Chiara Veronese

Swelling of the arcuate nerve fiber layer after internal limiting membrane peeling.

Purpose: The purpose of the study is to report the incidence of changes of the retinal nerve fiber layer in the early postoperative period after internal limiting membrane peeling for idiopathic macular hole and epiretinal membrane surgery. Methods: Interventional, noncomparative retrospective case series. Fifty-six eyes of 55 patients with an epiretinal membrane and 33 eyes of 31 patients with macular hole underwent pars plana vitrectomy and internal limiting membrane peeling. All patients received a complete ophthalmic examination, infrared and autofluorescence photography, and spectral-domain optical coherence tomography preoperatively and also at approximately 1 week, 1 month, and 3 months postoperatively. Vitrectomy and gas tamponade were performed with internal limiting membrane peeling after staining with Brilliant Peel. The main outcome measures were the presence of postoperative swelling of the arcuate retinal nerve fiber layer on spectral-domain optical coherence tomography, infrared and autofluorescence photographs, and its effect on best-corrected visual acuity. Results: On infrared and autofluorescence photographs, 28 (31.46%) of 89 eyes with internal limiting membrane peeling exhibited swelling of the arcuate retinal nerve fiber layer 1 week to 1 month postoperatively. Swelling of the arcuate retinal nerve fiber layer increased during the first month after surgery and resolved after a mean period of 2 months. These features were best visualized by autofluorescence imaging, as three to five dark striae originating from the optic nerve head, radiating in an arcuate pattern toward the macula. Simultaneous spectral-domain optical coherence tomography scanning through the striae indicated that they correspond to areas of focal swelling of the arcuate nerve fiber layer. No significant differences were found in eyes with or without swelling of the arcuate retinal nerve fiber layer for mean age or pre- and postoperative best-corrected visual acuity (P > 0.05). Conclusion: Swelling of the arcuate retinal nerve fiber layer often occurs after internal limiting membrane peeling for macular hole and epiretinal membrane. It is a transient feature after surgery that does not affect visual recovery. It is best visualized using infrared and autofluorescence imaging. Simultaneous infrared and spectral-domain optical coherence tomography imaging after macular surgery can detect transient inner retinal changes, which are not visible on clinical examination.

Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy.

Purpose: To describe the appearance of acute syphilitic posterior placoid chorioretinitis, a rare ocular manifestation of syphilis, on spectral domain optical coherence tomography (SD OCT) both before and after treatment. Methods: Ophthalmic examination and imaging studies of 30 eyes of 19 confirmed cases were analyzed both at the time of presentation and at each follow-up visit. Patients with SD OCT and fluorescein angiography at the time of presentation, and at least three documented follow-up visits after initiation of therapy, were included in the study. Standard treatment of neurosyphilis was given to each patient, including 4 million units of penicillin G administered intravenously every 4 hours for 14 days. Results: Fundus examination and imaging studies were consistent with previous reports and confirmed the diagnosis of acute syphilitic posterior placoid chorioretinitis. In 13 eyes (43.3%), baseline SD OCT scans were performed within 1 to 2 days of presentation and revealed a small amount of subretinal fluid, disruption of the inner segment/outer segment junction, and hyperreflective thickening of the retinal pigment epithelium (RPE). All 30 eyes were again scanned between Days 7 and 9 after presentation and revealed loss of the inner segment/outer segment and OS/RPE bands, and irregular hyperreflectivity of the RPE with prominent nodular elevations but without subretinal fluid. Early disruption of the external limiting membrane and punctate choroidal hyperreflectivity were seen in 1 of the 30 eyes (3.3%) and 14 of the 30 eyes (46.6%), respectively. Vision improved and the outer retinal abnormalities normalized in 28 of the 30 eyes (93.3%) after the treatment of neurosyphilis. The external limiting membrane, inner segment/outer segment band, and/or linear outer segment/RPE junction remained substantially abnormal despite treatment in 2 eyes left with 20/200 vision. Conclusion: Patients with acute syphilitic posterior placoid chorioretinitis show characteristic outer retinal abnormalities on SD OCT imaging, including disruption of the inner segment/outer segment band, nodular thickening of the RPE with loss of the linear outer segment/RPE junction, and, in some cases, loss of the external limiting membrane, accumulation of subretinal fluid, and punctate hyperreflectivity in the choroid. Vision improved and these abnormalities reversed after treatment of neurosyphilis in most of the patients. Persistently, poor vision despite treatment was associated with long-term loss or disruption of outer retinal anatomy on SD OCT.

Retinal nerve fiber layer thickness modification after internal limiting membrane peeling.

Purpose: To identify early and late retinal nerve fiber layer thickness (RNFLT) modification after internal limiting membrane peeling for idiopathic macular hole or epiretinal membrane and to correlate RNFLT to visual field indices. Methods: Single-center, prospective, interventional consecutive case series. Complete ophthalmic examination, fundus images, and spectral domain optical coherence tomography were performed in 30 eyes of 30 patients before and 1, 3, and 6 months after surgery. Six peripapillary sectors (superotemporal, temporal, inferotemporal, inferonasal, nasal, superonasal) and global RNFLT were evaluated. Visual field was performed preoperatively and 6 months postoperatively. Results: Significant RNFLT modification was found after surgery (P < 0.0001). Specifically, RNFLT significantly increased in all, but the temporal sectors, 1 month after surgery, and it returned to preoperative values at the third month. Six months after surgery, RNFLT was lower than basal values in the superotemporal, inferotemporal, and temporal sectors (P < 0.001, P < 0.05, and P < 0.001, respectively) with an average reduction of 18.2 ± 9.8 µm. No correlation was found between RNFLT and the visual field indices. Conclusion: The diffuse RNFLT increase 1 month postoperatively could be because of inflammatory responses. The reduction of RNFLT in the temporal sectors 6 months postoperatively could indicate damage to the macular retinal nerve fiber layer caused by internal limiting membrane peeling.

Perivenular Whitening in Central Vein Occlusion Described by Fundus Autofluorescence and Spectral Domain Optical Coherence Tomography

In the retinal tissue, transparency is a clinical index of tissue oxygenation. With central retinal vein occlusion, the most sensitive tissue for manifesting decreased oxygenation will be perivenular, especially in the posterior pole, where oxygen use is higher than in the retinal periphery. Patchy ischemic retinal whitening is located preferentially in a perivenular distribution near the macula. A 40-year-old man came to our institution complaining of loss of vision in the right eye, which had started 2 days previously. His medical and ophthalmologic histories were unremarkable. Initial examination in our department showed visual acuity of 20/32, right eye, and 20/20, left eye. Fundus examination showed dilated veins and scattered hemorrhages, along with a whitening of the macula (Figure 1A, white arrow) and of the perivenular retinal (Figure 1A, black arrow), and fluorescein angiogram showed delayed transit during the venous phase, but no capillary closure or blood retinal barrier rupture (Figure 1B), thus confirming the diagnosis of a mild form of central retinal vein occlusion with thrombus located more posteriorly relative to the lamina cribrosa. Autofluorescence imaging revealed a normal fundus autofluorescence corresponding to the arterioles and patchy hypoautofluorescence located preferentially in a perivenular distribution near the macula because of blockage of autofluorescence from inner retinal edema, which on spectral domain optical coherence tomographic scans corresponded to patchy retinal opacification extended from the inner to the outer plexiform layers (Figure 2, A and B) that may be secondary to ischemia of intermediary neurons within the inner nuclear and inner plexiform layers. One month later, autofluorescence and spectral domain optical coherence tomography showed improvement of symptoms (Figure 3, A and B) and visual acuity of 20/20. We report the presence of perivenular retinal edema in a patient with recent-onset central retinal vein occlusion, best visualized by autofluorescence as a hypoautofluorescent fern-like appearance. The perivenular location of ischemic edema is because of decreased arterial flow that leads to preferential oxygenation of the periarterial retina, which uptakes the available oxygen and subsequently desaturates hemoglobin before it can reach the perivenular space.

Combination Therapy With Dexamethasone Intravitreal Implant and Macular Grid Laser in Patients With Branch Retinal Vein Occlusion

PURPOSE To test a combination of dexamethasone intravitreal implant with macular grid laser for macular edema in patients with branch retinal vein occlusion (BRVO). DESIGN Prospective interventional, randomized, multicenter study. METHODS Patients with macular edema secondary to BRVO underwent an Ozurdex intravitreal implant at baseline. After 1 month, patients were randomly assigned to 2 study groups. Patients in Group 1 were followed up monthly and retreated with Ozurdex implant whenever there was a recurrence of macular edema or a decrease in best-corrected visual acuity (BCVA). In Group 2 patients macular grid laser was performed between weeks 6 and 8. After that, patients were followed up and retreated as for Group 1. RESULTS In Group 1 at 4 months, mean BCVA was 0.49 ± 0.35 logMAR and central retinal thickness (CRT) was 391 ± 172 μm; both improved significantly at 6 months, to 0.32 ± 0.29 logMAR and 322 ± 160 μm, respectively. In Group 2, CRT was reduced significantly to 291 ± 76 μm at 4 months, and BCVA improved to 0.25 ± 0.20 logMAR. At the final visit, BCVA was 0.18 ± 0.14 logMAR and mean CRT was 271 ± 44 μm. The number of Ozurdex implants at 4 months was 12 of 25 (48%) in Group 1 patients vs 3 of 25 (12%) in Group 2 patients (P = .012). At 6 months 3 of 25 patients (12%) in Group 1 vs 0 of 25 (0%) in Group 2 (P = .23) were retreated. CONCLUSIONS The combination of Ozurdex implant and macular grid laser is synergistic in increasing BCVA and lengthening the time between injections.

Multimodal Imaging in Hereditary Retinal Diseases

Introduction. In this retrospective study we evaluated the multimodal visualization of retinal genetic diseases to better understand their natural course. Material and Methods. We reviewed the charts of 70 consecutive patients with different genetic retinal pathologies who had previously undergone multimodal imaging analyses. Genomic DNA was extracted from peripheral blood and genotyped at the known locus for the different diseases. Results. The medical records of 3 families of a 4-generation pedigree affected by North Carolina macular dystrophy were reviewed. A total of 8 patients with Stargardt disease were evaluated for their two main defining clinical characteristics, yellow subretinal flecks and central atrophy. Nine male patients with a previous diagnosis of choroideremia and eleven female carriers were evaluated. Fourteen patients with Best vitelliform macular dystrophy and 6 family members with autosomal recessive bestrophinopathy were included. Seven patients with enhanced s-cone syndrome were ascertained. Lastly, we included 3 unrelated patients with fundus albipunctatus. Conclusions. In hereditary retinal diseases, clinical examination is often not sufficient for evaluating the patients condition. Retinal imaging then becomes important in making the diagnosis, in monitoring the progression of disease, and as a surrogate outcome measure of the efficacy of an intervention.

RETINAL ASTROCYTIC HAMARTOMA: Optical Coherence Tomography Classification and Correlation With Tuberous Sclerosis Complex.

Purpose: To propose a classification of retinal astrocytic hamartoma based on spectral domain optical coherence tomography and correlate each class with systemic manifestations of tuberous sclerosis complex. Methods: Retrospective chart review conducted at four international referral medical retina centers. There were 43 consecutive patients with an established diagnosis of tuberous sclerosis complex based on presence of at least 2 major or 1 major and 2 minor features of the diagnostic criteria. Clinical and spectral domain optical coherence tomography features regarding retinal astrocytic hamartoma were documented. Results: The mean patient age at presentation was 16.2 years. The retinal astrocytic hamartoma was classified as Type I (n = 41), Type II (n = 25), Type III (n = 20), or Type IV (n = 12). Patients with Type II showed greater number of cutaneous fibrous plaques (odds ratio = 64.8; 92% confidence interval: 64.2–65; P < 0.001); those with Type III displayed higher incidence of subependymal giant-cell astrocytomas (odds ratio = 43.2; 95% confidence interval: 43.0–43.3; P < 0.001); and those with Type IV showed higher incidence of pulmonary lymphangiomyomatosis (odds ratio = 126; 95% confidence interval: 122–128; P < 0.001). Conclusion: Retinal astrocytic hamartoma can be classified into four morphologic groups, based on spectral domain optical coherence tomography. There are important systemic tuberous sclerosis complex correlations with each class.

Double-vitrectomy for optic disc pit maculopathy.

Background: The origin of the fluid and precise pathophysiology of optic pit maculopathy remain unclear. It has been suggested that submacular fluid originates either from vitreous or cerebrospinal fluid. We report a case of optic pit maculopathy which was unsuccessfully treated with vitrectomy and internal limiting membrane (ILM) peeling, and subsequently resolved with revision of vitrectomy and gas tamponade. Methods: We report a case of optic disc pit maculopathy, well documented by spectral- domain optical coherence tomography, before and after pars plana vitrectomy with ILM peeling, and its revision with gas tamponade. Results: After ILM peeling, there was no improvement either in visual acuity or in the tomographic aspect of the retina. A revision of the surgery was then needed and gas tamponade was performed, which resulted in a complete resolution of the optic pit maculopathy. Conclusion: The absence of improvement after ILM peeling during the first surgical procedure, accompanied by resolution of the clinical picture with gas tamponade during the second surgical procedure, sustained the hypothesis of a subarachnoidal origin of the fluid.

Cystoid changes within astrocytic hamartomas of the retina in tuberous sclerosis.

PURPOSE The purpose of this study was to report two unusual cases of cavitations within astrocytic hamartoma, a feature that causes the tumor to appear cystic. To our knowledge, this is the first report on cavitary astrocytic hamartoma. METHODS In this observational case report, we identify two cases of cavitary astrocytic hamartoma imaged recently with spectral domain-optical coherence tomography. RESULTS In both of our patients, the cavitary portion was visible clinically as an empty cavity surrounded by translucent tissue. Optical coherence tomography examination of both cases confirmed the presence of a cavity surrounded by tumor tissue. CONCLUSION In these two patients, optical coherence tomography showed a new feature of cavitation within retinal astrocytic hamartoma.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY TO ASSESS PIGMENT EPITHELIAL DETACHMENT.

Retinal pigment epithelial detachment (PED) is the anatomical separation of the retinal pigment epithelium (RPE) from the underlying Bruch membrane. Retinal PEDs are most commonly associated with age-related macular degeneration (AMD), but are also seen in other retinal disorders, such as central serous chorioretinopathy, neoplastic, inflammatory, or iatrogenic conditions. In AMD, PED can be categorized into drusenoid, serous, vascularized, or mixed categories; however, multiple PED types may be found in the same patient. Drusenoid and serous PEDs are primarily a feature of nonneovascular AMD. In contrast, sub-RPE (Type 1) choroidal neovascularization (CNV) is associated with vascularized PEDs and is most commonly correlated with neovascular (wet) AMD. Early diagnosis and treatment of CNV is crucial to ensure superior visual outcomes. Therefore, it is important to be able to differentiate between various types of PED because their natural history, prognosis, and treatment options differ. Fluorescein angiography (FA) is the current gold standard for diagnosing and assessing PEDs. However, FA is invasive, time consuming, provides only a two-dimensional image, and has a minimal but significant risk profile that includes nausea, allergy, and, rarely, anaphylaxis. Although other imaging modalities such as indocyanine green angiography (ICGA), fundus autofluorescence, and spectral domain optical coherence tomography (SD-OCT) are useful in identifying specific characteristic findings of various types of PEDs, all modalities have unique limitations and drawbacks. Given the complex variety of clinical appearances and imaging characteristics of PEDs in AMD and other retinal disorders, it is often necessary to use multimodal imaging to assess PEDs. Optical coherence tomography angiography (OCTA) is a noninvasive, depth-resolved, non–dye-based, and rapid technique for visualizing retinal and choroidal vasculature and various planes of retinal tissues. It is capable of providing en face, detailed, threedimensional images of PEDs and CNV. The purpose of this study was to visualize, assess, and describe the spectrum of retinal findings associated with various types of PEDs through the use of OCTA without the need for invasive dyes.