To expand the multimodal imaging correlation of the concentric macular rings (CMR) sign seen on ultra-widefield fundus photography in patients with foveal hypoplasia.
Retrospective case series.
Thirty-two patients with foveal hypoplasia who demonstrated the CMR sign on ultra-widefield fundus photography.
Inclusion criterion was the presence of a CMR sign detected on ultra-widefield fundus photography.
Noninvasive multimodal retinal imaging, including ultra-widefield fundus photography, structural OCT, near-infrared reflectance, and blue fundus autofluorescence, were investigated. Horizontal dense B-scans and en face OCT images were acquired.
Evaluation of all patients (n = 32 patients) demonstrated a CMR sign on ultra-widefield fundus photography. Structural OCT scans were consistent with foveal hypoplasia in all patients. En face OCT images acquired at the level of the Henle fiber layer highlighted similar concentric rings around the location of the incipient fovea. The series of concentric rings was not visible at any other level of the macula. A significant correlation was found between the horizontal diameter of the largest outer ring and foveal hypoplasia grades (P < 0.0001).
The CMR sign seen on ultra-widefield fundus imaging may be a distinctive feature of foveal hypoplasia and can support this diagnosis, especially in patients in whom OCT cannot be performed (patients with poor fixation or nystagmus or young children). Multimodal imaging correlation suggests that these concentric rings occur exclusively at the level of the Henle fiber layer. This distinctive Henle fiber layer geometry may reflect an arrested development stage in the timeline of foveal maturation.