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 CMEvu 737 fois46-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 CMEvu 692 fois46-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 CMEvu 616 fois46-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 CMEvu 744 fois46-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 CMEvu 595 fois46-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 CMEvu 641 fois46-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 CMEvu 683 fois46-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 CMEvu 547 fois46-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 CMEvu 592 fois46-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 CMEvu 592 fois46-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 - FAFvu 548 fois46-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 - FAFvu 665 fois46-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 - FAFvu 548 fois46-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 - FAFvu 568 fois46-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 CMEvu 494 fois46-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 CMEvu 488 fois46-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 CMEvu 507 fois46-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 CMEvu 491 fois46-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 Earlyvu 578 fois46-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 Earlyvu 552 fois46-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 Earlyvu 522 fois46-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 Earlyvu 669 fois46-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 MIDvu 697 fois46-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 MIDvu 524 fois46-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 MIDvu 583 fois46-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 MIDvu 514 fois46-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 LATEvu 554 fois46-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 LATEvu 536 fois46-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 Earlyvu 473 fois46-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 Earlyvu 528 fois46-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 Earlyvu 534 fois46-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 Earlyvu 583 fois46-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 Earlyvu 510 fois46-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 Earlyvu 504 fois46-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 CMEvu 469 fois46-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 CMEvu 547 fois46-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 CMEvu 497 fois46-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 CMEvu 507 fois1 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 CMEvu 465 fois1 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 CMEvu 560 fois1 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 CMEvu 487 fois1 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 CMEvu 473 fois1 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 CMEvu 494 fois1 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 CMEvu 517 fois1 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 CMEvu 591 fois1 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 CMEvu 456 fois1 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 CMEvu 567 fois1 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 CMEvu 437 fois1 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 CMEvu 449 fois1 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 CMEvu 554 foisCME 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 CMEvu 678 foisCME 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 CMEvu 669 foisSubtenon's Kenlalog given right eye today.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 730 foisSubtenon's Kenlalog given right eye today.
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 591 foisComparison of Maps - CME is now cleared
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 662 foisComparison 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.
