B topics

Question 1

what is dystrophy and degeneration

Answer 1

dystrophy - hereditary that cause organ or tissue to waste away / weaken

degeneration - acquired loss of function due to aging, inflammation, infection, vascular disease, vitamin deficiency

Question 2

what are the types of corneal degeneration

Answer 2

crocodile shagreen
band keratopathy
corneal guttata

Question 3

what is band keratopathy and its ssx

Answer 3

deposition of calcium in bowman membrane

ssx:
affect vx when it block pupil
irritation, FB , pain

early is just white granular crescent at limbus
late - spread centrally a band chalky plaque

Question 4

causes of band keratopathy

Answer 4

chronic anterior uveitis
age related
systemic hypercalcemia
hereditary

Question 5

management of band keratopathy

Answer 5

lubrication for mild, chelation for severe and monitor for 3-6 month

Question 6

what is crocodile shagreen and ssx and mx

Answer 6

grey whate polygonal opacity in stroma with spaces in between

asymtom but may cause blur vision in severe

no mx

Question 7

what is EBMD and causes

Answer 7

epithelial dystrophy
seen in 20-30 yo

due to thickening of basement membrane - epithelium with protein deposit lead to weaken adhesion

Question 8

ssx of EBMD

Answer 8

dot like opacities, microcyst, maps, fingerprint
no symptoms
some may experience, irritation, photophobia, and reduced va

Question 9

mx of EBMD

Answer 9

lubrication, bandage cl, topical saline (5%)

Question 10

Types of epithelial dystrophy

Answer 10

meesman dystrophy
Epithelial basement membrane dystrophy

Question 11

what is the bowman membrane dystrophy

Answer 11

reis buckler dystrophy

Question 12

what is meesman dystrophy

Answer 12

juvenile epithelial dystrophy, presents before 2nd bdy

Question 13

ssx meesman dystrophy

Answer 13

small white intraepithelial cyst, corneal erosion
asymtom , irritation, photophobia, reduce va

Question 14

mx of meesman dystrophy

Answer 14

symptomatic relief, lubricants

Question 15

what is reis-buckler dystrophy

Answer 15

its a bowman membrane dystrophy, seen in early infancy

its autosomal dominant

Question 16

ssx of reis buckler

Answer 16

haze white, round polygonal opacity in the bowman progress to reticular pattern

severe recurrent cornea erosion in childhood

Question 17

mx of reis buckler

Answer 17

• lamellar keratoplasty
• excimer laser keratectomy

Question 18

Types of stroma dystrophy

Answer 18

lattice
macualr
granular

Question 19

what is lattice dystrophy and its ssx

Answer 19

• AD, TGFB1 type
  recurrent erosion @ end of 1st decade
  glass like filament lesion
  generalised stroma haze may impair vision

Question 20

mx of lattice dystrophy

Answer 20

penetrating keratoplasty / deep lamellar keratoplasty

Question 21

what is granular dystrophy and its ssx

Answer 21

• AD, TGFB1 type
• recurrent erosion uncommon
• glare and photophobia
  -discrete crumb like opacities in the central anterior stroma
  -gradual increase of size and number with deeper and outward spread
• impaired corneal sensation

Question 22

mx of granular dystrophy

Answer 22

penetrating keratoplasty / deep lamellar keratoplasty

Question 23

what is macular dystrophy and its ssx

Answer 23

AR, CHST6

early end of 1st decade visual deterioration
recurrent erosion is common
dense greyish white spot centrally in anterior stroma and peripherally in posterior stroma
eventually involvement of full thickness stroma

Question 24

mx of macular dystrophy

Answer 24

penetrating keratoplasty

Question 25

type of endothelial dystrophy

Answer 25

fuch dystrophy

Question 26

back ground of fuch dystrophy

Answer 26

women affected , ONSET in middle age or older
slow progressive disease in old age
bilateral endothelial cell loss
increase prevalence of POAG

Question 27

SSX of fuch dystrophy

Answer 27

cornea guttata
gradual worsening blur in the morning
specular reflection show tiny dark spot progressing to beaten metal appearance
endothelial decompensation - central stroma oedema, blur vision
oedema in advance cases form bullae = rupture = pain and expose nerves fibers

Question 28

mx of fuch

Answer 28

non urgent referral
hypertonic saline
evaporation of excess fluid off cornea
bandage cl for ruptured bullae
fail medical treatment - surgical tx
- penetrating
-decemet stripping endothelial keratoplasty
-decemet membrane endothelial keratoplasty

Question 29

what is stargardt disease

Answer 29

AR , ABCA4

-Common cause of central vx loss in adult <50
-accumulation of lipofusin in rpe
-present in childhood / adolescence
-prognosis is poor
-va reach below g/12 it will worsen rapidly before stabilizing at 6/60
-its bilateral

Question 30

ssx of stargardt disease

Answer 30

gradual impairment of central vision
some may complain reduce cv and impairment of dark adaptation

macula normal initially then show nonspecific mottling
progression to geographic atrophy / bull eye maculopathy
yellow-whitish lesion @RPE

Question 31

mx of stargradt

Answer 31

no tx, low vision aid, genetic counselling, avoid vitamin a supplement

Question 32

what is best disease

Answer 32

AD, BEST1 - found in plasma membrane of rpe and function as a transmembrane ion channel

second common macular dystrophy after stargradt
prognosis is generally good until middle age. which va declines in one/both eye due to cnv, scarring, geographic atrophy

Question 33

ssx of best disease

Answer 33

early - vitelliform appearance develop in childhood with round, sharply delineated macular lesion

late- slow progression to vitelliruptive , gradual fall in va

end - macular atrophy

Question 34

mx of best disease

Answer 34

no tx, genetic counselling, low vx aid for poor vx

Question 35

what is RP

Answer 35

AD,AR,XL , sporadic, many case due to mutation of rod genes

most common hereditary fundus dystrophy 1:5000
rod -cone dystrophy
group of inherited diffuse retinal degenerative disease

Question 36

ssx of rp

Answer 36

early - night problem, progressive peripheral visin restriction

late - tunnel vx

sign - rpe atrophy associated with arteriolar narrowing
bone spicule pigmentary changes @ periphery
waxy pallor optic disc
gradual increase in density and extent of pigmentary changes

Question 37

complication of rp

Answer 37

pscc, pvd, poag, keratoconus