Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome
Myelinated retinal nerve fibers (MNRF) are not a rare congenital anomaly but discovered as an incidental finding in approximately 1% of all eyes. This developmental defect presents as grey-white patches with feathery margins, especially in the peripapillary area. MNRF can be either static or dynamic...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-12-01
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| Series: | TNOA Journal of Ophthalmic Science and Research |
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| Online Access: | https://journals.lww.com/10.4103/tjosr.tjosr_107_24 |
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| author | Rajwinder Kaur Navdeep Kaur Balbir Khan Kirandeep Kaur Bharat Gurnani |
| author_facet | Rajwinder Kaur Navdeep Kaur Balbir Khan Kirandeep Kaur Bharat Gurnani |
| author_sort | Rajwinder Kaur |
| collection | DOAJ |
| description | Myelinated retinal nerve fibers (MNRF) are not a rare congenital anomaly but discovered as an incidental finding in approximately 1% of all eyes. This developmental defect presents as grey-white patches with feathery margins, especially in the peripapillary area. MNRF can be either static or dynamic, and may be congenital or acquired. Straatsma syndrome is a rare condition characterized by a traditional triad of unilateral MNRF, axial myopia, and amblyopia. We hereby present a case report of a 23-year-old male patient diagnosed with this syndrome. Cycloplegic retinoscopy revealed a refractive error of −2.00/−0.75 × 75 in the right eye and plano in the left eye. Best corrected visual acuity improved to 20/40 in the right eye and 20/20 in the left eye. Multimodal imaging of the right fundus showed extensive myelination encircling the disc 360 degrees, obscuring the disc margins. The left eye appeared normal. Type 2 MNRF, unilateral myopia, and amblyopia are poor prognostic factors in this case. |
| format | Article |
| id | doaj-art-e1fa4be1e6c64b1fbde3eec0eb9b0388 |
| institution | Kabale University |
| issn | 2589-4528 2589-4536 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | TNOA Journal of Ophthalmic Science and Research |
| spelling | doaj-art-e1fa4be1e6c64b1fbde3eec0eb9b03882025-01-16T07:07:48ZengWolters Kluwer Medknow PublicationsTNOA Journal of Ophthalmic Science and Research2589-45282589-45362024-12-0162446446710.4103/tjosr.tjosr_107_24Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma SyndromeRajwinder KaurNavdeep KaurBalbir KhanKirandeep KaurBharat GurnaniMyelinated retinal nerve fibers (MNRF) are not a rare congenital anomaly but discovered as an incidental finding in approximately 1% of all eyes. This developmental defect presents as grey-white patches with feathery margins, especially in the peripapillary area. MNRF can be either static or dynamic, and may be congenital or acquired. Straatsma syndrome is a rare condition characterized by a traditional triad of unilateral MNRF, axial myopia, and amblyopia. We hereby present a case report of a 23-year-old male patient diagnosed with this syndrome. Cycloplegic retinoscopy revealed a refractive error of −2.00/−0.75 × 75 in the right eye and plano in the left eye. Best corrected visual acuity improved to 20/40 in the right eye and 20/20 in the left eye. Multimodal imaging of the right fundus showed extensive myelination encircling the disc 360 degrees, obscuring the disc margins. The left eye appeared normal. Type 2 MNRF, unilateral myopia, and amblyopia are poor prognostic factors in this case.https://journals.lww.com/10.4103/tjosr.tjosr_107_24amblyopiamyelinated nerve fibersmyopiastraatsma syndrome |
| spellingShingle | Rajwinder Kaur Navdeep Kaur Balbir Khan Kirandeep Kaur Bharat Gurnani Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome TNOA Journal of Ophthalmic Science and Research amblyopia myelinated nerve fibers myopia straatsma syndrome |
| title | Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome |
| title_full | Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome |
| title_fullStr | Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome |
| title_full_unstemmed | Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome |
| title_short | Unilateral 360° Peripapillary Myelinated Nerve Fibers with Axial Myopia and Amblyopia: Straatsma Syndrome |
| title_sort | unilateral 360° peripapillary myelinated nerve fibers with axial myopia and amblyopia straatsma syndrome |
| topic | amblyopia myelinated nerve fibers myopia straatsma syndrome |
| url | https://journals.lww.com/10.4103/tjosr.tjosr_107_24 |
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