PMC5015608_01
The composite of colour photograph of the left eye shows angioid streaks in a spiderweb configuration centered around the optic nerve head and extending far in all retinal sectors. Note also an atrophic lesion (comet tail) in the nasal periphery. A fibrotic subfoveal membrane is seen with a more recent extension temporal of the macula. Note also temporal of the macula, a vertical traumatic linear break of Bruch's membrane.
OC-01-03-g-001.jpg
PMC5015608_01_OC-01-03-g-001.jpg
F1
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The autofluorescence imaging shows in the left eye hypoautofluorescence of angioid streaks, of the traumatic break, and of the longstanding choroidal neovascular membrane. The hypoautofluorescence suggest irreversible damage to RPE and overlying retina. Hyperfluorescence is noted in the recent extension of the membrane and at the borders of the CNV. Hyperautofluorescence is indicative of diseased RPE and risk for expansion of the lesion.
OC-01-03-g-002.jpg
PMC5015608_01_OC-01-03-g-002.jpg
F2
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The fluorescein angiography of the left eye shows at 30 seconds hyperfluorescence of the angioid streaks, the traumatic break, and the neovascular membrane.
OC-01-03-g-003.jpg
PMC5015608_01_OC-01-03-g-003.jpg
F3
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The 10 minutes angiogram of the left eye shows staining of these lesions without leakage and is indicative of low grade activity of the CNV.
OC-01-03-g-004.jpg
PMC5015608_01_OC-01-03-g-004.jpg
F4
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
A horizontal section of the Spectralis OCT shows a subretinal neovascular scar and overlying retinal degeneration without obvious oedema or other signs of active CNV.
OC-01-03-g-005.jpg
PMC5015608_01_OC-01-03-g-005.jpg
F5
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The composite of colour photographs of the right eye shows PXE-associated lesions including angioid streaks centered around the optic nerve and extending in all sectors, peau d'orange temporal of the macula, and a maculopathy with pigment dispersion.
OC-01-03-g-006.jpg
PMC5015608_01_OC-01-03-g-006.jpg
F6
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The autofluorescence imaging shows a few hypoautofluorescent spots and numerous hyperautofluorescent lesions.
OC-01-03-g-007.jpg
PMC5015608_01_OC-01-03-g-007.jpg
F7
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The fluorescein angiography shows at 50 seconds hyperfluorescence of the angioid streaks and of the vertical traumatic rupture in the papillomacular area and macular pigment clumping.
OC-01-03-g-008.jpg
PMC5015608_01_OC-01-03-g-008.jpg
F8
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
The 10 minute angiogram shows staining of the angioid streaks, and combined staining and leakage in the macular lesion, suggestive for low grade activity of the CNV.
OC-01-03-g-009.jpg
PMC5015608_01_OC-01-03-g-009.jpg
F9
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).
A horizontal section of the spectralis OCT shows in the right eye a subretinal lesion with a large overlying cystic lesion.
OC-01-03-g-010.jpg
PMC5015608_01_OC-01-03-g-010.jpg
F10
At last examination, in April 2011, VA was 20/60 in the right eye and 20/250 in the left eye, with in the left eye a large subfoveal CNV expanding on both sides of the fovea, and in the RE a smaller and mildly active subfoveal CNV requiring further treatment (Figure 1 (Fig. 1), Figure 2 (Fig. 2), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), Figure 6 (Fig. 6), Figure 7 (Fig. 7), Figure 8 (Fig. 8), Figure 9 (Fig. 9), Figure 10 (Fig. 10)).