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Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: A Distinctive Henle Fiber Layer Geometry.
Ophthalmol Retina. 2020 09; 4(9):946-953.OR

Abstract

PURPOSE

To expand the multimodal imaging correlation of the concentric macular rings (CMR) sign seen on ultra-widefield fundus photography in patients with foveal hypoplasia.

DESIGN

Retrospective case series.

PARTICIPANTS

Thirty-two patients with foveal hypoplasia who demonstrated the CMR sign on ultra-widefield fundus photography.

METHODS

Inclusion criterion was the presence of a CMR sign detected on ultra-widefield fundus photography.

MAIN OUTCOME MEASURES

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.

RESULTS

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).

CONCLUSIONS

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.

Authors+Show Affiliations

Centre Hospitalier Universitaire de l'Hôpital Nord, Marseille, France. Electronic address: pramtohul@me.com.Centre Hospitalier Universitaire de l'Hôpital Nord, Marseille, France.Centre Hospitalier Universitaire de l'Hôpital Nord, Marseille, France.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32418844

Citation

Ramtohul, Prithvi, et al. "Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: a Distinctive Henle Fiber Layer Geometry." Ophthalmology. Retina, vol. 4, no. 9, 2020, pp. 946-953.
Ramtohul P, Comet A, Denis D. Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: A Distinctive Henle Fiber Layer Geometry. Ophthalmol Retina. 2020;4(9):946-953.
Ramtohul, P., Comet, A., & Denis, D. (2020). Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: A Distinctive Henle Fiber Layer Geometry. Ophthalmology. Retina, 4(9), 946-953. https://doi.org/10.1016/j.oret.2020.03.022
Ramtohul P, Comet A, Denis D. Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: a Distinctive Henle Fiber Layer Geometry. Ophthalmol Retina. 2020;4(9):946-953. PubMed PMID: 32418844.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multimodal Imaging Correlation of the Concentric Macular Rings Sign in Foveal Hypoplasia: A Distinctive Henle Fiber Layer Geometry. AU - Ramtohul,Prithvi, AU - Comet,Alban, AU - Denis,Danièle, Y1 - 2020/04/06/ PY - 2020/01/10/received PY - 2020/03/18/revised PY - 2020/03/25/accepted PY - 2020/5/19/pubmed PY - 2021/7/28/medline PY - 2020/5/19/entrez SP - 946 EP - 953 JF - Ophthalmology. Retina JO - Ophthalmol Retina VL - 4 IS - 9 N2 - PURPOSE: To expand the multimodal imaging correlation of the concentric macular rings (CMR) sign seen on ultra-widefield fundus photography in patients with foveal hypoplasia. DESIGN: Retrospective case series. PARTICIPANTS: Thirty-two patients with foveal hypoplasia who demonstrated the CMR sign on ultra-widefield fundus photography. METHODS: Inclusion criterion was the presence of a CMR sign detected on ultra-widefield fundus photography. MAIN OUTCOME MEASURES: 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. RESULTS: 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). CONCLUSIONS: 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. SN - 2468-6530 UR - https://www.unboundmedicine.com/medline/citation/32418844/Multimodal_Imaging_Correlation_of_the_Concentric_Macular_Rings_Sign_in_Foveal_Hypoplasia:_A_Distinctive_Henle_Fiber_Layer_Geometry_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2468-6530(20)30124-X DB - PRIME DP - Unbound Medicine ER -