Unknown - White Spots Periphery - Recurrent CME - Maybe Retinitis Pigmentosa without Pigment
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46-year-old woman was seen in the office on January 23, 2012. She has a family history of retinitis pigmentosa. There is five children all together and to her knowledge three of her siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. Previous to that the vision was good and she notices a dark spot in the central vision over the last few weeks, more in the left eye than the right eye. She does notice some problem with glare. She was skiing the other day and had some difficulty functioning and her night vision has been not quite right for sometime. She has not had a peripheral vision test for a while and you noticed on a retinal scan recently her maculae are abnormal. Also she had a peripheral iridotomy laser back in 1993 for shallow angles.
VISUAL ACUITY: OD 20/40, OS 20/50. IOP: OD 11, OS 13. The anterior chamber is shallow in both eyes and she has peripheral iridotomies. She has 2+ nuclear sclerotic cataracts.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.3. There is no posterior vitreous separation. The fovea looks irregular. There are unusual looking white spots in the mid periphery, which radiate out and look to be superficial, in fact some of them are in front of the retinal vessels.
OS: Vertical C/D ratio is 0.3. There is no posterior vitreous separation. The macula looks irregular. Again, there is some superficial white spots radiating out in the periphery, but again they look to be in front of the retinal vessels. Her nerves are not pale and her retinal arteries are only minimally attenuated. Photos confirm clinical findings.
SPECTRALIS-SD-OCT SCAN: The OCT scan shows macular edema in both eyes with a serous macular detachment in the left eye. There are intraretinal cysts and retinal atrophy in the far periphery. The OCT scan does show atrophy of the outer retina.
FLUORESCEIN ANGIOGRAPHY: Fluorescein angiography shows hyperfluorescence in a pattern consistent with retinitis pigmentosa variance, where there is sparing of the central macula with increased hyperfluorescence in the periphery.
FUNDUS AUTO FLUORESCENCE: The fundus auto fluorescence images show patches of hypo auto fluorescence, predominately in the superior periphery with some hyper auto fluorescence around the macula in the mid periphery.
IMPRESSION:
1. RETINAL DYSTROPHY – BOTH EYES WITH WHITE FLECKS
2. POSSIBLE RETINITIS PUNCTATA ALBESCENS
3. FAMILY HISTORY OF RETINITIS PIGMENTOSA
DISCUSSION: I explained to the patient with her strong family history of retinitis pigmentosa, even though she has no pigment spots in the eye, she does have areas of retinal atrophy and geographic characteristics consistent with a retinal dystrophy and I think it would be reasonable for her to consider at least blood testing, if not a electroretinogram or both. I have given her information on the Carver Lab out in Iowa, in addition she may go to Bascom Palmer for further evaluation. In the meantime to treat her macular edema, I started her on Diamox 500 mg twice a day, which does work in retinitis dystrophic macular edema. Also topical angiotensin converting enzyme inhibitor will help it if she has trouble tolerating the systemic ones and I asked her to return for a check in one months or sooner if she should notice a problem.
As of 2/3/13, Patient initially responded to oral and topical ACE inhibitors, but then broke through therapy and required posterior subtenon's kenalog injections to clear edema in the right eye: 6/7/12, 12/17/12, and 1/23/13; left eye 7/5/12
Genetic testing from Carver Lab was negative for 16 genes previously associated with AR RP (ARRP,ABCA4, CERKL, CNGA1, CRB1, CHDDS, EYS, LRAT, MAK, NR2E3, PDE6B, RDH12, RLBP1, RPE65, SAG, TULP1, and USH2A.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME733 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME690 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME615 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME741 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME595 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME636 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME675 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME543 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME591 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME589 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FAF547 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FAF662 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FAF546 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FAF567 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME491 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME483 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME506 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME490 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early576 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early552 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early522 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early666 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA MID696 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA MID523 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA MID583 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA MID513 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA LATE553 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME- FA LATE535 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early472 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early526 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early533 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early583 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early510 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME - FA Early504 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME469 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME546 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME494 views46-year-old woman three of her 5 siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. OD 20/40, OS 20/50. IOP: Both eyes have CME
(0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME506 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME464 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME557 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME484 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME471 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME494 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME516 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME589 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME453 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME566 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME436 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME449 views1 month of treatment with diamox 500 mg BID- cme is a little better
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME553 viewsCME worse - patient received subtenon's kenalog injection right eye.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME677 viewsCME worse - patient received subtenon's kenalog injection right eye.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME667 viewsSubtenon's Kenlalog given right eye today.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME729 viewsSubtenon's Kenlalog given right eye today.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME589 viewsComparison of Maps - CME is now cleared
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CME661 viewsComparison of Maps - CME is now cleared
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46-year-old woman was seen in the office on January 23, 2012. She has a family history of retinitis pigmentosa. There is five children all together and to her knowledge three of her siblings have been diagnosed with retinitis pigmentosa. Her mother and father were seen and as far as everyone can tell, neither of them has it, although there was some thought they might be carriers. They were evaluated twenty five years ago and since then as far as she knows, there has not been a lot of testing done.
She has noticed a substantial decline in vision in both eyes over the last month or two. Previous to that the vision was good and she notices a dark spot in the central vision over the last few weeks, more in the left eye than the right eye. She does notice some problem with glare. She was skiing the other day and had some difficulty functioning and her night vision has been not quite right for sometime. She has not had a peripheral vision test for a while and you noticed on a retinal scan recently her maculae are abnormal. Also she had a peripheral iridotomy laser back in 1993 for shallow angles.
VISUAL ACUITY: OD 20/40, OS 20/50. IOP: OD 11, OS 13. The anterior chamber is shallow in both eyes and she has peripheral iridotomies. She has 2+ nuclear sclerotic cataracts.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.3. There is no posterior vitreous separation. The fovea looks irregular. There are unusual looking white spots in the mid periphery, which radiate out and look to be superficial, in fact some of them are in front of the retinal vessels.
OS: Vertical C/D ratio is 0.3. There is no posterior vitreous separation. The macula looks irregular. Again, there is some superficial white spots radiating out in the periphery, but again they look to be in front of the retinal vessels. Her nerves are not pale and her retinal arteries are only minimally attenuated. Photos confirm clinical findings.
SPECTRALIS-SD-OCT SCAN: The OCT scan shows macular edema in both eyes with a serous macular detachment in the left eye. There are intraretinal cysts and retinal atrophy in the far periphery. The OCT scan does show atrophy of the outer retina.
FLUORESCEIN ANGIOGRAPHY: Fluorescein angiography shows hyperfluorescence in a pattern consistent with retinitis pigmentosa variance, where there is sparing of the central macula with increased hyperfluorescence in the periphery.
FUNDUS AUTO FLUORESCENCE: The fundus auto fluorescence images show patches of hypo auto fluorescence, predominately in the superior periphery with some hyper auto fluorescence around the macula in the mid periphery.
IMPRESSION:
1. RETINAL DYSTROPHY – BOTH EYES WITH WHITE FLECKS
2. POSSIBLE RETINITIS PUNCTATA ALBESCENS
3. FAMILY HISTORY OF RETINITIS PIGMENTOSA
DISCUSSION: I explained to the patient with her strong family history of retinitis pigmentosa, even though she has no pigment spots in the eye, she does have areas of retinal atrophy and geographic characteristics consistent with a retinal dystrophy and I think it would be reasonable for her to consider at least blood testing, if not a electroretinogram or both. I have given her information on the Carver Lab out in Iowa, in addition she may go to Bascom Palmer for further evaluation. In the meantime to treat her macular edema, I started her on Diamox 500 mg twice a day, which does work in retinitis dystrophic macular edema. Also topical angiotensin converting enzyme inhibitor will help it if she has trouble tolerating the systemic ones and I asked her to return for a check in one months or sooner if she should notice a problem.
As of 2/3/13, Patient initially responded to oral and topical ACE inhibitors, but then broke through therapy and required posterior subtenon's kenalog injections to clear edema in the right eye: 6/7/12, 12/17/12, and 1/23/13; left eye 7/5/12
Genetic testing from Carver Lab was negative for 16 genes previously associated with AR RP (ARRP,ABCA4, CERKL, CNGA1, CRB1, CHDDS, EYS, LRAT, MAK, NR2E3, PDE6B, RDH12, RLBP1, RPE65, SAG, TULP1, and USH2A.
