Corneal Complications Related to Dupilumab Use.

OBJECTIVES: The aim of this report is to describe atypical corneal complications associated with dupilumab use. METHODS: This is a series of four cases of adult patients with infiltrative or ulcerative keratitis secondary to dupilumab use. RESULTS: All four patients in this series were prescribed dupilumab for the treatment of atopic dermatitis and developed infiltrative or ulcerative corneal lesions. In all cases, corneal disease was successfully managed with immediate discontinuation of dupilumab and topical steroid treatment. In two cases, the patient also received antibiotic eye drops for infection precautions. CONCLUSIONS: Although dupilumab is most commonly associated with conjunctivitis, physicians should be aware of potential severe corneal complications for early identification and intervention.

The evolution of refractive surgery training.

PURPOSE OF REVIEW: Although refractive surgery is one of the most commonly performed ophthalmic procedures, there is a relative paucity of literature on residency and fellowship refractive surgery education. The purpose of this article is to review the current state and recent updates to refractive surgery education and to evaluate the safety and visual outcomes of trainee performed refractive procedures. RECENT FINDINGS: Currently, there is no standard refractive surgery curriculum in the United States apart from required mandatory minimum refractive requirements for residents and fellows. Our survey of residency programs confirms that refractive training is extremely varied, ranging from a dedicated refractive rotation with direct surgical experience to only didactics or observation of procedures. There is a proposed standardized framework for refractive surgery training for the military, which may be a starting point in developing a more comprehensive refractive surgery curriculum in residency education. Multiple studies have reinforced the safety of resident and fellow performed refractive surgery. SUMMARY: Refractive surgery is an increasingly popular procedure and a more comprehensive refractive education is paramount. Future studies are needed to determine how best to provide the fundamental training and surgical experience for trainees in the fast-changing landscape of refractive surgery.

Incidence of postoperative week 1 management changes after resident-performed phacoemulsification cataract surgery.

BACKGROUND: The goal of this study was to investigate the incidence of departures from routine care at the postoperative week 1 (POW1) visit following uneventful resident-performed cataract surgery in asymptomatic patients who had a normal postoperative day 1 (POD1) examination. METHODS: A retrospective chart review of phacoemulsification surgeries performed by the senior resident class at Montefiore Medical Center between June 20, 2018 and April 1, 2019 was performed. The most recent preoperative visit note, operative report, POD1 visit note, and POW1 visit note were evaluated and variables were recorded. Exclusion criteria consisted of any complications that would have necessitated close follow-up and a POW1 visit, whether discovered preoperatively, intraoperatively, at the POD1 visit, or leading up to the POW1 visit. The primary outcome measure was the incidence of unanticipated management changes at the POW1 visit following resident-performed cataract surgery. RESULTS: The charts of 292 surgical cases of 234 patients that underwent phacoemulsification with intraocular lens implantation were reviewed. 226 cases (77%) had an uncomplicated pseudophakic fellow-eye history, with a routine surgery, and POD1 examination. 19 of these patients had symptomatic presentations at the POW1 timepoint, and an additional 30 had no POW1 visit at all. In total, 177 cases were included in the study, and only 4 of these cases (2.3%) had an unexpected management change at the POW1 visit. CONCLUSIONS: Asymptomatic patients who underwent uncomplicated cataract surgeries performed by resident surgeons followed by a routine POD1 visit had a low incidence of unexpected management changes at the POW1 visit. These results suggest that regularly scheduled POW1 visits could potentially be omitted for patients deemed to be at low risk for complications, and instead performed on an as-needed basis.

A Review of Pathologic Myopia.

ABSTRACT: Pathologic myopia is a leading cause of vision loss worldwide and is most prevalent in developed nations in East and Southeast Asia. Although the pathophysiologic mechanisms behind pathologic myopia have yet to be elucidated, the process is thought to stem from excessive axial elongation of the globe and subsequent degeneration of vital structures within the posterior segment. The array of complications that arise from this condition can lead to significant visual impairment. The purpose of this article is to review the classification, pathophysiology, complications, and management of pathologic myopia.

Neurotrophic keratopathy and refractive surgery.

PURPOSE OF REVIEW: Corneal nerve function is an important consideration for the refractive surgeon. In patients without a history of neuropathy, refractive surgery can lead to destruction of corneal nerves and may predispose to keratopathy. The purpose of this review is to provide an update on the effect of refractive surgery on corneal nerve function and the potential for an increased risk of neurotrophic keratopathy development. RECENT FINDINGS: Recent studies have reinforced that refractive surgery can cause anatomical and functional changes in corneal nerve function. Among the most commonly performed refractive surgery procedures are Laser Assisted In-situ Keratomileusis, Photorefractive Keratectomy, and Small Incision Lenticule Extraction. Due to differences in technique, these procedures can have varying effects on corneal nerve structure. Using in vivo confocal microscopy, postrefractive patients were found to have decreased total nerve density when compared with controls in the immediate postoperative period. In some studies, patients have been shown to undergo re-innervation and regain significant amounts of nerve function. The majority of studies have found that despite decreased nerve cell density, long-term outcomes of refractive surgery patients are generally favorable. However, loss of corneal nerve function can lead to a variety of serious effects ranging from dry eye disease, corneal hypoesthesia/anesthesia, neuropathic pain, to neurotrophic keratopathy and ulceration in the rare cases. SUMMARY: Neurotrophic keratopathy is a rare occurrence after keratorefractive surgery and is largely due to damage to corneal nerves. Proper patient selection and counseling are essential to decrease the risk of development of this devastating complication.

Acanthamoeba endophthalmitis during treatment for cutaneous disease in a renal transplant patient.

Acanthamoeba infections are difficult to diagnose and treat. We present a renal transplant patient who developed Acanthamoeba endophthalmitis on therapy with posaconazole and miltefosine for cutaneous acanthamobiasis. The patient was maintained on intracameral voriconazole injections, and oral azithromycin, fluconazole, and flucytosine. This case highlights novel presentations and treatments for acanthamoebic infection.

Improving accuracy for intraocular lens selection in cataract surgery.

PURPOSE OF REVIEW: To perform a literature review of articles published between 2016 and 2017 to assess recent advances in intraocular lens (IOL) selection. RECENT FINDINGS: A literature review revealed five areas of recent study including advances in biometric technology, formula selection for challenging eyes, accurate astigmatic correction, adjustment of IOL calculations for postrefractive eyes with and without prerefractive surgery data, and use of intraoperative IOL calculations. SUMMARY: As cataract surgery continues to be one of the most commonly performed surgeries in the United States, it is important to ensure that the most accurate technology, IOL formula, and surgical planning is utilized.

Recent advances in the treatment of corneal ectasia with intrastromal corneal ring segments.

PURPOSE OF REVIEW: To review the recent advances and reported outcomes in the use of intrastromal corneal ring segments (ICRS) for the treatment of corneal ectasia. RECENT FINDINGS: ICRS are a well-tolerated and effective treatment for patients with corneal ectasia, particularly keratoconus, offering long-term improvement in visual, refractive, and keratometric measures. ICRS do not consistently decrease corneal aberrations. Patients with mild-to-moderate keratoconus, known to have less predictable outcomes with ICRS, may be better selected and treated with the use of customized nomograms, accounting for factors such as internal astigmatism. Corneal collagen cross-linking performed after ICRS implantation is an important complementary treatment in preventing the progression of ectasia, whereas subsequent treatment with either photorefractive keratectomy or toric intraocular lens implantation offers a significantly improved visual and refractive result. SUMMARY: ICRS are an important component to the treatment of corneal ectasia. Knowledge of outcomes among specific groups of patients should improve treatment planning and nomograms. Combined treatments with ICRS allow for notable improvements in corneal stability and refractive error, in addition to the improvement in irregular astigmatism seen with ICRS.

Orbital granuloma annulare as presentation of systemic sarcoidosis.

PURPOSE: To report a unique case of an orbital mass with atypical histopathologic features that although overlap with other granulomatous disorders, likely represents orbital involvement of systemic sarcoidosis. METHODS: Observational case report. RESULTS: A 51-year-old woman presented with an orbital mass and was found to have mild proptosis and restriction of ocular motility. The remainder of her ophthalmic exam was unremarkable. Excisional biopsy revealed ill-defined granulomas with central necrosis, chronic inflammation and sclerotic collagen; several features consistent with granuloma annulare. Further systemic workup including laboratory and radiologic studies were compatible with a diagnosis of systemic sarcoidosis. CONCLUSION: Our case emphasizes the importance of a complete systemic workup with the detection of an orbital granuloma to ensure accurate diagnosis and appropriate management.

Umbilical Cord Graft for Refractory Neurotrophic Keratopathy.

PURPOSE: The purpose of this study was to evaluate whether an umbilical cord tissue graft (UCG) could promote reepithelialization in refractory cases of neurotrophic keratopathy, which failed traditional treatment modalities including amniotic membrane grafts. METHODS: This retrospective case series included 3 eyes of 3 patients who underwent UCG placement for refractory stage 3 neurotrophic keratopathy. Records were reviewed to evaluate the clinical course including previous treatments, time to UCG dissolution, time to corneal epithelialization, and recurrence of epithelial defects. RESULTS: The time of a nonhealing epithelial defect before UCG placement ranged from 7.7 to 30 weeks (mean 21.0). UCG dissolution time ranged from 1.7 to 8.1 weeks (mean 5.0) compared with the previous failed amniotic membrane dissolution time of 0.3 to 1.6 weeks (mean 0.95). The time to complete epithelialization after UCG placement was 7.7 and 8.1 weeks, respectively, for the first 2 cases, whereas the third case did not fully epithelialize. Only 1 eye did not have recurrence of an epithelial defect. The maximum time of maintained epithelialization without recurrence ranged from 5 to 86 weeks (mean 42.7). Follow-up time was 37 to 108.1 weeks (mean 62.2) after first UCG placement. CONCLUSIONS: UCG may allow for longer retention time of tissue grafts, provide a mechanical barrier for protection, and aid in regeneration of the ocular surface. UCG may be an option for re-epithelialization in recalcitrant cases of neurotrophic keratopathy, after conventional treatments such as amniotic membrane grafts have failed.

De novo malignant optic chiasm glioma with initial clinical response to steroids.

Malignant optic nerve glioma (MONG) is a rare but uniformly fatal disease that remains poorly understood. We describe a notable case of this rare disease occurring in the optic chiasm. Normal brain imaging and normal ophthalmic examination two years prior to diagnosis provide evidence for de novo genesis of MONG in our patient. Early response to steroids highlights the degree to which MONG can initially mimic inflammatory optic neuropathies and chiasmal syndromes. Our case also demonstrates a poor outcome with MONG even with current advanced therapy for glioblastoma including radiotherapy plus concomitant and adjuvant temozolomide (the EORTC/NCIC regimen) and bevacizumab.

Light Perception Vision and Xerophthalmia in an 11-Year-Old Boy.

The authors present a case of a child who had severe dietary restriction with consumption of french fries only. He presented to the ophthalmology clinic with light perception vision in both eyes, and was found to have dry, leathery conjunctivae with Bitot's spots and hazy corneas with diffuse punctate epithelial erosions. Electroretinography testing showed an absent rod response and a decreased cone response. Laboratory testing results were positive for vitamin A deficiency. After prompt initiation of treatment, he had improvement in his vision and anterior segment findings. This case serves as a reminder that vitamin A deficiency can present in high-income countries, and that ophthalmologists may play a key role in diagnosing the condition, initiating treatment, and sending patients to the appropriate subspecialists for further evaluation and management of the underlying condition. For patients with vitamin A deficiency, ophthalmologists can play an essential role in preventing permanent vision loss and the systemic consequences of deficiency such as infection or death. [J Pediatric Opthalmology & Strabismus. 2022;59(3):e35-e38.].

A child with dendritiform eye lesions and developmental delay.

Purpose: Tyrosinemia Type II (Richner-Hanhart syndrome) is a rare autosomal recessive disease that occurs due to deficiency in the enzyme tyrosine aminotransferase and can result in an ulcerated keratitis. We present a case of a young patient with oculocutaneous tyrosinemia despite a negative newborn screen. Observations: A 15 month old boy with an uncomplicated birth history and negative newborn screen presented with a unilateral central irregular epithelial defect and hyperkeratotic lesions on his fingertips and soles. A month later, the patient developed bilateral dendritiform epithelial erosions. Following a series of antiviral, antibiotic, and lubricating treatments, there was a waxing and waning course of epithelial healing. After the patient was lost to follow up for one year, the patient presented with a new global developmental delay prompting further workup. Tyrosine and phenylalanine levels were ordered which confirmed a diagnosis of Tyrosinemia Type II, and the patient was started on a low-protein diet. A month later, the patient's epithelial defects and ocular symptoms were resolved. Conclusion and Importance: Presentation of a dendritiform epithelial erosion, whether unilateral or bilateral, accompanied by symptoms of developmental delay and palmoplantar hyperkeratotic lesions should prompt measurement of tyrosine and phenylalanine levels. As dermatologic lesions and variable developmental delay may not appear until later in the course of disease, diagnosis may depend on early recognition of ocular signs and symptoms even with negative newborn screening. Prompt diagnosis and diet modification is necessary to prevent developmental delay in this disease. To our knowledge, this is the first Tyrosinemia Type II case in the literature manifesting as an asynchronous bilateral eye disease.

Unilateral vortex keratopathy of unknown etiology.

A 54-year-old man with noncontributory medical history presented to an ophthalmologist in January 2022 after 10 days of irritation in his right eye. The patient recounts having felt something get into his eye and under his contact lens (CL) while he was climbing into his car, but he was unsure what the foreign body may have been. Initial examination by the clinician found uncorrected distance visual acuity of 20/100-2 with a corneal abrasion, 4+ corneal edema, and 3+ conjunctival injection, for which he was placed on topical antibiotics (ocuflox and tobradex) with a bandage CL. 1 week later, visual acuity was 20/80, corneal edema had improved, and he was noted to have corneal scarring and an epithelial defect. Tobradex was continued while prednisolone drops and preservative-free artificial tears were started. 1 week later, the patient had worsening visual acuity to 20/250 and was referred to our tertiary center. On initial consultation, the patient had an uncorrected distance visual acuity of 20/500 and an uncorrected near visual acuity of >J10 in the right eye. Slitlamp examination of the right eye was significant for vortex keratopathy and mild corneal pannus with 360-degree subtle conjunctivalization of the limbus ( Figure 1JOURNAL/jcrs/04.03/02158034-202210000-00022/figure1/v/2022-10-03T121249Z/r/image-tiff ). The corneal topograph was obtained showing significant surface irregularity on the Placido image ( Figure 2JOURNAL/jcrs/04.03/02158034-202210000-00022/figure2/v/2022-10-03T121249Z/r/image-tiff ). Examination of the left eye was unremarkable. The ocular history is significant for myopia of -4.0 diopters and CL use for 20 years. The patient admits to regularly wearing soft CLs for several days straight and only removing them for a few hours. Antibiotics were discontinued, corticosteroid drops were reduced in frequency, and the patient was continued on preservative-free artificial tears. What imaging might you consider? What is your differential diagnosis at this point? What would be the most appropriate surgical and/or medical interventions? What would you counsel in prognosis for this patient?

CHORIORETINAL CHANGES IN A GENETICALLY CONFIRMED CASE OF BOUCHER-NEUHÄUSER SYNDROME.

PURPOSE: To describe the retinal findings in a 25-year-old white woman in whom a diagnosis of Boucher-Neuhäuser Syndrome (BNS) was supported by genetic testing, which identified a missense and novel nonsense mutation in the PNPLA6 gene. METHODS: Observational case report of a 25-year-old woman who presented with primary amenorrhea, cerebellar ataxia, and mild retinal pigmentary abnormalities. Neurologic, endocrine, and genetic evaluations established a diagnosis of BNS. RESULTS: Clinical examination and multimodal imaging documented focal outer retinal and retinal pigment epithelium changes including bilateral foveal stippling and a circular area of hypopigmentation in the superior macula of the left eye. Optical coherence tomography showed a linear area of outer retinal attenuation superonasal to the fovea and multiple foci of pinpoint outer retinal defects in the temporal macula of the left eye. Humphrey visual field 24-2 testing showed nonspecific defects in both eyes. Full-field electroretinography showed no evidence of a generalized retinal dysfunction. CONCLUSION: Recognition that the chorioretinal abnormalities occurring in BNS can be rather subtle is essential because the diagnosis of BNS may depend on their detection. To the best of our knowledge, this is the first report in the ophthalmic literature of mild chorioretinal changes in a patient with BNS testing positive for a mutation in the PNPLA6 gene.

Oral Miltefosine as Salvage Therapy for Refractory Acanthamoeba Keratitis.

PURPOSE: To report a case series of patients with treatment-resistant Acanthamoeba keratitis (AK) using oral miltefosine, often as salvage therapy. DESIGN: Descriptive, retrospective multicenter case series. METHODS: We reviewed 15 patients with AK unresponsive to therapy who were subsequently given adjuvant systemic miltefosine between 2011 and 2017. The main outcome measures were resolution of infection, final visual acuity, tolerance of miltefosine, and clinical course of disease. RESULTS: All patients were treated with biguanides and/or diamidines or azoles without resolution of disease before starting miltefosine. Eleven of 15 patients retained count fingers or better vision, and all were considered disease free at last follow-up. Eleven of 15 patients had worsening inflammation with miltefosine, with 10 of them improving with steroids. Six patients received multiple courses of miltefosine. Most tolerated oral miltefosine well, with mild gastrointestinal symptoms as the most common systemic side effect. CONCLUSIONS: Oral miltefosine is a generally well-tolerated treatment adjuvant in patients with refractory AK. The clinician should be prepared for a steroid-responsive inflammatory response frequently encountered during the treatment course.

Outcomes of Descemet Stripping Endothelial Keratoplasty in Eyes With Pars Plana Versus Anterior Chamber Glaucoma Drainage Devices.

PURPOSE: To compare visual outcomes and complications of Descemet stripping endothelial keratoplasty (DSEK) in eyes with anterior chamber (AC) versus pars plana (PP) glaucoma drainage devices (GDDs). METHODS: A retrospective chart review was conducted of 85 eyes that underwent DSEK and had previous or concurrent AC (37 eyes) or PP (48 eyes) GDD implantation. Outcome measures examined included visual acuity, donor dislocation rate, intraocular pressure, and secondary graft failure (SGF). RESULTS: The mean logarithm of the minimum angle of resolution vision improved from 1.50 (SD = 0.64) to 0.88 (SD = 0.71) in the AC group and from 1.37 (SD = 0.59) to 1.20 (SD = 0.76) in the PP group, with no significant group difference. Intraocular pressure did not differ between the groups preoperatively but was higher in the AC group compared with the PP group postoperatively [mean (SD) 14.5 (6.28) versus 11.5 (5.54) mm Hg, P = 0.03]. Graft dislocation occurred in 35.1% and 29.2% of eyes in the AC and PP groups, respectively. SGF occurred in 18.9% and 41.7% with median time 17.1 (interquartile range [IQR] 4.6-32.1) and 27.9 (IQR 15.1-34.7) months to development in the AC and PP groups, respectively. Multivariable logistic and Cox proportional hazards regression models found no statistically significant factors (tube location, age, sex, concurrent tube revision, new tube insertion) associated with either donor dislocation or SGF. CONCLUSIONS: There was no reduction in donor dislocation or SGF in eyes with PP compared with AC GDD, despite the decreased proximity of the shunt from the endothelial graft. Overall, GDD has a significantly adverse effect on long-term graft survival and close follow-up is warranted.

Clinical Outcomes of Descemet Stripping Endothelial Keratoplasty in Eyes With Glaucoma Drainage Devices.

PRECIS: In this retrospective case series of 85 eyes, 31.8% developed graft failure and graft survival was 50% at 3 years. The presence of an aqueous shunt has a significantly adverse effect on long-term graft survival. PURPOSE: To report visual outcomes and complications of Descemet stripping endothelial keratoplasty (DSEK) in eyes with glaucoma drainage devices (GDD). MATERIALS AND METHODS: A retrospective chart review was conducted of 122 procedures of 85 eyes of 83 patients who underwent DSEK and had prior or concurrent GDD implantation. Mean follow-up was 36.5±31.4 months. Patients with postoperative follow-up of <3 months were excluded from analysis. Outcome measures examined included visual acuity, donor dislocation rate, intraocular pressure (IOP), and graft survival. RESULTS: The mean logMAR vision improved to 1.07 postoperatively from 1.43 preoperatively, with 61% of eyes experiencing improved visual acuity. Graft dislocation occurred in 27 (31.8%) eyes and required repeat injection of air in the anterior chamber. Primary graft failure occurred in 2 (2.4%) eyes, whereas secondary graft failure occurred in 27 (31.8%) of eyes, with an average time of 24.3 months for development. Estimated survival rates at 1, 2, and 3 years were 89%, 78%, and 50%, respectively. Escalation of IOP occurred in 21 (24.7%) eyes postoperatively, where 14 eyes underwent medical therapy and 7 eyes required surgical intervention/laser for IOP control. CONCLUSIONS: DSEK after GDD is a viable alternative to treat endothelial dysfunction, with the majority of patients achieving visual improvement. However, aqueous shunt implantation has a significantly adverse effect on long-term graft survival and close follow-up is warranted.

Five year outcomes of Boston type I keratoprosthesis as primary versus secondary penetrating corneal procedure in a matched case control study.

Despite improved retention and reduced complication rates paving the way for the current expansion of applications and surge in prevalence for the Boston type I Keratoprosthesis (KPro), the most frequent indication for its implantation today remains prior graft failure. The purpose of this study is to evaluate the long-term outcomes of primary KPro and compare to secondary implantation in a matched cohort study. This study included patients who underwent KPro implantation in a single center by two surgeons between July 2008 and October 2014. All eyes with KPro implantation as the primary procedure with a minimum follow up of 12 months were matched with eyes with same preoperative diagnoses that underwent secondary KPro implantation. Main outcomes included visual acuity and device retention. A total of 56 eyes were included with 28 eyes in each group. Mean follow up was 5.0 years for both groups. Twenty-nine percent (8) of the eyes in the primary group had a diagnosis of chemical or thermal injuries, 25% (7) aniridia, 18% (5) autoimmune disease, 4% (1) infectious keratitis/neurotrophic cornea, 7% (2) gelatinous corneal dystrophy, 7% (2) ectrodactyly ectodermal dysplasia/limbal stem cell deficiency, and 11% (3) uveitis/hypotony. Sixty-one percent (17) of the eyes in the primary group and 39% (11) in the secondary group maintained a final best-corrected visual acuity of 20/200 or better at a mean follow up of 5.0 years; the probability of maintaining best-corrected vision is 0.83 and 0.49 for primary and secondary groups at 5.0 years (p = 0.02). There is no statistically significant difference between groups in device retention (p = 0.22) or postoperative complication rates (p >0.05). This study demonstrates that Boston KPro implantation may be successful as a primary procedure in patients at high risk of failure with traditional penetrating keratoplasty. The device has a good long-term retention rate and visual outcomes are promising however a larger study is needed for more definitive results.