Retina/Vitreous/Other Uveitis

Question 1

• Most common causes of vitreous hemorrhage

Answer 1

1) PDR; 2) retinal break without detachment; 3) PVD; 4) rhegmatogenous RD; 5) NV after RVO.

Question 2

• Causes of crystalline retinopathy

Answer 2

tamoxifen toxicity, methyoyfluorane toxicity, nitrofurantoin toxicity, canthaxanthine toxicity, talc, antifreeze ingestion, primary hereditary hyperoxyuria, bietti crystalline dystrophy

Question 3

• Gyrate atrophy:

Answer 3

mutation in OAT gene, gyrate chorioretinal lesions, PSC, high myopia with astigmatism, hyper ornithemia. Autosomal recessive inheritance. Tx is dietary argenine restriction and B6 supplementation.

Question 4

• Best dystrophy gene

Answer 4

BEST1 gene. Adult onset foveomacular vitelliform dystrophy (AFVD) is similar. Implied genes for that are PRPH2, IMPG1 and IMPG2

Question 5

• Stargardt’s gene

Answer 5

ABCA4

Question 6

• Gyrate atrophy gene

Answer 6

OAT

Question 7

• RD prognosis

Answer 7

best for small holes or retinal dialysis and those associated with demarcation lines. Worse for aphakic / pseudophakic eyes, worse for PVR, giant retinal tears, choroidal detachment, inflammation, or traumatic posterior breaks

Question 8

• DRS study

Answer 8

PRP vs no laser for severe NPDR or PDR—end point was severe vision loss (PRP helps).

Question 9

• EDTRS

Answer 9

focal laser reduces moderate vision loss and increased chance of 2 line gain in people with CSME. Early scatter PRP results in a small reduction in the risk of severe vision loss.

Question 10

• DRVS

Answer 10

(diabetic retinopathy vitrectomy study): early vitrectomy for nonclearing vitreous hemorrhage increased chances of 20/40 vision in type 1 diabetics

Question 11

• DCCT

Answer 11

(diabetes control complications trial): intensive control of blood glucose reduced risk of developing retinopathy by 76% and slowed progression by 54% in type 1 diabetics.

Question 12

• COMS study (large):

Answer 12

enucleation alone vs. XRT followed by enucleation—no difference in outcome.

Question 13

• COMS study (medium):

Answer 13

brachytherapy vs enucleation had similar mortality. Mets were found in approx. 10% of enucleated patients

Question 14

• COMS study (small):

Answer 14

mortality 1% at 5 years with observation only.

Question 15

• Choroideremia:

Answer 15

CHM gene, x-linked recessive. Starts mottled pigment then scalloped. Typically no change in vasculature or optic atrophy. Gene product is within the RPE not the choroid. Most have good vision until 30s-40s.

Question 16

• FEVR

Answer 16

genetic, autosomal dominant with variable expressivity. Looks like ROP but not preterm babies.

Question 17

• IP (incontinentia pigmenti):

Answer 17

otherwise known as bloch Sulzberger syndrome; x-linked dominant lethal in hemizygous form (lethal in males). Involves eyes, skin and brain. Skin findings include bullae on the extremities, hyperpigmented macules on the trunk. Brain abnormalities include microcephaly, seizures, intellectual disability. Eye findings are like ROP.

Question 18

• Chance of progression to PDR:

Answer 18

with severe NPDR it is 15% chance in 1 year, for very severe NPDR it is 45% chance to progress

Question 19

• Consideration for prophylactic barrier in people with lattice dystrophy:

Answer 19

usually lattice confers 1% risk of RD. It is found in 6-10% of eyes but in 20-30% of eyes with RDs. Consider laser if high myopia, history of RD in the fellow eye, presence of flap tears within the lattice, or aphakia (not pseudophakia).

Question 20

• Gyrate atrophy

Answer 20

OAT gene. Restrict argenine, supplement with B6. Autosomal recessive. Associated with PSC cataracts, high myopia with high astigmatism, hyperornithemia (if normal ornithine levels reconsider diagnosis). VA usually normal until approx. 10 years old, but night blindness usually starts within the first decade of life.

Question 21

• PHPV:

Answer 21

distinguish from RB by long ciliary processes. Also usually in microphthalmic eyes whereas RB is usually in normally sized eyes.

Question 22

• Von Hippel Landau

Answer 22

chromosome 3. Retinal hemangioblastomas with CNS / systemic disease. Vision loss can occur from exudate our serous RD. primary cause of death is cerebellar hemangioblastomas and renal cell carcinoma. A thorough systemic workup is needed. You can also get other findings like pheochromocytomas, meningiomas, liver cysts, spinal cord tumors, etc.