A unique presentation of subretinal fluid in a type III torpedo maculopathy phenotype.

Purpose: To report a rare type III torpedo maculopathy lesion with a unique manifestation of subretinal fluid. Observations: A nine-year-old patient was referred to retina for an evaluation of a hypopigmented oval-shaped lesion in the temporal macula with an area of inferior subretinal fluid in the right eye. The lesion demonstrated inner and outer retinal and retinal pigment epithelial attenuation, intraretinal and subretinal fluid, a serous neurosensory retinal detachment, and inner choroidal excavation on optical coherence tomography. Fundus autofluorescence showed a lane of downward-tracking fluid. Intravenously administered fluorescein angiography revealed a window defect in the area of the torpedo lesion suggesting choroidal flush. Conclusions and Importance: The case is the third documented case of torpedo maculopathy with subretinal fluid in the literature with a unique combination of intraretinal cystic changes and dependent descending subretinal fluid, somewhat akin to a Best disease outside of the fovea with choroidal excavation. The morphology of torpedo maculopathy continues to expand as more cases are revealed.

Pentosan Polysulfate Maculopathy: Prevalence, Spectrum of Disease, and Choroidal Imaging Analysis Based on Prospective Screening.

PURPOSE: To describe the prevalence and spectrum of disease of pentosan polysulfate (PPS) maculopathy in a large multimodal retinal imaging study and to report the results of choroidal vascularity index (CVI) analysis. DESIGN: Prospective cohort study Methods: Of 741 patients prescribed PPS within a large university database, 100 (13.4%) with any consumption agreed to participate in a prospective screening investigation. Multimodal retinal imaging including near-infrared reflectance (NIR), fundus autofluorescence (FAF), and spectral domain optical coherence tomography (SD-OCT) was performed in all patients. Characteristic findings of affected patients were identified, and affected and unaffected cohorts were compared. CVI, defined as stromal choroidal area (SCA) divided by the total choroidal area, was analyzed. RESULTS: The prevalence of PPS maculopathy was 16%. NIR illustrated punctate hyperreflective lesions with early presentation. FAF illustrated a speckled macular network of hypo- and hyperautofluorescence colocalized with multifocal hyperreflective retinal pigment epithelial lesions on SD-OCT. Advanced cases demonstrated varying degrees of atrophy. The affected cohort exhibited significantly greater mean PPS therapy duration, mean daily dosage, and mean cumulative dosage (19.5±5.5 years, 433.9±137.6 mg, 3,103.1±1,402.2 g) compared with the unaffected cohort (7.1±6.6 years, 291.6±177.6 mg, 768.4±754.8 g). SCA was significantly lower and CVI was significantly greater in the affected vs the unaffected group. CONCLUSIONS: This prospective cohort study identified a prevalence of PPS maculopathy of 15%-20% among PPS users who agreed to participate. A spectrum of findings may be observed with multimodal retinal imaging. Significant choroidal abnormalities associated with this characteristic maculopathy may provide surrogate markers of macular toxicity.

Identification and Characterization of Epivascular Glia Using En Face Optical Coherence Tomography.

PURPOSE: The purpose of this study was to describe the clinical features of epivascular glia (EVG) using en face optical coherence tomography (OCT). DESIGN: Retrospective cross-sectional study. METHODS: Single-institution en face OCT images were reviewed. Eyes displaying EVG were captured with manual internal limiting membrane (ILM) segmentation and analyzed with customized segmentation . A random age- and sex-matched control group was selected to determine relative epiretinal membrane (ERM) prevalence. RESULTS: Characteristic hyper-reflective ILM plaques with dendrite-like radiations were identified using en face OCT and displayed vascular predilection. A total of 161 eyes with EVG (the EVG group) and 2,315 eyes without EVG (control group) were identified from a total cohort of 1,298 patients (or 2,476 eyes). The prevalence of EVG was 161 of 2,476 eyes (6.5%) and 119 of 1,298 patients (9.2%) in the cohort. Mean age was 79.3 ± 10.7 years old in the EVG group and 55.9 ± 24.6 years old in the control group (P <.001). An advanced posterior vitreous detachment (PVD) stage was more common in the EVG group (grade 3: 41.7%; grade 4: 48.6%) than in the control group (grade 3: 18.5%; grade 4: 26.9%; P <.001). Contractile ERM was present in 71 of 161 eyes (44.1%) with EVG compared to 30 of 161 eyes (18.6%) in a random age- and sex-matched control cohort without EVG (P <.001). CONCLUSIONS: EVG previously described with histopathology and scanning electron microscopy can be identified using en face OCT. In this study, these lesions were associated with older age, pseudophakia, and advanced PVD, supporting the role of Müller cell activation through ILM breaks triggered by PVD, a pathogenic mechanism proposed by previous studies.

PAMM and the ischemic cascade associated with radiation retinopathy.

PURPOSE: To report a case of paracentral acute middle maculopathy (PAMM) and evidence of the ischemic cascade documented with spectral domain optical coherence tomography (SD-OCT) following radiation treatment of a choroidal melanoma. OBSERVATIONS: A healthy young patient was evaluated for an asymptomatic choroidal nevus in the left eye. Fundus examination was remarkable for a choroidal melanocytic lesion that measured 1.8 mm in thickness by initial B-scan ultrasound. Clinical examination 6 months later showed growth of the tumor at several margins with new subretinal fluid, and a B-scan measured thickness of 1.9 mm. The lesion was diagnosed as a small choroidal melanoma and treated with gamma knife radiation with a dose of 3000 cGy. Sixteen months later, examination showed signs of radiation retinopathy including cotton wool spots and PAMM via SD-OCT and OCT angiography and evidence of the ischemic cascade (i.e., alternating zones of middle and combined middle and inner retinal layer infarction). CONCLUSIONS: Radiation retinopathy can include signs of microvascular damage and ischemia including lesions such as cotton wool spots and PAMM. A case is presented in this report of radiation retinopathy with OCT evidence of PAMM and the ischemic cascade.

Choriocapillaris Flow Deficits and Treatment-Naïve Macular Neovascularization Secondary to Age-Related Macular Degeneration.

Purpose: To evaluate choriocapillaris (CC) flow deficits (FD) in eyes with treatment-naïve macular neovascularization (MNV) and to compare CC FD around exudative versus nonexudative MNV. Methods: Treatment-naïve eyes with a diagnosis of either exudative or nonexudative AMD and type 1 MNV were included. Normal control eyes were age-matched to each AMD eye one to one. En face optical coherence tomography angiograms were analyzed for percentage of CC FD (FD%) in two concentric 500 µm rings, ring 1 and ring 2, surrounding the dark halo around MNV. The mean CC FD% in ring 1 and ring 2 was evaluated for each eye. A secondary analysis was similarly carried out to investigate the differences in CC FD% in exudative versus nonexudative treatment-naïve MNV. Results: Twenty-three eyes with treatment-naïve MNV were age matched with 23 normal controls. The mean CC FD% was significantly greater in both rings in the MNV versus the normal control group (P < 0.05) and was significantly greater in the inner ring, closer to the lesion, than the outer ring. The mean FD% was also greater in both rings in the exudative versus the nonexudative MNV group, but this difference did not reach statistical significance. Conclusions: The CC FD% was greater in the area surrounding MNV versus age-matched normal controls and in the ring closer to the MNV lesion. Further, CC FD was greater in eyes with exudative versus nonexudative MNV in both rings surrounding the associated dark halo, although this difference was not statistically significant.

Relationship Between Nerve Fiber Layer Hemorrhages and Outcomes in Central Retinal Vein Occlusion.

Purpose: To evaluate the depth and pattern of retinal hemorrhage in acute central retinal vein occlusion (CRVO) and to correlate these with visual and anatomic outcomes. Methods: Retinal hemorrhages were evaluated with color fundus photography and fluorescein angiography at baseline and follow-up. Snellen visual acuity (VA), central foveal thickness (CFT), extent of retinal ischemia, and development of neovascularization were analyzed. Results: 108 eyes from 108 patients were evaluated. Mean age was 63.6 ± 16.1 years with a predilection for the right eye (73.1%). Average follow-up was 17.2 ± 19.2 months. Mean VA at baseline was 20/126 and 20/80 at final follow-up. Baseline (P = 0.005) and final VA (P = 0.02) in eyes with perivascular nerve fiber layer (NFL) hemorrhages were significantly worse than in eyes with deep hemorrhages alone. Baseline CFT was greater in the group with perivascular hemorrhages (826 ± 394 µm) compared to the group with deep hemorrhages alone (455 ± 273 µm, P < 0.001). The 10 disc areas of retinal ischemia was more common in patients with perivascular (80.0%) and peripapillary (31.3%) versus deep hemorrhages alone (16.1%, P < 0.001). Neovascularization of the iris was more common, although this differrence was not significant, in the groups with peripapillary (14.3%) and perivascular (2.0%) NFL versus deep hemorrhages alone (0.0%). Conclusions: NFL retinal hemorrhages at baseline correlate with more severe forms of CRVO, with greater macular edema, poorer visual outcomes, and greater risk of ischemia and neovascularization. This may be related to the organization of the retinal capillary plexus. The depth and pattern of distribution of retinal hemorrhages in CRVO may provide an easily identifiable early biomarker of CRVO prognosis.

Hyperreflective Stress Lines and Macular Holes.

Purpose: To determine the prevalence of a central hyperreflective line in eyes with full-thickness macular holes (FTMH) and lamellar macular holes (LMH) and to elucidate the pathoanatomic importance of this optical coherence tomography (OCT) sign. Methods: This retrospective analysis evaluated patients with FTMH and LMH at the Stein Eye Institute. Clinical data was collected and SD-OCT volume scans were analyzed for the presence of a central vertical hyperreflective line in 3 separate cohorts: patients with SD-OCT preceding FTMH development, patients with SD-OCT after pars plana vitrectomy (PPVT) treatment for FTMH, and patients with SD-OCT of LMH. Results: In total, 93 eyes with FTMH and 88 eyes with LMH were identified. Of the 93 FTMH eyes, SD-OCT volume scans were available before development of the FTMH in 12 eyes. Of these, 6 (50%) displayed a vertical hyperreflective line preceding the development of the FTMH. Fifty-one eyes underwent PPVT with resolution of the FTMH, and 26 displayed a hyperreflective line after resolution (51%). Of the 88 eyes with LMH, 22 displayed a hyperreflective line (25%). All hyperreflective lines were noted at the central fovea. Conclusions: SD-OCT illustrated the presence of a central vertical hyperreflective line preceding FTMH and after resolution of FTMH after PPVT in approximately one-half of cases, and concurrent with LMH in 25% of cases. This vertical hyperreflective line may represent an early SD-OCT marker for the development of FTMH, and may be a sign of central foveal dehiscence owing to disruption of the Muller cell cone.

Inner choroidal ischaemia and CNV due to handheld laser-induced maculopathy: a case report and review.

There has been a sharp rise of reported handheld laser-induced maculopathy (HLIM) cases over the past decade, a concerning trend that may continue due to unregulated online access to high power lasers. Though HLIM has distinct clinical features, not uncommonly it may masquerade as other retinal disorders. It is critical therefore to recognise the clinical and multimodal imaging characteristics of this important and potentially devastating condition. As HLIM patients are typically young, unique issues need to be considered, such as delayed presentation, difficult history, poor compliance and behavioural or psychiatric comorbidity. This article will review the clinical and diagnostic features of laser injury, with a special emphasis on the multimodal retinal findings. In addition, we present a unique case of HLIM, resembling the presentation of a placoid disease variant and illustrating choroidal ischaemia using advanced retinal imaging, that offers further insight into the mechanisms of laser injury and its complications. The issues addressed in this review aim to increase recognition of an increasingly important and trending condition with potentially profound visual complications.

Pentosan-associated maculopathy: prevalence, screening guidelines, and spectrum of findings based on prospective multimodal analysis.

OBJECTIVE: To describe the prevalence and spectrum of multimodal imaging findings of pentosan polysulfate sodium (PPS)-associated maculopathy and to recommend dosage-related screening guidelines. DESIGN: Cross-sectional study. METHODS: Patients previously or currently treated with PPS at University of California, Los Angeles, were randomly ascertained and prospectively screened for PPS-associated maculopathy with multimodal retinal imaging. Daily and cumulative dosages of PPS exposure were calculated for each patient. Images were studied to identify the characteristic findings of toxicity. The prevalence of PPS-associated maculopathy and screening guidelines were determined. RESULTS: The prevalence of PPS-associated maculopathy in this cohort was 20% (10/50 patients). Both average duration of PPS therapy and average cumulative dosage were significantly lower in the unaffected (6.3 ± 6.6 years, 691.7 ± 706.6 g) versus the affected groups (20.3 ± 6.6 years, 3375.4 ± 1650.0 g, p < 0.001). Near-infrared reflectance (NIR) illustrated characteristic punctate retinal pigment epithelium (RPE) macular lesions early. Fundus autofluorescence (FAF) showed speckled autofluorescence in the posterior pole with peripapillary extension. Co-localization with optical coherence tomography (OCT) displayed focal RPE thickening and, in more severe cases, RPE atrophy in the macula and even the periphery. CONCLUSIONS: A prevalence of 20% in this study cohort suggests a significant risk of macular toxicity for PPS-treated patients. Characteristic alterations are best detected with FAF and NIR. More significant PPS exposure was associated with more severe atrophy. We recommend an initial baseline eye examination to include OCT and, most importantly, NIR and FAF with annual retinal imaging thereafter especially with cumulative dosages approaching 500 g. Patients exposed to greater than 1500 g of PPS are at significant risk of retinal toxicity.

Selective posterior cerebral artery amobarbital test: a predictor of memory following subtemporal selective amygdalohippocampectomy.

BACKGROUND: The selective posterior cerebral artery (PCA) amobarbital test, or PCA Wada test, is used to predict memory impairment after epilepsy surgery in patients who have previously had a failed internal carotid artery (ICA) amobarbital test. METHODS: Medical records from 2012 to 2018 were retrospectively reviewed for all patients with seizures who underwent a selective PCA Wada test at our institution following a failed or inconclusive ICA Wada test. Standardized neuropsychological testing was performed before and during the Wada procedure and postoperatively in patients who underwent resection. RESULTS: Thirty-three patients underwent a selective PCA Wada test, with no complications. Twenty-six patients with medically refractory epilepsy had a seizure focus amenable to selective amygdalohippocampectomy (AHE). Six patients (23%, n=26) had a failed PCA Wada test and did not undergo selective AHE, seven (27%) declined surgical resection, leaving 13 patients who underwent subtemporal selective AHE. Hippocampal sclerosis was found in all 13 patients (100%). Twelve patients (92%) subsequently underwent formal neuropsychological testing and all were found to have stable memory. Ten patients (77%) were seizure-free (Engel Class I), with average follow-up of 13 months. CONCLUSION: The selective PCA Wada test is predictive of memory outcomes after subtemporal selective AHE in patients with a failed or inconclusive ICA Wada test. Furthermore, given the low risk of complications and potential benefit of seizure freedom, a selective PCA Wada test may be warranted in patients with medically intractable epilepsy who are candidates for a selective AHE and who have a prior failed or inconclusive ICA Wada test.

Digital subtraction cerebral angiography after negative computed tomography angiography findings in non-traumatic subarachnoid hemorrhage.

BACKGROUND: CT angiography (CTA) is widely used for the detection of vascular lesions in patients with non-traumatic subarachnoid hemorrhage (ntSAH); however, digital subtraction angiography (DSA) remains the gold standard for diagnosis. Our aim was to analyze the diagnostic yield of DSA after negative high-resolution CTA findings. METHODS: Records of patients with a CTA-negative ntSAH at a single institution from 2014 to 2018 were retrospectively analyzed. ntSAH patterns were categorized as cortical, perimesencephalic, or diffuse. Subsequent DSA findings were compared across the three cohorts. RESULTS: A total of 186 patients had CTA-negative ntSAH. The ntSAH pattern was identified as cortical (n=77, 41.4%), diffuse (n=60, 32.3%), or perimesencephalic (n=49, 26.3%). In eight patients (4%), DSA revealed a vascular lesion (one cervical arteriovenous fistula and seven atypical aneurysms) after negative CTA findings. All eight patients with positive DSA findings had diffuse SAH (13% of patients with a diffuse pattern). The seven aneurysms included four blister or dissecting (two basilar artery, one superior cerebellar artery, and one dorsal wall internal carotid artery), two fusiform (one posterior communicating artery and one anterior spinal artery), and one saccular aneurysm (middle cerebral artery). CONCLUSION: DSA identified a causative lesion in 4% of patients with CTA-negative ntSAH, but only in patients with diffuse ntSAH. Most of the lesions detected were atypical aneurysms and were found on delayed angiograms. These results suggest that DSA can help to diagnose CTA-negative ntSAH caused by unusual aneurysms, and repeat DSA may be needed only for patients with diffuse ntSAH.

Laparoscopic-Assisted Ventriculoperitoneal Shunt Placement and Reduction in Operative Time and Total Hospital Charges.

OBJECTIVE: In ventriculoperitoneal shunt (VPS) placement, distal placement of the peritoneal catheter will typically be performed by a neurosurgeon. More recently, laparoscopic-assisted (LA) placement of the distal peritoneal catheter by general surgeons has become common. The present study examined whether LA placement of a VPS (LAVPS) is associated with a reduced operative time, lower hospital costs, and fewer distal revisions. METHODS: A retrospective review was performed of the data from all patients who had received a new VPS at our institution from 2013 to 2016. Age, sex, diagnosis, previous abdominal surgery, operative time, anesthesia grade, incidence of 30-day shunt failure, and total hospital charges were analyzed. RESULTS: A total of 680 patients had undergone first-time VPS placement, including 199 with LAVPS and 481 with non-LAVPS placement (non-LAVPS). The mean age of the LAVPS patients was significantly older than that of the non-LAVPS patients (64.1 vs. 59.3 years; P = 0.002). The mean operative time was shorter in the LAVPS group than in the non-LAVPS group (55 vs. 75 minutes; P < 0.001). Distal shunt revision within 30 days occurred more often for the non-LAVPS patients (6 of 481 [1.2%]) than for the LAVPS patients (0 of 199 [0%]). A subset analysis of patients with normal-pressure hydrocephalus found decreased total hospital charges in the LAVPS group ($67,124 vs. $80,890; P = 0.009). CONCLUSIONS: Compared with non-LAVPS, LAVPS was associated with significantly shorter operative times and fewer distal shunt revisions within 30 days. The findings from a subset analysis supported a decrease in total hospital charges. Additional studies are needed; however, these data suggest that LAVPS is a safer, less-expensive alternative to non-LAVPS.