PMC5422748_01
a, b Colour fundus photography of Case 1 demonstrating bilateral angioid streaks (AS, green arrows), macular subretinal haemorrhage, and retinal pigment epithelial detachment (PED) (blue arrow). Case 1 at 3 years following the initiation of treatment. c Colour fundus photography demonstrates AS in the right eye (green arrows), with recurrence of a large subretinal haemorrhage, subretinal fluid (SRF), and PED. d OCT scan reveals subretinal haemorrhage, SRF, and PED. Fluorescein angiography (FA) demonstrates early filling of 2 grape-like polypoidal structures (e) with leakage in the late phase at the inferior region of the macula (f), corresponding to polypoidal choroidal vasculopathy (PCV) lesions at the edge of the AS (yellow arrows). Indocyanine green angiography (ICGA) also demonstrates early filling (g) and leakage in the late phase of the PCV lesions (yellow arrows) (h), as well as an associated branching vascular network (BVN) in both early and late phase. At 20 months after this visit, fundoscopy (i) and OCT (j) showed small SRF and PED with no subretinal haemorrhage; FA (k) and ICGA (m) in the early phase still revealed the PCV lesions and hyperfluorescence in the late phase in FA (l) and ICGA (yellow arrows) (n). The patient was then switched to intravitreal aflibercept, and at 27 months after the initial aflibercept injection, the OCT scan in p shows a small PED, and an absence of subretinal haemorrhage and SRF. Thin purple arrows in c, i, and o demonstrate the locations of the cross-sectional slides of the OCT images in d, j, and p, respectively.
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F1
Dilated fundus examination of both eyes showed dark brown irregularly radiating linear lines emanating from the peripapillary area including through the macula, corresponding to the characteristic clinical appearance of AS (Fig. 1e, f). Clinical examination revealed a large subretinal haemorrhage, subretinal fluid (SRF), and PED in the inferior macula (Fig. 1a, b). ICGA (Fig. 1e, f) demonstrated early filling of a grape-like polypoidal structure with leakage in the late phase suggestive of PCV secondary to AS. ICGA also revealed an associated BVN in both the early and late phase (Fig. 1e, f). OCT revealed an absence of SRF; however, a small but persisting PED (Fig. 1m). Over 84 months, a total number of 27 injections (19 ranibizumab and 8 aflibercept) were given in the right eye with an average of 90 days between injections (both types).
Red free fundus photos (a, b) of Case 2 (mother of Case 1) demonstrating bilateral angioid streaks (thick green arrows) and fibrotic disciform scars (purple triangles). Indocyanine green angiography (c, d) demonstrates polyp lesions at the edge of the disciform scars in the right eye (yellow arrow) and late geographic hypercyanescence in the left eye (red triangle) demonstrating the appearance of a branching vascular network, consistent with a diagnosis of advanced polypoidal choroidal vasculopathy.
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F2
FA and ICGA were performed and demonstrated bilateral PCV with polyp lesions and BVN (Fig. 2).
PMC5422748_02
Red free fundus photos (a, b) of Case 2 (mother of Case 1) demonstrating bilateral angioid streaks (thick green arrows) and fibrotic disciform scars (purple triangles). Indocyanine green angiography (c, d) demonstrates polyp lesions at the edge of the disciform scars in the right eye (yellow arrow) and late geographic hypercyanescence in the left eye (red triangle) demonstrating the appearance of a branching vascular network, consistent with a diagnosis of advanced polypoidal choroidal vasculopathy.
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F2
FA and ICGA were performed and demonstrated bilateral PCV with polyp lesions and BVN (Fig. 2).
PMC5422748_03
Case 3 at 3 years following treatment initiation. a Colour fundus photography demonstrates a discreet orange-red nodule in the nasal macula connecting to the angioid streaks (AS) in the right eye (green arrow; as described in Fig. 2A); b OCT reveals a PED with an underlying polypoidal lesion, with no subretinal or intraretinal fluid; fluorescein angiography (FA) in the early (e) and late phase (f) and ICGA in the early (g) and late phase (h) also reveal a grape-like polypoidal structure with leakage at late phase on the nasal aspect of the macula, corresponding to a PCV lesion secondary to the AS (yellow arrow). The patient was then switched to intravitreal aflibercept and the OCT scan at 2 months after the initial aflibercept injection revealed regression of the PCV lesion (c); the OCT scan at 7 months showed an absence of SRF and PED (d). The thin purple arrow in a demonstrates the locations of the cross-sectional slides of the OCT images in b, c, and d. Case 3 at the 3.5-year follow-up visit in the left eye. i Colour fundus photography demonstrates an orange-yellow nodule at the nasal edge of the macula and adjacent to the AS in the left eye (thick green arrow; as described in c), corresponding to a PCV lesion; j the OCT scan showed a PED associated with an underlying discreet polyp-like lesion and a small SRF next to the lesion; FA (m) and ICGA (n) demonstrate early filling of a grape-like structure suggestive of PCV, with leakage in the late phase in FA (o) and ICGA (p) (yellow arrow). OCT revealed a persisting PED with an underlying polypoidal lesion and the absence of SRF at 2 months (k) and 20 months (l) after this visit. The thin purple arrow in i demonstrates the locations of the cross-sectional slides of the OCT images in j, k, and l.
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F3
Clinical examination revealed a discreet orange-red nodule in the nasal right macula adjacent to the AS (Fig. 3a). OCT revealed typical features of PCV, including a PED with an underlying polypoidal lesion (Fig. 3b). FA (Fig. 3e, f) and ICGA (Fig. 3g, h) demonstrated a polypoidal lesion with terminal aneurysmal dilatations at the edge of the AS near the macula with an associated BVN. At 2 months' follow-up, the PED and PCV lesion had flattened (Fig. 3c). OCT (Fig. 3d) at 7 months showed an absence of SRF and PED, with complete regression of the polypoidal lesion. Fundoscopy revealed an orange nodule (Fig. 3i), and OCT examination demonstrated a PED and shallow SRF (Fig. 3j). FA (Fig. 3m, n) and ICGA (Fig. 3o, p) revealed early filling of a grape-like structure suggestive of PCV with leakage in the late phase and an associated small BVN in both the early and late phase. At 2 months after initiating aflibercept in the left eye, SRF was absent (Fig. 3k); however, the PED was still present. At the final follow-up, 26 months after aflibercept initiation (78 months following ranibizumab initiation), the PED was persisting; however, it had improved with the absence of SRF and intraretinal fluid (Fig. 3l).