Unusual Straatsma Syndrome - How dogmatic is a bad prognosis?
Purpose: To show that Straatsma Syndrome can have a good outcome and to highlight an unusual presentation of this disease. Observations: A four-year-old boy presents with severe right eye amblyopia in association with high myopia, esotropia, heterochromia iridum and extensive myelinated retinal fibe...
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| Format: | Article |
| Language: | English |
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Elsevier
2017-12-01
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| Series: | American Journal of Ophthalmology Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2451993616300755 |
| _version_ | 1818037087950077952 |
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| author | Ana Vide-Escada Helena Prior Filipe |
| author_facet | Ana Vide-Escada Helena Prior Filipe |
| author_sort | Ana Vide-Escada |
| collection | DOAJ |
| description | Purpose: To show that Straatsma Syndrome can have a good outcome and to highlight an unusual presentation of this disease.
Observations: A four-year-old boy presents with severe right eye amblyopia in association with high myopia, esotropia, heterochromia iridum and extensive myelinated retinal fibers involving both temporal arcades and the optic nerve head. Right eye initial visual acuity was less than 20/400 for distance and less than R6W10 for near. Left eye examination was unremarkable. Despite the indicators for bad prognosis, intensive occlusion therapy was prescribed. Parents were strongly involved in the treatment regimen. After four months, the patient presented an unexpected good visual recovery both for distance and near, that has persisted until present. Right eye visual acuity is 20/30 with −9.00 dioptres contact lens for distance and R2W1 for near. Esotropia also improved to 12 prism dioptres. Fundoscopic alterations and heterochromia iridum have remained stable. Spectral-domain optical coherence tomography images of the right eye showed thinner superior outer ring measurements.
Conclusions and importance: Straatsma Syndrome can present with heterochromia iridum. When strabismus is present, early surgery should be withheld. Intensive treatment of Straatsma Syndrome can yield an unexpected good result, despite initial high degree anisometropia and low vision acuity. |
| first_indexed | 2024-12-10T07:21:17Z |
| format | Article |
| id | doaj.art-2be924be97ea492e92c0f7c091db96f7 |
| institution | Directory Open Access Journal |
| issn | 2451-9936 |
| language | English |
| last_indexed | 2024-12-10T07:21:17Z |
| publishDate | 2017-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | American Journal of Ophthalmology Case Reports |
| spelling | doaj.art-2be924be97ea492e92c0f7c091db96f72022-12-22T01:57:48ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362017-12-018C717310.1016/j.ajoc.2017.10.003Unusual Straatsma Syndrome - How dogmatic is a bad prognosis?Ana Vide-Escada0Helena Prior Filipe1Integrated Responsibility Centre of Ophthalmology of Hospital Garcia de Orta EPE, Av. Torrado da Silva, 2805-267 Almada, PortugalHospital das Forças Armadas/Polo de Lisboa-EMGFA, Azinhaga dos Ulmeiros, 1690-020 Lisboa, PortugalPurpose: To show that Straatsma Syndrome can have a good outcome and to highlight an unusual presentation of this disease. Observations: A four-year-old boy presents with severe right eye amblyopia in association with high myopia, esotropia, heterochromia iridum and extensive myelinated retinal fibers involving both temporal arcades and the optic nerve head. Right eye initial visual acuity was less than 20/400 for distance and less than R6W10 for near. Left eye examination was unremarkable. Despite the indicators for bad prognosis, intensive occlusion therapy was prescribed. Parents were strongly involved in the treatment regimen. After four months, the patient presented an unexpected good visual recovery both for distance and near, that has persisted until present. Right eye visual acuity is 20/30 with −9.00 dioptres contact lens for distance and R2W1 for near. Esotropia also improved to 12 prism dioptres. Fundoscopic alterations and heterochromia iridum have remained stable. Spectral-domain optical coherence tomography images of the right eye showed thinner superior outer ring measurements. Conclusions and importance: Straatsma Syndrome can present with heterochromia iridum. When strabismus is present, early surgery should be withheld. Intensive treatment of Straatsma Syndrome can yield an unexpected good result, despite initial high degree anisometropia and low vision acuity.http://www.sciencedirect.com/science/article/pii/S2451993616300755Heterochromia iridumHigh myopiaAnisometropiaAmblyopiaMyelinated retinal fibresEsotropia |
| spellingShingle | Ana Vide-Escada Helena Prior Filipe Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? American Journal of Ophthalmology Case Reports Heterochromia iridum High myopia Anisometropia Amblyopia Myelinated retinal fibres Esotropia |
| title | Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? |
| title_full | Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? |
| title_fullStr | Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? |
| title_full_unstemmed | Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? |
| title_short | Unusual Straatsma Syndrome - How dogmatic is a bad prognosis? |
| title_sort | unusual straatsma syndrome how dogmatic is a bad prognosis |
| topic | Heterochromia iridum High myopia Anisometropia Amblyopia Myelinated retinal fibres Esotropia |
| url | http://www.sciencedirect.com/science/article/pii/S2451993616300755 |
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