Ocular Gene Therapy in a Patient with Dystrophic Epidermolysis Bullosa.

Dystrophic epidermolysis bullosa is a rare genetic disease caused by damaging variants in COL7A1, which encodes type VII collagen. Blistering and scarring of the ocular surface develop, potentially leading to blindness. Beremagene geperpavec (B-VEC) is a replication-deficient herpes simplex virus type 1-based gene therapy engineered to deliver functional human type VII collagen. Here, we report the case of a patient with cicatrizing conjunctivitis in both eyes caused by dystrophic epidermolysis bullosa who received ophthalmic administration of B-VEC, which was associated with improved visual acuity after surgery.

A Novel Cost-Effective Simulation Model for Continuous Curvilinear Capsulorhexis.

Introduction: We describe and validate a low-cost simulation model for practicing anterior lens capsule continuous curvilinear capsulorhexis (CCC). Methods: A simulation model for CCC was developed from widely available low-cost materials. Ophthalmologists attending the annual scientific meeting of the Research Institute of Ophthalmology, Giza, Egypt, were asked to perform a five CCC model task and then anonymously answer a questionnaire that assessed the realism and training utility of the model using a five-point Likert scale (1 = unacceptable, 2 = poor, 3 = acceptable, 4 = favorable and 5 = excellent). Results: Twenty-seven ophthalmologists completed the task and the anonymous questionnaire. Overall, participants felt that the model simulated CCC step in cataract surgery well (mean: 3.5) and was comparable to other kinds of CCC simulation models (mean: 3.3). The model scored highly for its overall educational value (mean: 4.00) and for enlarging a small CCC (mean:3.7), while the feasibility of this model in practicing the management of a runaway leading edge of CCC scored 2.9. Conclusion: This model may provide an alternative method for training for CCC and other anterior lens capsule-related maneuvers. This option may be particularly helpful for residency training programs with limited access to virtual reality simulators or commercially available synthetic eye models.

Case report: Spontaneous coronary artery rupture presenting with acute coronary syndrome: A rare diagnosis of common disease.

Acute coronary syndrome (ACS), myocardial infarction, and sudden death have all been linked to spontaneous coronary artery rapture (SCAR). Patients primarily afflicted by SCAR are those with or without cardiovascular risk factors, notably men, implying a mechanism distinct from the more prevalent atherosclerosis. Both medical and interventional treatment should consider the diverse causes of ACS as well as the patient's clinical stability. I herein report an unusual case of a 33 years old male who presented with acute chest pain to the emergency department. His physical exam was normal. The electrocardiogram showed non-specific ST segment changes in anterior leads, and the echocardiogram revealed mild anterior wall hypokinesia with no evidence of pericardial effusion. He underwent coronary angiography which revealed a contained rupture in the anterior descending coronary artery. The patient underwent uneventful lifesaving coronary artery perforation repair. It concluded that, though rare, SCAR should be considered as a differential diagnosis in patients with ACS, even in the absence of pericardial effusion in adult patients of all ages.

Evaluation of endothelial/Descemet membrane complex of eye bank donor corneas using enhanced depth imaging optical coherence tomography.

Objective: We present a novel method for screening eye bank donor corneas using high definition optical coherence tomography (HD-OCT). This technology allows for the quantification of endothelial/Descemet membrane (En/DM) complex thickness ex vivo. Design: Prospective interventional study. Participants: Fifty-two corneal grafts from 27 donors were included in this study. Twenty additional control eyes and 11 eyes with Fuchs' endothelial corneal dystrophy were also evaluated for comparison. Methods: A custom built, high speed HD-OCT device (Envisu R2210, Bioptigen, Buffalo Grove, IL, USA) was used to obtain images, and custom-made graph-based segmentation software was used to automatically deconstruct corneal images into micro-layers. HD-OCT imaging was used to scan through the sealed sterile case of donor corneas stored in McCarey-Kaufman medium to image their En/DM complex through the center of the cornea. Results: This technology allowed for quantification of En/DM complex thickness in all donor corneas through the sealed sterile container used to transport graft tissue. Mean En/DM complex thickness of donor corneas was 17±4 µm. The difference between donor cornea En/DM thickness and that of control subjects (16±2 µm) was not statistically significant (p=0.3), suggesting that the transport container and media do not affect measurements. There was a significant difference between En/DM thickness of Fuchs' endothelial corneal dystrophy eyes (25±5 µm) and both donor corneas (p<0.0001) and control subjects (p<0.0001). Conclusions: We have described a new technique to measure En/DM complex thickness in eye bank donor corneas stored in a sealed sterile case. This may represent a novel adjunctive approach to screen corneal grafts for early endothelial disease.

Comparison of phacoemulsification parameters between manual and femtosecond laser-assisted cataract surgery.

OBJECTIVE: To compare the nucleus removal time (NRT) and cumulative dissipated energy (CDE) outcomes of traditional phacoemulsification and femtosecond laser-assisted cataract surgery (FLACS) performed by cornea attendings and fellows. DESIGN: Prospective nonrandomized comparative study. PARTICIPANTS: A total of 410 eyes of 410 patients. METHODS: Nucleus removal time and CDE were recorded from patients who underwent cataract surgery using either FLACS (Catalys, LenSx, or Victus) or traditional phacoemulsification technique performed by 3 cornea attendings and 4 cornea fellows. One-way analysis of variance with Bonferroni post hoc tests and unpaired t tests were used to determine the differences between groups. RESULTS: There was no statistically significant difference in cataract grade between groups. NRT was significantly lower only when using the Catalys system compared with the LenSx and Victus platforms and the traditional surgery, in both the attending group (p = 0.006, p = 0.002, p < 0.000, respectively) and the fellow group (p = 0.049, p = 0.038, p = 0.011, respectively). With respect to CDE, there was no significant difference when using the laser systems compared with the traditional surgery in both attending and fellow groups (p > 0.05). NRT and CDE were significantly higher in the fellow group (NRT = 269.10 ± 117.67, CDE = 7.30 ± 4.83) compared with the attending group (NRT = 218.87 ± 109.67, CDE = 5.76 ± 3.66) in traditional cases; however, in FLACS cases, there was no significant difference in NRT and CDE between the fellow group and the attending group. CONCLUSIONS: Inexperienced surgeons seem to require more time and use more ultrasound energy during traditional phacoemulsification when compared with experienced surgeons. The use of FLACS seems to significantly improve the NRT of experienced and inexperienced surgeons.

Blue Nevi of the Ocular Surface: Clinical Characteristics, Pathologic Features, and Clinical Course.

PURPOSE: Blue nevus is a melanocytic tumor that is commonly found in the skin. Extracutaneous presentations, including the ocular surface, are rare. As such, the purpose of this study was to characterize the clinical features and clinical course of congenital melanocytic tumor (blue nevus) of the conjunctiva. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-one patients with 23 blue nevi of the ocular surface that were excised surgically between 2000 and 2016. METHODS: Chart review of patients identified from a database search of the Florida Lions Ocular Pathology Laboratory records. Pathologic diagnoses were confirmed by 2 pathologists (S.R.D. and G.E.). All specimens were bleached and, tissue permitting, stained using SOX10 (MilliporeSigma, Darmstadt, Germany) and CD68 (Leica Biosystems, Nussloch, Germany). MAIN OUTCOME MEASURES: Clinical characteristics, pathologic features, and clinical course. RESULTS: Mean age of the population was 55±15 years; 71.4% (n = 15) were white and 57.1% (n = 12) were men. One patient had 3 lesions, for a total of 23 lesions examined. Clinically, 13 lesions were on the bulbar conjunctiva, 3 were on the tarsal conjunctiva, 3 were in the fornix, 2 were caruncular, 1 was episcleral, and 1 was at the limbus. Before excision, 8 patients were thought to have primary acquired melanosis, 4 with concern for primary conjunctival melanoma, and 1 thought to have metastatic disease from a plantar melanoma. Five lesions were thought to be benign, and in 8 patients, the lesions were identified incidentally after other ocular surgeries, with no diagnosis of the lesions before excision. Pathologic features were consistent with simple blue nevi in 21 lesions and cellular blue nevus in 2 lesions. No malignant transformations were noted in any patient over the mean 20.2-month follow-up period (range, 2 weeks-103 months). CONCLUSIONS: Blue nevus is a rare deeply pigmented congenital melanocytic lesion with a benign clinical course that can appear clinically similar to primary acquired melanosis or melanoma.

Diagnosis and Medical Management of Ocular Surface Squamous Neoplasia.

INTRODUCTION: Topical chemotherapy has gained popularity among clinicians for the treatment of ocular surface squamous neoplasia (OSSN). The principal topical chemotherapy agents used in the management of OSSN are interferon-α2b, 5-fluorouracil, and mitomycin C. High-resolution optical coherence tomography (HR-OCT) is a non-invasive technique that can differentiate OSSN from other ocular surface lesions. AREAS COVERED: This review highlights the current regimens and diagnostic modalities used in managing OSSN. A review of the literature was performed using the keywords "conjunctival intraepithelial neoplasia", "ocular surface squamous neoplasia", "optical coherence tomography", "interferon-α2b", "5-fluorouracil" and "mitomycin C". EXPERT COMMENTARY: Topical chemotherapy for OSSN can be used as primary therapy, for chemoreduction prior to surgical excision, and postoperatively to prevent tumor recurrence. It has the advantage of treating microscopic disease as well as large tumors. HR-OCT provides an "optical biopsy" that can assist in diagnosis and guide management of OSSN lesions.

Second femtosecond laser treatment application for completion of partial capsulotomy caused by suction loss.

PURPOSE: To describe a case of a second application of femtosecond laser-assisted capsulotomy to achieve a complete treatment pattern after an initial incomplete capsulotomy creation due to loss of suction. METHODS: A 66-year-old woman presented with visually significant cataract in both eyes. She was scheduled to undergo femtosecond laser-assisted cataract surgery (FLACS) using the Victus platform. During FLACS capsulotomy of the right eye (capsulotomy diameter set at 5.0 mm), loss of suction occurred followed by laser treatment interruption, resulting in an incomplete, discontinuous capsulotomy treatment pattern. A second FLACS pretreatment was performed immediately after the first incomplete treatment, aiming for a capsulotomy diameter 0.1 mm larger than the previous (5.1 mm in diameter) and with placement of the capsulotomy treatment outside the prior incomplete capsulotomy. RESULTS: A complete continuous treatment pattern was achieved for the capsulotomy after second application of FLACS pretreatment. No intraoperative complications were noticed. CONCLUSION: A second application of femtosecond laser-assisted capsulotomy to achieve a complete treatment pattern after initial incomplete capsulotomy creation due to loss of suction is feasible during the same surgical setting.

Topical 5-Fluorouracil 1% as Primary Treatment for Ocular Surface Squamous Neoplasia.

PURPOSE: To determine the efficacy of topical 5-fluorouracil 1% (5-FU) as a primary treatment of ocular surface squamous neoplasia (OSSN). DESIGN: Retrospective study. PARTICIPANTS: Topical 5-FU was used as primary therapy in 44 patients with OSSN. METHODS: 5-Fluorouracil 1% administered topically 4 times daily for 1 week followed by a drug holiday of 3 weeks. Patients were identified through a pharmacy database. Patients were excluded if 5-FU was used as adjuvant therapy, if they did not complete therapy, or if they were still actively receiving treatment for OSSN at the time of last follow-up. MAIN OUTCOME MEASURES: The primary outcome measures were the frequency of complete resolution with topical 5-FU treatment and the rate of OSSN recurrence. RESULTS: Of the 44 patients identified, 32 were men and 12 were women. The mean age was 68 years. Complete resolution of OSSN was noted in 82% of patients (36/44); 18% (8/44) were considered treatment nonresponders. Patients were treated with a median of 4 cycles (range, 2-9 cycles). Nasal location was the only risk factor identified for nonresponse to therapy (P = 0.04). The median follow-up after resolution was 10 months (range, 2-77 months). In the 36 patients who showed complete resolution, 4 experienced tumor recurrence. Recurrence rates at 1 and 2 years were 6% and 15%, respectively, using Kaplan-Meier survival analysis. At least 1 side effect from the medication was reported by 61% of patients (21/44), but only 1 patient discontinued the medication because of intolerance. The most common side effect was pain (n = 17; 39%), followed by tearing (n = 10; 23%), photophobia (n = 6; 14%), itching (n = 4; 9%), swelling (n = 2; 5%), and infection (n = 1; 2%). No long-term complications were reported. CONCLUSIONS: 5-Fluorouracil is effective and well tolerated as a primary treatment for OSSN, with 82% of tumors responding completely to therapy.

Effects of Femtosecond Laser-Assisted Cataract Pretreatment on Pupil Diameter: A Comparison Between Three Laser Platforms.

PURPOSE: To assess pupil diameter before and after femtosecond laser-assisted cataract surgery (FLACS) pretreatment and compare the outcomes of three laser platforms. METHODS: This prospective observational case series included consecutive patients scheduled to undergo cataract extraction using FLACS between August 2013 and February 2015. All eyes received FLACS pretreatment using three laser platforms: LenSx (Alcon Laboratories, Inc., Fort Worth, TX), Catalys (Abbott Medical Optics Inc., Santa Ana, CA), and Victus (Bausch & Lomb, Inc., Rochester, NY). The same protocol for preoperative medical mydriasis was used for all patients, and pupil diameter was assessed immediately before and 3 minutes after FLACS using a surgical ruler. RESULTS: A total of 198 eyes of 161 patients were included in the study. Mean pupillary miosis was 1.42 ± 1.26 mm for the LenSx, 0.66 ± 0.89 mm for the Catalys, and 0.14 ± 0.34 mm for the Victus groups. Furthermore, 8 of the 198 eyes (4.0%) demonstrated a pupil diameter of less than 5 mm after FLACS and 48 eyes (24.24%) demonstrated a pupil diameter of 6 mm or less. There was a statistically significant decrease in pupil diameter for all groups individually (P < .05). There was also a statistically significant difference among the three groups (P < .05), with LenSx inducing the highest degree of miosis, followed by Catalys, and finally Victus. A correlation between the pupil diameter before FLACS and degree of FLACS-induced miosis was demonstrated (P < .05), with larger pupil diameter before FLACS associated with greater miosis. CONCLUSIONS: FLACS pretreatment seems to induce significant pupillary miosis with all laser platforms assessed in this study. The decrease in pupil diameter after FLACS reached clinical significance for cataract extraction (< 5 mm) in 4.0% of cases, whereas 20.2% of eyes demonstrated small pupil diameter (≤ 6 mm) after FLACS pretreatment.

Anterior Capsulotomy Outcomes: A Comparison Between Two Femtosecond Laser Cataract Surgery Platforms.

PURPOSE: To compare capsulotomy outcomes between two femtosecond laser platforms for cataract surgery. METHODS: This retrospective case series included 205 eyes of 162 consecutive patients (75 male and 87 female) aged 68.32 ± 10.27 years (range: 38 to 92 years) who underwent femtosecond laser-assisted cataract surgery (FLACS) using either the LenSx (Alcon Laboratories, Inc., Fort Worth, TX) or the Catalys (Abbott Medical Optics, Abbott Park, IL) laser platform. Intraoperative assessment of the capsulotomy was performed and graded as one of the following four types: complete treatment pattern (type 1), microadhesions (type 2), incomplete treatment pattern (type 3), and complete pattern but not continuous (type 4). RESULTS: Ninety-seven eyes underwent FLACS using the LenSx platform and 108 eyes using the Catalys platform. Ninety-four capsulotomies in the LenSx group were graded as type 1 (96.91%), and three were graded as type 2 (3.09%); all capsulotomies in the Catalys group were graded as type 1 (100%). There was no statistically significant difference between the LenSx and Catalys groups (P > .05). No intraoperative complications (specifically, no posterior capsular extension or vitreous loss) were noticed in any of the eyes included in the study. CONCLUSIONS: The LenSx and Catalys laser platforms demonstrate similar capsulotomy outcomes. The microadhesions that were demonstrated in three cases in the LenSx group did not lead to intraoperative or postoperative complications.

Comparison of surgically induced astigmatism between femtosecond laser and manual clear corneal incisions for cataract surgery.

PURPOSE: To assess the surgically induced corneal astigmatism (SIA) introduced by femtosecond laser-assisted clear corneal incisions (CCIs) for cataract extraction and to compare it with the SIA of manually created CCIs. SETTING: Bascom Palmer Eye Institute, Miller School of Medicine, Miami, Florida, USA. DESIGN: Prospective nonrandomized comparative case series. METHODS: Eyes received femtosecond laser-assisted CCIs (Group 1) or manual CCIs (Group 2). The surgical plan included 1 primary and 1 secondary port; the sites of the incisions were the same in both groups and were diametrically opposed between the right eye and left eye. The SIA was assessed using the preoperative and 1-month postoperative keratometric values obtained from corneal topography examinations. RESULTS: This study included 72 eyes of 68 patients with a mean age of 69.0 years ± 9.87 (SD) (range 36 to 90 years). Thirty-six eyes received femtosecond laser-assisted CCIs (Group 1) and 36 received manual CCIs (Group 2). The mean preoperative topographic corneal astigmatism was -1.19 ± 0.68 diopters (D) (range 0 to 2.50 D) and -0.92 ± 0.63 D (range 0.10 to 2.45 D) for Group 1 and Group 2, respectively, whereas, 1 month after cataract surgery, it was -1.16 ± 0.63 D (range 0.20 to 2.57 D) and -0.95 ± 0.64 D (range 0.21 to 2.37 D), respectively. Multivariate vector analysis revealed no statistically significant difference between the 2 groups for preoperative astigmatism, postoperative astigmatism, and SIA (P > .05 for all comparisons between Group 1 and Group 2). CONCLUSION: Femtosecond laser-assisted and manual corneal incisions for cataract surgery did not appear to significantly alter corneal astigmatism, whereas they showed comparable SIA. FINANCIAL DISCLOSURE: Drs. Yoo and Donaldson are speakers for and consultants to Alcon Surgical, Inc., and Abbott Medical Optics, Inc. No other author has a financial or proprietary interest in any material or method mentioned.