Table II.
Clinical characteristics of children with NF1 who received therapy for OPGs and final visual outcomes after therapy
| Patient number | Age (y) at detection of OPGs | Optic pathway MRI findings | Hypothalamic involvement | Vision abnormalities prior to therapy | Age (y) at treatment | Therapy | Relapse | OPGs resection | Final vision | Final VA | Endocrine | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
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| Ch | Pre-Ch | Post-Ch | VA | FD | PR | F | NC | I | W | |||||||||
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| 1 | 1.5 | Yes | No | Yes | No | − | − | − | − | 4 | V, C | No | No | − | + | − | 20/20; 20/30 | Precocious puberty |
| 2 | 2.5 | Yes | Yes (U) | No | No | − | − | − | − | 5 | V, C | Yes | No | − | + | − | 20/20; 20/20 | No |
| 3 | 1.5 | Yes | Yes (U) | No | No | − | − | − | − | 2 | V, C | No | No | + | − | − | 20/20; 20/20 | No |
| 4 | 3 | Yes | No | No | Yes | − | − | − | + | 8 | V, C | No | No | − | + | − | 20/15; 20/15 | Precocious puberty + HP |
| 5 | 1.5 | Yes | Yes (B) | No | Yes | − | − | − | − | 2 | V, C | Yes | No | + | − | − | 20/25; 20/25 | No |
| 6 | 1 | Yes | Yes (U) | No | Yes | − | − | − | − | 2.5 | V, C | No | No | + | − | − | 20/30; 20/25 | No |
| 7 | 2 | No | Yes (B) | No | No | − | − | − | − | 4 | V, C | No | No | − | + | − | 20/20; 20/20 | No |
| 8 | 5 | Yes | No | No | No | − | − | − | − | 8 | V, C | No | No | − | − | + | 20/25; 20/40 | No |
| 9 | 3 | No | Yes (B) | No | No | − | − | − | − | 3.2 | V, C | No | No | − | − | + | 20/20; 20/40 | No |
| 10 | 2.5 | Yes | Yes (B) | No | No | − | − | − | − | 3 | V, C | No | No | + | − | − | 20/20; 20/20 | No |
| 11 | 3.2 | Yes | Yes (B) | No | No | + | − | − | + | 5 | V, C | Yes | No | − | − | + (U) | NLP; 20/25 | Precocious puberty |
| 12 | 2 | Yes | No | No | No | + | − | − | + | 3 | V, C | No | No | − | − | + (U) | NLP; 20/25 | HP |
| 13 | 8 | Yes | No | No | ND | − | + | − | − | 12 | V, C | No | No | + | − | − | 20/20; 20/20 | HP |
| 14 | 1 | Yes | Yes (B) | No | Yes | + | − | − | − | 3 | V, C | Yes | No | − | − | + (U) | 20/20; 20/125 | No |
| 15 | 9 | Yes | Yes (B) | Yes | No | + | + | − | + | 12 | V, C | Yes | No | − | − | + (B) | 20/160; 20/160 | HP |
| 16 | 5 | Yes | No | No | ND | + | − | − | + | 8 | V, C | No | No | − | − | + (U) | 20/400; 20/20 | No |
| 17 | 3 | No | Yes (B) | No | No | + | + | − | − | 3.4 | V, C | No | Yes | − | − | + (U) | NLP; 20/25 | No |
| 18 | 5 | Yes | Yes (U) | Yes | No | + | + | − | + | 5.2 | V, C | Yes | No | − | − | + (B) | 20/125; 20/125 | Precocious puberty + HP |
| 19 | 3 | Yes | No | No | ND | + | + | − | + | 5 | V, D | No | No | − | − | + (B) | 20/200; 20/200 | Precocious puberty |
| 20 | 1.5 | Yes | Yes (B) | Yes | ND | + | − | − | + | 1.8 | V, C | Yes | Yes | − | − | + (U) | NLP; 20/30 | HP |
| 21 | 1.5 | Yes | Yes (B) | No | No | + | + | − | + | 4 | V, C | No | No | + | − | − | 20/25; 20/25 | No |
| 22 | 4 | Yes | Yes (B) | No | Yes | + | − | + | − | 5.4 | V, C | No | No | − | − | + (U) | 20/200; 20/30 | No |
B, bilateral; C, carboplatin; Ch, chiasmatic; D, dactinomycin; F, fundoscopy; FD, field defect; HP, hypopituitarism; I, improved; NC, no change; ND, no data; NLP, no light perception; Post-Ch, postchiasmatic; PR, pupillary response; Pre-Ch, prechiasmatic; U, unilateral; V, vincristine; VA, visual acuity; W, worsening.