وضعیت Hyaloid Face چشم مقابل در OCT چقدر میتواند در بیماران مبتلا به RRD مهم باشد

A cohort of 1049 individuals diagnosed with rhegmatogenous retinal detachment (RRD) were monitored over a mean duration of 5.7 ± 0.3 years.

In total, a diagnosis of bilateral sequential rhegmatogenous retinal detachment (RRD) was given to 153 patients, accounting for 14.6% of the study population during the specified follow-up period. A total of 582 fellow eyes had available OCT images.

Among these, 229 fellow eyes (39.3%) exhibited posterior vitreous detachment (PVD), while 353 fellow eyes (60.7%) showed an attached hyaloid.

An RRD was observed in seven fellow eyes, accounting for 3.1% of cases, where a posterior vitreous detachment (PVD) was present at the time of presentation.

Among the group of eyes with an attached hyaloid, a total of 28 eyes (7.9%) exhibited a retinal detachment (RRD) during the course of the study. However, when specifically considering those eyes in which a posterior vitreous detachment (PVD) occurred during the follow-up period, it was observed that 23.7% of these eyes also experienced an RRD.

During the period of PVD development in the contralateral eye, it was observed that an additional 21 eyes (17.8%) exhibited a retinal tear, which was successfully managed without subsequent progression to rhegmatogenous retinal detachment (RRD).

Findings: Optical coherence tomography (OCT) imaging of the unaffected eye at the initial diagnosis of a rhegmatogenous retinal detachment (RRD) provides valuable insights into the assessment of the risk level for RRD in that eye. Patients who exhibit a fully detached posterior hyaloid are observed to have a notably reduced risk of rhegmatogenous retinal detachment (RRD) compared to those with a visible posterior hyaloid. Consequently, individuals with a visible posterior hyaloid necessitate close monitoring during the development of posterior vitreous detachment (PVD).

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وجود پیگمان بر روی لامینا کریبروزا

A male child of Asian-American descent, aged 11, was brought in for assessment regarding the presence of pigmentation on the optic nerve. The visual acuity of both eyes, without any corrective measures, was measured to be 20/20.

The color fundus photography of the right eye exhibited a cup-to-disc ratio of 0.6, alongside the presence of a pigmented cribriform structure within the optic cup. It is postulated that the observed phenomenon corresponds to the presence of melanocytic pigmentation within the scleral fibers of the lamina cribrosa.

It is plausible that the condition under consideration may also represent an incipient optic nerve melanocytoma. Melanocytoma has been characterized as a pigmented lesion that partially or completely obscures the optic disc. The scleral fibers exhibit pigmentation, although they do not aggregate to form a distinct mass.

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Journal Club 21-3-1402

Journal Club 20-1-1402

Journal Club 17-2-1402

Novel Manifestation of Retinal Hemangioblastomas Detected by Optical Coherence Tomography Angiography in von Hippel-Lindau Disease

Published:February 10, 2023 DOI:https://doi.org/10.1016/j.ophtha.2023.02.008

Purpose
To elucidate the clinical characteristics of atypical retinal vascular proliferation in patients with von Hippel-Lindau (VHL) disease using optical coherence tomography angiography (OCTA).

Design
Prospective observational study.
Participants
Fifty-seven consecutive patients diagnosed with VHL disease who visited Kyoto University Hospital between January 2019 and March 2022.

Methods
Retinal hemangioblastomas (RHs) were assessed using multimodal imaging including OCTA. RHs were classified into two phenotypes: nodular and flat types. Nodular-type RHs were defined as typical RHs that were globular, well-circumscribed tumors, often accompanied with dilated feeder arterioles and draining venules. Flat-type RHs lacked a protruded red or colored mass, had variable and indistinct borders, and were not accompanied with feeder and draining vessels.
Main outcome measures
The prevalence, distribution, and description of atypical flat RHs.

Results
Among 57 consecutive patients with VHL disease, 37 patients (64.9%) showed RHs in at least 1 eye. Bilateral RHs were seen in 23 patients (62.2%). Among 58 eyes of 37 patients with RHs, typical nodular-type RHs were detected in 54 eyes. Nodular-type RHs were seen mainly in the peripheral retina and occasionally in the peripapillary region, and they showed exudative changes in some cases. Flat-type RHs were detected in 7 eyes (12.1%). Four eyes showed only flat-type RHs, and 3 eyes showed both types in the same eye. Most flat-type RHs appeared as retinal hemorrhage or faint flat abnormal retinal vessels in the inner retina on the fundus examination, often within the macula area or peripapillary. In all eyes with flat-type RHs, OCTA showed abundant blood flow in the lesions. OCT revealed that flat-type RHs were seen mainly between the retinal nerve fiber layer and the ganglion cell layer, and occasionally within the inner nuclear layer. During a mean follow-up period of 20.4 ± 15.0 months, no flat-type RHs accompanied exudative change or tractional retinal detachment, or progression in size.

Conclusions
Patients with VHL disease can present with two distinct types of RHs; the classic nodular-type and atypical flat-type RHs. OCTA can be useful in improving the detection of atypical flat-type RHs which can be difficult to detect clinically.

1-In the current study, 37 (64.9%) of 57 consecutive VHL patients presented with RHs in at least 1 eye. This rate is slightly higher than that in previous reports (25–60%)

2-flat-type RHs were observed in 7 (12.1%) of 58 eyes with VHL-related RHs
3- All the typical nodular-type RHs were observed peripherally or peripapillary. Flat-type RHs were observed mainly peripapillary or within the macula region

3-the lesions were completely undetectable during the fundus examination, but OCTA was very sensitive to detecting this type of RH

4-In 1 eye (case 4), OCTA clearly revealed a subtle flat-type lesion that even FA could not detect

Flat-type RHs sometimes appear as reddish flat lesions that look like a retinal hemorrhage in the macula area (case 3). However, OCTA findings indicated that these reddish lesions were actually atypical RHs with abundant blood flow. OCTA was very effective in detecting flat-type RHs within the macula region or around the optic disc.

Furthermore, because OCTA imaging is easily performed in the posterior pole where flat-type RHs frequently occur, OCTA should be valuable for screening the fundus of VHL patients.

Typical nodular-type RHs located above the RNFL protruded towards the vitreous cavity (Case 1). In contrast, flat-type RHs, located in various layers of the inner layers, remained flat and never protruded into the vitreous cavity. Detailed OCT analysis revealed that flat-type RHs were mainly seen between the RNFL and the GCL (Case 3, 5), and occasionally within the INL (Case 2)

Based on the OCT and OCTA evaluations, flat-type RHs associated with VHL mainly developed from the superficial capillary plexus of the retina, and occasionally from the deep capillary plexus of the retina.

No eyes with flat-type RHs showed exudative change during the follow-up, and all eyes maintained visual acuity.

They accompanied no exudation or tractional retinal detachment.no growth

اختلالات شبکیه در بیماران مبتلا به سندرم داون

The prevalence of retinal anomalies in children diagnosed with Down Syndrome (DS) has been estimated to range from 5.1% to 36.9%.

The aforementioned conditions encompass myopic retinal degeneration, tortuous vasculature, tigroid appearance of the fundus, preretinal hemorrhage, and localized hyperplasia of the retinal pigment epithelium.

The prevalence of proliferative diabetic retinopathy in individuals with Down syndrome (DS) appears to be lower, even in cases of prolonged diabetes duration. This observation may be linked to a diminished occurrence of angiogenesis in individuals with DS.

A study has found that children diagnosed with Down syndrome (DS) exhibit a higher central foveal thickness in comparison to a control group. On the other hand, a separate study revealed that there was no significant difference in foveal thickness between children diagnosed with Down syndrome (DS) and the control group. The peripapillary retinal nerve fiber layer in individuals with Down syndrome (DS) is notably thicker in comparison to individuals without the condition (controls). Differences in retinal abnormalities between individuals with Down syndrome (DS) and a control group were observed in various retinal layers, including the inner retina, outer plexiform layer, external limiting membrane, and outer segment of the interdigitation zone.

The hypothesis suggests that the atypical structure of the retina observed in Down syndrome (DS) is linked to neuro-sensory factors rather than vascular factors.

Research has demonstrated that the choroid in adults diagnosed with Down syndrome (DS) exhibits a greater thickness in comparison to individuals without DS, serving as a distinguishing characteristic.
In contrast, a separate study found no statistically significant disparities in subfoveal choroidal thickness between individuals diagnosed with Down syndrome (DS) and those without the syndrome. An increased level of choroidal tessellation has been observed in individuals with Down syndrome, even in the absence of myopia. The hypothesis posited that the presence of choroidal vasculature could potentially contribute to the observed manifestation.

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