The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness
Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR)...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2018-01-01
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| Series: | Taiwan Journal of Ophthalmology |
| Subjects: | |
| Online Access: | http://www.e-tjo.org/article.asp?issn=2211-5056;year=2018;volume=8;issue=4;spage=189;epage=195;aulast=Shapiro |
| _version_ | 1819262200061100032 |
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| author | Michael J Shapiro Michael P Blair Mark A Solinski David L Zhang Sayena Jabbehdari |
| author_facet | Michael J Shapiro Michael P Blair Mark A Solinski David L Zhang Sayena Jabbehdari |
| author_sort | Michael J Shapiro |
| collection | DOAJ |
| description | Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial. |
| first_indexed | 2024-12-23T19:53:54Z |
| format | Article |
| id | doaj.art-161dc0e6bfb0459abf205193ea66eced |
| institution | Directory Open Access Journal |
| issn | 2211-5056 2211-5072 |
| language | English |
| last_indexed | 2024-12-23T19:53:54Z |
| publishDate | 2018-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Taiwan Journal of Ophthalmology |
| spelling | doaj.art-161dc0e6bfb0459abf205193ea66eced2022-12-21T17:33:17ZengWolters Kluwer Medknow PublicationsTaiwan Journal of Ophthalmology2211-50562211-50722018-01-018418919510.4103/tjo.tjo_97_18The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindnessMichael J ShapiroMichael P BlairMark A SolinskiDavid L ZhangSayena JabbehdariStickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial.http://www.e-tjo.org/article.asp?issn=2211-5056;year=2018;volume=8;issue=4;spage=189;epage=195;aulast=ShapiroDiagnosisearly onset myopiaprimary careprophylaxissurveillance |
| spellingShingle | Michael J Shapiro Michael P Blair Mark A Solinski David L Zhang Sayena Jabbehdari The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness Taiwan Journal of Ophthalmology Diagnosis early onset myopia primary care prophylaxis surveillance |
| title | The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness |
| title_full | The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness |
| title_fullStr | The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness |
| title_full_unstemmed | The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness |
| title_short | The importance of early diagnosis of Stickler syndrome: Finding opportunities for preventing blindness |
| title_sort | importance of early diagnosis of stickler syndrome finding opportunities for preventing blindness |
| topic | Diagnosis early onset myopia primary care prophylaxis surveillance |
| url | http://www.e-tjo.org/article.asp?issn=2211-5056;year=2018;volume=8;issue=4;spage=189;epage=195;aulast=Shapiro |
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