Phakomatoses Flashcards

0
Q

Tuberous sclerosis
A) which chrom
B) inheritence
C) 4 findings

A
Chrom 9
Ad
1) adenoma sebaceum (malar like rash)
2) shagreen patch (skin finding)
3) ash leaf spot (hypopig macule)
4) retinal astrocytic hamartomas (mulberry lesion)

Kids usually have epilepsy

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1
Q

Name nf 1 findings (name 6 starting from skin to eye to bone)

A
  1. Cafe au lait spots
  2. Plexiform neurofibroma of lid
  3. Lisch nodules
  4. Combined hamartoma of rpe
  5. Cranial bone defects (absent sphenoid wing w pulsatile exophthalmos)
  6. Optic n glioma
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2
Q

Vhl
Chrom?
Inher?
5 tumors ass w it

A
Chrom 3 defect in tumor suppressor gene
Ad
1) retinal cap hemangioma
2) pheo
3) rcc
4) cerebellar hemangioblastoma
5) epididymal tumors
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3
Q

Whats the spahgetti crazy retinal vascular tumor?

A

Wyburn mason

Racemose hemangioma

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4
Q

What systemic findings do you worry about w wyburn mason?

A

Cns avm in the midbrain

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5
Q

Retinal cavernous hemangioma what does it look like?

A

Cluster of grapes

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6
Q

Whats organoid nevus syndrome? 3 findings?

A

1) alopecia
2) epibulbar mass like a dermoid
3) choroidal osteoma

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7
Q
Ataxia telangiectasia 
Inher
Other name
Main findings
Systemic risk?
A

Ar
Louis barr
Conj telangiectasia
Immunodeficiencies due to lack of iga

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8
Q

What do the numerals on a gvf mean?

A

I-V stimulous size
1-4 brightness of stimulous
e filter

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9
Q

Whats a scotoma?

A

Dark spot in vf surrounded by area of seeing

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10
Q

Whats a depression on vf?

A

Inward shift in a portion of an isopter

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11
Q

What causes halos at night in post lasik?

A

Pupil dilates at night
Spherical abberation light goes thru untreated k
Myopic defocus
This happens more in high refractive errors and small ablation zone

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12
Q

Whats abbe value?

A

High abbe value is good
It means less chromatic aberration
Its a property of glass

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13
Q

Steps in fitting rgp

A

Choses bc 0.5 steeper than low k
Switch to minus cyl
Drop cyl
Get ks

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14
Q

What do u do if cl is too steep?

A

Incr bc or decr diameter

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15
Q

If someone has induced astig what do u do?

A

Check topo

16
Q

Can u do duochrome on pt w 20/50?

A

No has to be better than 20/40

17
Q

What do you do if pt has myopic surprise after ce?

A
Consider pco and do yag
Ovd stuck behind iol
Iol put in backwards(capsular block syndrome)
Iol shift forward
Iol exchange
Piggyback lens
Refractive surgery
18
Q

How do u fit rgp?

A
Convert to minus cyl
Drop the cyl
Calc new vertex distance
Fit 0.5 steeper than flat k
Check the lens fits well w floursecin pattern 
Sam fap rule 
This is correct
19
Q

How do u check va if doesnt speak english?

A

Landolt c

Internet eye charts in theor own language

20
Q

Post lasik pt wants to be glasses independent what do u do?

A

Consider monovision for their iol choice

21
Q

What is distortion?

A

Due to curve of high rx lenses the loght curves at the edges

22
Q

Whats the problem w progressives

A

Can have oblique astigmatism at the edges

Theres a sweet spot

23
Q

Aphakic cl in one eye and glasses in phakic eye ghosting what do you do?

A

Can try scl ou but may have anisokonia

Give pt - glasses in eye w + cl

24
Q

If pt comes in and topo shows irreg astig what do you do?

A

Could be forme fruste kcn
Fit w rgp
Could become an ectasia
Follow w serial topo

25
Q

What do halos mean?

A

Could be very high iop

26
Q

What is ff? Monocular or binocular?

A

Monocular

27
Q

Whats csm monoc or binocular?

A

Cs- momocular

M- binocular

28
Q

What type of surprise can u get if so was in eye?

A

Hyperopic surprise bc so a length may be falsely long so you pock weaker iol

29
Q

What kind of lens to put in if so is staying?

A

Convex plano w plano side to so

30
Q

What does so do to biomtry?

A

It falsely lengthens eye

31
Q

Medical rx of ectropipb

A

Tape

32
Q

Surg options of ectropipb

A

1) Lateral tarsal strip w fixation to periosteum
2) Wedge excision w primary closure
3) Tarsortaphy

33
Q

What can happen to keratoacanthona?

A

Can involute

Or can excise

34
Q

What do u do for large >50% defect?

A

Lid sharing procedure

Highes or cutler beard