Background
Methods
Patients
Statistical analysis
Results and case descriptions
Subjects
Variable | Total (n = 48) | FEVR diagnosed pre-operatively (n = 23) | FEVR diagnosed intra−/post-operatively (n = 25) | P value |
---|---|---|---|---|
Age at initial clinic visit (years) | 14.22 ± 13.87 | 15.65 ± 10.17 | 12.90 ± 16.68 | 0.499* |
Age range (years), (median) | 0.25–58 (12) | 0.25–41 (14) | 0.25–58 (6) | |
Sex | ||||
Female | 13 (27.1) | 4 (17.4) | 9 (36.0) | 0.147# |
Male | 35 (72.9) | 19 (82.6) | 16 (64.0) | |
Clinical stage of patients at initial presentation, n (%) # | ||||
Stage 2 | 2 (4.2) | 0 (0.0) | 2 (8.0) | 0.398^ |
Stage 3 | 11 (22.9) | 7 (30.4) | 4 (16.0) | |
Stage 4 | 12 (25.0) | 7 (30.4) | 5 (20.0) | |
Stage 5 | 23 (47.9) | 9 (39.1) | 14 (56.0) | |
Clinical stage in contralateral eye, n (%) | ||||
NON-FEVR | 1 (2.1) | 0 (0.0) | 1 (4.0) | 0.216^ |
Stage 1 | 22 (45.8) | 10 (43.5) | 12 (48.0) | |
Stage 2 | 17 (35.4) | 7 (30.4) | 10 (40.0) | |
Stage 3 | 5 (10.4) | 3 (13.0) | 2 (8.0) | |
Stage 4 | 1 (2.1) | 1 (4.4) | 0 (0.0) | |
Stage 5 | 2 (4.2) | 2 (8.7) | 0 (0.0) | |
Patient FA | ||||
+ | 35 (72.9) | 15 (65.2) | 20 (80.0) | – |
N/A | 13 (27.1) | 8 (34.8) | 5 (20.0) | |
Referral diagnosis/pre-operative diagnosis, n (%) | ||||
Unilateral RD | 25 (52.1) | 14 (60.9) | 11 (44.0) | – |
Bilateral RD | 4 (8.3) | 3 (13.0) | 1 (4.0) | |
Bilateral PFV | 2 (4.2) | 2 (8.7) | 0 (0.0) | |
Unilateral PFV | 4 (8.3) | 1 (4.3) | 3 (12.0) | |
Ocular trauma | 1 (2.1) | 1 (4.3) | 0 (0.0) | |
Bilateral FEVR | 2 (4.2) | 2 (8.7) | 0 (0.0) | |
Unilateral PFV + ROP | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Unilateral RB | 2 (4.2) | 0 (0.0) | 2 (8.0) | |
Unilateral congenital cataract + VH + RD | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Unilateral RD + VH | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
VH + contralateral ERM | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Unilateral VH + aphakia | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Bilateral MF + LMH + HM | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Bilateral congenital cataract+ unilateral PFV | 1 (2.1) | 0 (0.0) | 1 (4.0) | |
Unilateral ERM | 1 (2.1) | 0 (0.0) | 1 (4.0) |
Risk factors
Risk factors | FEVR diagnosed pre-operatively (n = 23) | FEVR diagnosed intra−/post-operatively (n = 25) | Univariate Analysis | Multivariate Analysis | ||
---|---|---|---|---|---|---|
OR (95% CI) |
P
| OR (95% CI) |
P
| |||
Preoperative ocular manifestations, n (%) (no. of patients) | ||||||
RD only | 19 (82.6) | 8 (32.0) | 0.099 (0.025–0.389) | 0.001 | 0.104 (0.015–0.725) | 0.022 |
Other manifestations except/without RD | 4 (17.4) | 17 (68.0) | ||||
Father/mother FA, n (%) | ||||||
- | 2 (8.7) | 9 (36.0) | 0.141 (0.023–0.857) | 0.033 | 0.105 (0.013–0.855) | 0.035 |
+ | 11 (47.8) | 7 (28.0) | ||||
N/A | 10 (43.5) | 9 (36.0) | ||||
Year of diagnosis, n (%) | ||||||
2010–2015 | 7 (30.4) | 17 (68.0) | 0.161 (0.044–0.583) | 0.005 | ||
2016–2018 | 16 (69.6) | 8 (32.0) | ||||
Year at initial clinic visit (years), n (%) | ||||||
2007–2014 | 7 (30.4) | 19 (76.0) | 0.138 (0.039–0.496) | 0.002 | ||
2015–2018 | 16 (69.6) | 6 (24.0) | ||||
Wide-angle fundus imaging preoperatively, n (%) | ||||||
No | 6 (26.1) | 20 (80.0) | 0.088 (0.023–0.341) | 0.000 | ||
Yes | 17 (73.9) | 5 (20.0) | ||||
Ocular trauma, n (%) | ||||||
No | 17 (73.9) | 24 (96.0) | 0.118 (0.013–1.072) | 0.058 | ||
Yes | 6 (26.1) | 1 (4.0) | ||||
Clinical stage in contralateral eye, n (%) | ||||||
Stage 1-Stage 2 | 17 (73.9) | 23 (92.0) | 4.059 (0.728–22.637) | 0.110 | ||
Stage 3-Stage 5 | 6 (26.1) | 2 (8.0) | ||||
Age at initial clinic visit (years), n (%) | ||||||
0.25–12 | 10 (43.5) | 16 (64.0) | 0.433 (0.136–1.381) | 0.157 | ||
13–58 | 13 (56.5) | 9 (36.0) | ||||
Gender, n (%) | ||||||
Female | 4 (17.4) | 9 (36.0) | 0.374 (0.097–1.447) | 0.154 | ||
Male | 19 (82.6) | 16 (64.0) | ||||
Preterm birth, n (%) | ||||||
Yes | 0 (0.0) | 1 (4.0) | 0.000 (0.000-) | 1.000 | ||
No | 23 (100.0) | 24 (96.0) | ||||
Surgery in other hospital before first clinic visit, n (%) | ||||||
No | 21 (91.3) | 19 (76.0) | 3.316 (0.596–18.451) | 0.171 | ||
Yes | 2 (8.7) | 6 (24.0) | ||||
Ocular involvement, n (%) | ||||||
Bilateral | 6 (26.1) | 5 (20.0) | 0.908 (0.253–3.251) | 0.882 | ||
Unilateral | 17 (73.9) | 20 (80.0) | ||||
Clinical stage of patients at initial presentation, n (%)a | ||||||
Stage 1-Stage 4 | 14 (60.9) | 11 (44.0) | 1.980 (0.626–6.259) | 0.245 | ||
Stage 5 | 9 (39.1) | 14 (56.0) | ||||
Initial surgery in our hospital, n (%) | ||||||
SB/SE | 7 (30.4) | 5 (20.0) | 1.750 (0.466–6.568) | 0.407 | ||
PPV/L | 16 (69.6) | 20 (80.0) |
Reasons of missed diagnosis of FEVR preoperatively | Cases (n = 28)a |
---|---|
Age<3 years, unilateral leukocoria with invisible fundus + contralateral fundus seemingly normal | 8 |
Age ≥ 3 years, without ultra-wide-field scanning laser ophthalmoscopy at initial clinic visit or preoperatively | 9 |
Bilateral vitreoretinal surgery or peripheral retinal photocoagulation in other hospital before the first clinic visit | 2 |
Unilateral VH + contralateral phthisis bulbi | 2 |
Unilateral VH + contralateral ERM | 1 |
Unilateral RD + contralateral ERM | 1 |
Unilateral ERM | 1 |
Bilateral pathological myopia + myopic foveoschisis + lamellar macular hole | 1 |
3 years < age <4 years, poor cooperation, ultra-wide-field scanning laser ophthalmoscopy failed to capture the peripheral retina | 1 |
Premature birth history | 1 |
Ocular trauma | 1 |
Case descriptions
Other ocular manifestations except RD at the first clinic visit in our department
Patient no. (n = 21) | Age at initial clinic visit | Sex | Eye (n = 32) | FEVR stage | Ocular manifestations except RD | Operation numbers in other referral hospitals | Initial therapy in our hospital | Patient FA | Parents FA | Preoperative diagnosis | Final diagnosis | Diagnostic timing |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 3mos | F | OD | 5 | Corneal leucoma, AC (−), VH | 0 | L | N/A | + | OU FEVR | OU FEVR | Pre-op |
OS | 5 | Corneal leucoma, AC (−), VH | L | |||||||||
2 | 4mos | M | OD | 5 | Corneal leucoma, AC (−), a cord rising from the optic head to the lens, prolonged ciliary process | 0 | LV | N/A | + | OU FEVR | OU FEVR | Pre-op |
OS | 5 | Corneal leucoma, AC (−), a cord rising from the optic head to the lens, prolonged ciliary process | 0 | LV | ||||||||
3 | 10 yrs | M | OD | 5 | Repaired corneal laceration, hyphema | 1 | LV + SiO | N/A | N/A | OU FEVR | OU FEVR | Pre-op |
4 | 4mos | M | OD | 5 | Corneal leucoma, AC (−), falciform retinal fold | 0 | LV+ FA | + | + | OU FEVR | OU FEVR | Pre-op |
5 | 4mos | M | OD | 5 | Microphthalmia, retrolental membrane | 0 | LV + FA | + | – | OD PFV, OD ROP | OU FEVR | Intra-op |
6 | 3mos | M | OD | 5 | Corneal leucoma, AC (−), lens opacity | 0 | LV + FA | + | – | OU suspected FEVR | OU FEVR | Intra-op |
7 | 5mos | M | OS | 5 | AC (−), posterior iris adhesion | 0 | L + FA | + | – | OU suspected FEVR | OU FEVR | Intra-op |
8 | 3mos | M | OD | 5 | Corneal leucoma, AC (−) | 0 | L + FA | + | N/A | OD PFV | OU FEVR | Intra-op |
9 | 6mos | M | OD | 5 | Retrolental membrane | 0 | LV + FA | + | + | OD PFV | OU FEVR | Intra-op |
10 | 4mos | M | OD | 5 | AC (−), retrolental membrane | 0 | L + FA | + | – | OD Rb | OU FEVR | Intra-op |
11 | 3 yrs | F | OD | 5 | Cataract, VH | 0 | LV + FA | + | + | OD congenital cataract, VH, RD | OU FEVR | Intra-op |
12 | 4 yrs | F | OD | 3 | VH | 0 | V | N/A | + | OD RD, OD VH, OS phthisis | OU FEVR | Intra-op |
OS | 5 | Phthisis | 0 | |||||||||
13 | 5 yrs | F | OD | 2 | ERM | 0 | ||||||
OS | 2 | VH | 0 | LV | N/A | + | OD ERM, OS VH | OU FEVR | Intra-op | |||
14 | 58 yrs | F | OD | 5 | Phthisis bulbi | 0 | ||||||
OS | 2 | VH, nystagmus | 1 | V | N/A | N/A | OD phthisis, OS VH, aphakia | OU FEVR | Intra-op | |||
15 | 58 yrs | F | OD | 1 | MF, LMH, HM | 0 | ||||||
OS | 2 | MF, LMH, HM | 0 | Phaco+V + ILMpeeling+C3F8 | + | N/A | OU MF, LMH, PM | OU FEVR | Intra-op | |||
16 | 25 yrs | M | OD | 2 | ERM | 0 | V + SiO | |||||
OS | 4 | SiO tamponade | 1 | SiOR+LV + SiO | N/A | – | OD ERM, OS RD, OS SiO tamponade | OU FEVR | Intra-op | |||
17 | 6mos | F | OS | 5 | Shallow AC, anterior iris adhesion, esotropia | 0 | L | OS+, OD- | + | OS PFV | OS Unilateral FEVR | Post-op |
18 | 23 yrs | F | OD | 1 | Peripheral laser spots, HM | 0 | ||||||
OS | 3 | peripheral laser spots, HM | 0 | SB | + | – | OS RD | OU FEVR | Post-op | |||
19 | 18 yrs | M | OD | 4 | SiO tamponade, HM | 3 | SiOR+V + SiO | + | N/A | OD recurrent RD | OU FEVR | Post-op |
OS | 2 | Retinal tear surrounded by laser spots | 0 | |||||||||
20 | 40 yrs | M | OD | 5 | HM | 1 | V + SiO | + | N/A | OD RD | OU FEVR | Post-op |
OS | 2 | Superiotemporal retinal dry hole | 1 | PC | ||||||||
21 | 8 yrs | M | OD | 3 | RD occurred later | 0 | SB | OU FEVR | Post-op | |||
OS | 2 | ERM | 0 | V + ILMpeeling+C3F8 | + | N/A | OS ERM |
Main clue for diagnosing FEVR | Cases (n = 21) |
---|---|
Complicated FEVR patients diagnosed as FEVR preoperatively | |
Family history (+) | 3 |
Avascular peripheral area and peripheral vascular anomalies in the contralateral eye | 1 |
Complicated FEVR patients diagnosed as FEVR intraoperatively | |
Intraoperative FA (+) in the contralateral eye | 7 |
Avascular peripheral area and peripheral vascular anomalies were found when the peripheral sclera was impressed during the operation | 5 |
Complicated FEVR patients diagnosed as FEVR postoperatively | |
Radial retinal fold involved in all major retinal vessels during the follow-up | 1 |
Peripheral avascular zone was found during the follow-up | 4 |