Update on maculopathy secondary to pentosan polysulfate toxicity.

PURPOSE OF REVIEW: The aim of the present review is to provide a comprehensive summary of available knowledge regarding toxic maculopathy secondary to pentosan polysulfate sodium (PPS). RECENT FINDINGS: PPS toxicity was described in 2018, and additional studies characterize it as dysfunction of the retinal pigment epithelium centered on the posterior pole, which can progress despite drug cessation. Requisite exposure can be as little as 0.325 kg and 2.25 years but averages closer to 1-2 kg and 10-15 years. Multimodal imaging should include near-infrared reflectance, optical coherence tomography, and fundus autofluorescence. Cross-sectional studies demonstrate evidence correlating cumulative dosing and the likelihood/severity of maculopathy. Early estimates of prevalence range from 12.7 to 41.7% depending on dosing, with overall rates around 20%. SUMMARY: Reasonable evidence associates maculopathy with extended exposure to PPS, with an average reported incidence of around 20% in patients with long-term exposures. Patients with unexplained retinal pigment epithelium changes and difficulty with dark adaptation should be questioned regarding PPS exposure, and patients with known exposure to PPS should be examined. Further research is needed to refine screening protocols. Currently, providers should consider baseline examination and examination at 5 years and/or 500 g of exposure followed by yearly screening.

Intracapsular hemorrhage rates in non-fixated nylon sheet orbital implants for orbital fracture management.

PURPOSE: To examine the incidence of intracapsular hemorrhage in orbital fracture repair with non-fixated nylon sheet implants. METHODS: A retrospective chart review of 227 patients presenting from January 2013 to December 2016 for orbital fracture repair with nylon sheet implants. RESULTS: Of the 331 orbital fractures repaired over 4 years, a total of 227 met inclusion criteria. The average implant thickness was 0.38 mm and no implants were fixated. Four total implants (1.8%) were removed due to complications; one each secondary to exploration for ongoing postoperative diplopia, immediate post-operative orbital hemorrhage, a cystic mass anterior to the implant, and pain. There were no cases of intracapsular hemorrhage nor infection for any of the 227 patients over 4 years. CONCLUSIONS: To the authors knowledge, this represents the largest case series to date to assess the rate of intracapsular hemorrhage in non-fixated nylon sheet orbital implants. In the 227 cases reviewed over a 4-year period, there were no cases of intracapsular hemorrhage. This suggests a much lower complication rate than previously reported. PRéCIS: A case series of 227 patients who underwent orbital fracture repair with non-fixated nylon sheet implants.

Pseudotumor Cerebri Syndrome with Resolution After Discontinuing High Vitamin A Containing Dietary Supplement: Case Report and Review.

A 24-year-old non-obese, but slightly overweight, female presented with a two-week history of progressive severe headache associated with two days of blurry vision. Clinical exam was significant for bilateral papilledema and an enlarged blind spot on visual field testing. Contrast enhanced MRI head revealed no space occupying lesion. A lumbar puncture revealed an elevated opening pressure of 38 cm H2O with normal cerebrospinal fluid composition leading to a diagnosis of pseudotumor cerebri syndrome (PTCS). The patient lacked the typical risk factors of high body mass index or obvious antecedent medications; however, on subsequent questioning, she was chronically ingesting a high vitamin A containing weight loss dietary supplement (Thrive W® - Table 1), which we believe had caused intracranial hypertension. Discontinuation of the diet pill and treatment with acetazolamide led to marked improvement of her PTCS. This case highlights the fact that non-traditional products or medications with high vitamin A may cause pseudotumor cerebri, which treating physicians should assess for while dealing with non-obese PTCS patients.

Herpes-Zoster-Related Choroidopathy - A Report of Two Cases.

PURPOSE: To report a rare finding, choroidopathy, in herpes zoster ophthalmicus (HZO). METHODS: Report of two cases. RESULTS: Multiple, well-defined, choroidal depigmented lesions were demonstrated in two cases of HZO on fundus color imaging, optical coherence tomography, fundus angiography, and indocyanine green angiography at 6 months. CONCLUSION: This report demonstrates a very rare, late finding of choroidal involvement in two HZO cases.

Accuracy of Ophthalmology Clinic Follow-Up in the Incarcerated Patient Population.

Purpose Incarcerated patients represent a uniquely vulnerable population in the outpatient ophthalmology setting, and the reliability of follow-up in this group is undetermined. Methods This was a retrospective, observational chart review of consecutive incarcerated patients evaluated at the ophthalmology clinic of a single academic medical center between July 2012 and September 2016. For each encounter the following were recorded: patient age, gender, incarcerated status at the time of encounter (a subset of patients had encounters before/after incarceration), interventions performed, follow-up interval requested, urgency of follow-up, and actual time to subsequent follow-up. Primary outcome measures were no-show rate and timeliness, which was defined as follow-up within 1.5× the requested period. Results There were 489 patients included during the study period, representing a total of 2,014 clinical encounters. Of the 489 patients, 189 (38.7%) were seen once. Of the remaining 300 patients with more than one encounter, 184 (61.3%) ultimately did not return and only 24 (8%) were always on time for every encounter. Of 1,747 encounters with specific follow-up requested, 1,072 were considered timely (61.3%). Factors significantly associated with subsequent loss to follow-up include whether a procedure was performed ( p < 0.0001), urgency of follow-up ( p < 0.0001), incarcerated status ( p = 0.0408), and whether follow-up was requested ( p < 0.0001). Conclusion Almost two-thirds of incarcerated patients in our population requiring repeat examination were lost to follow-up, particularly those who underwent an intervention or required more urgent follow-up. Patients entering and exiting the penal system were less likely to follow-up while incarcerated. Further work is needed to understand how these gaps compare to those in the general population and to identify means of improving these outcomes.

Short-term outcomes in patients with center-involving diabetic macular edema after a single dose of intravitreal bevacizumab.

BACKGROUND: A significant portion of diabetic macular edema (DME) is refractory to anti-vascular endothelial growth factor (anti-VEGF) agents. This study investigates morphological and functional outcomes to a single intravitreal bevacizumab (IVB) injection in patients with center-involving DME (ciDME) at 4-6 weeks and compares treatment responders and non-responders based on spectral domain optical coherence tomography (SD-OCT) features. METHODS: IRB approved observational, retrospective chart review of patients with ciDME, identified by ICD-10 code, who received IVB and underwent baseline and 4-6 weeks follow-up SD-OCT imaging between January 1, 2016 and January 19, 2021. Patients who had received previous treatment with anti-VEGF or intraocular steroids within 1 year were excluded. Variables included best-corrected visual acuity (BCVA), central subfield thickness (CST) and total macular volume (TMV). Eyes were classified as responders if CST reduction was greater than 10%. OCT scans were graded qualitatively by two masked graders using Imagivault software. Paired Student's t-tests, Wilcoxon signed rank tests and Chi-Square tests were used for analysis. RESULTS: A total of 334 prospective subjects were identified, and after applying exclusion criteria 52 eyes from 46 patients (mean age 64.22 ± 8.12 years, 58.7% male) were included. Mean BCVA did not significantly change with treatment, 63.9 ETDRS letters (~ 20/50) at baseline and 65.9 ETDRS letters (~ 20/50) post-treatment (p = 0.07). Mean CST decreased from 466 ± 123 µm at baseline to 402 ± 86 µm post-treatment (p < 0.001). 22 (42.3%) of eyes were categorized as responders and 30 (57.7%) as non-responders. Average change in CST from baseline in responders was -164 µm (p < 0.001) and + 9 µm in non-responders (p = 0.47). Vitreomacular adhesion (VMA) was more prevalent in non-responders (28.7% vs. 4.8%, p = 0.03). In addition, cyst location in the inner nuclear layer (INL) was present more frequently in responders (95.5% vs. 73.3%, p = 0.037) as was subretinal fluid (45.5% vs. 13.3%, p = 0.01). CONCLUSION: The short-term response to a single IVB was sub-optimal with structural but no functional improvements. Greater baseline CST, presence of INL cysts and subretinal fluid may represent factors indicative of a better treatment response.

New Insights Into Pentosan Polysulfate Maculopathy.

BACKGROUND AND OBJECTIVE: To provide new insights into toxic maculopathy secondary to pentosan polysulfate (PPS) utilizing multimodal testing. PATIENTS AND METHODS: Retrospective case-series of four patients from two academic centers evaluated with multimodal imaging, electrophysiology, dark adaptometry (DA), and genetic testing. RESULTS: Median age was 58 years, exposure to PPS was 18.5 years, and cumulative dose of was 2,025 grams. Seven of eight eyes had visual acuity of 20/40 or better. Optical coherence tomography (OCT) angiography demonstrated increased choriocapillaris flow voids (54.25%) in cases compared to controls (13.2%). Two subjects had abnormal foveal avascular zone configurations. Two subjects demonstrated collapse of the retinal pigment epithelium nodular excrescences and progressive retinal thinning over 4 to 5 years on OCT. Electrophysiology was normal (3/3 patients), but DA was delayed (2/2 patients). CONCLUSIONS: The authors describe novel findings of PPS maculopathy, including flow voids in the choriocapillaris. Progressive retinal thinning may suggest a secondary retinal effect. These findings may improve understanding of the pathophysiology. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:13-22.].

Glaucoma therapy by extended release of timolol from nanoparticle loaded silicone-hydrogel contact lenses.

Glaucoma is the second major cause of blindness in the world after cataract. Glaucoma management through eye drops that reduce the intraocular pressure (IOP) has major deficiencies including low patient compliance and low bioavailability. Extended wear contact lenses that deliver glaucoma drugs for extended periods could increase patient compliance, while also increasing the bioavailability. To develop extended wear contact lenses that can also provide extended glaucoma therapy, we disperse nanoparticles of PGT (propoxylated glyceryl triacylate) that contain a glaucoma drug timolol. The particles can also be loaded into prefabricated lenses by soaking the lenses in a solution of particles in ethanol. The particle loaded gels can release timolol in phosphate buffered saline (PBS) for about a month at room temperature. The most likely rate controlling mechanism is hydrolysis of the ester bond that links timolol to the PGT matrix, but other mechanisms such as water and drug diffusion, drug dissolution, drug-polymer chain cleavage, time-dependent drug permeability within the polymeric matrix, etc. may also be important. Nanoparticle incorporation in the silicone hydrogels results in reduction in ion and oxygen permeabilities, and an increase in modulus, and the impact on each of these properties is proportional to the particle loading. A gel with 5% particle loading can deliver timolol at therapeutic doses for about a month at room temperature, with a minimal impact on critical lens properties. Preliminary animal studies in Beagle dogs conducted with lenses in which particles are loaded by soaking the lenses in ethanol show a reduction in IOP.