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Supplemental Figure1: RetCam fundus pictures of the right and left eye of case 1with accompanying
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cartoons that pictorially show the corresponding subtle retinal vascular changes. The numbers at the left
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hand corner of each image refer to the postmenstrual age (PMA) at examination in weeks.
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a and b. One week post intravitreal bevacizumab (IVB), media clarity better (had hazy media and poor
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mydriasis at presentation), decrease in vessel dilatation and tortuosity and thinning of the shunts.
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Appearance of retinal haemorrhage near superotemporal shunt in OS (b)
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c and d. Retinal images two weeks post IVB showing further reduction in disease activity. The retinal
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haemorrhage superotemporally (d) slightly more than the previous visit
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Supplemental Figure 2: RetCam fundus picture of the right eye of case 1 and the corresponding cartoon
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showing saw toothed arcade and a demarcation line anterior to it. The numbers at the left hand corner of
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each image refer to the postmenstrual age (PMA) at examination in weeks. The retinopathy remained
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status quo in this stage from 32 to 37 weeks PMA.
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Supplemental Figure 3: RetCam fundus pictures of both eyes of case 2 and accompanying cartoons that
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pictorially show the corresponding subtle retinal vascular changes. The numbers at the left hand corner of
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each image refer to the postmenstrual age (PMA) at examination in weeks.
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a to c. Retinal features after laser in the right eye. While the lasered right eye regressed within 2 weeks
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(a,b) with no recurrence till 52weeks PMA (c), abnormal circumferential retinal vessel (yellow arrow)
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was seen as late as 52 weeks of post menstrual age (PMA) in superior and nasal quadrants in left eye (d).
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Supplemental Figure 4: Serial RetCam fundus images of the left eye of case 2 and the corresponding
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cartoons to show the vascular changes. The numbers at the left hand corner of each image refer to the
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postmenstrual age (PMA) at examination in weeks.
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a. Dilatation and tortuosity of the posterior pole vessels at presentation.
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b. Remarkable reduction in vascular dilatation and tortuosity within a week of intravitreal bevacizumab
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(IVB). The shunts became thinner and invisible with few fresh retinal hemorrhages in inferotemporal
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quadrant (ITQ).
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c. Fine small vascular twigs appeared at the site of previous shunts with a demarcation line anterior to it
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d. Fine saw toothed bridging vessels and demarcation line appeared inferiorly while the arcade in ITQ
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was replaced with closely packed bridging vessels in 2 to 3 rows.
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e. Saw toothed arcade and demarcation line in superotemporal quadrant (STQ) and multiple arcades as
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before in ITQ
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f. Saw toothed arcade superior and inferiorly with multilayered arcade nasally and infero-temporally. The
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vessels were more tortuous than previous visits.
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From 40 to 44 weeks PMA (Pictures not shown), the disease remained status quo with slight increase in
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tortuosity and early ridge formation.
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g. Ridge formation in all quadrants (picture shows the superotemporal quadrant) except inferonasally.
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h. Doubling of the ridge temporally (arrow mark) with non-dichotomously branching vessels crossing it
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at places. Long abnormal circumferential vessels formed nasally (not shown).
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i. Further ridge regression temporally while ridge in all other quadrants disappeared. Abnormal
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circumferential vessels (ACV) appeared superiorly and that in the nasal quadrant persisted.
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The ACVs persisted, looked more mature, displaced peripherally and appeared as if continuation of the
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major vascular arcade up to 52 wks. PMA (j and k).The temporal ridge regressed completely.
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j. The previous changes could not be observed as far as the retina could be visualized at 62 wks. of PMA.
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Supplemental Figure 5: Serial RetCam fundus images of the right eye of case 3 and the corresponding
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cartoons to show the vascular changes. The numbers at the left hand corner of each image refer to the
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postmenstrual age (PMA) at examination in weeks.
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a. Tortuosity of posterior pole vessels with shunts, retinal haemorrhage superotemporally and a ridge
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nasally in zone 1.
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b. One week post bevacizumab showing marked reduction in tortuosity and caliber of the posterior pole
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vessels, thinning and invisibility of the shunts. Retinal haemorrhages and ridges (at places) disappeared
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with appearance of demarcation line at places (represented by dotted blue line in cartoon).
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c. Demarcation line temporally, multiple minute vascular twigs at vascular-avascular junction in supero
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and inferonasal quadrant and closely packed multiple retinal vessel nasally.
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d. Appearance of saw-toothed shunts temporally, other changes remaining same as in c. These shunts
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were less curved as their counterpart before treatment.
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The disease remained status quo in this stage for 7 week till 42 weeks PMA
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e. Large abnormal circumferential vessel (ACV) in supero and inferotemporal quadrant.
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f. ACV as before with early ridge formation
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g. Ridge in anterior zone III temporally and superiorly
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h. Large and mature looking ACV
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i. No vascular anomaly could be detected as far as the fundus was seen.
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Supplemental Figure 6: Serial RetCam fundus images of the left eye of case 3 and the corresponding
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cartoons to show the vascular changes. The numbers at the left hand corner of each image refer to the
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postmenstrual age (PMA) at examination in weeks.
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a. Tortuosity of the retinal vessels at posterior pole with shunts. There were demarcation line and ridges at
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places. The retinopathy was extending up to posterior zone I nasally and zone II temporally.
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b. Posterior pole vessels attained normalcy, thinning and disappearance of arcades and retinal
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haemorrhages. Fine saw toothed arcades superonasally and superotemporally. Demarcation line all
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around except inferotemporally.
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c. Demarcation line and saw toothed arcade inferotemporally.
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d. Closely packed multiple arcades in supero and inferotemporal quadrant. Saw toothed arcades bridging
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between radial vessels and demarcation line in all other quadrants
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e. Same as above but saw toothed arcades got replaced with single mature looking arcades
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f. Single anomalous arcades in almost all quadrants except closely packed multiple arcades
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superotemporally
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g. Status quo except prominent closely packed multiple vessels superiorly
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h. Single high arched circumferential mature looking vessel in place of multiple arcades seen superiorly in
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previous visit
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i. No anomalous vasculature could be observed as far as the retina could be seen.
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Supplemental Figure 7:
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RetCam images (anterior and posterior segment) of the left eye of case 4 and the corresponding cartoons
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to show the vascular changes. The numbers at the left hand corner of each image refer to the
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postmenstrual age at examination in weeks.
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a. Anterior segment as imaged with RetCam showing poor mydriasis and 360 degree neovascularization
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at pupillary margin.
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b. Superficial retinal hemorrhages in the vascularized retina and pre retinal hemorrhages overlying the
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vascular avascular junction and demarcation line temporally
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c and d. Increase in retinal and pre retinal hemorrhages with a prominent ridge temporally
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e. Post lasered regressed retinopathy without any significant vascular anomaly.
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Supplemental Figure 8: RetCam images (anterior and posterior segment) of the right eye of case 4 and the
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corresponding cartoons to show the vascular changes. The numbers at the left hand corner of each image
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refer to the postmenstrual age at examination in weeks.
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a. Anterior segment as imaged with RetCam showing poor mydriasis and 360 degree neovascularization
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at pupillary margin.
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b. Demarcation line temporally with saw toothed shunts posterior to it along its entire length
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c. Saw toothed shunts temporally and early ridge formation nasally
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d. Temporal ridge with early specs of hemorrhage overlying it.
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e. Lasered regressed retinopathy without any vascular anomaly
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