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Abhishek Karra and Parth Saraf
This 24YO male presented with a history of poor vision in his left eye since childhood. Best-corrected vision was 20/20 in his normal OD and 20/80 OS. Axial length was 23.95 mm OD and 26.02 mm OS.
Fundus photography shows a large area of dense myelinated nerve fiber layer (NFL) extending outward from the tilted disc, sparing the macula. Fainter myelination extends into the nasal periphery.
Learning Points:
Myelinated NFL occurs when retinal nerve fibers develop a myelin sheath, which usually stops posterior to the lamina cribrosa. The myelination may represent an oligodendrocytic choristoma (Rao et al, Retina 2019;39:1125-1132).
Myelinated NFL is an uncommon, either unilateral or bilateral, usually isolated finding present at birth. The myelinated NFL is often connected to the optic nerve. Most patients are asymptomatic, although some may have a corresponding relative scotoma.
Our patient’s findings were consistent with Straatsma syndrome, which consists of the triad of unilateral axial myopia, amblyopia, and myelinated NFL (Tarabishy et al, Surv Ophthalmology 2007;52:588-596). Bilateral and hyperopic (“reverse Straatsma”) variants have also been reported (Shenoy et al, Middle East Afr J Ophthalmol 2011;18:65-66).
Ophthalmol Retina. 2026 Oct;10(10):1047-1055. doi: 10.1016/j.oret.2026.06.001.
RAM Clinical features, management and outcomes. Most spontaneously improve, BUT selective treatment for more severe presentations may be beneficial — Retrospective, BPEI, 161 eyes
Purpose: To describe baseline characteristics, management patterns, and long-term outcomes in a contemporary cohort of eyes with retinal arterial macroaneurysms (RAM) managed with observation versus treatment.
Design: Retrospective cohort study.
Subjects: Adult patients (aged ≥18 years) diagnosed with RAM at Bascom Palmer Eye Institute between January 2013 and December 2025.
Methods: Baseline demographics, ocular findings, ancillary optical coherence tomography imaging, treatments, and visual outcomes were recorded. Continuous variables were compared using two-tailed Student t-tests and Wilcoxon signed-rank tests, as appropriate. Categorical variables were compared using chi-square or Fisher exact tests. Statistical significance was defined as P < 0.05.
Main outcome measures: Resolution of RAM, time to resolution, and changes in best-corrected visual acuity (BCVA) at final follow-up.
Results: A total of 161 eyes (156 patients) met inclusion criteria. The mean age was 73.7 years (range 19-97), and 104 (67%) were female. Hypertension was present in 126 (81%) patients. Overall, 110 eyes (68%) were managed with observation, and 51 eyes (32%) were managed with early treatment. The treatment cohort had, on average, worse BCVA compared to the observation cohort (20/160 vs. 20/50, P < 0.0001) and more frequently reported macula-involving hemorrhage (78% vs. 52%; P = 0.0024) at the initial visit. In the observation cohort, 6% of eyes developed symptomatic hemorrhage prompting treatment, which occurred an average of 250 days after the initial diagnosis. In the treatment cohort, 59% of eyes were treated with antivascular endothelial growth factor monotherapy, 22% were treated with focal laser monotherapy, 16% were treated with combination therapy, and 4% were treated with vitrectomy with tissue plasminogen activator. Resolution of RAM, defined as fibrosis of the RAM with resorption of surrounding hemorrhage and/or edema, occurred in 100% of eyes in both cohorts of patients. BCVA at last follow-up was not statistically different between groups (20/50 observation vs. 20/70 treatment, P = 0.0730). However, time to resolution of RAM was more rapid in the treatment cohort (362 vs. 536 days; P = 0.0170).
Conclusions: This descriptive cohort of patients with RAM suggests that most cases will improve spontaneously; however, selective treatment of eyes with more severe initial presentation may be beneficial.
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