Acute Zonal Occult Outer Retinopathy - AZOOR - Cystoid Macular Edema
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58-year-old man in the office on March 25, 2008. He developed a peripheral vision problem about 25 years ago. He had a bump on the head with a refrigerator door, but it was nothing significant and there was no loss of consciousness, and then the very next day he woke up with severe side vision loss in the left eye. He had an extensive evaluation done in Dallas, Texas where he saw a neuro-ophthalmologist and as best he could tell, there was never any diagnosis made. He since then has had poor side vision, however, the central vision in the left eye has been good. His left eye is his better eye and his dominant eye until about three to four weeks ago when he started to develop a smudge in the central vision, which waxed and waned. His vision in the right eye is fine.
VISUAL ACUITY: OD 20/30, OS 20/40. IOP: OD 17, OS 18. The right eye has normal peripheral vision. The left eye has loss of peripheral vision. The left eye also has a 1+ afferent pupillary defect. The color vision is normal in each eye with 9/10 plates correct.
SLIT LAMP EXAM: There is 1+ nuclear sclerosis in both eyes.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.4. There is no posterior vitreous separation. There is mild peripapillary atrophy.
OS: Vertical C/D ratio is 0.4. There is peripapillary atrophy, which is more extensive than the fellow eye. There is 3+ cystoid macular edema. There are bone spicules starting at the macula and extending out to the periphery.
OCT SCAN: The OCT scan of the right eye was normal. The left eye shows cystoid macular edema.
Photos confirm clinical findings.
FLUORESCEIN ANGIOGRAM: FA shows petaloid staining of the macula in the late frame. There is no evidence of any choroidal neovascularization. There is hypopigmentation around each optic nerve, consistent with peripapillary atrophy.
IMPRESSION:
1. PROBABLE ACUTE ZONAL OCCULT OUTER RETINOPATHY (AZOOR) – LEFT EYE
2. BONE SPICULES IN THE PERIPHERY – LEFT EYE
3. CYSTOID MACULAR EDEMA – LEFT EYE
4. PERIPAPILLARY ATROPHY CONSISTENT WITH HISTORY OF POSSIBLE ACUTE IDIOPATHIC ENLARGEMENT BLIND SPOT SYNDROME – BOTH EYES
DISCUSSION: I explained to the patient I explained to the patient that although 25 years ago they did not know the diagnosis, the findings in the left eye are most consistent with acute zonal occult outer retinopathy, which was described about 15 years ago by Dr. Guess. The left eye looks exactly like eye with retinitis pigmentosa and since patients with retinitis pigmentosa can develop cystoid macular edema, it makes sense that his eye could also develop cystoid macular edema.
Patients with retinitis pigmentosa will sometimes recover on their own, however, sometimes it is helpful to treat the eye and the treatments, which are helpful, involve either Kenalog treatments or oral Diamox pills. Oral Diamox is more standard of care in patients with retinitis pigmentosa. I gave him a brochure about all the risks involved with oral Diamox and I suggested for now we leave the eye alone. He will return for a check in six weeks, sooner should he notice any problem. If the eye looks better at that point, I will leave the eye alone. I asked him to return urgently should he notice any problem. He will see you back regularly. He already had a full medical examination because one thing I was concerned about whether he is a little anemic or his blood pressure is high, which can also cause macular swelling, but as far as he can tell at this point, things are going pretty well medically.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 462 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 553 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 390 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 422 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 397 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 389 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 442 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 463 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 357 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 372 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 419 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 348 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 326 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 283 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 309 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 340 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 403 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye 400 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye Post Treatment for CME469 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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Acute Zonal Occult Outer Retinopathy with Severe Peripheral Vision Loss Left Eye Post Treatment for CME418 views 58-year-old man developed a peripheral vision problem about 25 years ago. OD 20/30, OS 20/40.
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58-year-old man in the office on March 25, 2008. He developed a peripheral vision problem about 25 years ago. He had a bump on the head with a refrigerator door, but it was nothing significant and there was no loss of consciousness, and then the very next day he woke up with severe side vision loss in the left eye. He had an extensive evaluation done in Dallas, Texas where he saw a neuro-ophthalmologist and as best he could tell, there was never any diagnosis made. He since then has had poor side vision, however, the central vision in the left eye has been good. His left eye is his better eye and his dominant eye until about three to four weeks ago when he started to develop a smudge in the central vision, which waxed and waned. His vision in the right eye is fine.
VISUAL ACUITY: OD 20/30, OS 20/40. IOP: OD 17, OS 18. The right eye has normal peripheral vision. The left eye has loss of peripheral vision. The left eye also has a 1+ afferent pupillary defect. The color vision is normal in each eye with 9/10 plates correct.
SLIT LAMP EXAM: There is 1+ nuclear sclerosis in both eyes.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.4. There is no posterior vitreous separation. There is mild peripapillary atrophy.
OS: Vertical C/D ratio is 0.4. There is peripapillary atrophy, which is more extensive than the fellow eye. There is 3+ cystoid macular edema. There are bone spicules starting at the macula and extending out to the periphery.
OCT SCAN: The OCT scan of the right eye was normal. The left eye shows cystoid macular edema.
Photos confirm clinical findings.
FLUORESCEIN ANGIOGRAM: FA shows petaloid staining of the macula in the late frame. There is no evidence of any choroidal neovascularization. There is hypopigmentation around each optic nerve, consistent with peripapillary atrophy.
IMPRESSION:
1. PROBABLE ACUTE ZONAL OCCULT OUTER RETINOPATHY (AZOOR) – LEFT EYE
2. BONE SPICULES IN THE PERIPHERY – LEFT EYE
3. CYSTOID MACULAR EDEMA – LEFT EYE
4. PERIPAPILLARY ATROPHY CONSISTENT WITH HISTORY OF POSSIBLE ACUTE IDIOPATHIC ENLARGEMENT BLIND SPOT SYNDROME – BOTH EYES
DISCUSSION: I explained to the patient I explained to the patient that although 25 years ago they did not know the diagnosis, the findings in the left eye are most consistent with acute zonal occult outer retinopathy, which was described about 15 years ago by Dr. Guess. The left eye looks exactly like eye with retinitis pigmentosa and since patients with retinitis pigmentosa can develop cystoid macular edema, it makes sense that his eye could also develop cystoid macular edema.
Patients with retinitis pigmentosa will sometimes recover on their own, however, sometimes it is helpful to treat the eye and the treatments, which are helpful, involve either Kenalog treatments or oral Diamox pills. Oral Diamox is more standard of care in patients with retinitis pigmentosa. I gave him a brochure about all the risks involved with oral Diamox and I suggested for now we leave the eye alone. He will return for a check in six weeks, sooner should he notice any problem. If the eye looks better at that point, I will leave the eye alone. I asked him to return urgently should he notice any problem. He will see you back regularly. He already had a full medical examination because one thing I was concerned about whether he is a little anemic or his blood pressure is high, which can also cause macular swelling, but as far as he can tell at this point, things are going pretty well medically.
