Truxima (rituximab-abbs) for Periocular Xanthogranuloma with Adult-Onset Asthma and Systemic IgG4-Related Disease.

A 58-year-old female with a 3-year history of adult-onset asthma, bilateral blepharoptosis, dry eye, and yellow-orange xanthelasma-like plaques extensively involving both upper eyelids presented with a diagnosis of adult-onset asthma with periocular xanthogranuloma (AAPOX) and systemic IgG4-related disease. Over the next 8 years, she received 10 intralesional triamcinolone injections (40-80 mg) in the right upper eyelid, 7 intralesional triamcinolone injections (30-60 mg) in the left upper eyelid, underwent right anterior orbitotomy twice followed by 4 doses of rituximab (1000 mg intravenous infusion) without regression of the AAPOX. The patient was then treated with 2 monthly doses of Truxima (1000 mg intravenous infusion), a biosimilar to rituximab. At the most recent follow-up, 13 months later, the xanthelasma-like plaques and orbital infiltration had markedly improved. To the best of the authors' knowledge, this is the first report of Truxima being used to treat AAPOX with systemic IgG4-related disease and to generate a sustained clinical response.

Thinning of the Skull Base and Calvarial Thickness in Patients With Idiopathic Intracranial Hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a disorder characterized by elevated intracranial pressure without secondary causes on neuroimaging. IIH typically occurs in young, obese female patients and, when severe, can cause permanent and irreversible vision loss. The association between skull base thinning in patients with intracranial hypertension and obesity has been previously reported; however, no study has reported these findings in IIH. The goal of our study is to determine whether IIH is independently associated with skull base and calvarial thinning. METHODS: A retrospective, matched case-control study was performed. Each patient diagnosed with IIH (case) was matched with a patient diagnosed with headache (control) by age, gender, and race. Patients were included if they underwent computed tomographic imaging of the head, maxillofacial, or orbits within 3 months of their diagnosis. Exclusion criteria were history of skull base or frontal bone pathology because of surgery or skull trauma, central nervous system infections, or incomplete radiologic data. Patient demographics, medical history, clinical examination, and skull base, calvarial, and zygoma thickness were recorded. Skull base thickness was measured by the height of the auditory canal in the coronal plane. Calvarial thickness was measured just anterior to the foramen rotundum in the coronal plane. Extracranial zygoma thickness was measured and used as an internal imaging control because the zygoma is not subject to intracranial forces. RESULTS: One hundred twenty-six patients were included in the study, 63 cases and 63 controls. Each group comprised 61 female patients (97%), 24 (38%) Caucasian, 23 (37%) black, 1 (2%) Asian, and 15 (24%) others. The average age was 31.5 ± 8.7 years. Patients with IIH were more likely to be obese (n = 60, 95%) compared with the control patients (n = 23, 37%, P < 0.001). All patients with IIH underwent lumbar puncture (LP) with an average opening pressure (OP) of 40.5 ± 15.6 cm H2O, whereas only 13 (20%) controls underwent an LP with a mean OP of 19.5 ± 8.5 cm H2O. There was no statistical difference in mean visual acuity between the IIH and control groups (logMar 0.22 [20/30] ± 0.45 vs logMar 0.09 [20/25] ± 0.30, P = 0.093, respectively). Compared with the controls, patients with IIH were more likely to have headache (97% vs 74%, P = 0.001), pulsatile tinnitus (48% vs 7%, P < 0.001), horizontal binocular diplopia (24% vs 4%, P = 0.006), confrontational visual field deficit (23% vs 2%, P = 0.003), and papilledema (74% vs 0%, P < 0.001). Patients with IIH had thinner skull base and calvarium width compared with the controls (mean skull base thickness 4.17 ± 0.94 mm vs 5.05 ± 1.12 mm, P < 0.001 and mean calvarial width 1.50 ± 0.50 mm vs 1.71 ± 0.61 mm, P = 0.024). Zygoma thickness was similar in both groups (mean zygoma thickness 1.18 ± 0.30 mm in the IIH group vs 1.26 ± 0.35 mm in the control group, P = 0.105). In a subgroup analysis controlling for obesity (body mass index >30 kg/m2), there was no statistically significant difference in skull base, calvarial, or zygoma thickness between obese and nonobese patients. CONCLUSIONS: Patients with IIH have thinner mean skull base and calvarial thickness compared with the controls. There was no difference in the mean extracranial zygoma thickness, which was the internal imaging control. Contrary to previous reports, we did not find an association between obesity and skull base or calvarial thinning. These findings suggest that IIH is associated with skull base and calvarial thinning.

Impact of Tumor Pigmentation in 6934 Patients with Uveal Melanoma at a Single Center.

Purpose: To evaluate clinical features and outcomes associated with degree of tumor pigmentation in patients with uveal melanoma (UM) of the choroid and ciliary body. Design: Retrospective observational study. Subjects: Six thousand nine hundred thirty-four consecutive patients with choroidal or ciliary body melanoma between 1971 and 2007 from a single ocular oncology center. Methods: Data on patient demographics, tumor characteristics, treatment approach, and clinical outcomes were collected. Comparisons between pigmented (>80% pigmentation by surface area), partially pigmented (20%-80%), and nonpigmented tumors (<20%) were performed using relevant hypothesis testing. Survival analyses for metastasis and melanoma-related death were conducted using the Kaplan-Meier method with log-rank tests for univariate comparisons. A multivariate Cox regression analysis was performed to assess the independent effects of multiple covariates on time-to-metastasis. Main Outcome Measures: Extraocular extension, ocular melanocytosis, time to tumor recurrence, tumor location, and melanoma-related metastasis and death. Results: There were 6934 eyes with UM and the degree of tumor pigmentation was classified as pigmented (n = 3762; 54%), partially pigmented (n = 2115; 31%), or nonpigmented (n = 1057; 15%). Pigmented UM was associated with extraocular extension (P < 0.001), ocular melanocytosis (P = 0.003), earlier tumor recurrence (P < 0.001), and more anterior tumor epicenter location (ciliary body, and equator to ora serrata) (P < 0.001). Pigmented UMs also exhibited the highest 10-year metastasis rate at 26%, compared with 19% for partially pigmented UMs and 16% for nonpigmented UMs (P < 0.001). Kaplan-Meier survival curves demonstrated differences among the tumor pigmentation groups for melanoma-related metastasis (P < 0.001) and melanoma-related death (P < 0.001). Multivariate Cox regression analysis for melanoma-related metastasis showed that pigmented UMs had a 29% higher relative risk of developing metastasis compared with partially pigmented UMs (P = 0.002) and a 54% higher relative risk of developing metastasis compared with nonpigmented UMs (P < 0.001). Conclusions: Pigmented choroidal and ciliary body melanoma is more often associated with ocular melanocytosis, extraocular extension, anterior tumor epicenter, and earlier tumor recurrence. We also revealed that patients with pigmented UMs demonstrate a higher 10-year rate of metastatic disease and have decreased metastatic survival relative to partially pigmented and nonpigmented UMs. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Mechanism and outcomes of recreational and sports-related open globe injuries.

OBJECTIVES: To describe the mechanisms and visual outcomes of recreational and sports-related open globe injuries (OGIs). METHODS: A retrospective case series of eyes experiencing OGI secondary to recreational and sports-related activities at Memorial Hermann Hospital - Texas Medical Center (MHH-TMC) from January 1st, 2010 through March 31st, 2015 was conducted. Exclusionary criteria included no documented ophthalmologic examination upon presentation and repairs performed by services other than ophthalmology. A two-tailed t-test and Fisher's exact test were utilized to assess for statistical significance (p < 0.05). RESULTS: A total of 20 eyes from 20 patients experiencing OGIs secondary to recreational and sports-related activities were included. Thirteen eyes (65 %) presented with OGIs from penetrating objects while seven eyes (35 %) had injuries from blunt injuries. Males comprised most of the total study group (17 of 20 patients), and zone 3 injuries were the most common location of OGI. While eyes with OGIs from blunt trauma underwent evisceration/enucleation procedures more frequently than OGIs from penetrating mechanisms (71% vs 23 %) (p = 0.10), the final visual outcomes were similarly poor between groups. Only three eyes in this series experienced an improvement from baseline VA; all three eyes had lacked initial findings consistent with severe injury. CONCLUSIONS: Recreational and sports related OGIs most commonly occur in zone 3 and in young males, regardless of injury type. OGIs due to both penetrating and blunt trauma mechanisms implicate poor functional outcomes, but the absence of certain presenting injury features may indicate possibility of eventual visual recovery.

Correlation of fitzpatrick skin type and iris color with tumor size in 823 patients with uveal melanoma.

PURPOSE: To determine the correlation of Fitzpatrick Skin Type (FST) and iris color with tumor size (tumor thickness and basal diameter) in patients with uveal melanoma. DESIGN: Retrospective Cohort METHODS: Retrospective cohort from a single ocular oncology center of 823 patients with uveal melanoma and documented FST, iris color, and tumor size. Patients were classified by FST (type I, II, and III-V) and iris color (blue, green, and brown) on the basis of external facial photography. There were no FST type VI patients. Tumor thickness was classified into small [< 3 millimeter (mm)], medium (3.1-8.0 mm), or large (> 8.0 mm), and basal diameter into small (< 10 mm), medium (10.1-15 mm) or large (> 15 mm). The correlation of FST and iris color with tumor thickness and basal diameter was evaluated using the Kruskal-Wallis H test. RESULTS: The FST classification was type I (n = 92, 11%), type II (n = 643, 78%), or III-V (n = 88, 11%), and iris color was blue (n = 472, 57%), green (n = 102, 12%), or brown (n = 249, 30%). A comparison of FST revealed differences in mean tumor thickness (P = 0.04) and basal diameter (P = 0.006). Iris color showed no difference for mean tumor thickness (P = 0.41) or basal diameter (P = 0.48). There was a statistically significant difference with brown iris color relative to FST III-V for mean tumor thickness (P = 0.003) and basal diameter (P = 0.001) but no difference with blue or green iris color (P > 0.05). CONCLUSIONS: Iris color alone showed no difference in tumor size, but those with brown iris color and FST type III-V demonstrated larger tumor thickness and basal diameter.

Association of Fitzpatrick Skin Type with metastatic risk from uveal melanoma in 854 consecutive patients at a single center.

OBJECTIVE: To assess the association of skin color using Fitzpatrick Skin Type (FST) with metastatic risk of uveal melanoma. SUBJECTS: 854 consecutive patients with uveal melanoma and documented FST. METHODS: Retrospective detailed review of patient charts was performed for FST (type I- white, II-fair, III-average, IV-light brown, V-brown, VI-black), clinical details of the patient and the uveal melanoma, tumor cytogenetic classification according to The Cancer Genome Atlas (TCGA), and outcome of melanoma-related metastasis and death. RESULTS: The FST classification was type I (n = 97 patients), type II (n = 665), type III (n = 79), type IV (n = 11), type V (n = 2), type VI (n = 0). A comparison of patient FST (type I vs. II vs. III-V) revealed significant differences in mean age at presentation (64.1 vs. 58.5 vs. 49.8 years, p < 0.001), race white (100% vs. 98% vs. 75%, p < 0.001), presence of ocular melanocytosis (3% vs. 3% vs. 10%, p = 0.01), visual acuity <20/200 at presentation (6% vs. 7% vs. 13%, p = 0.03), genetic results showing TCGA group B tumors (11% vs. 14% vs. 26%, p = 0.01) or TCGA group D tumors (22% vs. 11% vs. 9%, p = 0.01), 10-year incidence of melanoma-related metastasis (25% vs. 15% vs. 14%, p = 0.02) and 10-year incidence of melanoma-related death (9% vs. 3% vs. 4%, p = 0.04). FST was a significant predictor of melanoma-related metastasis (p = 0.02, Hazard ratio 2.3). CONCLUSIONS: Fitzpatrick skin type may be a predictor of melanoma-related metastasis, with metastasis and TCGA Group D tumors being more common in patients with FST I.

Cavitary Retinoblastoma Imaged on Magnetic Resonance Imaging.

Cavitary retinoblastoma is a rare subtype, characterized by the presence of translucent cavities within the solid malignancy. A 2-year-old boy with bilateral retinoblastoma was found to have a unique cavitary retinoblastoma with seven cavities, more than previously reported, and large enough to be imaged on magnetic resonance imaging. [J Pediatric Ophthalmol Strabismus. 2023;60(1):e1-e4.].

The historical development and an overview of contemporary keratoprostheses.

Corneal blindness is a major cause of ocular morbidity that affects 4.5 million people worldwide. Though penetrating keratoplasty is an excellent option for most patients with corneal blindness, there are various conditions for which corneal transplantation carries a low likelihood of success. In cases of multiple failed transplants, the keratoprosthesis, an artificial cornea, is a well-documented surgical option, though few models are commercially available. Keratoprostheses also provide a solution to those in developing areas of the world who do not have access to penetrating keratoplasty owing to limitations in the supply of corneal donor tissue. We summarize the history of keratoprostheses, examine the various keratoprosthesis models used across the globe, and highlight efforts to improve the accessibility and biointegration of keratoprosthesis through novel technological developments.

Controversy and Consideration of Refractive Surgery in Patients with Heritable Disorders of Connective Tissue.

Heritable Disorders of Connective Tissue (HDCTs) are syndromes that disrupt connective tissue integrity. They include Osteogenesis Imperfecta (OI), Ehlers Danlos Syndrome (EDS), Marfan Syndrome (MFS), Loeys-Dietz Syndrome (LDS), Epidermolysis Bullosa (EB), Stickler Syndrome (STL), Wagner Syndrome, and Pseudoxanthoma Elasticum (PXE). Because many patients with HDCTs have ocular symptoms, commonly myopia, they will often present to the clinic seeking refractive surgery. Currently, corrective measures are limited, as the FDA contraindicates laser-assisted in-situ keratomileusis (LASIK) in EDS and discourages the procedure in OI and MFS due to a theoretically increased risk of post-LASIK ectasia, poor wound healing, poor refractive predictability, underlying keratoconus, and globe rupture. While these disorders present with a wide range of ocular manifestations that are associated with an increased risk of post-LASIK complications (e.g., thinned corneas, ocular fragility, keratoconus, glaucoma, ectopia lentis, retinal detachment, angioid streaks, and ocular surface disease), their occurrence and severity are highly variable among patients. Therefore, an HDCT diagnosis should not warrant an immediate disqualification for refractive surgery. Patients with minimal ocular manifestations can consider LASIK. In contrast, those with preoperative signs of corneal thinning and ocular fragility may find the combination of collagen cross-linking (CXL) with either photorefractive keratotomy (PRK), small incision lenticule extraction (SMILE) or a phakic intraocular lens (pIOL) implant to be more suitable options. However, evidence of refractive surgery performed on patients with HDCTs is limited, and surgeons must fully inform patients of the unknown risks and complications before proceeding. This paper serves as a guideline for future studies to evaluate refractive surgery outcomes in patients with HDCTs.

A One Year Longitudinal Comparative Analysis of Visual Outcomes Between Femtosecond Laser-Assisted Cataract Surgery and Standard Phacoemulsification Cataract Surgery.

PURPOSE: To assess visual outcomes over time of femtosecond laser-assisted cataract surgery compared to standard phacoemulsification cataract surgery. DESIGN: A retrospective, single-center comparative study. METHODS: Patient data including demographics, ocular biometry, pre- and postoperative visual acuity, postoperative complications, primary (uncorrected distance visual acuity over time) and secondary visual outcomes (uncorrected near visual acuity, best distance visual acuity, patient complaints, satisfaction, and postoperative surgery) were gathered and statistically analyzed. Demographic differences between patients receiving femtosecond-laser assisted cataract surgery (FLACS) versus standard phacoemulsification cataract surgery (PCS) were corrected for outcome comparison. Safety, efficacy, predictability, and stability were analyzed for each procedure and compared. RESULTS: A total of 155 eyes in PCS and 143 eyes in FLACS were analyzed at 1 week, 3 months, and 1 year using odds ratio. The odds ratio of being 20/20 or better and 20/40 or better at the specified time periods were similar and statistically insignificant at all time periods analyzed except 20/20 or better for uncorrected distance visual acuity (UDVA) at 1 year (p=0.0001) and uncorrected near visual acuity (UNVA) at 1 week (p=0.02). In both cases, the odds of being 20/20 or better favored FLACS. Mean UDVA, UNVA, and best distance visual acuity (BDVA) were all similar and statistically insignificant between the two groups. Postoperative patient complaints, safety, efficacy, predictability, and stability between the two groups showed no statistical significance. CONCLUSION: Despite the odds ratio of being 20/20 or better favoring FLACS for UDVA at 1 year and UNVA at 1 week, the mean logMAR UDVA, BDVA, and UNVA were similar and statistically insignificant between the FLACS and PCS groups at 1 week, 3 months, and 1 year. Differences in visual acuity were likely due to differences other than surgical approaches. While both FLACS and PCS are appropriate approaches to cataract surgery, one does not appear to be superior when assessing longitudinal markers for visual acuity, safety, efficacy, predictability and stability.

Gravitational Shifting of a Pigmented Iris Lesion in a Young Boy.

A 13-year-old boy with a history of sports-related blunt trauma to the left eye was referred for evaluation of an asymptomatic, pigmented iris lesion. Slitlamp examination revealed a round, pigmented lesion measuring 3 × 3 mm in basal dimension and with gravitational shifting within the anterior chamber fluid with patient head tilt. What would you do next?

Abemaciclib and Fulvestrant for Bilateral Choroidal Metastasis From Breast Carcinoma.

This case report discusses the follow-up of a woman with HR+/ERBB2 invasive ductal breast carcinoma metastatic to the lungs, brain, and spine.