Myelinated Retinal Nerve Fibers Research Articles

Reverse straatsma syndrome and lamellar cataract: a case report and review of the literature

Myelinated retinal nerve fiber (MRNF) is a developmental anomaly that is often detected during routine ophthalmological examination, but can be an ocular finding in a wide variety of diseases. An important example of this group is the Straatsma Syndrome. Straatsma Syndrome is an entity that presents with the triad of myopia, amblyopia, and MRNF. When this triad is accompanied by hyperopia instead of myopia, it is referred to as the Reverse Straatsma Syndrome. Here, we present a case of Reverse Straatsma Syndrome accompanied by lamellar congenital cataracts.

Unilateral Myelinated Retinal Nerve Fibers Associated with Ipsilateral Myopia, Amblyopia and Exotropia

This report describes a case of unilateral myelinated retinal nerve fibers associated with ipsilateral myopia, amblyopia and exotropia. A 3-year-old boy presented with the complaint of diminished vision in his right eye. Visual acuity testing with a picture at a distance of 2.5 m revealed 1.5 in the left eye, but right visual acuity was not available. Near visual acuity with dot cards was 0.2 in the right eye and 0.6 in the left eye. The refractive error was -7.25D and +1.50D in the right and left eyes, respectively. Fundus examination revealed extensive myelinated retinal nerve fibers in the right eye, but no abnormal findings in the left eye. The axial length was 24.10 mm and 22.90 mm in the right and left eyes, respectively. Cycloplegic retinoscopy revealed a refractive error of a −7.00D in the right eye and plane in the left eye. Glasses with full optical correction were prescribed, and the patient was evaluated one month later, presenting a best-corrected visual acuity of 0.6 in the right eye and 2.0 in the left eye with a picture at a distance of 2.5 m. Three months later, his visual acuity was 0.7 in the right eye. At this point, amblyopia treatment was started with two hours daily of left eye occlusion. One year following the initial presentation, his visual acuity improved 0.8 in the right eye. The type of fixation, the initial visual acuity, the high myopia and the high anisometropia indicated a most likely bad prognosis. However, the age of diagnosis and wearing status of glasses were advantage for treatment effect in this case. Even though this condition is associated with poor visual prognosis, intensive occlusion therapy should always be attempted, along with the prescription of a cycloplegic refraction, due to unexpected and variable responses to treatment.

Without a Cause: Bilateral Acquired Progressive Myelinated Retinal Nerve Fiber Layers.

Without a Cause: Bilateral Acquired Progressive Myelinated Retinal Nerve Fiber Layers.

Macular Imaging Characteristics in Children with Myelinated Retinal Nerve Fiber and High Myopia Syndrome.

To investigate the macular imaging features in patients with unilateral myelinated retinal nerve fiber (MRNF) and high myopia syndrome. Six patients with unilateral MRNF and high myopia syndrome and 13 myopic controls were enrolled in this study. Spectral domain (SD) optical coherence tomography (OCT), SD enhanced depth imaging OCT, and OCT angiography (OCTA) imaging results of MRNF-affected eyes were compared with the fellow eyes and myopic controls. All patients had abnormal foveal reflex and/or ectopia. No significant difference in retinal thickness parameters were noted between the groups. In OCT scans, posterior vitreous detachment (PVD) was observed in 4 out of the 6 MRNF-affected eyes. Regarding OCTA parameters, only a significant increase in acircularity index was noted in myelinated eyes (p=0.01). All patients demonstrated normal foveal contours, macular structure, and OCTA features except for a higher acircularity index. The incidence of PVD was notably increased in the myelinated eyes.

Myelinated retinal nerve fibers associated with retinal vascular abnormalities and retino-vitreal neovascularization complicated by recurrent vitreous hemorrhage (clinical case)

Myelinated retinal nerve fibres are a rare, benign and asymptomatic lesion that is detected incidentally at fundus examination. In some cases, they may be associated with various abnormalities of the eye or have a complicated course. The article presents a clinical case of myelinated retinal nerve fibers associated with retinal vascular abnormalities and retino-vitreal neovascularization complicated by recurrent vitreous hemorrhage.

Myelinated Retinal Nerve Fibers, a Complicating Factor in Amblyopia Rehabilitation?

Myelinated Retinal Nerve Fibers, a Complicating Factor in Amblyopia Rehabilitation?

Myelinated Retinal Nerve Fibers

We report a case of a 25-year-old-woman, with no pathological history. The patient presented to ophthalmology consultation for a routine eye examination.

Multimodal imaging in myelinated retinal nerve fiber layers

Multimodal imaging in myelinated retinal nerve fiber layers

Myelinated Retinal Nerve Fiber Layer in Idiopathic Intracranial Hypertension: A Rare and Challenging Scenario.

Myelinated Retinal Nerve Fiber Layer in Idiopathic Intracranial Hypertension: A Rare and Challenging Scenario.

Extensive myelinated retinal nerve fibres and bilateral foveal hypoplasia: A specific clinical entity.

Extensive myelinated retinal nerve fibres and bilateral foveal hypoplasia: A specific clinical entity.

Visual outcomes and retinal characteristics in children with myelinated retinal nerve fibers, myopia and amblyopia

Myelinated retinal nerve fibers are a rare congenital developmental anomaly, which may be associated with high myopia and resulting severe amblyopia in unilateral cases. Early detection, refractive correction, and amblyopia treatment can result in improvement of visual function in cases without optic nerve hypoplasia. Limited data regarding macular thickness and lamination are available in such children.

Myelinated Retinal Nerve Fiber Layer

The axons of the retinal ganglion cells compose the retinal nerve fiber layer, located in the inner (closest to the vitreous) retina. These axons coalesce at the optic disc and traverse the scleral canal to become the optic nerve. Posterior to the globe, the axons of the optic nerve become myelinated by supporting oligodendrocytes. Within the retina, however, myelination of these axons is present in only about 0.5% of autopsy eyes and has the appearance of a feathery, white opacity in the inner retina.

Hypermetropia, myelinated retinal nerve fiber and amblyopia: Reverse straatsma syndrome

Myelinated retinal nerve fibers (MRNF) are a common developmental anomaly. MRNF is commonly associated with myopia. The association of MRNF with myopia, amblyopia, and strabismus is well documented. The amblyopia may be refractory to treatment. We report a case of a child with MRNF and amblyopia in the more hyperopic eye, a case of reverse Straatsma syndrome.

Swept-source optical coherence tomography of myelinated retinal nerve fiber layer.

Swept-source optical coherence tomography of myelinated retinal nerve fiber layer.

Myelinated retinal nerve fiber layer: an extensive presentation.

Myelinated retinal nerve fiber layer is rare. It is estimated to occur in 0.5% to 1% of the population and is often detected on routine examination without symptoms. It consists of white or gray-white well-demarcated patches on the outermost surface of the retina that obscure the underlying retinal vessels. It can be associated with axial myopia, amblyopia, and strabismus. A 12-year-old girl with normal development presented with ocular deviation of the right eye. There was no past medical or ocular history. Automatic refraction found in the right eye -9 DP and -1 DP in the left eye. The best Visual acuity was 20/600 in the right eye and 20/20 in the left one. Slit lamp examination of the right eye (A) revealed two vast whitish zones extending from the papilla towards the temporal sector sparing the posterior pole. These lesions covered the temporal vessels with fuzzy and scalloped boundaries. Fundus examination of the left eye was without abnormalities (B). Scan of Optical coherence tomography showed isolated hyper reflectivity of the fiber optic layer, an abnormality of the vitreoretinal interface with posterior shadow cone effect (C).

Straatsma Syndrome: Should Visual Prognostic Factors Be Taken into Account? A Case Report

Straatsma syndrome is the triad of myelinated retinal nerve fibers, myopia, and amblyopia and may be associated with strabismus, nystagmus, hypoplastic optic nerve, and heterochromia iridum. The degree of anisometropia, presence of strabismus, extent of myelination, and macular involvement have been reported to be associated with poor visual acuity after occlusion therapy for amblyopia in this syndrome. Here we present two cases of Straatsma syndrome with different responses to occlusion therapy and discuss their treatment responses according to prognostic factors for post-occlusion visual acuity.

Progressive Myelinated Retinal Nerve Fibers in Children With Craniosynostosis.

The authors found the changes of myelinated retinal nerve fibers in one pair of identical twins with nonsyndromic craniosynostosis and two patients with Crouzon syndrome, who were at risk for impaired lamina cribrosa barrier function. This is the first report of progressive and presumed acquired myelinated retinal nerve fibers in craniosynostosis. [J Pediatr Ophthalmol Strabismus. 2021;58(6):e40-e43.].

Myelinated Retinal Nerve Fiber Progression in a 10-Year Follow-Up. The Beijing Eye Study 2001/2011

To assess the prevalence of myelinated retinal nerve fibers (MRNFs), the rate of their change in a 10-year follow-up, and associations with ocular and systematic parameters in a population-based cohort. Longitudinal population-based cohort study. The Beijing Eye study including 4,439 participants aged 40+ years in 2001 and was repeated in 2011, with 2,695 individuals (66.4% of the surviving) being re-examined in 2011. All participants underwent detailed physical and ocular examinations. MRNFs were diagnosed on fundus photographs and their change was assessed using a flicker method of fundus photographs. Out of 35 eyes (29 participants) with detected MRNFs at baseline (mean prevalence: 0.4% ± 0.26% per eye or 0.7% ± 0.41% per individual), 23 eyes from 20 individuals (17 [85%] participants with unilateral MRNFs) were re-examined in 2011. MRNF enlargement was detected in all 19 eyes (100%) with clear fundus photographs. The mean MRNF area increased from 4,233 ± 3,670 µm2 (range: 178-11,643 µm2) at baseline to 5,243 ± 4,092 µm2 (range: 196-13,297 µm2) at follow-up (P < .001), by 1,010 ± 1,026 µm2 (18-3,967 µm2) or by 47% ± 74% (9%-315%). A larger MRNF increase was associated with an MRNF location distant from the optic disc as compared to a juxtapapillary location (P=.001, standardized regression coefficient beta: -0.53), smaller MRNF area at baseline (P=.006, beta: -0.34), and higher serum concentration of low-density lipoproteins (LDL) (P < .001, beta: 0.57). MRNFs (mean prevalence per eye: 0.4%) showed, in association with higher LDL serum concentration and peripheral located MRNF, an enlargement during a 10-year follow-up, while in the same period no new MRNFs were detected in the total study cohort.

The long-term observation in Chinese children with monocular myelinated retinal nerve fibers, myopia and amblyopia.

BackgroundThe syndrome of monocular myelinated retinal nerve fibers (MRNF), myopia and amblyopia threatens visual development of infants and young children. The efficacy of part-time occlusion therapy remains controversial, and the long-term prognosis of the syndrome remains unclear.MethodsTen children (4.40±2.22 years, 4 boys and 6 girls) with monocular MRNF, myopia and amblyopia were recruited. Both the affected eyes (treatment group) and the fellow eyes (control group) underwent routine ophthalmic examinations, including AL, cycloplegic refraction, best-spectacles-corrected distance visual acuity (BSCDVA), cover test, simultaneous perception, corneal curvature, anterior segment and fundus examinations. Refractive error was corrected by either spectacles or rigid gas permeable (RGP) contact lenses. Part-time occlusion therapy was employed for amblyopia treatment. The retinal characteristics were examined only on the final visit with a Fourier-domain optical coherence tomography (FD-OCT). The speed of myopia progression and axial elongation were calculated. Statistical analysis was performed using SPSS 20 software. Cut-off P values were 0.05.ResultsOn the final visit, six children had normal simultaneous perception but three had strabismus. The mean AL and spherical equivalent (SE) values of the affected eyes were 28.05±1.59 mm and −12.60±4.20 D, respectively, while those of the fellow eyes were 23.67±1.13 mm and −0.66±1.99 D, respectively. AL and SE were significantly different over time and between groups (P<0.0001), but no time*group interaction effect (P>0.05) was observed. The differences in the corneal curvature, mean speed of myopia progression, axial elongation, foveal thickness and parafoveal thickness between the two groups were not significant (P>0.05). However, the perifoveal thickness in the affected eyes was significantly higher than that of the control eyes (P=0.047). In the treatment group, the occlusion dosage positively correlated with the final BSCDVA (R=0.764, P=0.016) but did not correlate with the speed of myopia progression or axial elongation (both P values >0.05).ConclusionsThe affected eyes have longer AL, severer myopia and thicker perifoveal retina when compared with the fellow eyes. Both the affected and the fellow eyes have similar speed of myopia progression as well as that of axial elongation.

Prevalence and Clinical Characteristics of Myelinated Retinal Nerve Fibres in a Chinese Teleophthalmology System

ABSTRACT Purpose: To investigate the prevalence and clinical characteristics of myelinated retinal nerve fibre (MRNF) in a large teleophthalmology system. Methods: All records between January 2015 and December 2015 from Daheng Prust teleophthalmology system were reviewed by 2 ophthalmologists independently. MRNF was classified into continuous group and discontinuous group according to the relationship between MRNF patches and optic disc. The number, total area and location of MRNF patches were analysed. Concomitant ocular diseases were documented. Results: Out of 51469 subjects, MRNF was detected in 304 eyes of 263 subjects with a prevalence rate of 0.51 ± 7.1% per subject and 0.30 ± 5.4% per eye. Among 304 eyes with MRNF, 239 (78.6%) eyes were in continuous group and 65 (21.4%) eyes were in discontinuous group. Single MRNF patch was found in 249 (81.9%) eyes and multiple MRNF patches were found in 55 (18.1%) eyes. MRNF of small size was found in 150 (49.3%) eyes. The ratios of multiple MRNF patches and small-sized MRNF in the continuous group were significantly higher than those in the discontinuous group (P = .014 and P < .001). In continuous group, the MRNF patches were located most frequently in the superior region (68.6%) of the optic disc; In discontinuous group, the MRNF patches were located most frequently in the inferotemporal region (38.5%) of the retina. Epiretinal membrane (12 eyes, 3.9%) was the most common concomitant ocular disease. Conclusion: MRNF is uncommon in China. MRNF usually presents unilaterally and as a single small whitish patch that is connected with optic disc.