RETINAL VASCULOPATHY in FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY
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59-year-old woman was seen in the office on 3-24-08. This patient was in for a comprehensive evaluation. She had a blood pressure spike to 170/90 and was on medications for that. She has not had any specific visual complaints. This was her first visit with you and you saw problems with the retinal vasculature and suggested she come in here for evaluation. She has been questioned multiple times and she is not diabetic. She did recently have a 50 pound weight loss, but she says even prior to the 50 pound weight loss she was at that time also not diabetic.
She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
AMSLER GRID: Normal OU.
SLIT-LAMP EXAMINATION: There is 2+ nuclear sclerosis in both eyes.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.3. There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
OS: Vertical C/D ratio is 0.2. There is no posterior vitreous separation or patchy retinal hemorrhages at all levels. They are rare and there is no edema or proliferation.
The OCT scan was normal in both eyes. Photos confirm clinical findings.
Fluorescein angiogram shows blockage where the hemorrhages are as well as a few microaneurysms near the fovea in the both eyes.
IMPRESSION: RETINAL VASCULOPATHY IN BOTH EYES ASSOCIATED WITH FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY.
DISCUSSION: I explained to the patient that she does have retinal hemorrhages and microaneurysms, which look exactly like you would see in a patient with diabetic retinopathy.
There is an association of 50 to 75% of patients with facioscapulohumeral muscular dystrophy do develop abnormalities of the retinal blood vessels. Most of the reports suggest that it is a Coats-like response where there is sometimes retinal exudate and sometimes areas of retinal non-perfusion and capillary drop out, but the descriptions I found were not too dissimilar to what the patient manifests. I explained to the patient that I think it is unlikely she will suffer vision loss, but it seems like these eyes tend to behave similar to eyes with Coat’s disease and can sometimes develop problems. I asked her to return here for a check in 6 months, sooner should she notice any problem and I suggest she also have her cousin come in for retinal evaluation.
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy line os428 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy 465 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
59-year-old woman had a blood pressure spike to 170/90 and was on medications for tha     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy 456 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy 494 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy 385 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy very early fa344 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy early fa478 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy early fa276 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy early fa283 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy early fa330 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy mid fa363 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy mid fa281 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy late fa348 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy late fa355 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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Retinal Vasculopathy associated with Facioscapulohumeral Muscular Dystrophy OCT right eye line scan395 views59-year-old woman had a blood pressure spike to 170/90 and was on medications for that. She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
     (0 votes)
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59-year-old woman was seen in the office on 3-24-08. This patient was in for a comprehensive evaluation. She had a blood pressure spike to 170/90 and was on medications for that. She has not had any specific visual complaints. This was her first visit with you and you saw problems with the retinal vasculature and suggested she come in here for evaluation. She has been questioned multiple times and she is not diabetic. She did recently have a 50 pound weight loss, but she says even prior to the 50 pound weight loss she was at that time also not diabetic.
She does have facioscapulohumeral muscular dystrophy, which runs in her family. She has a cousin with the same disorder.
VISUAL ACUITY: Her vision is 20/25 OU. IOP: OD 21, OS 18.
AMSLER GRID: Normal OU.
SLIT-LAMP EXAMINATION: There is 2+ nuclear sclerosis in both eyes.
EXTENDED OPHTHALMOSCOPY:
OD: Vertical C/D ratio is 0.3. There is no posterior vitreous separation, telangiectatic vessels near the macula. There are patchy retinal hemorrhages. There is no edema or proliferation.
OS: Vertical C/D ratio is 0.2. There is no posterior vitreous separation or patchy retinal hemorrhages at all levels. They are rare and there is no edema or proliferation.
The OCT scan was normal in both eyes. Photos confirm clinical findings.
Fluorescein angiogram shows blockage where the hemorrhages are as well as a few microaneurysms near the fovea in the both eyes.
IMPRESSION: RETINAL VASCULOPATHY IN BOTH EYES ASSOCIATED WITH FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY.
DISCUSSION: I explained to the patient that she does have retinal hemorrhages and microaneurysms, which look exactly like you would see in a patient with diabetic retinopathy.
There is an association of 50 to 75% of patients with facioscapulohumeral muscular dystrophy do develop abnormalities of the retinal blood vessels. Most of the reports suggest that it is a Coats-like response where there is sometimes retinal exudate and sometimes areas of retinal non-perfusion and capillary drop out, but the descriptions I found were not too dissimilar to what the patient manifests. I explained to the patient that I think it is unlikely she will suffer vision loss, but it seems like these eyes tend to behave similar to eyes with Coat’s disease and can sometimes develop problems. I asked her to return here for a check in 6 months, sooner should she notice any problem and I suggest she also have her cousin come in for retinal evaluation.