Term
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Definition
• Loss of pericytes
• Endothelial cell damage
• Leaky tight junctions
• Loss of blood-retinal barrier
-> proliferative retinopathy |
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Term
| Four Stages of Diabetic Retinopathy |
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Definition
• Mild Non-proliferative Retinopathy
• Moderate Non-proliferative Retinopathy
• Severe Non-proliferative Retinopathy or Pre- proliferative Retinopathy
• Proliferative Retinopathy |
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Term
| Nonproliferative (NPDR) diabetic retinopathy |
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Definition
– Earlier
– Usually asymptomatic |
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Term
| Proliferative (PDR) diabetic retinopathy |
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Definition
– Later
– Can cause sudden loss of vision |
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Term
| Early NPDR Ophthalmoscopic Signs |
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Definition
• Increased vascular permeability
• Microaneurysms
• Intraretinal hemorrhages
• Hard exudates |
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Term
|
Definition
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Term
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Definition
• High risk of imminent PDR
• No immediate treatment
• Patient needs re-evaluation in 2 to 4 months |
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Term
| Severe NPDR Ophthalmoscopic Signs |
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Definition
• Retinalischemia
• Microaneurysms
• Intraretinalhemorrhagesextensive • Hardexudates
• Cottonwoolspots
• Venousbeading
50% will develop PDR within 1 year |
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Term
| Diabetic Macular Edema Prevalence |
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Definition
In type II diabetics
• Diabetic Dx less than 5yrs = 5%
• Diabetic Dx more than 15yrs = 15% |
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Term
| PDR Ophthalmoscopic Signs |
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Definition
• All NPDR findings including macular edema • Ischemia induced neovascularization
• Vitreous hemorrhage
• Fibrous proliferation
• Retinal traction, tears, detachment • Blindness |
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Term
|
Definition
iris neovascularization
This is an image showing us blood vessels within the iris. This is known as iris neovascularization and can occur secondary to the ischemia that we described and proliferation of blood vessels to try and deal with the ischemic conditions in diabetes. Neovascularization of the iris is often accompanied by neovascularization of what we call the angle or the area where the cornea as well as the iris meet.
And this is the area where fluid from the eye flows out, maintaining normal eye pressures. If we have neovascularization of the angle as well as iris neovascularization, patients may be at risk for elevated intraocular pressures, which is known as neovascular glaucoma. Patients may lose vision, because of damage to the optic nerve in this case. |
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Term
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Definition
tractional retinal detachment
And this fibrous membrane may contract and pull on the retina, causing what is called a tractional retinal detachment. These blue arrows outline the tractional retinal detachment in this eye. And you can see all the neovascularization that's occurring adjacent to the tractional retinal detachment. |
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Term
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Definition
pdr vitreous hemorrhage
This is a picture of a vitreous hemorrhage. A vitreous hemorrhage can occur acutely, because of leakage from the neovascular vessels in the retina or around the optic nerve. These vessels are very fragile and can rupture at any time, causing an acute vitreous hemorrhage. The patient often describes an acute loss of vision and many floaters.
And this, in a patient with proliferative diabetic retinopathy, is usually a symptom of acute vitreous hemorrhage. Vicious hemorrhages are usually monitored unless they are not clearing. And if so, a retina surgeon may operate on the eye to remove the vitreous hemorrhages. |
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Term
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Definition
• Related to
– Duration of diabetes
– Patient's age at time of Dx |
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Term
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Definition
Laser surgery most benefits patients with NVD or NVE with Vitreous hemorrhage.
It is also helpful in diabetic macular edema
Interventions include intra-vitreal injections Anti –VEGF antibodies
- pegaptanib, ranibizumab, bevacizumab, aflibercept Steroids
- triamconolone, dexamethasone, fluocinolone |
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Term
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Definition
laser photocoagulation
This picture shows us scars from the laser photocoagulation that I mentioned.
You can see that there are very well-circumscribed round scars in the periphery of the retina and these help, as I mentioned, decrease the load of oxygen demand. Intra-vitreal injections are performed in the office with a very short needle. And whichever agent, bevacizumab or aflibercept that's injected is injected about 3 millimeters to 4 millimeters behind the limbus of the eye. And these injections are usually administered on a monthly basis and, in some instances, every three to four months, depending on the treatment that the patient requires. |
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Term
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Definition
Intravitreal Injection
It is very common for patients to have combination therapy as well. So, for example, they may have laser as well as injections and steroid injections, depending on the progression of their disease. The best treatment is prevention of diabetic retinopathy. And this involves a very strict glucose control or aiming for a hemoglobin A1C less than 7.0. |
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Term
|
Definition
• Affects over 73 million people in the United States
• It is the most common retinal vascular disease |
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Term
|
Definition
• Manifestation of end organ disease
• Bilateral
• Symmetric process |
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Term
| Hypertensive retinopathy grades |
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Definition
0
No findings
1
Arteriolar thickening
2
Focal arteriolar constriction, venous
3
Hemorrhages, cotton wool spots, exudates
4
Optic nerve swelling |
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Term
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Definition
av nicking seen in hypertensive retinopathy
In this picture, we see that as the artery crosses over the vein, there's a little bit of space here, and this is what we refer to as arteriovenous nicking, or AV nicking. |
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Term
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Definition
hypertensive retinopathy
In this picture here, here are all the cotton wool spots that we mentioned in one of the grades of hypertension. There's some exudates here as well. You can go back to your chart and guess or try and work out what grade this type of hypertensive retinopathy is based on the findings of cotton wool spots, exudate, and arteriovenous changes. |
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Term
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Definition
• Autosomal recessive disorder
• In the USA the incidence of sickle cell trait (AS) is about 8%, while that of SCD is 0.4% |
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Term
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Definition
| • SS has the most severe systemic disease • Most retinopathy is in SC trait |
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Term
|
Definition
sickle cell retinopathy
The pathophysiology of sickle cell retinopathy actually turns out to be similar in a way to some of the retinopathy we've seen in diabetes. Sickle cell retinopathy occurs because of ischemia. So as you recall from our lecture on diabetes, you can see all these new blood vessels that are growing here-- so proliferation of blood vessels. And we use a dye called fluorescein dye and injected it in this patient's vein, and took pictures of the retina so we could highlight these areas of vascular proliferation.
We can also appreciate that the blood vessels come along the retina and end abruptly. So in sickle cell disease, there's infarct of the retina and attenuation of the vessels, as well as hyperperfusion in the peripheral retina. And this again, just like in diabetes, drives up VEGF levels and results in proliferation.
Just like in diabetes, these areas of neovascularization can bleed. So these patients can also present with acute loss of vision secondary to vitreous hemorrhage. Any patient you have with sickle cell disease or the SC trait should be seen by an ophthalmologist on a regular basis to monitor for these possible changes. |
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Term
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Definition
Neurofibromatis 1
This is an autosomal dominant condition. And the defect is found on chromosome 17.
If you look at this child here, you can tell that the right eyelid is lower than the left eyelid, or ptotic, as we call it. And you can tell that it's a little fuller than the left eyelid. I'll get my pointer set up here. I also want to point out that this eyelid is a little bit S-shaped.
So the condition that causes the ptotic eyelid to have this little fullness as well as the S-shape is called a plexiform neurofibroma. And this is found in neurofibromatosis type 1. It is very important to monitor patients with neurofibromatosis type 1 and give them thorough eye exams, because in young children this ptotic eyelid may cover their visual access or their pupil and cause amblyopia and cause a permanent decrease in vision in that particular eye.
Even if the eyelid is not covering the visual axis, the pressure from the eyelid or the tumor may cause astigmatism in that eye causing a refractive type of amblyopia and having the same consequence of permanent vision loss secondary to amblyopia. So it is important, once again, to examine these patients thoroughly and on a regular basis as these fibromas can develop at any time. |
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Term
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Definition
Lisch Nodules
This picture here shows us Lisch nodules. So Lisch noddles are hamartoma that occur on the iris. These are little nodules. They do not affect vision and do not affect the eye. However, when you examine an individual with neurofibromatosis type 1, you might see these nodules. In some instances, an opthalmic examination and observation of these nodules may assist in the diagnosis of neurofibromatosis type 1.
These nodules do not appear before the age of two. And they do increase throughout childhood. Therefore, if you suspect neurofibromatosis in a child under the age of two, it will not be beneficial to look for Lisch nodules. However, in children older than the age of two, it may be helpful to look for Lisch nodules. |
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Term
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Definition
• Optic nerve gliomas are low-grade pilocytic astrocytomas
• occur in up to 15% of patients with NF • Can be bilateral and involve chiasm
[image] |
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Term
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Definition
• Autosomal dominant condition
• Caused by abnormal NF2 gene which is located on chromosome 22
• It is diagnosed clinically with findings such as bilateral acoustic neuromas, meningioma, glioma or early-onset posterior subcapsular cataract
• NF-2 patients usually present in their teens or early adulthood with symptoms related to acoustic neuromas (tinnitus, decreased hearing)
• The ophthalmologist can be helpful in the diagnosis of NF 2 in detecting cataracts early |
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Term
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Definition
neurofibromatosis type 2
And this is a picture of a posterior subcapsular cataract, which can be found in neurofibromatosis type 2. So these cataracts are on the posterior aspect of the lens. The most common visual complaints with a posterior subcapsular cataract are glare in bright light conditions. So fluorescent lighting or outdoors, these patients tend to have a decrease in their vision because these cataracts scatter the light and diminish their vision. Treatment is surgical if they are visually disturbing and inhibit activity, such as night driving. |
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Term
|
Definition
• 1:6000-10000
• Occurs as a result of mutations in the TSC1 and TSC 2 tumor suppressor genes found on chromosome 9 and 16 respectively
• Autosomal dominant, most often sporadic
• The Vogt triad of seizures, cognitive impairment and facial angiofibromas occurs in about 30% of patients with tuberous sclerosis
• Characterized by benign tumor growth in multiple organs such as the skin, brain, heart and eye
• A life threatening cardiac manifestation is a rhabdomyoma
• The most frequent ocular manifestation is a retinal phakoma also known as retinal astrocytic harmatoma. |
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Term
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Definition
| The next series of pictures shows you multiple manifestations of tuberous sclerosis, including the facial angiomas. And sometimes if you see these in young individuals, you may mistake it for acne. They can also have periangle lesions as well as lesions on their guns. And we see these hypo pigmented lesions. And the ocular manifestation is the astrocytic harmatoma, which is this elevated light lesion. |
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Term
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Definition
Astrocytic Harmatoma
This one happens to be on the optic nerve. A lot of them can be seen in the periphery. They are usually solitary lesions. However, patients may present with several astrocytic harmatomas of the retina. Fortunately, they are not vision threatening. And we observe them and they do not require any treatment. |
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Term
|
Definition
• The incidence is approximately 1:36 000 births
• Autosomal dominant condition
• caused by a mutation in the VHL gene located on chromosome 3
• It causes vascular tumors in the retina and CNS (usually cerebellum). Cysts and tumors may also occur in the kidneys (RCC), pancreas, liver and adrenal glands (pheochromocytoma)
• The retinal lesions are called retinal capillary hemangiomas
• Usually visible around 25 years of age
• The tumors have a pair of dilated vessels running
between the optic nerve and the lesion
• These represent arterio-venous communication |
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Term
|
Definition
retianl capillary hemangioma
So as I mentioned, they run from the optic nerve. And you can see that the arterio-venous anastomoses occurs here. And these vessels are very dilated. Here's another example of a retinal capillary hemangioma.
If they're in the periphery, they don't tend to cause any vision threatening situation. However, this one has become so leaky, that there are exudates in the macula. So this patient will have visual compromise.
And treatment of these is not standardized. However, people have used laser to try and cut off the supply to the hemangioma. Some people are using anti-VEGF injections, as well. The same injections that we talked about in the diabetic retinopathy lecture. |
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Term
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Definition
• Mutations in the fibrillin gene located on chromosome 15
• Patients are tall, have long limbs and fingers and have chest abnormalities
• Cardiovascular abnormalities are the main cause of mortality and morbidity. These include dilated aortic root, dilation of the ascending aorta and dissecting aneurysm
• Ocular abnormalities occur in up to 80% of individuals
• Lens dislocation (most common), myopia, and retinal detachment |
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Term
|
Definition
pectus excacatum, arachnodactyly, dilation of aorta
• Myopia
• Retinal Detachment
• Lens Subluxation |
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Term
|
Definition
Marfan’s - Lens Subluxation
And to summarize, the patients who have myopia, retinal detachments, and Len subluxation-- which you can see in this picture-- when the pupil is dilated, the lens is usually centered within the pupil. In this case, there is this empty space here. And you can see the edge of the lens. This is showing you that the lens has detached from its usual support structures called zonules and is actually elevated in this situation. The patient with a sublux lens will often present with a decrease in vision. And because the lens can be mobile, the vision can fluctuate quite significantly.
The treatment for this is cataract removal or lens removal and then implantation of an intraocular lens, either in the front of the eye, or the antechamber, or with using sutures on to the sclera.
If the patient have retinal detachments, a retina surgeon is very helpful in repairing those. So Marfan's patients require very close of ophthalmic surveillance because they do have very frequent ophthalmic complications and severe ones like retinal detachment. |
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Term
|
Definition
galactosemia
So galactosemia is one entity. And in this case, the clue that the patient might have an inborn metabolic error is a failure to thrive. These are patients that are diagnosed very young, so within the first few months of life, over failure to thrive. And ophthalmic exam can be helpful in determining the cause of the failure to thrive.
In patients with galactosemia, we see this cataract that you can see is centrally located in the lens. And we call it an oil droplet cataract, because of the appearance it has as if it is an oil droplet. These patients are given a galactose free diet to treat them. And it has been shown that with adequate treatment, the oil droplet cataract can completely regress. So ophthalmic exams can be helpful in monitoring the success of the therapy along, of course, with the patient's growth pattern and energy levels. |
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Term
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Definition
Cystinosis is also a metabolic error that can occur. In these patients, there's an accumulation of cystine because that amino acid is not able to be metabolized in the body. So these patients are placed on low diets, or low cystine diets, and can also use Cysteamine systemically to break down the cystine.
The ocular symptoms that patients with cystinosis experience are extreme light sensitivity and foreign body sensation and tearing. And this is because of cystine deposits throughout the entire cornea. And this gives them this specific discomfort.
Unfortunately, systemic Cysteamine does not penetrate the cornea, so we use topical Cysteamine. And this has been shown to be very effective. Cysteamine is not a very tasty medication or neither does it have a good smell, so most patients have difficulty with compliance. However, it has shown to be pretty effective and patients with cystinosis, with appropriate treatment, can live well into their 50s. |
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Term
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Definition
HIV/AIDS
In this section, we'll review ocular manifestations of HIV and AIDS, metastatic conditions, as well as uveitis. So this is the most common manifestation in the eye of HIV/AIDS. By now, you guys are probably experts at describing or noticing cotton wool spots. These are infarcts in the nerve fiber layer, and they're creamy lesions, small, white, or pale cream lesions. And you can see them running along the vessels or the vascular arcades. HIV retinopathy often is just cotton wool spots and maybe a few hemorrhages here and there. This is asymptomatic. |
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Term
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Definition
HIV/AIDS patients may also have periocular lesions. You can see these umbilicated little lesions along the eyelid margin. If you're doing your physical exam on a patient and you notice these, you should probably get an HIV test, because molluscum is not common, and this is what this is. It's called molluscum contagiosum. It's caused by a pox virus. And again, these umbilicated little lesions are pathognomonic usually for molluscum contagiosum. You can treat these with cautery or resection. However, you want to be careful around the eyelid not to cause scarring.
On this image, you see this red, beefy lesion with blood vessels emanating from it. This lesion is caused by Kaposi's sarcoma. So HH8 causes, the Human Herpes Virus Number Eight. And sometimes, it is confused with pterygium, or chronic conjuncitivitis. In patients with HIV/AIDS with a non-resolving reddish conjunctiva or eyelid lesion, you should suspect Kaposi's sarcoma, and you can send these patients to an ophthalmologist for resection of this tumor. |
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Term
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Definition
Patients with AIDS or with CD4 of count less than 50 can have retinal infections. CMV retinitis is the most common retinal infection at these very high levels of immunocompromised, or levels of very low CD4 counts. In this picture, you can see that the retina is damaged. There's a lot of hemorrhage and there's a lot of whitening, here, of the retina. This is necrosis of the retina. And this is CMV retinitis.
CMV retinitis can also appear as a frosted branch angiitis, which means that it's affecting the blood vessels. So you can see that along these blood vessels, there's inflammation, or an angiitis. And this is also one presentation of CMV. Again, these patients are usually severely immunocompromised, so you need to work them up for other opportunistic infections in addition to he CMV retinitis. Therapy for CMV retinitis is systemic, and also, we can treat them with intravitreal injections or local injections if systemic therapy is causing them significant side effects. |
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Term
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Definition
• The most common intraocular cancers of the eye are metastatic
• Spread hematogenously to the eye, mainly to the choroid due to its rich blood supply
• The most common cancers to metastasize to the eye are lung and breast |
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Term
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Definition
lung cancer metastases
This is a picture demonstrating multiple lesions within the retina. Because this lesion is within the macula area, the patient did have visual complaints, and we can see these other lesions throughout the retina. Because these are metastases, the patient needs systemic therapy. We don't often treat metastases in the eye with a local therapy, however, in rare cases, or on a case-by-case basis, if the metastases are close to the central vision, or close to the macula, we may initiate local therapy. But because these are metastases, these patients require systemic therapy. |
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Term
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Definition
Occasionally, lung cancer is first diagnosed in an ophthalmologist's office because of findings, such as the ones in this picture. Here's another example of a lung cancer metastasis. It looks a little bit different from the one I showed you previously.
This is a solitary lesion, again, because it's close to the macula of this patient, like we did have visual symptoms. This one also has overlying pigment changes here. In this case, the patient may be offered local therapy to try and improve their vision, or cheat the visual symptoms. But overall, they do need systemic therapy to control the cancer. |
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Term
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Definition
beast cancer metastasis
This is an example of a solitary metastasis from breast cancer. You can, see, again it's a white lesion, and it's underneath the retina. You can tell because you can see the vessels very clearly. And as we mentioned, these are usually choroidal metastases. So take home message is-- cancer can metastasize to the eye, the two most common are lung and breast cancer. Many others can, such as leukemia and they're much more rare, but you can see them in the eye, as well. |
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Term
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Definition
• Inflammation of the uvea; iris, ciliary body, choroid
• Pain
• Redness
• Light sensitivity (photophobia) • Floaters
• Decreased Vision |
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Term
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Definition
On exam, you might notice injection of the eye. And on the cornea, you can notice some deposits of inflammatory cells on the inner part of the cornea. Occasionally, there's so much inflammation they develop a hypopyon, which you can see in your lectures about endophthalmitis, or corneal ulcer.
And then in this case, there's been so much inflammation that the pupil margin is irregular because the iris is stuck to the lens, and this is called synechia. So on your exam, when you do a pupillary exam, you'll notice that the patient's pupil is irregular, or does not constrict uniformly, or at all, depending on how severe the synechiae are. |
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Term
| Systemic Associations w ueitis |
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Definition
• Autoimmune Conditions
• Children
– Juvenille Idiopathic Arthritis
• Adults
– HLA-B27
– Sarcoidosis
– Lupus
• Infections – Syphilis
– Tuberculosis
– Herpes Zoster – Herpes Simplex |
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