Straatsma Syndrome: A Case Series

Karakosta, Christina; Paraskevopoulos, Konstantinos; Bisoukis, Anastasios; Βουγιούκας, Κωνσταντίνος; Kokolaki, Anna

doi:10.7759/cureus.29779

Cureus

2022

DOI: 10.7759/cureus.29779

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Straatsma Syndrome: A Case Series

Christina Karakosta¹,

Konstantinos Paraskevopoulos²,

Anastasios Bisoukis³

et al.

Abstract: The aim of this article is to report two cases of Straatsma syndrome, a rare disease characterized by the traditional triad of unilateral myelinated retinal nerve fibres, axial myopia, and amblyopia.The cases were a five-year-old and a three-year-old girl, both with unilateral myopia, visual acuity of 0.1 (decimal), and myelinated retinal nerve fibres in fundoscopy. Prescription of cycloplegic refraction findings and occlusion of the involved eye was attempted in both cases, but visual acuity did not improve. … Show more

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2024

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Cited by 2 publications

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A case of bilateral straatsma syndrome

Gupta,

Chawla,

Garg

et al. 2024

SAJCRR

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Straatsma syndrome is characterized by a constellation of conditions including amblyopia, nystagmus, and myopia. We present the case of a 10-year-old boy who complained of involuntary back-and-forth eye movements and reduced vision since birth. His visual acuity was measured at 2/60 in both eyes. Examination revealed horizontal pendular nystagmus without ocular misalignment. Fundus examination showed myopic changes, including an anomalous optic disc with extensive myelinated nerve fibers extending from the optic disc along the superior and inferior arcades to the fovea. Axial length measurements were 28.34 mm and 27.96 mm in the right and left eyes, respectively. SD-OCT imaging confirmed thickening of the retinal nerve fiber layer. Treatment included cycloplegic refraction and occlusion therapy, with regular follow-ups every three months utilizing contact lenses with cycloplegic refraction. The etiology of retinal fiber myelination remains unclear; however, it is hypothesized that blurred retinal imaging during critical ocular development stages may lead to axial elongation and subsequent myopia, potentially delaying the development of the lamina cribrosa and facilitating myelination extension.

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A case of bilateral straatsma syndrome

Gupta,

Chawla,

Garg

et al. 2024

SAJCRR

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Leukocoria in a 4-year-old boy

Altamirano,

Gonzalez,

Oke

2024

Digit J Ophthalmol

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A healthy 4-year-old boy referred for evaluation of an abnormal red reflex in the left eye was noted, on fundus examination, to have extensive white, striated lesions surrounding the optic nerve that involved the superior and inferior macular arcades. On further examination, he was found to have ipsilateral high myopia and amblyopia. The triad of unilateral myelinated retinal nerve fibers, myopia, and amblyopia led to a diagnosis of Straatsma syndrome, which requires early treatment to prevent permanent vision loss. Treatment comprised a contact lens for refractive correction of the left eye, patching of the right eye, and full-time polycarbonate protective lenses. With 2 years’ follow-up, the left eye failed to improve appreciably.

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Straatsma Syndrome: A Case Series

Cited by 2 publications

References 9 publications

A case of bilateral straatsma syndrome

A case of bilateral straatsma syndrome

Leukocoria in a 4-year-old boy

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