DIAGNOSTIC EVALUATION AND SURGICAL TREATMENT OF EXOTROPIA IN A PATIENT WITH STRAATSMA SYNDROME - CASE REPORT

Authors

  • Bekim Teteshi University Clinic for Eye Diseases, Skopje, Republic of North Macedonia, Faculty of Medical Sciences, "Goce Delchev" University in Shtip, Shtip, Republic of North Macedonia
  • Stefan Pandilov University Clinic for Eye Diseases, Skopje, Republic of North Macedonia, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Era Tateshi Veliu City General Hospital "8th September", Ophthalmology Department, Skopje, Republic of North Macedonia, Faculty of Medical Sciences, "Goce Delchev" University in Shtip, Shtip, Republic of North Macedonia

Keywords:

amblyopia, myopia, myelinated retinal nerve fibers, Straatsma syndrome, strabismus

Abstract

Straatsma syndrome is a rare ophthalmic condition characterized by the triad of myelinated retinal nerve fibers, high myopia, and amblyopia. It is often associated with strabismus, nystagmus, heterochromia of the iris, and optic nerve hypoplasia.

We present a case of a 22-year-old patient with this syndrome, who presented with high unilateral myopia, amblyopia, and myelinated retinal nerve fibers along the inferior temporal arcade of the affected eye. In addition, the patient had a hypoplastic optic nerve and a large-angle exotropia, which required surgical correction for cosmetic reasons. Due to untreated amblyopia in childhood, the patient had very poor visual function in the affected eye.

This case highlights the importance of early recognition and diagnosis of Straatsma syndrome, as delayed diagnosis may prevent effective treatment of amblyopia.

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 Pt 1): 506-510. doi: 10.1016/0002-9394(79)90655-x.

Tarabishy AB, Alexandrou TJ, Traboulsi EI. Syndrome of myelinated retinal nerve fibers, myopia, and amblyopia: a review. Surv Ophthalmol 2007; 52(6): 588-596. doi: 10.1016/ j.survophthal.2007.08.016.

Juhn AT, Houston SK 3rd, Mehta S. Bilateral Straatsma syndrome with nystagmus. Retin Cases Brief Rep 2015; 9(3): 198-200. doi: 10.1097/ICB.0000000000000137.

Wang Y, Gonzalez C. Unilateral myelinated nerve fibers associated with hypertropia, strabismus and amblyopia? Reverse straatsma syndrome? Binocul Vis Strabismus Q 2008; 23(4): 235-237. PMID: 19132949.

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. doi: 10.4274/tjo.galenos.2021.32470.

Parulekar MV, Elston JS. Acquired retinal myelination in neurofibromatosis 1. Arch Ophthalmol 2002; 120(5): 659-655. PMID: 12003622.

Ramkumar HL, Verma R, Ferreyra HA, Robbins SL. Myelinated Retinal Nerve Fiber Layer (RNFL): A Comprehensive Review. Int Ophthalmol Clin 2018; 58(4): 147-156. doi: 10.1097/IIO.0000000000000239.

Hunter SF, Leavitt JA, Rodriguez M. Direct observation of myelination in vivo in the mature human central nervous system. A model for the behaviour of oligodendrocyte progenitors and their progeny. Brain 1997; 120 (Pt 11): 2071-2082. doi: 10.1093/ brain/120.11.2071.

Ellis GS Jr, Frey T, Gouterman RZ. Myelinated nerve fibers, axial myopia, and refractory amblyopia: an organic disease. J Pediatr Ophthalmol Strabismus 1987; 24(3): 111-119. doi: 10.3928/0191-3913-19870501-04.

Schmidt D, Meyer JH, Brandi-Dohrn J. Wide-spread myelinated nerve fibers of the optic disc: do they influence the development of myopia? Int Ophthalmol 1996-1997; 20(5): 263-268. doi: 10.1007/BF00131921.

Choudhary A, Kathare R, Gandhi P, Yadav NK, Prabhu V, Bavaskar S et al. Characteristics of myelinated retinal nerve fibres in associated straatsma syndrome. Eur J Ophthalmol 2025; 35(2): 689-694. doi: 10.1177/11206721241272182.

Kee C, Hwang JM. Visual prognosis of amblyopia associated with myelinated retinal nerve fibers. Am J Ophthalmol 2005; 139(2): 259-265. doi: 10.1016/j.ajo.2004.09.018.

Ben Addou Idrissi S, Moutei H, Bennis A, Chraibi F, Abdellaoui M, Benatiya Andaloussi I. Insights into Straatsma syndrome: a pediatric case report. Strabismus. 2025; 1-7. doi: 10.1080/09273972.2025.2564760.

Hittner HM, Antoszyk JH. Unilateral peripapillary myelinated nerve fibers with myopia and/or amblyopia. Arch Ophthalmol 1987; 105(7): 943-948. doi: 10.1001/archopht. 1987.01060070079033.

Bass SJ, Westcott J, Sherman J. OCT in a Myelinated Retinal Nerve Fiber Syndrome with Reduced Vision. Optom Vis Sci 2016; 93(10): 1285-1291. doi: 10.1097/OPX. 0000000000000951.

Yalcın E, Balcı O, Akıngol Z. Association of extensive myelinated nerve fibers and high degree myopia: case report. Indian J Ophthalmol 2013; 61(10): 606-607. doi: 10.4103/ 0301-4738.118455.

Karakosta C, Paraskevopoulos K, Bisoukis A, Bougioukas K, Kokolaki A. Straatsma Syndrome: A Case Series. Cureus 2022; 14(9): e29779. doi: 10.7759/cureus.29779.

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Published

2025-12-16

Issue

Vol. 5 No. 3 (2025): Acad Med J

Section

Case Reports

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Copyright (c) 2025 Bekim Teteshi, Stefan Pandilov, Era Tateshi Veliu

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