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Dense white proliferation observed in periodic exam
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Images: Morier AM
In July 2011, a 22-year-old man presented for a periodic eye examination. His ocular history was significant only for myopia in both eyes that was first diagnosed at the age of 9 years. Otherwise, his medical and family ocular history were unremarkable.
On examination, the patient’s best-corrected visual acuity was 20/20 in each eye. His manifest refraction was -7.75 D -1.00 D x 40 OD and -7.00 D -0.75 D x 175 OS. His anterior segment examination was unremarkable in both eyes.
We observed a region of dense white proliferation in the right eye that obscured the retinal vessels of the inferior arcade but did not involve the optic nerve head. The posterior segment of the left eye was unremarkable. Baseline fundus photo documentation was obtained, and the patient was observed.
On follow-up examination in July 2015, significant enhancement of the previously documented area was seen. There was no reported change otherwise in ocular or medical history.
On the most recent follow-up in July 2016, the patient’s clinical presentation still showed continual progression without evidence of inflammation, remodeling or vascular changes of note. Corresponding visual fields from baseline to current were also obtained and analyzed.
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Myelinated retinal nerve fibers (MNFL) are developmental anomalies that are present in approximately 1% of eyes (Straatsma et al.). Myelination of the visual pathway has been demonstrated to occur by as early as 32 weeks of gestation and continues for several years into early childhood (Ali et al.).
Most cases involving the intraocular portion of the retinal neural pathway are asymptomatic although occasionally can cause symptomatic scotomas when significant myelination is present. Previous reports have noted the rare phenomenon of MNFL that can be progressive and have suggested that they be considered among the peripapillary choristomas (Rosen et al.).
In these cases, etiology such as trauma, status post optic nerve sheath fenestration, history of optic nerve drusen, or conditions such as Arnold-Chiari malformation or von Recklinghausen’s disease have been associated as the primary factor. However, in our case, there were no known associations detected that could lead to such a dramatic progression in clinical appearance.
We propose that there may be a subset of patients with progression of MNFL as a natural progression of this condition. Further studies are needed to understand all significant risk factors for progression and additional clinical associations. We suggest that the prevalence of this subset is underdiagnosed, and the need for regular monitoring with fundus photographs and serial visual fields over time is essential.
Conversely, optic atrophy of any etiology may result in attenuation or disappearance of the myelination appearance. Reports of such occurrences have been published but are rare (Sowka et al., Katz et al.).
We present a case of clinical progression of a MNFL of idiopathic etiology in an otherwise healthy patient.
- References:
- Ali BH, et al. Am J Ophthalmol. 1994;118:515–517. doi: 10.1016/S0002-9394(14)75805-2.
- Katz SE, et al. J Glaucoma. 1996;5(6):406-409.
- Rosen B, et al. Am J Ophthalmol. 1999;127:471-473. doi.org/10.1016/S0002-9394(98)00377-8.
- Sowka JW, et al. Optom Vis Sci. 2013;90(7):e218-220. doi: 10.1097/OPX.0b013e3182968b1a.
- Straatsma BR et al. Am J Ophthalmol. 1979;88:506-510.
- For more information:
- Joshua O. Mali, MD, is a board-certified ophthalmologist and vitreoretinal surgeon practicing at The Eye Associates, a private multispecialty ophthalmology practice in Sarasota, Fla. He can be reached at: joshuamalimd@gmail.com.
- Albert M. Morier, OD, MA, is in private group practice in Schenectady, N.Y. He works in the Glaucoma Consultants of the Capital Region and is an associate clinical professor of Ophthalmology at Albany Medical Center. He can be reached at: amorier1@nycap.rr.com.
- Edited by Leo P. Semes, OD, FAAO, a professor of optometry, University of Alabama at Birmingham and a member of the Primary Care Optometry News Editorial Board. He may be reached at leopsemes@gmail.com.
Disclosures: Mali and Morier reported no relevant financial disclosures. Semes is an advisor or on the speakers bureau for Alcon, Allergan, Bausch + Lomb, Genentech, Maculogix, OptoVue, Shire and ZeaVision. He is a stockholder with HPO.