Persistent syphilitic ocular manifestations despite treatment: a case series.

BACKGROUND: Penicillin has remained the most effective treatment for syphilis for several decades. Syphilitic retinal manifestations may persist following treatment and cause visual problems. In this case series, we describe three syphilis patients with persistent posterior segment manifestations due to chronic inflammation, incomplete treatment, and reinfection. CASE SERIES: Recommended initial treatment for all patients was 14 days of intravenous penicillin. Oral prednisone was added 48 h after initiation of penicillin therapy. Case 1: A 48-year-old female presented with gradual vision loss for two months. Fundus imaging revealed syphilitic outer retinopathy (SOR), papillitis, and acute syphilitic posterior placoid chorioretinopathy (ASPPC). After treatment, she had persistent cystoid macular edema (CME) and was treated with intravitreal triamcinolone injections and ketorolac drops. CASE 2: A 24-year-old male presented with sudden vision loss for two days. On imaging, he had ASPPC, papillitis, and SOR. IV penicillin treatment was given for 10 days only. He had persistent SOR and was retreated with doxycycline and prednisone. Case 3: A 52-year-old male presented with eye pain and visual loss for one week. There was evidence of ASPPC and papillitis on imaging. One month after treatment, he had persistent papillitis and was restarted on oral prednisone. One year later, he was found to have recurrent ASPPC and was confirmed to be reinfected with syphilis, for which he was retreated. CONCLUSION: When treating persistent syphilitic ocular manifestations, we recommend checking that the penicillin treatment was complete and the RPR titers are declining. If both hold true, then the affected eye should be treated with anti-inflammatory therapy. Other factors that contribute to poor visual prognosis include treatment delay, poor initial visual acuity, macular edema, and HIV coinfection.

Early Optical Coherence Tomography Signs of Erdafitinib-Induced Retinopathy.

A 64-year-old male presented for a baseline ophthalmic exam before beginning erdafitinib, a fibroblast growth factor receptor (FGFR) inhibitor, for stage 4 papillary urothelial cancer. Baseline optical coherence tomography (OCT) and ophthalmic examination were unremarkable. After one month of treatment, his OCT demonstrated a significant thickening of the ellipsoid zone and prominence of the interdigitation zones along with a small amount of subretinal fluid. Two months after discontinuation of the medication, the OCT returned to baseline. Erdafitinib is a Food and Drug Administration (FDA)-approved treatment for unresectable or metastatic urothelial cancer with FGFR2 or FGFR3 mutations. However, retinal toxicity can ensue with the initiation of the drug and cause subjective vision changes and OCT abnormalities. The drug may exert toxic effects on retinal pigment epithelium, which may be seen through interval OCTs and visualization of the interdigitation zone. Therefore, pronunciation of the ellipsoid and interdigitation zone on OCT may allow for surveillance of early manifestations of erdafitinib-induced retinal toxicity.

Factors affecting the development of cystoid macular edema following pars plana vitrectomy with silicone oil placement: a retrospective cohort study.

BACKGROUND: Cystoid macular edema (CME) can develop following silicone oil placement in complex vitreoretinal surgeries, contributing to poor visual outcomes. In this study, we investigated the clinical and surgical characteristics associated with the development of CME following the use of silicone oil (SO) in pars plana vitrectomy (PPV) surgeries. METHODS: We conducted a retrospective chart review of patients who underwent implantation of SO during PPV from 2010 to 2020 by a single surgeon. Patient demographics, type of oil, duration of oil tamponade, retinectomy size, diabetic status, lens status, prior panretinal photocoagulation, visual acuity, and incidence of CME were reviewed. RESULTS: This study included 43 eyes from 40 patients who underwent SO tamponade for retinal detachment (RD) surgery. The mean duration of SO tamponade was 15.7 ± 12.7 months (range: 1-58 months). The most common indication for surgery was diabetic tractional RD (32.7%), followed by traumatic RD (16.3%) and rhegmatogenous RD with proliferative vitreoretinopathy (11.6%). Of the 43 eyes, 18 (41.9%) developed CME for the first time after PPV with SO placement, with 8 (44%) resolving within a year of oil removal. The mean duration for the development of CME was 9 months. A logistic regression model showed that a scleral buckle procedure and poor initial vision were statistically significant factors for predicting the development of CME (ORs: 11.65 and 16.06, respectively). Overall, 91% of the patients had stable or improved vision after surgery. CONCLUSIONS: The use of a scleral buckle procedure and poor initial vision are significant factors for predicting CME following silicone oil tamponade in PPV surgeries, with 41.9% of patients developing CME with an average duration of 9 months. Recognizing such factors can lead to early monitoring and prompt management of CME. MEETING PRESENTATION: Partial analyses were presented at the ASRS 2020 conference. CLINICAL TRIAL NUMBER: Not applicable.

Utility of pan-bacterial and pan-fungal PCR in endophthalmitis: case report and review of the literature.

BACKGROUND: Endophthalmitis is a clinical diagnosis but identification of the disease-causing agent or agents allows for a more tailored treatment. This is routinely done through intraocular fluid cultures and staining. However, culture-negative endophthalmitis is a relatively common occurrence, and a causative organism cannot be identified. Thus, further diagnostic testing, such as pan-bacterial and pan-fungal polymerase chain reactions (PCRs), may be required. BODY: There are now newer, other testing modalities, specifically pan-bacterial and pan-fungal PCRs, that may allow ophthalmologists to isolate a causative agent when quantitative PCRs and cultures remain negative. We present a case report in which pan-fungal PCR was the only test, amongst quantitative PCRs, cultures, and biopsies, that was able to identify a pathogen in endophthalmitis. Pan-PCR has unique advantages over quantitative PCR in that it does not have a propensity for false-positive results due to contamination. Conversely, pan-PCR has drawbacks, including its inability to detect viruses and parasites and its increased turnaround time and cost. Based on two large retrospective studies, pan-PCR was determined not to be recommended in routine cases of systemic infection as it does not typically add value to the diagnostic workup and does not change the treatment course in most cases. However, in cases like the one presented, pan-bacterial and pan-fungal PCRs may be considered if empiric treatment fails or if the infective organism cannot be isolated. If pan-PCR remains negative or endophthalmitis continues to persist, an even newer form of testing, next-generation sequencing, may aid in the diagnostic workup of culture-negative endophthalmitis. CONCLUSION: Pan-bacterial and pan-fungal PCR testing is a relatively new diagnostic tool with unique advantages and drawbacks compared to traditional culturing and PCR methods. Similar to the tests' use in non-ophthalmic systemic infections, pan-bacterial and pan-fungal PCRs are unlikely to become the initial diagnosis test and completely replace culture methods. However, they can provide useful diagnostic information if an infectious agent is unable to be identified with traditional methods or if empiric treatment of endophthalmitis continues to fail.

CARING: Cannula for Alleviation of Retinal Injury Caused by Needle Fluidic Gashing.

Infusion-related iatrogenic retinal breaks (IRBs) are a significant complication in vitrectomies, particularly when smaller-gauge cannulas are used during fluid infusion. Using two-dimensional finite element analysis (FEA), we analyzed forces exerted on the retina from different cannulas: traditional 25-gauge, 20-gauge, 23-gauge, and 27-gauge, then investigated four alternative new cannula designs: (A) oblique orifices, (B) external obstruction, (C) side ports, and (D) perpendicular orifices. The analysis revealed that the standard 25-gauge cannula had a force of 0.546 milli-Newtons (mN). Optimized cannulas demonstrated decreased forces: 0.072 mN (A), 0.266 mN (B), 0.417 mN (C), and 0.117 mN (D). While all the designs decrease fluid jet force, each has unique challenges: Design A may complicate manufacturing, B requires unique attachment techniques, C could misdirect fluid toward the lens and peripheral retina, and D requires a sealed trocar/cannula design to prevent unwanted fluid ejection. These four innovative cannula designs, identified with detailed engineering simulations, provide promising strategies to reduce the risk of IRBs during vitrectomy, bridging the gap between engineering insights and clinical application.

Fractal Changes of the Retinal Microvasculature in Birdshot Chorioretinopathy.

BACKGROUND AND OBJECTIVE: Retinal vascular disorders are associated with lower fractal dimension (FD). We analyzed FD in birdshot chorioretinopathy (BSCR). PATIENTS AND METHODS: This was a retrospective cohort study. We performed optical coherence tomography angiography (OCTA) and analyzed FD of the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) using ImageJ. For each vascular layer, we analyzed the presence of BSCR, subjects' age, sex, and presence of diabetes mellitus to determine which may predict lower FD. RESULTS: We compared 28 eyes (14 patients) with BSCR to 34 control eyes (17 patients). Mean FD of BSCR was lower in SCP (1.584 [± 0.126] vs 1.706 [± 0.118], P < 0.001), DCP (1.637 [± 0.134] vs 1.780 [± 0.096], P < 0.001), and CC (1.884 [± 0.063] vs 1.917 [± 0.047], P = 0.036). FD of SCP was lower per increasing year (0.005 [P = 0.014]). Male patients had lower FD-DCP (0.101 [P = 0.043]). CONCLUSION: In BSCR, fractal analysis showed significant involvement of the SCP, DCP, and to a lesser extent the CC. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

Phacofragmentation of Posteriorly Dislocated Lens Fragments: Limbal versus Pars Plana Approach.

PURPOSE: To assess intra-operative complications and feasibility of removing crystalline lens fragments from the vitreous cavity through a limbal incision compared to a pars plana approach. DESIGN: Retrospective cohort study. SUBJECTS: 16 eyes underwent phacofragmentation via a limbal approach (Group A) and 9 eyes through a pars plana approach (Group B) at an academic center over a 10-year period. METHODS: We collected pre-operative, intra-operative, and post-operative data. We compared rates of intraoperative complications, including corneal wound burn, iris or capsular damage, retinal tears, and hemorrhage, and recorded post-operative BCVA and IOP measurements at the one-month post-operative appointment. We also compared rates of post-operative complications, including corneal edema, choroidal detachment, or retinal detachment. MAIN OUTCOME MEASURES: Primary outcomes of the study were the rates of intraoperative complications and the feasibility of crystalline lens removal with the limbal approach. We defined the latter outcome as the ability to complete lens removal without switching to the pars plana route. RESULTS: Mean BCVA for group A was 1.6, and for group B was 2.0 (p = .19). There was no significant difference between the two groups in the incidence of intraoperative complications, including corneal wound burn, iris damage, anterior capsular tear, iatrogenic retinal tear, or suprachoroidal hemorrhage (p > .99). There was no significant difference in the incidence of intra-operative vitreous hemorrhage (p = .36). Additionally, there was no significant difference in post-operative corneal edema (p = .27), choroidal detachment (p = .52), or retinal detachment (p > .99). The mean post-operative BCVA was 1.0 in group A and 1.0 in group B (p = .75). We completed all cases in group A using the limbal approach without switching to the pars plana route. CONCLUSION: Phacofragmentation through a limbal incision provides a feasible option for dropped nuclear fragment removal and is not associated with a higher risk of complications than the pars plana route.

Posterior Segment Manifestations of Syphilis and Correlation With Serologic Markers of Infection.

BACKGROUND AND OBJECTIVE: Retrospective analysis correlating serologic titers of ocular syphilis with posterior segment manifestations. PATIENTS AND METHODS: This study consisted of 40 patients (80 eyes imaged, 68 affected) with positive rapid plasma reagin (RPR) and Treponema Pallidum immunoglobulin G. We collected demographic and presentation data including HIV status, absolute CD4 count, RPR, cerebrospinal fluid-venereal disease research laboratory (CSF-VDRL) test, and retinal zone. We categorized imaging into syphilitic outer retinopathy (SOR), acute syphilitic posterior placoid chorioretinopathy, retinitis/chorioretinitis (RC), and papillitis. Multivariate analysis correlated HIV status, RPR, and VDRL titers with posterior segment findings and zone. RESULTS: Mean age of 42.8 ± 10.7 years, with 70% male patients. Presenting visual acuity (logMAR) 0.66 ± 0.74 did not correlate with RPR, nor was it associated with papillitis, RC, or acute syphilitic posterior placoid chorioretinopathy. Higher RPR (≥ 1:128) positively associated with SOR (P = 0.031) and zone 1 (odds ratio [OR], 1.62; P = 0.02), but negatively associated with zone 2 (OR 0.35; P = 0.005). HIV positivity increased RC odds (OR, 4.45; P = 0.047). CONCLUSION: Higher RPR correlated with SOR and zone 1, whereas HIV positivity correlated with RC. [Ophthalmic Surg Lasers Imaging Retina 2024;55:511-516.].

A patient with albinism and retinitis pigmentosa, a case report.

Purpose: To present a case of molecularly confirmed oculocutaneous albinism (OCA) and retinitis pigmentosa (RP). Observations: A 46-year-old male with a lifelong established diagnosis of OCA and baseline best corrected visual acuity (BCVA) of 20/200, presented for worsening visual acuity over the last few years. BCVA was light perception and hand motion at face for the right and left eye, respectively. Fundus exam showed hypopigmented fundi with visible choroidal vessels and blunted foveal reflexes in both eyes. Optical coherence tomography showed foveal hypoplasia and outer retinal degenerative changes not typical of OCA. Fundus autofluorescence (FAF) imaging showed focal areas of decreased signal at the fovea, similar to areas of atrophy in an age matched patient with PDE6A-RP. Genetic testing identified a homozygous disease-causing variant in TYR c.1467dup, p. (Ala490Cysfs*20) causing OCA, and a homozygous pathogenic variant c.304C > A, p. (Arg102Ser) in PDE6A causing autosomal recessive RP. Conclusions and importance: This is the first report of a patient with OCA and RP. The lack of pigmentary changes can make the diagnosis of RP challenging in patients with albinism. FAF can show features suggestive of RP and genetic testing can establish the diagnosis. The findings described herein may help physicians diagnose an extremely rare phenotype.

Sclerectomy Reverses Nanophthalmic Optic Neuropathy.

Introduction: Nanophthalmos is characterized by a short axial length, a thick choroid, and a thick sclera. Unilateral symptomatic disc swelling in nanophthalmos presents both a diagnostic and a therapeutic challenge. Case Presentation: A healthy 59-year-old man reported a two-week-long abrupt vision reduction in his right eye. 20/100 best spectacle (+17.25 diopter) corrected visual acuity, unilateral widespread disc enlargement, central scotoma, and a slight color vision disruption without an afferent pupillary defect were among the positive findings in the right eye. Workup for neuro-ophthalmology was negative. Numerous consultations did not suggest any form of treatment for the patient. Review of the optical coherence tomography (OCT) indicated a small, crowded optic nerve head and substantial diffuse choroidal thickening with dome-shaped temporal peripapillary area with choroidal expansion. In addition to circumferential anterior four-quadrant 95%-deep sclerectomy from recti insertion to the vortices, radial nasal posterior sclerotomy reaching the optic nerve sheath was performed on the patient. After the procedure, 2 weeks later, the patient's vision returned, and it persisted until the 6-month follow-up. By OCT, the two eyes were comparable as far as disc contour and nerve fiber layer thickness. Conclusion: This form of sclerectomy, which aims at decompressing the oncotic choroidal pressure, is an effective treatment for compressive optic neuropathy in the context of nanophthalmos. Could sclerectomy assist in treating other optic neuropathies associated with peripapillary pachychoroid?

The impact of the COVID-19 pandemic on ocular trauma in American infants and toddlers.

PURPOSE: To assess the impact of the COVID-19 pandemic on infant and toddler ocular trauma in the United States. METHODS: This retrospective analysis of ocular injury data for children ≤3 years of age focused on epidemiologic trends in pediatric ocular injuries in the United States caused by consumer products from 2017 to 2021 and compared differences between pre-pandemic (2017-2019) and pandemic (2020-2021) time periods. Data were collected from the US Consumer Product Safety Commission National Electronic Injury Surveillance System, which includes emergency department visits caused by consumer product-related injuries from a nationally representative sample of hospitals. RESULTS: The national-level estimate of ocular injuries in infants and toddlers was 51,250 (95% CI, 30471-72030). Most injuries occurred at home. We found a significant decline in the proportion of projectile ocular injuries from 0.89% (95% CI, 0.35-2.25) to 0.12% (95% CI, 0.03-0.45) (P = 0.037). The proportion of patients diagnosed with chemical-burn-related injuries increased significantly, from 23.34% (95% CI, 19.73-27.38) in the pre-pandemic period to 31.63% (95% CI, 26.98-36.69) in the pandemic period (P = 0.048), with 71.75% (95% CI, 65.25-77.46) due to cleaning products. After adjusting for confounding variables, the odds of sustaining a chemical-burn-related injury in the post-pandemic period were 1.51 times higher than in the pre-pandemic period (95% CI, 1.10-2.08). CONCLUSIONS: The proportion of children diagnosed with chemical-burn-related injuries increased significantly in the post-pandemic period, with a large portion due to cleaning products.

Effect of Oral Prednisone on the Prevention and Management of Proliferative Vitreoretinopathy After Open-Globe Injury.

Purpose: To determine the impact of oral prednisone on the final visual acuity (VA) and prevention of proliferative vitreoretinopathy (PVR) in patients having pars plana vitrectomy (PPV) for globe injuries. Methods: A retrospective chart review was performed of all globe injuries with an initial repair and subsequent PPV between 2009 and 2018. Data included the initial VA, zones of injury, initial closure date, time to secondary intervention (PPV), oral prednisone (1 mg/kg/day) use, the final VA, and enucleation rate. Multivariable regression models were used to assess the impact of oral prednisone use on anatomic and functional outcomes. Results: The mean (±SD) patient age was 46.25 ±18.56 years (range, 13-92); 131 (83.9%) were men. Oral prednisone intake was recorded in 81 patients (52.3%). The prednisone group had significantly more zone 3 involvement (P = .001), worse initial VA (2.28 vs 1.92 logMAR; P = .003), and a greater mean number of surgeries (P = .020) than the no-steroids (control) group but an equivalent final logMAR VA (1.57 vs 1.52; P = .881). The prednisone group had significant VA improvement (P = .025); however, oral prednisone use did not predict the development of PVR (29.23% vs 12.90%; odds ratio [OR], 2.81; 95% CI, 0.89-8.85) or retinal detachment (27.27% vs 29.58%; OR, 0.59; 95% CI, 0.23-1.56). Conclusions: Despite a worse initial clinical presentation, patients who received oral prednisone had significant visual improvement compared with the control group. However, oral prednisone (1 mg/kg/day) use at the time of injury did not decrease the PVR rate.

Maculopathy in a 52-Year-Old Patient With Diabetes.

A 52-year-old woman with severe nonproliferative diabetic retinopathy had decreased vision and macular edema in both eyes that was not responding to treatment. What would you do next?

Belzutifan-induced regression of retinal capillary hemangioblastoma: A case-series.

Purpose: To report a series of three patients with von Hippel-Lindau (VHL) disease who demonstrated regression of their retinal hemangioblastomas (RH) using belzutifan in conjunction with photocoagulation therapy. Observations: Patient 1, a 23-year-old female, presented with multiple RHs in her right eye (OD) that were lasered. Her left eye (OS) revealed a large inferotemporal RH that measured approximately 2.1 mm2. Systemic belzutifan was administered. Four months after initiation of treatment, the lesion regressed to 1.4 mm2, but belzutifan was not well-tolerated and was discontinued due to side effects. At the date of belzutifan discontinuation, the lesion measured about 1.1 mm2. Focal laser photocoagulation was applied. The lesion regressed to around 0.6 mm2. Two additional laser treatments were applied one month later. On the most recent follow-up, the lesion was completely fibrosed.Patient 2, a 32-year-old male, presented with one RH OD and two RHs OS. Belzutifan was administered for one month before the patient began experiencing side effects of the medication. Consequently, the dose of belzutifan was decreased. After one month with the lowered dose, laser coagulation was applied to OS. In the most recent follow-up, five months after the initial presentation, the lesions remain less vascularized and reduced in size.Patient 3, is a 44-year-old male with a large RH OD. Following seven months of belzutifan daily, there was a significant reduction in the RH size. Conclusions: Belzutifan, a hypoxia-inducible factor inhibitor, is an FDA-approved medication for VHL disease associated with renal cell carcinoma, central nervous system hemangioblastomas, or pancreatic neuroendocrine tumors that do not require immediate surgical resection. Because of the high incidence of VHL-associated RHs, adjuvant laser photocoagulation therapy when belzutifan is suspended or withheld can allow for the regression of large lesions. In this case series, we also propose a reproducible and technically simple method to measure RH lesions size, using Optos fundus imaging.

Fractal Changes of the Retinal Microvasculature in Syphilitic Uveitis.

PURPOSE: To quantify chorioretinal microvascular damage and recovery post-treatment in patients with acute syphilitic posterior placoid chorioretinitis (ASPPC) using fractal dimension (FD). METHODS: Retrospective cohort study of patients with serologically confirmed syphilitic uveitis. We obtained optical coherence tomography angiography (OCTA) scans at baseline and follow-up after intravenous penicillin treatment and computed FD of the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) using ImageJ. RESULTS: We enrolled seven patients with ASPPC (11 eyes), and 17 control subjects (34 eyes). Pre-treatment averages of FD-SCP, FD-DCP, and FD-CC were: 1.672 (±0.115), 1.638 (±0.097), and 1.72 (±0.137); post-treatment: 1.760 (±0.071), 1.764 (±0.043), and 1.898 (±0.047). After treatment FD-CC increased in all 11 eyes with an average of 0.163 (p = 0.003); FD-DCP increased in 10 (91%) eyes with an average of 0.126 (p = 0.003); and FD-SCP increased in seven (64%) eyes with an average of 0.089 (p = 0.059). Compared to the post-treatment FD values in the syphilitic group, controls had similar FD-SCP (p = 0.266), FD-DCP (p = 0.078), and FD-CC (p = 0.449). CONCLUSIONS: CC and DCP are mostly affected in ASPPC with minimal changes in the SCP. All vascular layers FD recovered after completing antibiotic treatment.