Dataset Information

Progressive myelinated retinal nerve fibers in a 10-year-old boy with Crouzon syndrome after craniofacial surgery.

ABSTRACT:

Purpose

We present a case of a 10-year-old boy with Crouzon syndrome that demonstrates progressive myelinated retinal nerve fibers (MRNF).

Observations

A 10-year-old boy was referred for ophthalmic examination due to clusters of opaque white fibers around his optic nerve. Past surgical history includes craniofacial surgery at 3 years of age secondary to the deteriorating vision from increased intracranial pressure and papilledema. Upon examination (now 6.5 years post-craniofacial surgery), the patient denied any ocular complaints. The fundus examination showed progressively enlarged myelination of the retinal nerve fiber layer (right eye > left eye). Although previous cases of MRNF with Crouzon syndrome have been reported, our case is unique given its post-operative status with early onset of MRNF.

Conclusion and importance

This case report documents the photographic progression of bilateral myelinated retinal nerve fibers (MRNF) in a pediatric case of Crouzon syndrome post-craniofacial surgery secondary to increased intracranial pressure and papilledema. Based on our patient, craniofacial decompression surgery may not prevent the development of MRNF. The exact mechanisms of MRNF are still being studied. Further investigations correlating craniofacial surgeries, increased intracranial pressure, and progression of myelinated retinal nerve fibers are needed to understand this process.

SUBMITTER: Saffra NA

PROVIDER: S-EPMC10374455 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature

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Publications

Progressive myelinated retinal nerve fibers in a 10-year-old boy with Crouzon syndrome after craniofacial surgery.

Saffra Norman A NA Emborgo Trisha S TS Ranka Milan P MP Kirsch David S DS

American journal of ophthalmology case reports 20230720

<h4>Purpose</h4>We present a case of a 10-year-old boy with Crouzon syndrome that demonstrates progressive myelinated retinal nerve fibers (MRNF).<h4>Observations</h4>A 10-year-old boy was referred for ophthalmic examination due to clusters of opaque white fibers around his optic nerve. Past surgical history includes craniofacial surgery at 3 years of age secondary to the deteriorating vision from increased intracranial pressure and papilledema. Upon examination (now 6.5 years post-craniofacial ...[more]

PMID: 37521804

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Project description: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.

Dataset Information

Progressive myelinated retinal nerve fibers in a 10-year-old boy with Crouzon syndrome after craniofacial surgery.

Purpose

Observations

Conclusion and importance

Publications

Progressive myelinated retinal nerve fibers in a 10-year-old boy with Crouzon syndrome after craniofacial surgery.

Similar Datasets

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