BACKGROUND: Fovea plana is indicative of an immature inner retina, yet its association with epimacular membrane (ERM) remains unexplored. This study aims to investigate the prevalence of fovea plana in the contralateral eye of patients diagnosed with ERM.
METHODS: A retrospective analysis was conducted on consecutive patients in a monocentric tertiary ophthalmic department between January and April 2021. The study included all patients referred for ERM, with dense optical coherence tomography (OCT) B-scans utilized to assess the incidence of fovea plana in the contralateral eye. Patients with bilateral ERM were excluded from the analysis.
RESULTS: Out of 181 patients, 26 exhibited fovea plana in the contralateral eye, resulting in an incidence rate of 14.3%. Demographic characteristics, OCT patterns, and indications for surgery did not differ significantly between patients with and without fovea plana.
CONCLUSIONS: The prevalence of fovea plana in patients with ERM did not exhibit a statistically significant increase compared to the reported incidence in patients undergoing preoperative cataract surgery.
CONCLUSIONS: In our cohort, the incidence of fovea plana in patients with ERM was determined to be 14.3%.

This study aimed to evaluate foveal avascular zone (FAZ) features and macular flow density (FD) in various retinal layers in a cohort of patients with foveal hypoplasia (FH) using optical coherence tomography angiography (OCTA), in order to characterize microvascular abnormalities and explore their potential clinical significance. FAZ parameters and FD, as well as retinal thickness and volume values were analyzed and compared between patients with FH and an age- and gender-matched control cohort. Correlations between disease severity and visual acuity (VA), as well as between disease severity and FAZ features were evaluated. A total of 19 eyes with FH and 19 control eyes were included. The study group showed significantly higher FD values in the foveal sectors of the superficial and deep capillary plexus compared to controls. FAZ area, perimeter, and acircularity index (ACI) were noticeably altered in eyes with FH; however, they did not correlate with disease severity. Visual acuity was negatively correlated with disease severity. The results of this study provide evidence of altered microvasculature architecture specifically in the foveal sectors of patients with FH. The higher FD values in the foveal sectors of FH patients suggest a potential compensatory response of the retinal microvasculature. FAZ parameters and FD values of the foveal sectors could be used as part of an OCTA-based grading system in FH patients.

OBJECTIVE: To report the association of tilted disc (TD) with fovea plana.
METHODS: Monocentric retrospective study of consecutive eyes diagnosed with fovea plana, assessed by spectral-domain optical coherence tomography. Analysis of the medical charts and imaging findings of patients to collect demographics, the visual acuity, and the clinical context. The presence of associated conditions was checked by two independent readers in order to classify fovea plana as isolated or part of other conditions.
RESULTS: Twenty-one patients, 9 men and 12 women, aged 12 to 91 years, were included. Fovea plana was isolated and asymptomatic in 10 (47.6%) patients. In 6 (28.5%) patients, fovea plana was associated with ocular albinism and/or nystagmus. In 6 (28.5%) patients, fovea plana was associated with an obliquity of the optic disc typical of TD, isolated (5 cases), or associated with nystagmus (1 case).
CONCLUSIONS: An association between TD and fovea plana had been reported only once in the literature and had been considered likely coincidental. However, this association could be more common than initially reported and suggests a common pathological process in eye development during embryogenesis.

OBJECTIVE: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A).
METHODS: A retrospective observational study including patients diagnosed with Best disease.
METHODS: Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), p = 0.9) diagnosed with Best disease were included. Patients\' eyes were categorized into two groups: Eyes with a fovea plana appearance (\'FP group\') and eyes without fovea plana appearance (\'no FP group\'), based on the foveal appearance on B-scan SD-OCT.
METHODS: Cross-sectional OCT images were assessed for the persistence of inner retinal layers (IRL) and OCT-A was analyzed for the presence of a foveal avascular zone (FAZ), the size of which was determined when applicable.
RESULTS: Overall, 16 eyes (27.1%) of 9 patients had a fovea plana appearance (\'FP group\') with the persistence of IRL, and 43 eyes (72.9%) of 23 patients did not have fovea plana appearance (\'no FP group\'). Among FP eyes, OCT-A performed in 13 eyes showed bridging vessels through the FAZ in 100% of eyes with OCT-A. Using Thomas classification, 14 out of the 16 eyes with fovea plana (87.5%) had atypical foveal hypoplasia, and the 2 others (12.5%) had a grade 1b fovea plana.
CONCLUSIONS: In our series, foveal hypoplasia was present in 27.1% of patients with Best disease. OCT-A showed bridging vessels through the FAZ in all eyes. These findings highlight the microvascular changes associated with Best disease, which can be an early sign of the disease in patients with a family history.

BACKGROUND: Fovea plana is defined as an immature macula diagnosed by OCT, showing the unusual shunt of the inner retinal layers into the fovea. The incidence of fovea plana in the adult population remains to be determined. The aim of this study was to determine the incidence of fovea plana in the French population with age-related cataract.
METHODS: Consecutive patients who underwent cataract surgery in Rothschild Foundation Hospital, France, between January and March 2021, with preoperative analyzable OCT scans available, were retrospectively screened in order to determine the incidence of fovea plana in these population. Ophthalmological characteristics of patients were reported, and detailed.
RESULTS: Fovea plana was encountered in 20 out of 204 patients during the 3 months corresponding to an incidence of 9.8%. One of those patients had stage 2 fovea plana.
CONCLUSIONS: Although fovea plana is defined as an immature macula, it is not rare in preoperative population. This macular aspect was not associated with poor visual acuity in our cohort.

Albinism is a group of genetic disorders characterized by general skin and retinal hypopigmentation. It is in most cases an autosomal recessive condition. Foveal hypoplasia (FH) is one of the main criteria for the diagnosis of albinism. The aim of this study was to analyze the macular profile of the parents of patients with albinism.
This study included a case series of 27 patients with albinism seen in Rothschild Foundation between April 2017 and February 2020. Spectral-domain optical coherence tomography (SD-OCT) and OCT angiography (OCT-A) were performed in every patient when possible and in every available parents. FH was graded according to Thomas\' classification based on OCT. Next generation sequencing-based gene panel testing was performed in parents and children when a FH was detected on OCT in a parent.
Twenty-seven patients with albinism were examined. Nine parents had FH based on the OCT B-scan (33%). In parents without FH based on the SD-OCT B-scan (67%), OCT-A showed a reduced avascular zone in the deep vascular plexus in 4 parents. Six parents carried variants that could explain their phenotype, including TYR R402Q hypomorphic alleles.
This study showed the presence of FH in parents of patients with albinism, and aimed to genetically explain this phenotype.

A pathognomonic macular ripple sign has been reported with scanning laser ophthalmoscopy images in patients with foveal hypoplasia, though the optical basis of this sign is presently unknown. Here we present a case series of seven individuals with foveal hypoplasia (based on spectral domain optical coherence tomography). Each patient underwent infrared scanning laser ophthalmoscopy retinal imaging in both eyes, acquired with and without a polarization filter and assessment for a ripple-like effect in the fovea. On imaging, macular ripples were present in all eyes with foveal hypoplasia when using a polarization filter, but not when imaged without the filter. We conclude that the macular ripple sign is an imaging artifact attributable to the unique pattern of phase retardation of the Henle fiber layer in the setting of foveal hypoplasia. By utilizing a polarization filter with retinal photography, this feature can be exploited to promptly identify foveal hypoplasia in settings where OCT is not possible due to nystagmus.

This paper aims to study adaptative vascular arrangements in idiopathic fovea plana with volume-rendered optical coherence tomography angiography (OCTA). A retrospective review of two cases of idiopathic fovea plana (mean age: 26.5 years) and two age-matched controls imaged with OCTA was conducted using spectral-domain OCTA (RTVue XR Avanti, Optovue, Inc., Fremont, CA) equipped with the AngioVue software. Both en face OCTA slabs and OCTA b scans were processed through Fiji software (http://fiji.sc; software version 2.0.0-rc-68/1.52e), and then extracted as image sequences for volume rendering reconstructions using the ImageVis3D volume rendering system (3.1.0 release). Eyes with idiopathic fovea plana demonstrated a regular superficial vascular plexus connecting to a single vascular monolayer representing the deeper vascular plexuses. At this location, several vertical short path connections were demonstrated, in contraposition with normal eyes where short path connections were infrequently observed. Advances in three-dimensional OCTA reconstruction increase the understanding of vascular connections and arrangement in retinal plexuses and possible anatomical variations that cannot be detected with conventional two-dimensional b scans.

Fovea plana is a congenital condition characterized by anatomic absence of the foveal pit. It may be isolated or associated with congenital ocular anomalies. In this report, we present a case of fovea plana associated with situs inversus of the optic disc, optic disc hypoplasia, tilted optic disc, and prepapillary vascular loop and with best corrected visual acuity of 20/32. The aim of this report is to demonstrate the coexistence of very rare multiple optic disc anomalies and fovea plana, and also to emphasize that the use of multimodal imaging methods facilitates the identification of rare anomalies.

Albinism can present with a wide range of ophthalmic findings and variable expressivity. With the use of optical coherence tomography, there has been increasing awareness of the variability of macular findings in this condition.
Case report.
We present a case of oculocutaneous albinism with bilateral atypical lamellar holes which may represent part of the spectrum of retinal abnormalities in this condition.
Optical coherence tomography can be helpful in diagnosing albinism. Variable expressivity leads to a range of macular pathology in albinism which may include atypical lamellar holes as described in this patient.