Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity.

PURPOSE: To describe the optical coherence tomography features of pachyvitelliform maculopathy (PVM), an acquired vitelliform lesion (AVL) associated with pachychoroid disease. METHODS: This study was a retrospective, multicentre, observational analysis.Medical records and multimodal imaging were reviewed in all patients with pachychoroid disease and AVL. Visual acuity, central choroidal thickness (CCT), AVL dimensions, total choroidal area, luminal choroidal area, stromal choroidal area and choroidal vascular index were measured in all eyes with PVM and compared with normal age-matched control eyes. RESULTS: Mean age of the PVM group (17 eyes of 17 patients) was 71.41 years. Average follow-up was 33.15 months. Baseline VA was 20/40 in the PVM group and declined to 20/100 (p=0.006). AVLs were all detected overlying pachyvessels with optical coherence tomography and were all hyperautofluorescent with fundus autofluorescent imaging. Mean CCT in the PVM group was significantly greater (352.35 µm) than the CCT in the control group (226.88 µm, p<0.001). Retinal pigment epithelium (RPE) disruption was present in 64.71% of eyes with PVM at baseline and 41.18% developed macular atrophy at the end of follow-up. CONCLUSIONS: PVM, defined by the presence of AVL associated with pachychoroid features, is a distinct novel entity of the pachychoroid disease spectrum. This study suggests a possible pathogenesis of RPE dysfunction secondary to a thick choroid, leading to accumulation of undigested photoreceptor outer segments and AVL. Clinicians should be aware of this common cause of vitelliform lesions and the poor visual prognosis due to the high risk of atrophy development.

Recurrent Anterior Uveitis Associated with Major Fluctuations in Choroidal Thickness in Patient with Pachychoroid Disorder.

PURPOSE: To describe remarkable choroidal thickness fluctuations corresponding to episodes of recurrent anterior uveitis with subretinal fluid development when exceeding a choroidal thickness threshold. METHODS: A patient with pachychoroid pigment epitheliopathy and unilateral acute anterior uveitis of the left eye was evaluated over a period of 3 years with multimodal retinal imaging including optical coherence tomography (OCT). Longitudinal changes in subfoveal choroidal thickness (CT) were measured and correlated with episodes of recurrent inflammation. RESULTS: Over the course of 5 recurrent episodes of inflammation in the left eye treated with oral antiviral and topical steroid therapy, subfoveal CT increased as much as 200 um or more. Subfoveal CT in the fellow quiescent right eye by contrast, was within normal limits and minimally changed throughout the follow up. Increased CT occurred with each episode of anterior uveitis and decreased by 200 µm or more during periods of quiescence in the affected left eye. Subretinal fluid and macular edema developed with a maximum CT of 468 um and spontaneously resolved when CT decreased after treatment. CONCLUSION: In eyes with pachychoroid disease, anterior segment inflammation may lead to marked increases in subfoveal CT and the development of subretinal fluid at a threshold thickness value.

NINE-YEAR PROGRESSION OF PENTOSAN MACULOPATHY WITH MULTIMODAL RETINAL IMAGING.

BACKGROUND/PURPOSE: The purpose of this study was to describe the characteristic pattern of progression of pentosan polysulfate (PPS) maculopathy with multimodal retinal imaging in two patients, including one with over 9 years of follow-up. METHODS: Two patients with PPS maculopathy were sequentially evaluated with near-infrared reflectance (NIR) and optical coherence tomography. RESULTS: Near-infrared reflectance showed characteristic centrifugal progression of the parafoveal hyperreflective lesions toward the vascular arcades with the development of hyporeflective areas in both cases. Optical coherence tomography demonstrated focal retinal pigment epithelium (RPE) thickening that corresponded to the hyperreflective lesions on NIR. On subsequent optical coherence tomography scans, the hyperreflective areas resolved with the development of ellipsoid zone attenuation, RPE disruption, and atrophy, which colocalized with hyporeflectivity on NIR. CONCLUSION: This report describes the progression of pentosan polysulfate maculopathy over almost 10 years of PPS treatment and highlights the importance of NIR as a tool for the diagnosis and monitoring of PPS maculopathy. Pentosan polysulfate lesions present as areas of focal RPE thickening with ensuing development of ellipsoid zone loss and RPE drop-out. The pathophysiology of PPS toxicity is unknown and may either result from primary RPE or choroidal toxicity.

Epidemiology of Pars Plana Vitrectomy in the Elderly: A Retrospective 10-Year Survey of 592 Cases.

PURPOSE: To report the epidemiology of the surgical indications of pars plana vitrectomy in patients aged 75 years or older. PATIENTS AND METHODS: The medical records of patients who underwent vitrectomy surgery in a single center between 2008 and 2018 were evaluated retrospectively for demographics, medical background, and surgical indications. Data on patients aged 75-85 were compared to those of patients older than 85 years and those recorded between 2008-2013 and 2014-2018. RESULTS: A total of 592 patients were included, of whom 55% were males. The mean±standard deviation age of the cohort at presentation was 80.4±4.60 years, and the mean visual acuity was 1.3±0.93 logMAR. The main indications for surgery were retinal detachment (n=117, 19.7%), epiretinal-membrane/vitreomacular traction (n=140, 23.6%), dropped intraocular lens/retained lens (n=89, 15%), macular hole (n=64, 11%), submacular hemorrhage (n=40, 6.7%), diagnostic vitrectomy (n=37, 6.2%), non-diabetic vitreous hemorrhage (n=30, 5.1%), and diabetic vitreous hemorrhage and other diabetic complications (n=33, 5.6%). Patients aged 85 years and older had higher rates of urgent surgeries (65%), such as for submacular hemorrhage and trauma (p=0.0000039 and p=0.001, respectively), and lower rates of non-urgent surgeries, such as for epiretinal membrane and macular hole (p=0.000032 and p=0.02, respectively), compared to patients aged 75-84 years. Additionally, the surgical rate for submacular hemorrhage decreased during 2008-2014 compared to 2014-2018 (p=0.000014). CONCLUSION: Understanding the unique distribution of indications for vitrectomy among elderly patients is essential for appropriate management and treatment. Urgent cases represent 65% of the surgeries performed in the older population.

Pediatric retinal damage due to soccer-ball-related injury: Results from the last decade.

OBJECTIVE: To outline the incidence of posterior segment injuries related to soccer-ball blunt trauma in children. METHODS: Retrospective search of the computerized hospital medical database between the years 2007 and 2017. All pediatric trauma cases were reviewed and cases with blunt trauma related to direct orbital/ocular hit from a soccer-ball were included. Cases were divided into two groups (non-severe and severe) based on the presence of sight-threatening findings on presentation (e.g. retinal tear, vitreous hemorrhage, retinal detachment, and macular edema). RESULTS: Out of 343 pediatric patients with relevant diagnoses, 14 (4.1%) were treated for injuries related to soccer-ball trauma. All patients were males at their early-to-mid teens (14.3 ± 2.1 years). The most common funduscopic finding was peripheral commotio retina (13, 93%). There was equal distribution between the two groups (seven each). Retinal injury in the severe group included retinal tear (3), vitreous hemorrhage (4), retinal detachment (1), and macular hole (1). Five patients in this group presented with visual acuity of 20/25 or better. Rate of external signs of injury were similar in both groups. CONCLUSION: Soccer-ball blunt trauma in children can cause significant posterior segment injuries regardless of the presence of external injury or ocular complaints. A thorough ocular exam is mandatory in all cases for the detection of vision-threatening retinal injuries.

Blunt ocular trauma in patients over 70: Clinical characteristics and prognosis.

PURPOSE: To describe the mechanisms and outcomes of open globe injuries (OGIs) in patients 70 years or older. METHODS: A retrospective chart review of patients from two large academic centers in Israel who were underwent surgery for OGI. Data was collected from surgical and clinic charts. Anatomical and functional results were recorded as well any additional surgeries and complications. RESULTS: Thirty-two eyes of 32 patients were included. The mechanism of OGI consisted of blunt trauma with ocular rupture secondary to falls. All eyes underwent immediate primary closure of the corneal and scleral lacerations. Additional surgeries were performed in 34.3% of cases. In 31.2% of cases additional surgery was not required, and in 21.9% of cases the damage was so devastating that additional procedures were not recommended. Four patients (12.5%) chose not to undergo additional surgery. Mean visual acuity at presentation was 2.24 ± 0.72 logMAR and it improved to 1.92 ± 0.89 logMAR at final follow-up (p = 0.002). VA at presentation was significantly correlated to final VA (p < 0.001). CONCLUSIONS: OGI in the elderly represents a unique group. It is mostly related to falls, with a female predominance and a poor visual prognosis. In this patient population, general health concerns and increased risks of anesthesia require special attention. Care should be taken to educate elderly patients and their caretakers on how to avoid falls.

Microarchitecture of Schlemm Canal Before and After Cataract Extraction Surgery.

PRECIS: Schlemm canal (SC) expands after cataract extraction (CE), both in the area and in volume by 25% as was measured using enhanced-depth imaging optical coherent tomography (EDI-OCT) in patients before and 1 week after CE. PURPOSE: This study aims to characterize the structural and volume changes on the microstructure of SC in patients before and after uneventful phacoemulsification CE by using EDI-OCT. MATERIALS AND METHODS: Forty-one serial horizontal EDI-OCT B-scans (interval between B-scans, 69 µm) were obtained in the nasal corneoscleral limbus before and 1 week after CE. The structure of aqueous channels, conjunctival blood vessels and iris anatomy in each scan were used as landmarks to select for overlapping scans taken before and following CE. The SC cross-section area was measured in each of the selected scans and SC volume was determined following a 3-dimensional reconstruction. RESULTS: Eleven eyes (6 females and 5 males) were imaged successfully before and after CE. Mean age was 70.54±11.38 years. The mean axial length was 23.10±0.87 mm. After CE, the mean best-corrected visual acuity in logMAR improved from 0.4±0.13 to 0.2±0.13 (P=0.028). There was no significant change in the mean intraocular pressure before and after CE (15.09±1.33 to 15.0±2.16 mm Hg; P=0.39). The mean SC cross-section area increased by 25%, from 4744±376 to 5941±1048 µm (P<0.001). SC volume in the analyzed region increased by 25% from 6,641,473±585,954 to 8,317,909±1,328,809 µm (P<0.001). CONCLUSION: CE expands SC dimensions in healthy eyes. EDI-OCT imaging of SC may prove useful in the evaluation of the SC dimensions in vivo before and after CE.

A comparison of plusoptiX A12 measurements with cycloplegic refraction.

PURPOSE: To test the accuracy and reliability of the plusoptiX A12 in detecting amblyogenic risk factors. METHODS: We prospectively collected data on children undergoing screening with the plusoptiX A12, cycloplegic refraction, and complete ophthalmic examination. American Association for Pediatric Ophthalmology and Strabismus (AAPOS) 2013 guidelines for the detection of amblyogenic risk factors were used for plusoptiX A12 screening and comparison of the results of both examination modes. RESULTS: Data on 402 eyes of 201 children (mean age, 7.63 ± 3.41 years) was collected. Mean (with standard deviation) cycloplegic refraction results were as follows: sphere, 0.88 ± 1.5 D; cylinder, -0.61 ± 0.74 D; axis, 71.17 ± 71.04; and spherical equivalent, 0.68 ± 2.63. The plusoptiX A12 measurements were as follows: sphere, 0.58 ± 1.4 D; cylinder, -0.66 ± 0.77 D; axis, 77.3 ± 68.9; and spherical equivalent, 0.25 ± 1.3. We found a strong correlation (Pearson) for sphere (r = 0.91), cylinder (r = 0.81), and axis (r = 0.7). The mean difference of the myopic spherical component between the plusoptiX and cycloplegic refraction was -0.048 ± 0.55 (95% LoA, +1.04 to -1.14 D); for the hyperopic spherical component, 0.37 ± 0.93 (LoA, +2.20 to -1.45 D); and for the cylindrical component, 0.05 ± 0.32 (LoA, +0.68 to -0.57D). The sensitivity, specificity, positive and negative predictive values for myopia were, respectively, 86%, 93%, 82%, and 94%; for astigmatism, 85%, 98%, 88% and 98%; and for hyperopia, 40%, 100%, 100%, and 98%. CONCLUSIONS: The plusoptiX A12 accuracy is high in all subgroups but better in the myopic, astigmatic, and anisometropic subgroups. Reliability was lower in the hyperopic eyes, possibly resulting in underestimation of hyperopic refractive error.