Age Sex Race Symptoms Laterality Visual acuity Location Hemorrhage RD Exudates Other system involvement Treatment Follow-up Follow-up  outcome Family history
1 21y M white asymptomatic right 20/20 360o of midperipheral yes no no cutaneous observation 28m unchanged no
2 25y F white asymptomatic right 20/20 360o of midperipheral no no no no observation 3y unchanged no
3 9y F n/a acute loss of vision right 6/60 macula yes no no no observation 1y unchanged no
4 10y M n/a asymptomatic right 20/20 superonasal midperipheral no no no brain observation n/a unchanged yes
5 23y n/a n/a asymptomatic unilateral n/a n/a no no no brain laser 33y slowly increased no
6 16y M caucasian asymptomatic left 20/20 inferiorly no no no cutaneous observation 7y unchanged yes
7 44y F white asymptomatic bilateral 20/20 optic disc  superiotemporal yes no no brain observation n/a unchanged yes
8 17y F n/a asymptomatic left 20/20 optic disc no no no no observation 1.5y unchanged no
9 13y M n/a strabismus and decreased vison right  6/60 superonasal no no no no observation 1.5y unchanged no
10 6y M n/a esotropia right 20/30 optic disc yes no no no observation 5y larger no
11 74y F n/a decreased vision right 20/25 optic disc yes no no no vitrectomy 30y increase in numbers no
12 38y M n/a decreased vision right CF optic nerve yes no no no vitrectomy 7y VA improved no
13 35y M n/a decrease vision and pain right LP optic nerve yes no no no enucleation n/a unchanged no
14 38y M n/a n/a right n/a inferior temporal n/a n/a no no n/a n/a n/a yes
15 13y F white asymptomatic right 20/20 superiotemporal no no no no observation n/a n/a no
16 9y M white seizures right 20/20 optic disc no no no brain observation n/a n/a yes
17 14y F white headaches left 20/20 peripheral retina no no no brainstem observation n/a n/a yes
18 22y F n/a asymptomatic left 20/20 nasally yes no no n/a photocoagulation 4y hemorrhage no
19 28y F n/a asymptomatic right 20/20 superior no no n/a no n/a n/a n/a no
20 13y M n/a asymptomatic bilateral 20/20 n/a no no np no observation 4y unchanged yes
21 10y F n/a asymptomatic right 20/20 midperiphery no no no brain observation 16y increased fibrous tissue yes
22 8y F n/a asymptomatic left 20/20 n/a no no no no observation n/a n/a yes
23 43y F n/a decreased vision right  1/10 macula no no no no obs n/a n/a no
24 18y M n/a Squint poor vision left CF macula inferotemporal no no no no observation n/a n/a no
25 32y M n/a transient visual disturbance right 20/20 inferior no no no cutaneous brain observation n/a n/a yes
26 55y F n/a asymptomatic left 20/20 n/a n/a n/a n/a n/a n/a n/a n/a n/a
27 14y M n/a asymptomatic right 20/20 nasal no no no no observation 15y aneurysm  occlusion n/a
28 3y F n/a asymptomatic left 20/400 n/a n/a n/a n/a n/a n/a n/a n/a  n/a
29 23y F n/a decrease vision right 20/100 macula n/a  n/a n/a n/a n/a n/a n/a n/a
30 50y M n/a asymptomatic left 20/20 n/a n/a n/a n/a n/a n/a n/a n/a n/a
31 10y M n/a asymptomatic left 20/20  n/a n/a n/a n/a n/a n/a n/a n/a n/a
32 1y F n/a asymptomatic right normal superior nasal no no no no observation 1y unchanged no
33 12y M n/a decreased vision left 20/100 inferotemporal no no no no observation 2y unchanged no
34 18y F n/a decreased vision right 20/50 throughout the fundus no no no no observation 1y unchanged no
35 6-month F n/a white pupillary reflex left NLP n/a no yes yes no enucleation n/a n/a no
36 37y M n/a decreased vision right CF inferior yes no no no observation 6y improved no
37 44y F white decreased vision left   4/10 macula no no no no obs n/a n/a no
38 4-month M n/a skin lesion left n/a macula yes no no cutaneous observation 2y epithelial defect no
39 25y M white asymptomatic left 20/20 optic disc no no no no observation n/a n/a no
40 16y F n/a asymptomatic right 20/20 temporal yes no no no observation 21y vitrous hemorrage no
41 27y M n/a asymptomatic left 20/20 temporal no no no no observation n/a n/a no
42 26y M n/a asymptomatic bilateral 20/20 temporal no no no cutaneous observation 8y unchanged yes
43 16y M n/a asymptomatic right 20/20 peripheral no no no no observation 6y Increased fibrous tissue no
44 70y M n/a grand mal left normal peripheral no no no no observation n/a n/a yes
45 10y M n/a astigmatism right 0.4 peripheral no no no no observation 2y unchanged no
46 4y F n/a asymptomatic right normal nasal peripheral no no no no observation 1y unchanged no
47 39y F n/a diplopia right 20/20 superonasal no no no cutaneous brain observation 2y unchanged yes
48 10y F n/a asymptomatic bilateral 20/20 inferonasal superotemporal no no no no observation 4y unchanged yes
49 10y F white asymptomatic left 20/20 superotemporal no no no n/a observation n/a n/a no
50 18y F white asymptomatic left   1/20 optic nerve no no no no observation n/a n/a no
51 7y F white decreased vision left   1/20 macula no no no no observation n/a n/a no
52 37y n/a white diplopia right 20/20 inferior no no no no observation n/a n/a no
53 40y n/a white decreased vision left  9/10 superotemporal no no no no observation n/a n/a no
54 63y n/a white asymptomatic right 20/20 inferior no no no no obs n/a n/a no
55 25y n/a white asymptomatic right 20/20 temperal no no no no obs n/a n/a no
56 19y M white decreased vision right  6/10 nasal no no no no obs n/a n/a no
57 48y M white asymptomatic right 20/20 superonasal no no no cutaneous brain observation 5y unchanged no
58 19y M white asymptomatic left 14/21 superior no no no brain observation 5y aneurysm  occlusion no
59 50y M black decreased vision left 20/200 macula no no no no observation n/a n/a no
60 71y M white reading discomfort right 20/30 temporal yes no no no photocoagulate 5 m unchanged no
61 19y M white asymptomatic left 20/20 temporal no no no no photocoagulation 5y subretinal hemorrhage no
62 53y M white poor vision right HM inferior yes no no no no n/a n/a no
63 8y F n/a asymptomatic n/a n/a n/a no no no no observation 7y slightly changed no
64 63y M white asymptomatic right 20/30 temporal no no no no observation 34y slightly changed no
65 35y M black asymptomatic right 20/20 inferotemporal no no no no observation 3y unchanged no
66 45y F white asymptomatic right 20/15 nasal no no no no observation n/a n/a n/a
67 37y F white blurred vision strabismus left 0.1 macula temporal yes yes no no observation 13y slight changed no
68 2.5y F white esotropia right n/a superotemporal yes yes no no photocoagulation 4.5y hemorrhage no
69 33y F white asymptomatic right 20/20  inferior nasal no no no cutaneous observation 5y aneurysm  occlusion yes
70 51y F white asymptomatic right 20/15 optic  nerve no no no no observation 6y unchanged no
71 21y F white decreased vision right 20/30 temporal no no no cutaneous brain observation 4y unchanged no
72 47y F n/a decrease vision left n/a n/a yes no no no photocoagulation 10y hemorrhage absorbed no
73 13y M white keratitis bilateral 20/20 peripheral no no no no observation 2y increased in numbers yes
74 10y F white asymptomatic right 20/20 superotemporal no no no no observation n/a n/a yes
75 24y F n/a asymptomatic bilateral n/a inferior no no no no n/a n/a n/a n/a
76 14y M n/a asymptomatic left normal optic nerve yes no no n/a n/a n/a n/a n/a
77 2y F white n/a n/a n/a midperiphery yes no no n/a enucleate n/a n/a n/a
78 34y M white decreased vision right n/a optic nerve n/a n/a n/a no n/a n/a n/a n/a
79 26y F white routine refraction left 20/20 n/a yes yes no cutaneous n/a n/a n/a n/a
80 11y F n/a decreased vision right 5/100 macula no no no n/a n/a n/a n/a n/a
81 4y M n/a decreased vision,esotropia left 3/200 nasal yes no no n/a n/a n/a n/a n/a
82 54y M n/a retrobulbar pain left 20/20 n/a n/a n/a n/a cutaneous enucleation n/a n/a n/a
83 7y F n/a asymptomatic left 20/20  n/a n/a n/a n/a n/a n/a  n/a n/a   n/a
84 17y F n/a headaches unilateral n/a superotemporal no n/a no cutaneous brain n/a n/a n/a yes
85 21y M n/a headaches decreased vision bilateral 15/400  n/a no no no no observation 7 m unchanged n/a
86 32y M n/a mild decreased vision right n/a optic nerve yes yes n/a no n/a n/a n/a n/a
87 11y F asian asymptomatic right 20/200

inferotemporal

 no no  no  no observation 5 y unchanged no
88 19y F n/a Decreased vision and floaters left 20/20 superotemporal yes no no no observation 5 m improved no
89 63y M n/a Decreased vision right 20/400 macula no no no no infliximab 6 m improved no
90 0d F white hyphema left n/a inferior yes n/a no no enucleation 52 y n/a no
91 18y M mestizo scotoma Right n/a inferotemporal no no no n/a observation 48 m unchanged n/a
92 43y M n/a asymptomatic left 20/30 supertemporal n/a n/a n/a no n/a n/a n/a no
93 28y F n/a asymptomatic right 20/30 inferior yes n/a n/a no n/a n/a n/a no
94 29y F n/a paracentral right 20/20 Optic disc yes n/a n/a no n/a 1 m hemorrhage absorbed n/a
95 12y F n/a asymptomatic right 6/6 nasal n/a n/a n/a n/a n/a n/a n/a n/a
96 30y F n/a asymptomatic right 20/20 macula yes n/a n/a brain n/a n/a n/a yes
Table 1: Summary of CHR cases from the literature.