- Visibility 173 Views
- Downloads 204 Downloads
- Permissions
- DOI 10.18231/j.ijceo.v.11.i.3.32
-
CrossMark
- Citation
Straatsma syndrome: A case report
- Author Details:
-
Ashwini G
-
Aditi Dhamgaye *
-
Jyothis Chandy
-
Manjunath K R
The aim of our articleis to report a case of Straatsma syndrome, a rare disease characterized by characteristic triad of axial myopia, unilateral myelinated retinal nerve fibres and amblyopia. The patients will be usually asymptomatic, however few cases may present with significant visual abnormalities, commonlyaxial myopia and amblyopia, constituting the Straatsma syndrome. We report a case of 13 year old boy who presented to us with this rare syndrome. A 13 year old boy presented with a history of decreased vision in right eye since childhood. On examination his BCVA in right eye was 6/24, N6, while left eye it was 6/6, N6. Cycloplegic refraction revealed refractive error of -6.50DS, -1.50DC at 168° in right eye. Fundoscopy was fundamental in diagnosing this case. Dilated fundus examination of right eye revealed blurred optic disc margin and presence of myelinated retinal nerve fiber layer along superior- temporal arcade and inferio- temporal arcade,contiguous with optic nerve head, more extensivesuperiorly. B scan report of right eye revealed vitreous detachment. Straatsma syndrome may be included in the differential diagnosis of leucokoria and can be suspected in patients presenting with refractive errors.
Though the prognosis is poor to moderate, better results may be achieved with an early diagnosis and managing amblyopia. It can yield good results, despite high degree of anisometropia and low visual acuity.
References
- Straatsma BR, Heckenlively JR, Foos RY, Shahinian JK. Myelinated retinal nerve fibers associated with ipsilateral myopia, amblyopia, and strabismus. Am J Ophthalmol. 1979;88(3):506–10.
- Juhn AT, Houston SK 3rd, Mehta S. Bilateral Straatsma syndrome with nystagmus. Retin Cases Brief Rep. 2015;9(3):198–200.
- Tarabishy AB, Alexandrou TJ, Traboulsi EI. Syndrome of myelinated retinal nerve fibers, myopia, and amblyopia: a review. Surv Ophthalmol. 2007;52(6):588–96.
- Vide-Escada A, Prior Filipe H. Unusual Straatsma syndrome - how dogmatic is a bad prognosis? Am J Ophthalmol Case Rep. 2017;8:71–73.
- Shenoy R, Bialasiewicz AA, Al Barwani B. Bilateral hypermetropia, myelinated retinal nerve fibers, and amblyopia. Middle East Afr J Ophthalmol. 2011;18(1):65–6.
- Yalcın E, Balcı O, Akıngol Z. Association of extensive myelinated nerve fibers and high degree myopia: case report. Indian J Ophthalmol. 2013;61(10):606–7.
- Kee C, Hwang JM. Visual prognosis of amblyopia associated with myelinated retinal nerve fibers. Am J Ophthalmol. 2005;139(2):259–65.
- Prakalapakorn SG, Buckley EG. Acquired bilateral myelinated retinal nerve fibers after unilateral optic nerve sheath fenestration in a child with idiopathic intracranial hypertension. J AAPOS. 2012;16(6):534–8.
- Naghib J. Triad of myelinated retinal nerve fibers, axial myopia and amblyopia. J Ophthalmic Vis Res. 2010;5(4):284–5.
- Sevik MO, Aykut A, Karaman NF, Şahin Ö. Straatsma Syndrome: should visual prognostic factors be taken into account? a case report. Turk J Ophthalmol. 2021;51(6):398–402.
- You Q, Xu L, Jonas JB. Prevalence of myelinated retinal nerve fibres in urban and rural adult Chinese populations: the Beijing Eye Study. Acta Ophthalmol Scand. 2007;85(6):631–2.
- Funnell CL, George NDL, Pai V. Familial myelinated retinal nerve fibres. Eye (Lond). 2003;17(1):96–7.
- Lam DSC, Leung KS, Mohamed S, Chan WM, Palanivelu MS, Cheung CYL, et al. Regional variations in the relationship between macular thickness measurements and myopia. Invest Ophthalmol Vis Sci. 2007;48(1):376–82. Ashwini G / Indian Journal of Clinical and Experimental Ophthalmology 2025;11(3):563–567 567
- Gharai S, Prakash G, Kumar DA, Jacob S, Agarwal A, Arora V. Spectral domain optical coherence tomographic characteristics of unilateral peripapillary myelinated retinal nerve fibers involving the macula. J AAPOS. 2010;14(5):432–4.
- Nogueira G, Heringer J, Paulo Filho L, Raupp N, Morizot E. Straatsma syndrome: two case reports. Rev Bras Oftalmol. 2017;76(5):262–4.
How to Cite This Article
Vancouver
G A, Dhamgaye A, Chandy J, R MK. Straatsma syndrome: A case report [Internet]. Indian J Clin Exp Ophthalmol. 2025 [cited 2025 Oct 02];11(3):563-567. Available from: https://doi.org/10.18231/j.ijceo.v.11.i.3.32
APA
G, A., Dhamgaye, A., Chandy, J., R, M. K. (2025). Straatsma syndrome: A case report . Indian J Clin Exp Ophthalmol, 11(3), 563-567. https://doi.org/10.18231/j.ijceo.v.11.i.3.32
MLA
G, Ashwini, Dhamgaye, Aditi, Chandy, Jyothis, R, Manjunath K. "Straatsma syndrome: A case report ." Indian J Clin Exp Ophthalmol, vol. 11, no. 3, 2025, pp. 563-567. https://doi.org/10.18231/j.ijceo.v.11.i.3.32
Chicago
G, A., Dhamgaye, A., Chandy, J., R, M. K.. "Straatsma syndrome: A case report ." Indian J Clin Exp Ophthalmol 11, no. 3 (2025): 563-567. https://doi.org/10.18231/j.ijceo.v.11.i.3.32