Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus · 2026
Concentric macular rings: ultrawide-field biomarker in pediatric albinism.
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Caminal-Caramés Marta, Català Mora Jaume, Díaz-Cascajosa Jesus, Pedemonte Sarrias Eduard
Аннотация
PURPOSE: To evaluate the prevalence and clinical relevance of the concentric macular rings (CMR) sign on ultrawide-field (UWF) imaging as a noninvasive marker of foveal hypoplasia (FH) in pediatric patients with albinism, in whom optical coherence tomography (OCT) acquisition is limited by nystagmus and poor fixation.
METHODS: The medical records of pediatric patients with albinism who underwent UWF imaging, green-filter UWF, and OCT were reviewed retrospectively. CMR presence, morphology and size were assessed using standard UWF imaging, green-filter, and red-filter modalities. Best-corrected visual acuity (BCVA), cycloplegic refraction, FH grade, macular pigmentation, genetic mutations, and associated features (nystagmus, strabismus) were analyzed.
RESULTS: A total of 128 eyes of 64 patients (mean patient age, 11.3 years; 55% males) were analyzed. Mean BCVA was 0.5 logMAR; and 62.5% of eyes had nystagmus. FH stages 3 and 4 were observed in 48.3% and 45.7% of eyes, respectively. CMR sign was identified in 70.4% on UWF retinography and in 75% using the green filter. Mean largest ring diameter was 3.4 ± 1.05 mm; median ring count was 10 per eye. Interobserver agreement was substantial on standard UWF and almost perfect on green-filter UWF (κ = 0.815). Ring number correlated with FH grade (P = 0.006) and BCVA (P = 0.034). The presence of this sign was significantly associated with hyperopia (P = 0.001), macular hypopigmentation, and FH grade (P < 0.001).
CONCLUSIONS: The CMR sign on UWF retinography, particularly with a green filter, may serve as a noninvasive diagnostic aid for FH in albinism, especially in pediatric patients or those with fixation difficulties.
METHODS: The medical records of pediatric patients with albinism who underwent UWF imaging, green-filter UWF, and OCT were reviewed retrospectively. CMR presence, morphology and size were assessed using standard UWF imaging, green-filter, and red-filter modalities. Best-corrected visual acuity (BCVA), cycloplegic refraction, FH grade, macular pigmentation, genetic mutations, and associated features (nystagmus, strabismus) were analyzed.
RESULTS: A total of 128 eyes of 64 patients (mean patient age, 11.3 years; 55% males) were analyzed. Mean BCVA was 0.5 logMAR; and 62.5% of eyes had nystagmus. FH stages 3 and 4 were observed in 48.3% and 45.7% of eyes, respectively. CMR sign was identified in 70.4% on UWF retinography and in 75% using the green filter. Mean largest ring diameter was 3.4 ± 1.05 mm; median ring count was 10 per eye. Interobserver agreement was substantial on standard UWF and almost perfect on green-filter UWF (κ = 0.815). Ring number correlated with FH grade (P = 0.006) and BCVA (P = 0.034). The presence of this sign was significantly associated with hyperopia (P = 0.001), macular hypopigmentation, and FH grade (P < 0.001).
CONCLUSIONS: The CMR sign on UWF retinography, particularly with a green filter, may serve as a noninvasive diagnostic aid for FH in albinism, especially in pediatric patients or those with fixation difficulties.