Subretinal drusenoid deposits (SDD) are findings that can be observed in age-related macular degeneration as well as in ischemic ocular diseases. These deposits are believed to be of prognostic importance, as they have been shown to be associated with choroidal neovascularization. HELLP (hemolysis, elevated liver enzymes, low platelet) syndrome is a condition linked with severe preeclampsia, and it presents ocular findings such as hypertensive retinopathy, serous retinal detachment, and cortical visual impairment. This case report discusses the presence and course of SDD in a female patient who presented with hypertensive retinochoroidopathy secondary to HELLP syndrome.

OBJECTIVE: To investigate the distribution of hyperreflective foci (HRF) in eyes with dry age-related macular degeneration (AMD).
METHODS: We retrospectively reviewed optical coherence tomography (OCT) images of 58 dry AMD eyes presenting HRF. The distribution of HRF according to the early treatment diabetic retinopathy study area was analyzed according to the presence of subretinal drusenoid deposits (SDDs).
RESULTS: We classified 32 eyes and 26 eyes into the dry AMD with SDD group (SDD group) and dry AMD without SDD group (non-SDD group), respectively. The non-SDD group had higher prevalence and density of HRF at the fovea (65.4% and 1.71 ± 1.48) than the SDD group (37.5% and 0.48 ± 0.63, P = 0.035 and P < 0.001, respectively). However, the prevalence and density of HRF in the outer circle area of the SDD group (81.3% and 0.11 ± 0.09) were greater than those of the non-SDD group (53.8% and 0.05 ± 0.06, p = 0.025 and p = 0.004, respectively). The SDD group showed higher prevalence and mean densities of HRF in the superior and temporal area than in the non-SDD group (all, p < 0.05).
CONCLUSIONS: HRF distributions in dry AMD varied according to the presence of SDDs. This might support that the degenerative features may be different between dry AMD eyes with and without SDDs.

To determine whether reticular pseudodrusen (RPD) status, ARMS2/HTRA1 genotype, or both are associated with altered geographic atrophy (GA) enlargement rate and to analyze potential mediation of genetic effects by RPD status.
Post hoc analysis of an Age-Related Eye Disease Study 2 cohort.
Eyes with GA: n = 771 from 563 participants.
Geographic atrophy area was measured from fundus photographs at annual visits. Reticular pseudodrusen presence was graded from fundus autofluorescence images. Mixed-model regression of square root of GA area was performed by RPD status, ARMS2 genotype, or both.
Change in square root of GA area.
Geographic atrophy enlargement was significantly faster in eyes with RPD (P < 0.0001): 0.379 mm/year (95% confidence interval [CI], 0.329-0.430 mm/year) versus 0.273 mm/year (95% CI, 0.256-0.289 mm/year). Enlargement was also significantly faster in individuals carrying ARMS2 risk alleles (P < 0.0001): 0.224 mm/year (95% CI, 0.198-0.250 mm/year), 0.287 mm/year (95% CI, 0.263-0.310 mm/year), and 0.307 mm/year (95% CI, 0.273-0.341 mm/year) for 0, 1, and 2, respectively. In mediation analysis, the direct effect of ARMS2 genotype was 0.074 mm/year (95% CI, 0.009-0.139 mm/year), whereas the indirect effect of ARMS2 genotype via RPD status was 0.002 mm/year (95% CI, -0.006 to 0.009 mm/year). In eyes with incident GA, RPD presence was not associated with an altered likelihood of central involvement (P = 0.29) or multifocality (P = 0.16) at incidence. In eyes with incident noncentral GA, RPD presence was associated with faster GA progression to the central macula (P = 0.009): 157 μm/year (95% CI, 126-188 μm/year) versus 111 μm/year (95% CI, 97-125 μm/year). Similar findings were observed in the Age-Related Eye Disease Study.
Geographic atrophy enlargement is faster in eyes with RPD and in individuals carrying ARMS2/HTRA1 risk alleles. However, RPD status does not mediate the association between ARMS2/HTRA1 genotype and faster enlargement. Reticular pseudodrusen presence and ARMS2/HTRA1 genotype are relatively independent risk factors, operating by distinct mechanisms. Reticular pseudodrusen presence does not predict central involvement or multifocality at GA incidence but is associated with faster progression toward the central macula. Reticular pseudodrusen status should be considered for improved predictions of enlargement rate.
Proprietary or commercial disclosure may be found after the references.

To determine the frequency of multiple OCT biomarkers of intermediate age-related macular degeneration (iAMD) and their relationship with the development of complete retinal pigment epithelium and outer retinal atrophy (cRORA) after 2 years.
Retrospective cohort study.
This retrospective analysis included 330 eyes of 330 consecutive patients with iAMD in ≥ 1 eye who had 24 months of follow-up data.
Spectralis OCT volume scans (49 B-scans over 6 × 6 mm, automatic real time = 6, fovea-centered) at baseline were evaluated for the previously described iAMD biomarkers, including a high-central drusen volume (DV; ≥ 0.03 mm3), intraretinal hyper-reflective foci (IHRF), subretinal drusenoid deposits (SDDs), hypo-reflective drusen cores (hDCs), and a thin or thick (multilayered) double-layer sign (DLS). The age-related macular degeneration (AMD) status in the fellow eye was also assessed and classified as normal or early AMD, iAMD, exudative macular neovascularization, or cRORA.
Incidence of cRORA, odds ratio for demographics, and OCT features.
At month 24, 16.36% (54/330) of the iAMD eyes developed cRORA. Several baseline features, including high-central DV, IHRF, SDD, hDC, thin DLS, and cRORA in the fellow eye, were associated with a significantly greater risk for development of cRORA at 2 years. The odds ratio, 95% confidence interval, P value, and baseline frequencies of these biomarkers were DV (6.510, 2.467-17.176, P < 0.001, 49.1%), IHRF (12.763, 4.763-34.202, P < 0.001, 38.8%), SDD (2.307, 1.003-5.304, P = 0.049, 34.2%), hDC (3.012, 1.152-7.873, P = 0.024, 13.0%), thin DLS (4.517, 1.555-13.126, P = 0.006, 11.8%), and cRORA in the fellow eye (7.184, 1.938-26.623, P = 0.003, 8.2%).
In addition to the 4 previously reported factors that are present in a significant proportion of iAMD (DV, IHRF, hDC, and SDD), a thin DLS and cRORA in the fellow eye were associated with an increased risk of progression to cRORA over 2 years. These biomarkers may aid in prognostication, risk stratification, and selection of patients for clinical trials.
Proprietary or commercial disclosure may be found after the references.

BACKGROUND: We sought to investigate the chorioretinal thickness and retinal pigment epithelial (RPE) degenerative features of eyes with early age-related macular degeneration (AMD) and subretinal drusenoid deposits (SDDs) according to the presence of macular neovascularization (MNV) in the fellow eyes.
METHODS: We classified 70 eyes into two groups of 47 eyes with non-neovascular AMD and 23 eyes with neovascular AMD, respectively, according to the presence of MNV in the fellow eyes. The mean macular retinal, ganglion cell-inner plexiform layer (GCIPL), and choroidal thickness values and RPE features of the 6-mm-diameter zone were compared. RPE degeneration was defined as a lesion with an incomplete RPE and outer retinal atrophy (iRORA) or attenuated RPE reflectivity with diffuse basal laminar deposits, which was defined as when the eye showed an attenuated RPE line with granular features and mixed reflectivity in combination with sub-RPE deposits with a lesion ≥ 1,000 µm in length.
RESULTS: Mean retinal, GCIPL, and choroidal thickness values (286.69 ± 15.02 µm, 64.36 ± 4.21 µm, and 156.11 ± 33.10 µm) of the neovascular AMD group were greater than those (278.61 ± 13.96 µm, 61.44 ± 4.63 µm, and 133.59 ± 34.33 µm) of the non-neovascular AMD group (all P < 0.05). RPE degeneration was more prevalent in the neovascular AMD group (65.2%) than the non-neovascular AMD group (38.3%; P = 0.034). Greater mean GCIPL and choroidal thickness values and the presence of RPE degeneration were associated with type 3 MNV in fellow eyes (all P < 0.05).
CONCLUSIONS: Different degenerative features according to MNV in fellow eyes of patients with AMD and SDDs suggest that variable degenerative features might be present during disease progression and have an association with the phenotype.

A wide spectrum of phenotypic manifestations characterizes age-related macular degeneration (AMD). Drusen is considered the hallmark of AMD and is located underneath the retinal pigment epithelium (RPE). In contrast, subretinal drusenoid deposits (SDDs), also known as reticular pseudodrusens, are located in the subretinal space, on top of the RPE. SDDs are poorly detected by clinical examination and color fundus photography. Multimodal imaging is required for their proper diagnosis. SDDs are topographically and functionally related to rods. SDDs cause a deep impairment in retinal sensitivity and dark adaptation. SDDs are dynamic structures that may grow, fuse with each other, or regress over time. An intermediate step in some eyes is the development of an acquired vitelliform lesion. The presence of SDD confers an eye a high risk for the development of late AMD. SDD leads to macular neovascularization, particularly type 3, geographic atrophy, and outer retinal atrophy.

To describe the incidence of subretinal deposits that are similar in structure and stage on OCT imaging to subretinal drusenoid deposits (SDDs) in age-related macular degeneration (AMD) in patients with hypertensive choroidopathy secondary to severe pre-eclampsia and malignant hypertension (MHT) and the implications of this ischemic choroidopathy for the pathophysiologic characteristics of SDDs in AMD.
Retrospective cross-sectional study.
Thirty-three pre-eclampsia patients and 25 MHT patients with serous retinal detachment (SRD) in at least 1 eye were included.
Serial multimodal images, including enhanced depth imaging spectral-domain OCT of eyes with hypertensive choroidopathy secondary to pre-eclampsia and MHT, were reviewed at 2 time points, the acute phase (within 4 weeks of initial hypertensive insult) and the recovery phase (beyond 4 weeks).
Incidence of SDD-like lesions in patients with hypertensive choroidopathy secondary to pre-eclampsia and MHT.
Subretinal drusenoid deposit-like lesions were observed exclusively in eyes with SRD. Serous retinal detachment occurred in 87.87% of eyes of pre-eclampsia patients and in 94% of eyes of MHT patients. Subretinal drusenoid deposit-like lesions occurred in 28.57% of all eyes with SRD, in 32.76% of eyes with SRD from the pre-eclampsia group, and in 23.40% of eyes with SRD from the MHT group. Vascular imaging suggested underlying choroidal ischemia in all patients (12 eyes) in which it was performed.
Choroidal ischemia may be the underlying mechanism of SDD-like lesions in patients with pre-eclampsia and MHT choroidopathy. These findings potentially are of utmost importance in understanding the mechanism of the reticular macular disease subtype of AMD. Reticular macular disease is characterized by the known association of choroidal insufficiency and SDD, with choroidal insufficiency postulated, but not proven, to be causative. Pre-eclampsia and MHT choroidopathy seems to be a model for lesions similar to SDD in AMD developing based on choroidal insufficiency and, as such, may offer further insights into the pathoetiologic features of SDD in AMD.

We describe a patient with oral-facial-digital syndrome (OFDS) with the following anomalies: cleft lip, cleft palate, micrognathia, hypertelorism, nasal septum deviation, thumb polydactyly in the right hand, and partial agenesis of the corpus callosum. In addition, the patient had optic disc coloboma in the left eye and subfoveal drusenoid deposit in the right eye, features of OFDS type IX. Subfoveal drusenoid deposit has not been previously reported in OFDS type IX. Evaluation of the fundus is necessary for diagnosis of OFDS.

A distinction between conventional drusen and pseudodrusen was first made in 1990, and more recently knowledge of pseudodrusen, more accurately called subretinal drusenoid deposits (SDDs), has expanded. Pseudodrusen have a bluish-white appearance by biomicroscopy and color fundus photography. Using optical coherence tomography, pseudodrusen were found to be accumulations of material internal to the retinal pigment epithelium that could extend internally through the ellipsoid zone. These deposits are more commonly seen in older eyes with thinner choroids. Histologic evaluation of these deposits revealed aggregations of material in the subretinal space between photoreceptors and retinal pigment epithelium. SDDs contain some proteins in common with soft drusen but differ in lipid composition. Many studies reported that SDDs are strong independent risk factors for late age-related macular degeneration. Geographic atrophy and type 3 neovascularization are particularly associated with SDD. Unlike conventional drusen, eyes with SDD show slow dark adaptation and poor contrast sensitivity. Outer retinal atrophy develops in eyes with regression of SDD, a newly recognized form of late age-related macular degeneration. Advances in imaging technology have enabled many insights into this condition, including associated photoreceptor, retinal pigment epithelium, and underlying choroidal changes.

Age-related macular degeneration (AMD) is a leading cause of vision loss in patients >50 years old. The hallmark of the disease is represented by the accumulation of extracellular material between retinal pigment epithelium and the inner collagenous layer of Bruch\'s membrane, called drusen. Although identified almost 30 years ago, reticular pseudodrusen (RPD) have been recently recognized as a distinctive phenotype. Unlike drusen, they are located in the subretinal space. RPD are strongly associated with late AMD, especially geographic atrophy, type 2 and 3 choroidal neovascularization, which, in turn, are less common in typical AMD. RPD identification is not straightforward at fundus examination, and their identification should employ at least 2 different imaging modalities. In this narrative review, we embrace all aspects of RPD, including history, epidemiology, histology, imaging, functional test, natural history and therapy.