Purpose: This case report presents a rare case of bilateral Straatsma syndrome with nystagmus and documents additional findings related to spectral domain optical coherence tomography and ocular fundus images.
Methods: Case report with fundus photography, fluorescein angiography, indocyanine green angiography, and spectral domain optical coherence tomography correlations.
Results: A 22-year-old man presented with extensive bilateral myelination of the retinal nerve fiber layer that encompassed both posterior poles. The patient had concomitant ocular findings of high myopia, strabismus, amblyopia, and congenital nystagmus.
Conclusion: This case describes an unusual presentation of Straatsma syndrome and investigates the clinical features of this disease. The patient's constellation of findings is most easily explained as bilateral myelination serving as the catalyst for the associated findings of myopia and nystagmus.
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http://dx.doi.org/10.1097/ICB.0000000000000137 | DOI Listing |
Ophthalmic Surg Lasers Imaging Retina
November 2024
A 34-year-old asymptomatic woman with a history of amblyopia in her right eye presented for a routine examination. Fundus examination revealed broad myelinated nerve fibers along with telangiectatic vessels. Wide-field fluorescein angiography revealed no evidence of ischemia or active retinal neovascularization.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
December 2024
Department of Ophthalmology, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-0033, Japan.
J AAPOS
October 2024
Department of Ophthalmology, Mayo Clinic Rochester, Rochester, Minnesota. Electronic address:
Straatsma syndrome is an uncommon ocular condition generally comprised of myelinated retinal nerve fiber layer, myopia, and amblyopia. Associated ocular disorders include strabismus, nystagmus, optic nerve hypoplasia, and iris heterochromia. We report the case of a 6-year-old girl with unilateral Straatsma syndrome and an acquired cataract that required surgical extraction.
View Article and Find Full Text PDFJ Vitreoretin Dis
April 2024
Massachusetts Eye and Ear, Harvard Medical School, Boston, MA, USA.
To report a case of macular hole (MH)-associated retinal detachment (RD) with Straatsma syndrome. A clinical case was retrospectively reviewed. A 39-year-old woman with Straatsma syndrome was treated for an MH-associated RD.
View Article and Find Full Text PDFEur J Ophthalmol
August 2024
Department of Retina and Vitreous, Narayana Nethralaya, Rajaji Nagar, Bengaluru. Karnataka, India.
Purpose: To study cases diagnosed of myelinated retinal nerve fibres (MRNF) continuous with the optic disc and describe the plausible pathogenic mechanism for the ocular features in Straatsma syndrome.
Methods: This retrospective observational study includes clinically diagnosed MRNF cases. MRNF, myopia, and amblyopia defined Straatsma syndrome.
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