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 833 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 790 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 709 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 862 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 671 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 733 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 766 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 638 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 679 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 688 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 648 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 763 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 628 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 681 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 565 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 566 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 593 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 589 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 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 - FA Earlyvu 626 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 613 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 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 CME - FA MIDvu 779 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 591 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 654 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 599 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 622 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 590 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 544 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 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 CME - FA Earlyvu 609 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 642 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 574 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 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 541 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 625 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 589 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 532 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 663 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 583 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 563 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 564 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 593 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 663 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 548 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 633 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 505 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 538 fois1 month of treatment with diamox 500 mg BID- cme is a little better     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 612 foisCME worse - patient received subtenon's kenalog injection right eye.     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 744 foisCME worse - patient received subtenon's kenalog injection right eye.     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 746 foisSubtenon's Kenlalog given right eye today.     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 819 foisSubtenon's Kenlalog given right eye today.     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 669 foisComparison of Maps - CME is now cleared     (0 votes)
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Retinitis Pigmentosa Sine Pigmento or Unknown Dystrophy - Peripheral Superficial White Spots and Recurrent CMEvu 762 foisComparison of Maps - CME is now cleared     (0 votes)
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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.