates around the disc are a useful clue to diagnose cap hemangioma

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14
Q

Inherited optic atrophy types

A
  • primary hereditary optic atrophy: specific defect directly results in axonal atrophy . Lebers, dominant optic atrophy
  • secondary hereditary optic atrophy: optic atrophy is linked t ocausal genes which alter other bodily processes which in turn affect the retina and the ONH. Wolframs syndrome, also called DIDMOAD
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15
Q

Pathophysiology of inherited optic atrophy

A
  • most of the heritable optic atrophied occur due to defects in nuclear DNA
  • lebers occurs due to defects in mitochondrial DNA
  • compromised mitchdonriral function can result from defects in mitochondrial and or nuclear genome
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16
Q

Domaintnt optic atrophy (DOA)

A
  • first decade of life (4-5)
  • slow visual decline and stable around 50
  • dominant inheritcen
  • OPA-1 gene mutation leads to defective mitochrdrian in RGC
  • thinning of the RNFL and atrophy of the disc
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17
Q

DOA presentation

A

Bialteral and symmetric

  • 20/20-20/200
  • varying extend of atrophy and excavation
  • classic CV/VF defect
  • blue dyschromatopsia
  • scotomas
  • mixed VF defects are actually more common
18
Q

Management of DOA

A

Low vision aids

Genetic counseling

19
Q

Dominant optic atrophy plus

A

Additional defects compared to pure DOA. Hearing problem, muscle problem, and neuropathies
-eventually progressive external ophthalmoplegia (CPEO)

20
Q

Lebers hereditary optic neuropathy (LHON)

A
  • 10-40 onset age
  • bialteral and asymmetrical presentation
  • transmitted maternally mtDNA
  • no father-son transmission
  • males affected 4:1 over females
  • wide range of phenotypic expression due to heteroplasmy and homoplasmy of mtDNA
21
Q

LHON plus disease

A

Involves MS like whit matter lesions in the brain

22
Q

Presentation of LHON

A
  • painless VA loss in one eye
  • fellow eye in a couple weeks to a year
  • pseudodisc edema, hyperemia, and telangectatic vessels are present at onset
  • loss of visual function is typically permanent
  • spared pupil function
23
Q

Manamgntof of LHON

A

No treatment

Genetic counseling

24
Q

Typical finding for LHON

A

Pseudodisc edema

25
Q

Difference between LHON and foster Kennedy syndrome

A
  • LHON mimics FKS in which one eye shows optic atrophy and the fellow eye shows disc edema
  • LHON may be easily differnt is Ted from FKS when central VF defects are present OU

In FKS a frontal lobe tumor compresses the ONH leading to atrophy. The tumor also causes raised ICP which leads to disc edema in the fellow eye

  • FKS usually does not show vision loss in the eye not compressed by the tumor
  • LHON does not leak dye on FA
26
Q

Neoplasms of ON

A 
Glioma 
Meningioma 
Cap hemangioma 
Astrocytic hamartoma 
Melanocytoma
27
Q

Optic nerve glioma

A
  • childhood onset
  • benign
  • if in adult age, its more aggressive and usually fatal
  • neoplasm arising out of ON glial tissue
28
Q

Presentation of optic nerve glioma

A

Blurry vision, disc pallor, APD

Late
-pallor, optociliary shunts, strab, proptosis

Complications: NICRVO, ICRVO 9iris rubeosis, NVG)

29
Q

Managment of optic nerve neoplasms

A

Observe in kids

Surgery if tumor grow intracrnaially

30
Q

The best clue for optic nerve glioma

A

Proptosis eye and muscel restciton

31
Q

Optic nerve sheath meningioma

A

Middle age
More in women
Usually benign
-neoplasms arising out meningiothelial cp cells of the ON sheath

32
Q

Optic nerve sheath meningioma

A

Proliferation of meningothelial cells within the nerve sheath of the orbital intracanalicular portion of the optic nerve

33
Q

Nerve sheath meningioma cause

A

Less rapid deterioration of vision than do gliomas as the tumor grows around the nerve and slowly compresses it

34
Q

Vision and optic nerve sheath meningioma

A

In early stages it is still pretty good unless it compresses the ON

35
Q

Optociliary shunt

A

Can be seen in optic nerve sheath meningioma

36
Q

Childhood gliomas

A

Rarely malignant whereas adult gliomas are almost always fatal

37
Q

Meningioma and benign gliomas are observed how often

A

3-6m F/U

38
Q

Melanocytoma optic nerve

A

Interdigitates with the NFL at the optic nerve

-nothing we can do, definite referral

39
Q

Astrocytic hamartoma

A

Assocaited with tuberous sclerosis or NF

  • right on the ONH
  • composed of astrocytes, calcium and other material
40
Q

Association of astrocytic hamartoma and systemic disease

A

Tuberous sclerosis

41
Q

Tuberous SLE Rossi

A

Rare multisystem genetic disease that causes benign tumors to grow int he brain and on other vital organs such as the kindest, heart, liver, eyes, lungs, and skin

3F: Neuro Final (8 decks)

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