Q Angioid Streaks
What is the classic DFE appearance of angioid streaks?
1 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
2 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Why are they called ‘angioid’ streaks?
3 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Why are they called ‘angioid’ streaks? Because the streaks resemble blood vessels
4 Angioid Streaks
Angioid streaks (arrowheads). Note that only a few of the many present have been marked. Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally?
6 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally? Bilaterally
7 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally? Bilaterally
With what symptom(s) does/do angioid streaks present?
8 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally? Bilaterally
With what symptom(s) does/do angioid streaks present? Nothing—it is asymptomatic (until/unless the fovea is involved)
9 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally? Bilaterally
With what symptom(s) does/do angioid streaks present? Nothing—it is asymptomatic (until/unless the fovea is involved)
Can angioid streaks occur congenitally?
10 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region bilaterally? unilaterally?
Does angioid streaks present unilaterally, or bilaterally? Bilaterally
With what symptom(s) does/do angioid streaks present? Nothing—it is asymptomatic (until/unless the fovea is involved)
Can angioid streaks occur congenitally? Apparently not—there has never been a reported case of their presence in the fundus of an infant
11 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal?
12 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
13 Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
Let’s briefly review the anatomy of Bruch’s membrane…
14 Q Angioid Streaks
What are the five layers of Bruch’s membrane?
1) (Start here) Innermost 2) 3) 4)
Bruch’s membrane Bruch’s 5) Outermost
15 Q/A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basementtwo wordsmembrane of RPE Innermost 2) 3) 4)
Bruch’s membrane Bruch’s 5) Outermost
16 A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) (Next) 3) 4)
Bruch’s membrane Bruch’s 5) Outermost
17 Q/A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenousone word layer 3) 4)
Bruch’s membrane Bruch’s 5) Outermost
18 A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) (Next) 4)
Bruch’s membrane Bruch’s 5) Outermost
19 Q/A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elasticdiff one word layer 4)
Bruch’s membrane Bruch’s 5) Outermost
20 A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer
4) (Next)
Bruch’s membrane Bruch’s 5) Outermost
21 Q/A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenousone familiar word layer
Bruch’s membrane Bruch’s 5) Outermost
22 A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) (Next) Outermost
23 Q/A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer Bruch’s membrane Bruch’s 5) Basementtwo familiar membrane words of choriocapillaris Outermost
24 A Angioid Streaks
What are the five layers of Bruch’s membrane?
1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
25 Q Angioid Streaks
What are the five layers of Bruch’s membrane? 0) ? What (non-Bruch’s) structure goes here? The RPE cells themselves 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
26 A Angioid Streaks
What are the five layers of Bruch’s membrane? 0) RPE cells What (non-Bruch’s) structure goes here? The RPE cells themselves 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
27 Q Angioid Streaks
What (non-RPE) structures go here? What-1) ? are the five layers of Bruch’sThe photoreceptor membrane? outer segments 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
28 A Angioid Streaks
What (non-RPE) structures go here? What-1) PR are outerthe five segs layers of Bruch’sThe photoreceptor membrane? outer segments 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
29 Q Angioid Streaks
What cell type is this? -2) ? Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
30 A Angioid Streaks
What cell type is this? -2) Bipolar cells Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost
31 Q Angioid Streaks
-2) Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost What structure is this? -6) Chorio ? The choriocapillaris
32 A Angioid Streaks
-2) Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost What structure is this? -6) Choriocapillaris The choriocapillaris
33 Q Angioid Streaks
-2) Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost -6) Choriocapillaris What structure is this? -7) Choro ? The choroid 34 A Angioid Streaks
-2) Bipolar cells
What-1) PR are outerthe five segs layers of Bruch’s membrane? 0) RPE cells RPE cells 1) Basement membrane of RPE Innermost 2) Inner collagenous layer 3) Elastic layer 4) Outer collagenous layer
Bruch’s membrane Bruch’s 5) Basement membrane of choriocapillaris Outermost -6) Choriocapillaris What structure is this? -7) Choroid The choroid 35 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? -- --
36 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…[thickened vs thinned] --It is…[common pathologic process]
37 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
38 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
39 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
40 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane
41 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
42 QA Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
To be clear: Does this refer to hemorrhage not related to a CNVM?
43 QA Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
To be clear: Does this refer to hemorrhage not related to a CNVM? Yes 44 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Indeed it is—estimates of CNVM prevalence in angioid streaks run as high as 80% 45 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Indeed it is—estimates of CNVM prevalence in angioid streaks run as high as 80%% 46 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Indeed it is—estimates of CNVM prevalence in angioid streaks run as high as 80% 47 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 48 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane
What’s abnormal about Bruch’s in angioid streaks? --It is…thickened --It is…calcified (and thus brittle)
How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 49 Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane Does the brittleness of Bruch’s… predispose these eyes to traumatic choroidal rupture with subsequent CNVM What’s abnormal about Bruch’s in angioid streaks? development? --It is…thickened Indeed it does --It is…calcified (and thus brittle) Are there steps the managing ophthalmologist should take in this regard? How are angioid streaks related to Bruch’s? Encourage the use of safety glasses, and Angioid streaks represent breaks in the brittle Bruch’s the avoidance of risky activities What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 50 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane Does the brittleness of Bruch’s… predispose these eyes to traumatic choroidal rupture with subsequent CNVM What’s abnormal about Bruch’s in angioid streaks? development? --It is…thickened Indeed it does --It is…calcified (and thus brittle) Are there steps the managing ophthalmologist should take in this regard? How are angioid streaks related to Bruch’s? Encourage the use of safety glasses, and Angioid streaks represent breaks in the brittle Bruch’s the avoidance of risky activities What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 51 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane Does the brittleness of Bruch’s… predispose these eyes to traumatic choroidal rupture with subsequent CNVM What’s abnormal about Bruch’s in angioid streaks? development? --It is…thickened Indeed it does --It is…calcified (and thus brittle) Are there steps the managing ophthalmologist should take in this regard? How are angioid streaks related to Bruch’s? Encourage the use of safety glasses, and Angioid streaks represent breaks in the brittle Bruch’s the avoidance of risky activities What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 52 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane Does the brittleness of Bruch’s… predispose these eyes to traumatic choroidal rupture with subsequent CNVM What’s abnormal about Bruch’s in angioid streaks? development? --It is…thickened Indeed it does --It is…calcified (and thus brittle) Are there steps the managing ophthalmologist should take in this regard? How are angioid streaks related to Bruch’s? Encourage the use of safety glasses, and Angioid streaks represent breaks in the brittle Bruch’s the avoidance of risky activities What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 53 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane Does the brittleness of Bruch’s… predispose these eyes to traumatic choroidal rupture with subsequent CNVM What’s abnormal about Bruch’s in angioid streaks? development? --It is…thickened Indeed it does --It is…calcified (and thus brittle) Are there steps the managing ophthalmologist should take in this regard? How are angioid streaks related to Bruch’s? Encourage the use of safety glasses, and Angioid streaks represent breaks in the brittle Bruch’s the avoidance of risky activities What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 54 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 55 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluorescehypo- v hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to stainingmechanism --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 56 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 57 Angioid Streaks
Early Late
FA Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluorescehypo- v hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 59 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 60 Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from the (atrophic)? peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 61 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 62 Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown ? Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 63 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layer of the retina is abnormal? Bruch’s membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 64 Angioid Streaks
Angioid streaks: Lacquer cracks: ‘Reddish-brown’ ‘Yellowish’ Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’s? membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 66 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 67 Angioid Streaks
Angioid streaks: Lacquer cracks: ‘Reddish-brown’ ‘Yellowish’ ‘Peripapillary’ ‘Macular’ Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes ? What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 69 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 70 Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? Locus for CNV? Yes ? The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 71 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? Locus for CNV? Yes Yes The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to staining --It is…thickened --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 72 Q Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? Locus for CNV? Yes Yes The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to stainingAssociated with… We’ll-- Itsee is…thickened shortly ? --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 73 A Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? Locus for CNV? Yes Yes The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to stainingAssociated with… We’ll-- Itsee is…thickened shortly High/pathologic myopia --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 74 Angioid Streaks
Angioid streaks: Lacquer cracks: ‘Reddish-brown’ ‘Yellowish’ ‘Peripapillary’ ‘Macular’ ‘High myopia’ (note the optic disc) Angioid Streaks There is another Bruch’s-based condition that, similar to angioid streaks, Whatpresents is the with classic a linear DFEfinding onappearance DFE. What is that of condition? angioid streaks? Reddish-brown lines radiatingAngioid streaks out from Lacquerthe (atrophic) cracks peripapillary Colorregion Reddish-brown Yellowish Which layerLocation of the retina isPeripapillary abnormal? Bruch’sMacula membrane Subretinal heme? Yes Yes What is the typical appearance of angioid streaks on FA? Locus for CNV? Yes Yes The streaks hyperfluoresce early due to a What’swindow abnormal defect, about Bruch’s in angioid streaks? and late due to stainingAssociated with… We’ll-- Itsee is…thickened shortly High/pathologic myopia --It is…calcified (and thus brittle) Of course, if a CNVM develops, it will hyperfluoresce during the choroidal phase How are angioid streaks related to Bruch’s? Angioid streaks represent breaks in the brittle Bruch’s
What serious complications can result from the breaks in Bruch’s? Choroidal neovascular membrane and subretinal hemorrhage
Is CNVM a common occurrence in angioid streaks? Does CNVM in angioid streaks carry a good, Indeed it is—estimates of CNVM prevalence in or poor prognosis? angioid streaks run as high as 80% Poor 76 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities?
77 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half
78 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? P seudoxanthoma elasticum (PXE) E hlers-Danlos syndrome P aget’s disease of bone S ickle-cell disease diopathic (I know, it’s not a systemic association) I 79 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? P E P S I 80 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? P E P S I 81 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE) Ehlers-Danlos syndrome Paget’s disease of bone Sickle-cell disease diopathic (I know, it’s not really an association) I 82 Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE) Ehlers-Danlos syndrome Paget’s disease of bone Sickle-cell disease Mnemonic caveat #1: A number of hemoglobinopathies (eg, β-thal) diopathic I besides sickle-cell dz are associated with 83 angioid streaks Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE) Ehlers-Danlos syndrome Mnemonic caveat #2: The list of conditions that have been Paget’s disease of bone associated with angioid streaks is much longer than the PEPSI mnemonic suggests. ickle-cell disease S Caveat emptor. diopathic I 84 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)?
Ehlers-Danlos syndrome? Of these, which has the strongest association with angioid streaks? Paget’s disease of bone? Idiopathic Sickle-cell disease? What proportion of cases are idiopathic? diopathic? About half I 85 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)
Ehlers-Danlos syndrome Of these, which has the strongest association with angioid streaks? Paget’s disease of bone Idiopathic Sickle-cell disease What proportion of cases are idiopathic? diopathic! About half I 86 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)
Ehlers-Danlos syndrome Of these, which has the strongest association with angioid streaks? Paget’s disease of bone Idiopathic Sickle-cell disease What proportion of cases are idiopathic? diopathic! About half I 87 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)
Ehlers-Danlos syndrome Of these, which has the strongest association with angioid streaks? Paget’s disease of bone Idiopathic Sickle-cell disease What proportion of cases are idiopathic? diopathic! About half (as implied above) I 88 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)
Ehlers-Danlos syndrome Of these, which has the strongest association with angioid streaks? aget’s disease of bone P Idiopathic Sickle-cell disease What should you do Whatif you seeproportion an angioid of- streakcases pt arewho idiopathic? does diopathic! not carry the dx of a Aboutknown associatedhalf systemic condition? I Refer her for a workup, to make certain 89 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)
Ehlers-Danlos syndrome Of these, which has the strongest association with angioid streaks? aget’s disease of bone P Idiopathic Sickle-cell disease What should you do Whatif you seeproportion an angioid of- streakcases pt arewho idiopathic? does diopathic! not carry the dx of a Aboutknown associatedhalf systemic condition? I Refer her for a workup, just to make certain 90 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)?
Ehlers-Danlos syndrome? Of the cases associated with a condition, which condition is most likely? aget’s disease of bone? P PXE, by a mile Sickle-cell disease? diopathic I 91 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 92 Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ associations? What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P Paget’sPXE, and bysickle a mile-cell are a distant Sickle-cell disease second and third diopathic I 93 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ Of those angioid-streaks pts with a systemic association, associations? Whatwhat are proportion the have associations? PXE? Well over half Pseudoxanthoma elasticum (PXE)! Of pts with PXE, what proportion have angioid streaks? Ehlers-Danlos syndromeEssentially all of them,Of the eventually cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 94 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ Of those angioid-streaks pts with a systemic association, associations? Whatwhat are proportion the have associations? PXE? Well over half Pseudoxanthoma elasticum (PXE)! Of pts with PXE, what proportion have angioid streaks? Ehlers-Danlos syndromeEssentially all of them,Of the eventually cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 95 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ Of those angioid-streaks pts with a systemic association, associations? Whatwhat are proportion the have associations? PXE? Well over half Pseudoxanthoma elasticum (PXE)! Of pts with PXE, what proportion have angioid streaks? Ehlers-Danlos syndromeEssentially all of them,Of the eventually cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 96 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonic for angioid streaks’ Of those angioid-streaks pts with a systemic association, associations? Whatwhat are proportion the have associations? PXE? Well over half Pseudoxanthoma elasticum (PXE)! Of pts with PXE, what proportion have angioid streaks? Ehlers-Danlos syndromeEssentially all of them,Of the eventually cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 97 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 98 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 99 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 100 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 101 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 102 Q/A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer MvF♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 103 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 104 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic-- abnormalities? About half -- What is-- the well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 105 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is--GI the tract well-known mnemonic for angioid streaks’ associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 106 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye --The neck associations?-- TheWhat axillae are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 107 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye --The neck associations?-- TheWhat axillae are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 108 Angioid Streaks
PXE skin Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye --The neck associations?-- TheWhat axillae are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 110 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye --The neck associations?-- TheWhat axillae are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 111 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye -- associations?-- What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 112 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are ofWhat twice the is the asretina appearancelikely to is be of abnormal?affected affected skin? Bruch’s membrane An area of waxy-yellow, papule-like lesions What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with What is the classic informal descriptor for this appearance? systemic--Skin abnormalities?‘Chicken skin‘ About half --Vascular system What is--GI the tract wellIn what-known two locations mnemonic is ‘chicken skin’ formost angioidoften found? streaks’ --Eye --The neck associations?-- TheWhat axillae are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 113 Angioid Streaks
PXE: ‘Chicken skin’ Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities?How doesAbout PXE affecthalf the vascular system? --Vascular system In a number of ways; the two mentioned in the Retina book What is--GI the tract well-knownare mnemoniccalcific coronary artery for dz angioid, and an increasedstreaks’ risk of CVA associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 115 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities?How doesAbout PXE affecthalf the vascular system? --Vascular system In a number of ways; the two mentioned in the Retina book What is--GI the tract well-knownare mnemoniccalcific coronary artery for dz angioid, and an increasedstreaks’ risk of CVA associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 116 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is the classic GI manifestation of PXE? What is--GI the tract wellHemorrhage,-known which mnemonic is common, and for life -angioidthreatening instreaks’ severity associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 117 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system What is the classic GI manifestation of PXE? What is--GI the tract wellHemorrhage,-known which mnemonic is common, and for life -angioidthreatening instreaks’ severity associations?--Eye What are the associations? Pseudoxanthoma elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 118 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are the associations? -- Pseudoxanthoma-- elasticum (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 119 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are the associations? --RPE mottling Pseudoxanthoma--Optic disc elasticum drusen (PXE)!
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 120 Q Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are Whatthe mellifluousassociations? name is used to describe --RPE mottling the RPE mottling? Pseudoxanthoma--Optic disc elasticum drusen Peau (PXE)!d’orange
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 121 A Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are Whatthe mellifluousassociations? name is used to describe --RPE mottling the RPE mottling? Pseudoxanthoma--Optic disc elasticum drusen Peau (PXE)!d’orange
Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 122 QA Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are Whatthe mellifluousassociations? name is used to describe -- the RPE mottling? RPE mottling What does peau d’orange mean in English? Peau d’orange Pseudoxanthoma--Optic disc elasticum drusen (PXE)! ‘Orange peel’--refers to a fine stippling like that found on the skin of an orange Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 123 QA Briefly, what sort of disorderAngioid is PXE?Streaks An elastorrhexis, ie, a condition characterized by progressive calcification and fragmentation of elastic tissues What is the classic DFE appearance of angioid streaks? ReddishIs it- common,brown orlines rare? radiating out from the (atrophic) Rare peripapillary region Is there a gender predilection? WhichYes, layer ♀ are of twice the asretina likely to is be abnormal?affected Bruch’s membrane What proportionOther than the eye, of angioidwhat organ-streakssystems are are affected? associated with systemic--Skin abnormalities? About half --Vascular system There are three classic eye findings in PXE, one of What is--GI the tract wellwhich -isknown angioid streaks. mnemonic What are the forother angioidtwo? streaks’ associations?--Eye --Angioid Whatstreaks are Whatthe mellifluousassociations? name is used to describe -- the RPE mottling? RPE mottling What does peau d’orange mean in English? Peau d’orange Pseudoxanthoma--Optic disc elasticum drusen (PXE)! ‘Orange peel’--refers to a fine stippling like that found on the skin of an orange Ehlers-Danlos syndrome Of the cases associated with a condition, which condition is most likely? aget’s disease of bone P PXE, by a mile Sickle-cell disease diopathic I 124 Angioid Streaks
PXE: Peau d’orange fundus Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder associations? What are theis Ehlersassociations?-Danlos? It is a connectivetwo-words-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 126 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 127 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome -- -- Paget’s disease of bone --
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 128 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruisingtwo related/bleeding words
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 129 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 130 Angioid Streaks
Ehlers-Danlos syndrome Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 132 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 133 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layer of the retina is abnormal? Bruch’s membrane What proportion of angioid streaks are associated with systemic abnormalities? About half What is the well-known mnemonicIn two, hyphenated for angioid words, whatstreaks’ sort of disorder is Ehlers-Danlos? associations? What are theIt isassociations? a connective-tissue disorder Pseudoxanthoma elasticum (PXE)! Briefly, what are its main nonocular findings? Ehlers-Danlos syndrome --Fragile skin -- Hyperextensible joints Paget’s disease of bone --Easy bruising/bleeding
Sickle-cell disease What colorful eye finding is associated with Ehlers-Danlos? diopathic I Blue sclera 134 Angioid Streaks
Blue sclera in Ehlers-Danlos Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 136 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 137 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 138 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 139 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 140 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 141 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 142 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only 10% will develop angioid streaks diopathic I 143 Q/A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only ~10%% will develop angioid streaks diopathic I 144 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region
Which layer of the retina is Isabnormal? Paget’s a common, Bruch’s or uncommon membrane condition? Quite common; it is the most common bone What proportion of angioiddiseasestreaks after are osteoporosis associated with systemic abnormalities? About half Briefly, what is the pathophysiology? What is the well-known mnemonicIncreased osteoclastic for angioid and osteoblasticstreaks’ activity associations? What are thelead associations? to focal abnormal bone structure Pseudoxanthoma elasticum (PXE)!How does it present clinically? It is usually asymptomatic, but can present with Ehlers-Danlos syndrome skeletal deformities pain Paget’s disease of bone As noted previously, essentially all PXE pts develop angioid streaks. Is the same true of Sickle-cell disease Paget’s pts? No--only ~10% will develop angioid streaks diopathic I 145 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half --SS What is-- theSC well-known mnemonic for angioid streaks’ associations?--S-Thal What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 146 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half --SS What is-- theSC well-known mnemonic for angioid streaks’ associations?--S-Thal What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 147 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half -- What is-- the well-known mnemonic for angioid streaks’ associations?-- What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 148 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half --SS What is-- theSC well-known mnemonic for angioid streaks’ associations?--S-Thal What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 149 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half --SS What is-- theSC well-known mnemonic for angioid streaks’ associations?--S-Thal What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 150 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half --SS What is-- theSC well-known mnemonic for angioid streaks’ associations?--S-Thal What are the associations? --SA Pseudoxanthoma elasticum (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 151 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemic abnormalities? About half What are theWhat four is common the key difference genotypes? between SS, SC and S-Thal vs SA disease? -- What is theSS wellThe-known first three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC asymptomatic (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 152 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemic abnormalities? About half What are theWhat four is common the key difference genotypes? between SS, SC and S-Thal vs SA disease? -- What is theSS wellThe-known first three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC asymptomatic (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 153 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemic abnormalities? About half What are theWhat four is common the key difference genotypes? between SS, SC and S-Thal vs SA disease? -- What is theSS wellThe-known first three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC asymptomatic (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 154 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemic abnormalities? About half What are theWhat four is common the key difference genotypes? between SS, SC and S-Thal vs SA disease? -- What is theSS wellThe-known first three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC asymptomatic (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 155 Q Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 156 A Angioid Streaks
What is the classic DFE appearance of angioid streaks? Reddish-brown lines radiating out from the (atrophic) peripapillary region Which layerBroadly ofspeaking, the retina what sort is of abnormal? disease is sickle Bruch’s-cell anemia? membrane What proportionA hemoglobinopathy of angioid streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 157 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 158 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 159 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 160 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 161 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 162 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR), DFE and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) Nonproliferative and proliferative—just like diabetic retinopathy? peripapillaryIndeed region WhichLike layer BroadlyDBR, does ofspeaking, thesickle retina retinopathywhat sort is of abnormal?tend disease to occur is sickle in theBruch’s-cell posterior anemia? membranepole? No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 163 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 164 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 165 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 166 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 167 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 168 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) In a veryNonproliferative few words, whatand is the proliferative pathogenesis— justof NPSR? like diabetic retinopathy? peripapillaryCapillaryIndeed and/or arteriolar region occlusion WhichNPSRLike manifests layer BroadlyDBR, as does ofthreespeaking, thesickle lesions. retina retinopathywhat What sort are is ofthey? abnormal?tend disease What to occur is theirsickle in appearance? theBruch’s-cell posterior anemia? membranepole? --Salmon patches: Retinal hemorrhage trapped under the internal limiting membrane No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What--Refractile(usually proportion spots anterior: Iridescent to of the retinal angioid equator) areas streaks are associated with --Sunburst lesions: Hyperpigmented patches, usually somewhat stellate systemicWh abnormalities?at are the four common About genotypes? half WhatWhat is isthe the incidence key difference of significant between retinopathy SS, SC and in: S-Thal vs SA disease? --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 169 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 170 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 171 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 172 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 173 Q Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 174 A Angioid Streaks Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? ‘Sea fans’ No—Aunlike hemoglobinopathy DBR, sickle retinopathy tends to occur well into the periphery What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? half No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinically for apparent angioid dz, whereasstreaks’ SA is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal What are the associations? --SA What percent of African-Americans test positive for sickle trait? Pseudoxanthoma8% elasticum (1 in 12) (PXE)! In America, people of which two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American Paget’s disease of bone Sickle-cell disease diopathic I 175 Angioid Streaks
Sickle-cell retinopathy comes in two basic forms—what are they? WhatNonproliferative is the classic(NPSR DFE), and proliferativeappearance (PSR) of angioid streaks? Reddish-brown lines radiating out from the (atrophic) NonproliferativeWhat is the pathogenesisand proliferative of PSR?—just like diabetic retinopathy? peripapillaryIndeedAs with NPSR, region it’s vascular occlusion, but severe enough to produce significant ischemia WhichLike layer BroadlyByDBR, what does appearanceofspeaking, thesickle retina -retinopathywhatbased sort name is of abnormal?tendare disease sickle to occur- cellis sickle neovascular in theBruch’s-cell posterior anemia? lesions membranepole? known? No—‘Seaunlike fans’ DBR, sickle retinopathy tends to occur well into the periphery A hemoglobinopathy DBR SR What(usually proportion anterior to of the angioid equator) streaks are associated with Do sea fans always require treatment? systemicWh abnormalities?at are the four common About genotypes? halfPosterior to the Anterior to the No, they mayWhat Whatspontaneously is isthe the incidence key regress, difference of significant and between laser retinopathy may SS, precipitate SC and in: S -AThal rhegmatogenousvs SA disease?RD --SS Location equator (usually in equator (ie, What is the well--SSThe-known dz first? 3% three mnemonicmanifest as clinicallythe for posteriorapparent angioid dzpole), whereasstreaks’ SAperipherally) is an --SC --SCasymptomatic dz? 33% (usually) carrier state--aka ‘sickle trait’ associations?--S-Thal WhatProliferative are the lesions associations? --SA What percentregress of African -Americans test Nopositive for sickle trait? Yes Pseudoxanthoma8% elasticum (1 spontaneously?in 12) (PXE)! In America, peopleProne of whichto developing two ethnic heritages are at greatest risk? EhlersAfrican-Danlos-American; syndrome Hispanic-American retinal tears when No Yes Paget’s disease of bone lasered? Sickle-cell disease Review slide—no questions diopathic I 176