Refractive outcomes of immediately sequential bilateral cataract surgery in eyes with long and short axial lengths.

PURPOSE: To report the refractive outcomes of long (≥25.00 mm) and short (≤22.00 mm) axial length (AL) eyes undergoing immediately sequential bilateral cataract surgery (ISBCS). METHODS: In this retrospective cohort study, patients who underwent ISBCS were identified and eyes of patients with bilateral long and short ALs were included. Pre- and postoperative biometry, autorefraction, and ocular comorbidities or complications were recorded. The primary outcome was the mean refractive prediction error. RESULTS: Thirty-seven patients (74 eyes) with long ALs and 18 patients (36 eyes) with short ALs were included. The means ± standard deviations of the ALs were 26.40 ± 1.38 mm and 21.44 ± 0.46 mm in the long and short AL groups, respectively. In long AL eyes, the mean absolute error from the biometry-predicted refraction was - 0.16 ± 0.46 D, corresponding to 74% of eyes achieving a refraction within ±0.50 D of the predicted value. In short AL eyes, the mean absolute error was - 0.63 ± 0.73 D, corresponding to 44% of eyes achieving a refraction within ±0.50 D of the predicted value. Eight (44.4%) patients with short AL eyes had a myopic deviation greater than ±0.50 D from the predicted result in both eyes. CONCLUSIONS: Compared to patients with long AL eyes, ISBCS in patients with short ALs had a wider variance in refractive outcome and a lower rate of achieving a postoperative refraction within ±0.50 D of the predicted target.

Quality of hydroxychloroquine retinopathy screening at a Canadian teaching hospital.

PURPOSE: To assess the quality of hydroxychloroquine (HCQ)-induced retinopathy screening at a Canadian tertiary center, we concentrate on risk factor documentation within the electronic health record, in accordance with the 2016 AAO guidelines. METHODS: We performed a retrospective quality assessment study based on chart review of patients who underwent screening for HCQ-induced retinopathy at the Centre Hospitalier de l'Université de Montréal (CHUM) from 2016 to 2019. We evaluated four key risk factors for HCQ-induced retinopathy: daily dose, duration of use, renal disease, and tamoxifen use, using a three-tier grading system (ideal, adequate, inadequate) for documentation assessment. Pareto and root cause analyses were conducted to identify potential improvement solutions. RESULTS: Documentation quality varied in our study: daily dosage was 33% ideal, 31% appropriate, and 36% inappropriate. Duration of use documentation was 75% ideal, 2% adequate, and 24% inadequate. Renal disease documentation was only 6% ideal, with 62% adequate and 32% of charts lacking any past medical history. Among women's charts, tamoxifen use wasn't documented at all, with 65% adequately documenting medication lists. Pareto analysis indicated that improving renal disease and tamoxifen documentation could reduce 64% of non-ideal records, and enhancing daily dose documentation could decrease this by up to 90%. CONCLUSION: Accurate documentation of key risk factors is critical for HCQ-induced retinopathy screening, impacting both exam initiation and frequency. Our study identifies potential improvements in the screening process at the hospital, referring physician, and ophthalmologist levels. Implementing integrated pathways could enhance patient experience and screening effectiveness.

The management of neurotrophic keratitis.

PURPOSE OF REVIEW: The management of neurotrophic keratitis (NK) has evolved in the last decade. The present article reviews updated management guidelines of this entity, as well as future innovations in the field. RECENT FINDINGS: The advent of confocal microscopy has allowed for the first time to image corneal nerves. In addition, multiple novel topical treatments such as nerve growth factor have improved the prognosis of this disease, with many other in the pipeline. Finally, corneal nerve restoration is now possible with corneal neurotization procedures. SUMMARY: Many novel treatments based on agents that stimulate nerve regrowth are now available to treat NK. Improvement in neurotization procedures could also address advanced stages of this disease with surgery.

Visual Outcomes and Complications of Type I Boston Keratoprosthesis in Children: A Retrospective Multicenter Study and Literature Review.

PURPOSE: To report outcomes and complications of Boston type 1 keratoprosthesis (KPro) implantation in children. DESIGN: Retrospective, multicenter case series. PARTICIPANTS: All children 16 years of age or younger who underwent KPro surgery at 3 ophthalmology centers in Canada between January 2010 and November 2014. METHODS: Records of patients having undergone KPro implantation were reviewed. Data on preoperative characteristics, surgical procedure(s) performed, and postoperative outcomes were collected and analyzed. MAIN OUTCOME MEASURES: Intraoperative and postoperative complications, device retention, and best-corrected visual acuity (BCVA). RESULTS: The KPro was implanted in 11 eyes of 11 patients 0.9 to 15.5 years of age, with 6 being primary corneal procedures. Best-corrected visual acuity recorded before surgery ranged from 20/600 to light perception (LP), and vision in 2 eyes was fix and follow. All patients had been diagnosed with glaucoma and 6 eyes had glaucoma drainage devices (GDDs) inserted before KPro implantation. At last follow-up (mean, 41.8 months; range, 6.5-85.0 months), 2 eyes retained BCVA of 20/400 or better, whereas 5 eyes lost LP. Postoperative complications included retroprosthetic membrane (9 eyes), corneal melt (5 eyes), infectious keratitis (3 eyes), endophthalmitis (3 eyes), GDD erosion (2 eyes), and retinal detachment (5 eyes). The initial KPro was retained in 4 eyes (36.4%). CONCLUSIONS: Boston type 1 keratoprosthesis implantation in children is associated with a substantially higher rate of complications, higher chance of device failure, and worse visual outcomes than observed in adults. In view of these results, the authors do not recommend the use of the KPro in the pediatric population.

The role of novel DMEK graft shapes in facilitating intraoperative unscrolling.

PURPOSE: To evaluate whether the preparation of Descemet membrane endothelial keratoplasty (DMEK) grafts into various shapes affect their propensity to scroll as compared with the conventional circular graft design. METHODS: Prospective randomized laboratory-based study, using nine pre-stripped DMEK corneal grafts unfit for transplantation. Each graft was trephinated into a standard circular 8-mm shape, stained with trypan blue, and then immersed in balanced salt solution (BSS). The width of the DMEK graft scroll was measured, and photographs were obtained. The graft was then cut into one of three pre-selected shapes (four peripheral punches, four radial cuts, Maltese cross). These newly shaped grafts were then replaced in BSS and again photographed. The scroll widths, as well as pre- and post-preparation scroll width-to-height ratios, were calculated and compared. RESULTS: Nine pre-stripped DMEK corneal grafts (mean donor age ± SD 73.1 ± 9.3 years, range 58-85 years) were included. The mean pre-cut scroll widths for the three selected shapes were statistically similar. Following graft preparation into their assigned shapes, the mean post-preparation scroll widths (and corresponding change from pre-preparation measurements) were 1.73 ± 0.16 mm (- 3.6%) for the 4-peripheral punches, 2.59 ± 0.35 mm (+ 0.2%) for the 4-radial cuts and 4.13 ± 0.63 mm (+ 20.1%) for the Maltese cross (P = 0.0013). Therefore, the Maltese cross design resulted in a wider scroll than its pre-preparation control. CONCLUSIONS: Certain DMEK graft shapes may be less prone to scrolling than others. The Maltese cross graft design scrolled less tightly than the other experimental graft shapes. Future studies may elucidate intraoperative scrolling behavior of these DMEK graft shapes within the anterior chamber.

Outcomes and Early Complications Using an Endothelium-in Pull-Through Descemet Membrane Endothelial Keratoplasty Technique With Preloaded Versus Surgeon-Loaded Donor Tissue in Fuchs Patients.

PURPOSE: This study aimed to compare outcomes and early complications using an endothelium-in pull-through Descemet membrane endothelial keratoplasty (DMEK) technique with preloaded versus surgeon-loaded donor tissue. METHODS: Data from 163 eyes of 125 patients at the Wilmer Eye Institute diagnosed with Fuchs endothelial corneal dystrophy who underwent DMEK with or without cataract extraction using surgeon-loaded tissue (n = 83) or preloaded tissue (n = 80) were reviewed. Best-corrected visual acuity and early postoperative complications including small graft detachment (less than one third of the graft area), large graft detachment (more than one third), graft failure, and rebubbling were compared. RESULTS: Baseline characteristics including age, sex, and visual acuity were not statistically different between the groups. Small graft detachment was observed in 18.1% of the surgeon-loaded and 22.5% of the preloaded group ( P = 0.48), whereas large detachment occurred in 12.0% and 5.0%, respectively ( P = 0.11). Among these, rebubbling was performed in 18 (21.7%) in the surgeon-loaded compared with 12 (15.0%) in the preloaded group ( P = 0.27). The rebubbling rate of the combined procedure (cataract surgery and DMEK) was 21.8% and of DMEK alone was 7.7% ( P = 0.048). Primary graft failure occurred in 2 surgeon-loaded cases (2.4%) and 1 preloaded case (1.3%) ( P = 0.58). There was no difference in postoperative best-corrected visual acuity at 1 year (logarithm of the minimum angle of resolution 0.21 ± 0.25 for the surgeon-loaded vs. 0.16 ± 0.16 for the preloaded group, P = 0.23). CONCLUSIONS: DMEK surgery using preloaded endothelium-in tissue has comparable outcomes with surgeon-loaded endothelium-in tissue. However, there was a trend toward the lower rebubbling rate in DMEK alone compared with combined procedures.

Survey: Preferred practice patterns in the diagnosis of mucous membrane pemphigoid amongst cornea specialists.

PURPOSE: To evaluate preferred diagnostic tools and treatment decision-making factors in cases suspicious of mucous membrane pemphigoid (MMP) amongst ophthalmologists and cornea specialists. METHODS: Web-based survey, consisting of 14 multiple choice questions, posted to the Cornea Society Listserv Keranet, the Canadian Ophthalmological Society Cornea Listserv, and the Bowman Club Listserv. RESULTS: One hundred and thirty-eight ophthalmologists participated in the survey. Eighty-six percent (86%) of respondents were cornea trained and practiced in either North America or Europe (83%). Most respondents (72%) routinely perform conjunctival biopsies for all suspicious cases of MMP. For those who do not, fear that biopsy will exacerbate inflammation was the most common reason to defer investigation (47%). Seventy-one percent (71%) performed biopsies from perilesional sites. Ninety-seven percent (97%) ask for direct (DIF) studies and 60% for histopathology in formalin. Most do not recommend biopsy at other non-ocular sites (75%), nor do they perform indirect immunofluorescence for serum autoantibodies (68%). Immune-modulatory therapy is started following positive biopsy results for most (66%), albeit most (62%) would not let a negative DIF influence the choice of starting treatment should there be clinical suspicion of MMP. Differences in practice patterns as they relate to level of experience and geographical location are contrasted to the most up-to-date available guidelines. CONCLUSION: Responses to the survey suggest that there is heterogeneity in certain practice patterns for MMP. Biopsy remains an area of controversy in dictating treatment plans. Identified areas of need should be targeted in future research.

Efficacy of ab-interno gelatin microstent implantation in primary and refractory glaucoma.

OBJECTIVE: To evaluate the outcomes of ab interno gelatin microstent implantation alone and in combination with phacoemulsification for the reduction of intraocular pressure (IOP). DESIGN: Retrospective cohort study. PARTICIPANTS: 141 eyes of 141 patients with any glaucoma subtype, including refractory glaucoma, operated in the Centre Hospitalier de l'Université de Montréal (CHUM) from 2015-2018. Patients were included if they were over 40 years of age and had a preoperative IOP of >18 mm Hg on maximum tolerated medical therapy. METHODS: All patients received ab-interno microstent implantation (XEN-45, Allergan, Madison, NJ) with mitomycin C +/- combined phacoemulsification. The primary outcome was complete surgical success (IOP 6-18 mm Hg and <20% reduction from baseline without IOP medications or reoperations or cyclophotocoagulation); secondary outcomes included qualified success allowing for medications, percentage reduction in mean IOP and medications, and reduction in number of complications, interventions, and reoperations. RESULTS: Mean follow-up was 30.5 ± 10.2 months (±SD). Mean IOP was 23.3 ± 7.0 mm Hg on 3.4 ± 0.8 medications at baseline and 13.3 ± 4.7 mm Hg on 1.9 ± 1.5 medications at 24 months of follow-up (p < 0.001). From 24-month survival analysis estimates, complete success was achieved in 34.1% of microstent eyes versus 20.7% with combined phacoemulsification (p = 0.02); 79.1% versus 75.1% achieved qualified success, respectively (p = 0.86). Cases with combined phacoemulsification had a higher rate of failure (hazard ratio [HR] = 1.6, 95% CI 1.1-2.3, p = 0.02). Needling with mitomycin-C or 5-fluorouracil postoperatively occurred in 54 eyes (38.3%). Complications included transient hypotony (10.6%), transient hyphema (6.4%), macular edema (4.3%), and microstent exposure (2.8%). There were 33 eyes (23.4%) with reoperations and 14 (9.9%) requiring subsequent cyclophotocoagulation lasers. CONCLUSIONS: Microstent implantation required topical therapy in most cases 24 months following surgery in primary and refractory glaucoma and, when combined with phacoemulsification, had a higher risk of failure.

Extended Depth of Focus Versus Trifocal for Intraocular Lens Implantation: An Updated Systematic Review and Meta-Analysis.

PURPOSE: To compare the extended depth of focus (EDOF) vs trifocal intraocular lenses (IOLs) in patients undergoing IOL implantation. DESIGN: Systematic review and meta-analysis. METHODS: An electronic search was conducted as per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to include studies comparing EDOF vs trifocal IOLs. Refraction and visual acuity were primary outcomes. Secondary outcomes included defocus curves, intraocular aberrations, contrast sensitivity (CS), quality of vision (QoV) questionnaire score, haloes and glare, spectacle independence, and patient satisfaction. RESULTS: A total of 22 studies enrolling 2200 eyes were identified. Trifocal IOL showed a significant improvement in sphere (mean difference [MD] = -0.23; P = .001) and spherical equivalence (MD = -0.11, P = .0001) compared to EDOF IOL. No difference was observed in cylinder (MD = -0.03, P = .25) or astigmatism. Trifocal IOL had superior near visual acuity outcomes, namely uncorrected near visual acuity (MD = 0.12, P < .00001) and distance-corrected near visual acuity (MD = 0.12, P = .002). Postoperative corrected distance visual acuity (MD = -0.01, P = .01) was significantly improved for the EDOF group, although no difference was noted in postoperative uncorrected distance visual acuity (MD = 0.00, P = .84), uncorrected intermediate visual acuity (MD = 0.01, P = .68) or distance-corrected intermediate visual acuity (MD = -0.01, P = .39). Defocus curve favored trifocal IOLs at near vision and EDOF IOLs at intermediate vision. Ocular aberration, CS, haloes (odds ratio = 0.64, P = .10), glare, and patient satisfaction were not statistically significant between the groups. The trifocal IOL was associated with an improved QoV questionnaire score (MD = 1.24, P = 0.03) and spectacle independence (odds ratio = 0.26, P = .02). CONCLUSIONS: Trifocal IOLs improved uncorrected near visual acuity compared to EDOF IOLs. Uncorrected distance and intermediate visual acuity, halos, and glare were not statistically different between both groups.

Staged hepatectomy for bilobar colorectal hepatic metastases.

OBJECTIVES: This study describes the management of patients with bilobar colorectal liver metastases (CRLM). METHODS: A retrospective collection of data on all patients with CRLM who were considered for staged resection (n= 85) from January 2003 to January 2011 was performed. Patients who underwent one hepatic resection were considered to have had a failed staged resection (FSR), whereas those who underwent a second or third hepatic resection to produce a cure were considered to have had a successful staged resection (SSR). Survival was calculated from the date of diagnosis of liver metastases. Complete follow-up and dates of death were obtained from the Government of Quebec population database. RESULTS: Median survival was 46 months (range: 30-62 months) in the SSR group and 22 months (range: 19-29 months) in the FSR group. Rates of 5-year survival were 42% and 4% in the SSR and FSR groups, respectively. Fifteen of the 19 patients who remained alive at the last follow-up date belonged to the SSR group. CONCLUSIONS: In patients in whom staged resection for bilobar CRLM is feasible, surgery would appear to offer benefit.

Corneal Perforation in Patients Under Treatment With Dupilumab for Atopic Dermatitis.

PURPOSE: We report, for the first time, 2 cases of corneal ulceration and perforation after treatment with dupilumab for atopic dermatitis. METHODS: A 30-year-old woman and a 44-year-old man developed unilateral severe corneal ulceration and perforation while on dupilumab therapy after 3 and 9 months, respectively. RESULTS: Corneal cultures were negative in both cases except for scanty growth of Staphylococcus species on enrichment. Both cases progressed to perforation despite intensive topical antibiotic treatment. The first case required a tectonic keratoplasty to restore globe integrity after failed attempts of corneal gluing and multilayer amniotic membrane transplantation, and the second case was managed successfully with a cyanoacrylate glue patch. CONCLUSIONS: Although there have been previous reports of conjunctival injection and dry eye after dupilumab, these are the first 2 reports of corneal ulceration with rapid progression to perforation in patients under treatment with dupilumab. The underlying pathophysiology for ulcerative keratitis in these cases remains unknown, but there is no doubt that cessation of dupilumab prevented progression of the melting. Severe ocular symptoms while on dupilumab require a prompt discussion with the dermatology team to potentially switch treatment and halt further keratitis progression.

Corneal thinning following bevacizumab intrastromal injection for the treatment of idiopathic lipid keratopathy.

Purpose: To describe the occurrence of corneal thinning in a patient following intrastromal injection of bevacizumab to treat lipid keratopathy. Observations: A 36-year-old female presented with decreased vision in her right eye with central posterior corneal haze and underwent a treatment regimen including artificial tears, cyclosporine 0.05% drops, prednisolone 1% and oral Valacyclovir 1g with no improvement. Neovascularization was noted at 18 months follow up and treated with intrastromal bevacizumab injections at 24 months. The feeder vessel was attenuated at 3- and 6-months post-injection, but tomography indicated sustained thinning and flattening of the cornea at the injection site contributing to the development of irregular astigmatism. Conclusions and Importance: Corneal thinning is an uncommon potential side effect of intrastromal bevacizumab injection that may affect postoperative visual acuity.

Reliability of Several Glaucoma Tests in Patients With Boston Type 1 Keratoprosthesis.

PURPOSE: Managing glaucoma after Boston type 1 keratoprosthesis (KPro) surgery remains challenging. We herein assessed the fitness of commonly used clinical tests to evaluate glaucoma in KPro eyes versus eyes with penetrating keratoplasty (PK) as controls. METHODS: Sixteen patients with KPro and 14 patients with PK tested in an identical manner. After the 10-2 visual field with size V stimulus, intraocular pressure (IOP) was estimated with palpation by the first observer. Then, retinal nerve fiber layer (RNFL) thickness analysis was performed twice using optical coherence tomography by an ophthalmic photographer, before and after a short break. After the second observer estimated the IOP, the visual field was repeated. Finally, color photographs of the optic disk were captured by an ophthalmic photographer. The cup-to-disk ratio was assessed by 2 masked observers, at 2 different time points, in a random manner. Agreements between and within observers and reliability of repeated measurements were evaluated using the intraclass correlation coefficient (ICC) and Bland-Altman plots. RESULTS: Inter-rater agreement of palpation IOP estimate was moderate for eyes with KPro (ICC = 0.47) and fair for eyes with PK (ICC = 0.27). Visual field and RNFL thickness showed high test-retest reliability in both KPro and PK eyes (ICC > 0.80 for both). Inter-rater agreement of cup-to-disk ratio assessments was substantial in eyes with both KPro (ICC = 0.62) and PK (ICC = 0.70). CONCLUSIONS: The 10-2 visual field and RNFL thickness seem sufficiently repeatable and might allow the detection of glaucoma progression in KPro eyes. Such testing is important, given limited inter-rater agreement regarding the palpation IOP estimate.

Three-Year Outcomes of Implantable Collamer Lens Followed by Excimer Laser Enhancement ("Bioptics") in the Treatment of High Myopic Astigmatism.

PURPOSE: Describe three-year outcomes of spherical implantable Collamer lens (ICL) followed by excimer laser enhancement (bioptics) in eyes with high myopic astigmatism. PATIENTS AND METHODS: Retrospective case series of thirty-four cases that underwent bioptics enhancement at the Johns Hopkins Wilmer Eye Institute. All eyes had a preoperative sphere of -6.00 D or more with a cylinder of at least 2.00 D. Uncorrected and corrected distance visual acuity (UDVA and CDVA), manifest spherical equivalent refraction (MSE), ICL vault measurements and central corneal thickness (CCT) were collected. Endothelial cell counts (ECC), root mean square (RMS) of higher order aberrations (HOAs), adverse events and subsequent surgeries were also assessed. RESULTS: All patients had a minimum follow-up of 3 years. Preoperative UDVA was 2.29 ± 0.46 logMAR and improved to 0.03 ± 0.23 logMAR at 3 years (p<0.05). MSE was -12.30 ± 4.05 preoperatively and changed to -0.21 ± 0.46 at 3 years (p<0.05). The efficacy and safety indices were 1.28 ± 0.32 and 1.47 ± 0.27 at 3 years post-enhancement. HOA did not significantly change throughout the follow-up (p<0.05). Endothelial cell loss at 12 months was calculated at 5.7%. Two eyes required ICL exchange due to vault-related issues. CONCLUSION: Bioptics offered excellent long-term safe, predictable, and efficient outcomes for high myopic astigmatism and can be considered an option if toric ICL is not available. Results confirm that wavefront-guided photoablation remains an excellent option to manage residual refractive error after phakic IOL.

Ocular mucous membrane pemphigoid: novel treatment strategies.

PURPOSE OF REVIEW: To describe current treatments of ocular mucous membrane pemphigoid (OMMP), including conventional systemic immunosuppression (CIST) and novel biologic agents. RECENT FINDINGS: CIST remains the main stay therapy for the management of OMMP. The choice of agents is tailored to disease severity - mild disease is treated with dapsone, whereas moderately severe cases are often treated with mycophenolate mofetil or sulfa-based drugs and severe cases are treated with cyclophosphamide. In recalcitrant disease newer biologics such as Rituximab, intravenous immunoglobins and tumour necrosis alpha antagonists have proven to be effective in disease control. Control of inflammation with topical therapy and halting conjunctival fibrosis remain as areas of unmet clinical needs. SUMMARY: OMMP is largely treated with CIST. The recent introduction of new biologic agents could shape the way the disease is treated in the near future.

Presumed Herpes Zoster Ophthalmicus Reactivation Following Recombinant Zoster Vaccination.

PURPOSE: To report a case of herpes zoster ophthalmicus (HZO) reactivation after recombinant zoster vaccination. METHODS: A 78-year-old woman, with a history of HZO 20 years ago, was referred for progressive corneal thinning in her left eye that started 1 week after her second dose of recombinant zoster vaccination. RESULTS: At presentation, visual acuity was counting fingers. Corneal sensation was markedly decreased. Slit lamp examination revealed a temporal paracentral epithelial defect 1.5 × 2.0 mm in size with 40% thinning and surrounding stromal inflammation suggestive of stromal keratitis with ulceration. The patient was started on oral valacyclovir, topical erythromycin ointment, and hourly topical lubrication. A bandage contact lens was placed and was replaced 1 week later with self-retained cryopreserved amniotic membrane ring. The ring was removed in the following week when the thinned area was epithelialized with no further evidence of stromal lysis. CONCLUSIONS: HZO reactivation after recombinant zoster vaccination is uncommon but possible. Ophthalmologists should remain aware of potential risks of zoster vaccination and take special precautions in patients with HZO history.

Clinical Characteristics of Patients With Chronic Stevens-Johnson Syndrome Treated at a Major Tertiary Eye Hospital Within the United Kingdom.

The purpose of this study is to provide a comprehensive review of the clinical characteristics in chronic Stevens-Johnson syndrome (SJS) patients within the United Kingdom population, their causative factors, treatment profile and prognosis. This retrospective series included 91 patients with chronic SJS treated at Moorfields Eye Hospital (London, United Kingdom). A chart review included visual acuity and presence of clinical findings (including lid abnormalities and ocular surface findings). All medical and surgical treatments were also recorded. Approximately a half of patients were White British but there were significant numbers of patients from other ethnic groups, South Asian and Black in particular. Oral antibiotics were the causative agent in almost a half of the patients with SJS, systemic infections in 14%, non-steroidal anti-inflammatory drugs in 8% and anticonvulsants in 7%. The age of onset was varied but a significant proportion of patients developed acute SJS in childhood. There was a significant correlation between visual acuity at initial referral to final recorded vision. Vision was found to continue to significantly deteriorate over time despite therapeutic interventions. Our regression model shows that ~62% of the variance in final vision can be explained by the initial vision and duration disease. The majority of our patients were on advanced ocular surface treatments including serum drops, topical ciclosporin and retinoic acid drops. Of particular significance, approximately a third of our patient cohort was also on systemic immune suppression. In conclusion, chronic SJS within the UK population under tertiary care remains an area of unmet clinical need. Current medical and surgical modalities prevent worsening of vision in severe ocular disease from SJS.

Descemet Membrane Endothelial Keratoplasty Using a Pull-Through Technique With Novel Infusion Forceps.

PURPOSE: To describe a surgical technique of Descemet membrane endothelial keratoplasty (DMEK) using an endothelium-in pull-through technique with novel infusion forceps and report 6-month clinical outcomes. METHODS: This is a retrospective case series of 33 cases with Fuchs endothelial corneal dystrophy who underwent either DMEK alone (n = 5), DMEK combined with phacoemulsification (n = 27), or DMEK combined with intraocular lens exchange (n = 1) performed by surgeons at the beginning of the DMEK learning curve. Best-corrected visual acuity (BCVA), manifest refraction, central corneal thickness, endothelial cell density, intraoperative and postoperative complications, and need for additional surgeries including rebubbling were evaluated through 6 months postoperatively. RESULTS: Preoperative BCVA was 0.37 ± 0.34 logarithm of the minimum angle of resolution and improved to 0.09 ± 0.10 logarithm of the minimum angle of resolution at 6 months (P < 0.001). Eighty-two percent of eyes obtained a BCVA of 20/25 or better. Central corneal thickness decreased significantly at 6 months (P < 0.001). Endothelial cell loss was 29.1% at 6 months (available for 20 of 33 eyes). All surgeries were uneventful. Six eyes required rebubbling for graft detachments. There were no primary or secondary graft failures at 6 months. CONCLUSIONS: DMEK with infusion microforceps is efficacious and safe in eyes with Fuchs endothelial corneal dystrophy and could offer a more controlled surgery, making it suitable for both novice and experienced DMEK surgeons.

Allgrove Syndrome: A Report of New Pathological Variants in the AAAS Gene.

PURPOSE: To report 2 novel variants in the AAAS gene consistent with the diagnosis of Allgrove syndrome. METHODS: A 12-year-old girl was referred to our clinic for progressive bilateral decrease in visual acuity. She was known for achalasia that had been surgically treated at a very early age. On examination, she was found to have dry eye disease secondary to lacrimal insufficiency. She also had anisocoria, light-near dissociation, and bilateral optic nerve atrophy. RESULTS: Neurological examination and brain magnetic resonance imaging were within normal limits. Genetic workup revealed compound heterozygosity for 2 novel variants in the AAAS gene, confirming the diagnosis of Allgrove syndrome. The patient was referred to endocrinology to screen for adrenocorticotropic hormone insufficiency. She was started on topical lubricating therapy that improved her symptoms. CONCLUSIONS: Allgrove syndrome is a rare genetic disease that is characterized by the triad of achalasia, alacrima, and adrenal insufficiency. Early diagnosis, confirmed with genetic testing, is essential to initiate an appropriate follow-up and prevent a life-threatening addisonian crisis. Report of novel mutations is important to further characterize this disease.