A Rapidly Expanding Hemorrhagic BRAF-Mutant Orbital Atypical Glomus Tumor.

A healthy 32-year-old woman presented with the acute onset of left sided eye pain, upper eyelid fullness, and binocular diplopia during light weightlifting. Examination elevated intraocular pressure, proptosis, upper eyelid ptosis, and motility deficits. CT demonstrated a well-circumscribed, homogeneous-appearing extraconal mass in the superior left orbit. The patient underwent an urgent orbitotomy with the excision of a hemorrhagic mass. Histopathology showed a glomus tumor with atypical features and hemorrhagic infarction, best classified as having uncertain malignant potential. A B-Raf proto-oncogene V600E mutation was detected with immunohistochemistry, which suggests a more aggressive tumor behavior yet presents an opportunity for targeted primary or adjunctive therapy. This is the first reported case of a B-Raf proto-oncogene-mutant atypical glomus tumor arising in the orbit.

Outcomes of Anterior Chamber, Sulcus, and Pars Plana Glaucoma Drainage Device Placement in Glaucoma Patients.

Purpose: To assess outcomes of anterior chamber (AC), sulcus, and pars plana (PP) glaucoma drainage device (GDD) placement in glaucoma patients. Patients and Methods. Retrospective evaluation of glaucoma patients who underwent GDD insertion in the AC, sulcus, or PP at Massachusetts Eye and Ear between November 2016 and May 2021. Patients who received AC, sulcus, and pars plana tubes were selected using simple random sampling, and the first 40 patients meeting inclusion criteria were analyzed. Main outcome measures were cumulative success probabilities from Kaplan-Meier (KM) analyses, intraocular pressure (IOP), medication burden, and complication rates. Results: The PP group had a larger proportion of Ahmed GDDs and was younger on average with less severe glaucoma compared to patients with AC or sulcus tubes. The PP group had a higher proportion of mixed-mechanism glaucoma and lower proportion of primary open-angle glaucoma. With success defined as IOP reduction ≥20% and 5 < IOP ≤ 21 mm Hg, the Kaplan-Meier cumulative success probabilities for all three GDD locations were not significantly different. No significant differences were found in complication rates between all groups after 3 months. Patients with PP GDD had significantly lower medication burden than those with AC or sulcus GDDs up to 1.5 years postoperatively (1.7 ± 1.1, 3.0 ± 1.4, and 2.8 ± 1.2 for PP, AC, and sulcus, respectively; P=0.017). Conclusion: PP GDDs may be more effective in lowering medication burden than AC or sulcus tubes without compromising long-term safety.

Risk Factors for Glaucoma Drainage Device Revision or Removal Using the IRIS Registry.

PURPOSE: To elucidate risk factors for revision or removal of glaucoma drainage devices (GDD) in glaucoma patients in the United States. DESIGN: Retrospective cohort study. METHODS: IRIS Registry (Intelligent Research in Sight) patients who underwent GDD insertion between January 1, 2013 and December 31, 2018 were included. Various demographic and clinical factors were collected. Kaplan-Meier survival plots, Cox proportional-hazard models utilizing Firth's Penalized Likelihood, and multivariate linear regression models were used. The main outcome measures were hazard ratios (HR) and beta coefficient (ß) estimates. RESULTS: A total of 44,330 distinct patients underwent at least 1 GDD implantation, and 3354 of these underwent subsequent GDD revision or removal surgery. With failure defined as GDD revision/removal, factors significantly associated with decreased failure included unknown race (HR = 0.83; P = .004) and unknown ethnicity (HR = 0.68; P < .001). Factors associated with increased risk of GDD revision/removal surgery included presence of chronic angle-closure glaucoma (HR = 1.32; P < .001) and dry eye disease (HR = 1.30; P = .007). Additionally, factors associated with a decreased average time (in days) to GDD revision/removal included male sex (ß = -25.96; P = .044), unknown race (ß = -55.28; P = .013), and right-eye laterality (ß = -38.67; P = .026). Factors associated with an increased average time to GDD revision/removal included having a history of a past eye procedure (ß = 104.83; P < .001) and being an active smoker (ß = 38.15; P = .024). CONCLUSIONS: The size and scope of the IRIS Registry allows for detection of subtle associations between risk factors and GDD revision or removal surgery. The aforementioned demographic and clinical factors may all have an impact on GDD longevity and can inform the treatment options available for glaucoma patients.

Outcomes of phacoemulsification and endoscopic cyclophotocoagulation performed with dual blade ab interno trabeculectomy or trabecular micro-bypass stent insertion.

BACKGROUND/OBJECTIVE: To report the initial outcomes of phacoemulsification, endoscopic cyclophotocoagulation, and dual blade ab interno trabeculectomy (PEcK), and compare them to those of phacoemulsification, endoscopic cyclophotocoagulation, and trabecular micro-bypass stent insertion (ICE-1). SUBJECTS/METHODS: Patients from January 2018 to December 2019 that underwent PEcK or ICE-1 at a tertiary referral centre were included in this retrospective comparative case series. Patients were excluded if they had additional concomitant procedures, less than 6 weeks (42 days) of follow-up or were not at least 18 years old. Intraocular pressure (IOP), number of glaucoma medications, and best-corrected visual acuity were collected preoperatively and postoperatively at 6 weeks, 3, 6, and 12 months. Kaplan-Meier survival analysis and Cox proportional-hazards regression were conducted to elucidate any factors associated with survival time. RESULTS: The mean preoperative IOP was 18.3 ± 5.9 mmHg in the PEcK group (53 eyes) and 14.7 ± 4.3 mmHg in the ICE-1 group (23 eyes) (p = 0.004) on 3.3 ± 1.3 and 1.7 ± 0.93 glaucoma medications (p < 0.001), respectively. Twelve months postoperatively the mean IOP reduction was 5.1 ± 4.4 mmHg and 2.3 ± 4.0 mmHg (p = 0.08), and the mean medication reduction was 1.6 ± 1.5 and 0.97 ± 0.66 (p = 0.10), in the PEcK and ICE-1 groups, respectively. Kaplan-Meier survival analysis did not reveal any differences in treatment survival. CONCLUSIONS: Both PEcK and ICE-1 provide clinically relevant reductions in IOP and glaucoma medication burden, however the PEcK procedure may confer greater reductions in IOP. The procedures did not differ with regard to Kaplan-Meier survival probability.

Effects of Postoperative Intravitreal Injections on Outcomes of Traditional Glaucoma Surgery in Patients with Preoperative Intravitreal Injections.

PURPOSE: To compare outcomes of glaucoma drainage device (GDD) implantation and trabeculectomies with and without postoperative intravitreal injections (IVIs) in glaucoma patients with a history of preoperative IVIs. DESIGN: Retrospective cohort study. PARTICIPANTS: A total of 133 eyes of 133 glaucoma patients who underwent GDD implantation or trabeculectomy with at least 1 IVI preoperatively between January 2005 and October 2020 at Massachusetts Eye and Ear. METHODS: Chart review of glaucoma patients with traditional glaucoma surgery and at least 1 IVI before surgery. All statistical analyses were conducted with R statistical programming software. MAIN OUTCOME MEASURES: Intraocular pressure (IOP), medication burden, best-corrected visual acuity (BCVA), Kaplan-Meier success rates, adjusted hazard ratios (HRs), and complications. RESULTS: Baseline demographics were similar between the groups with and without postoperative IVIs. The group with postoperative IVIs had a higher proportion of diabetic retinopathy and retinal vascular occlusions than the group without postoperative IVIs, which had more eyes with age-related macular degeneration. Intraocular pressure, medication burden, and visual acuity were similar between groups at all time points except for IOP at 6 weeks, which was lower in the group with postoperative IVIs. The group with postoperative IVIs had significantly more preoperative IVIs than the group without postoperative IVIs (6.6 vs. 3.3, P = 0.017). For success defined as IOP reduction ≥ 20% with 5 < IOP ≤ 21 mmHg, Kaplan-Meier analyses demonstrated similar success rates between groups with and without IVIs. When stratified by the number of IVIs, success rates for the group with 7 or more IVIs were significantly higher than the success rates for the group with 0-6 IVIs (P = 0.005). Each additional postoperative IVI resulted in a 7.2% decrease in the hazard of failure to achieve our stated success criteria. With regard to late complications, the group with postoperative IVIs had a higher incidence of vitreous hemorrhage (18.5% vs. 3.2%, P = 0.039) than the group without postoperative IVIs. CONCLUSIONS: A higher number of postoperative IVIs, specifically 7 or more IVIs, may be associated with improved success rates of traditional glaucoma surgery in glaucoma patients who received IVIs before surgery.