Unilateral Granular Type 2 Corneal Dystrophy With Exacerbation After LASIK.

PURPOSE: The aim of this study was to report a case of unilateral granular corneal dystrophy type 2 (GCD2) with exacerbation after bilateral laser in situ keratomileusis (LASIK). METHODS: Clinical evaluation, Scheimpflug imaging, anterior segment optical coherence tomography (AS-OCT), cytology, and genetic testing were used to confirm the diagnosis of unilateral GCD2 with exacerbation after bilateral LASIK. Detailed literature review for possible unilateral GCD2 presentations was performed. RESULTS: A 54-year-old White woman presented with blurred vision in her left eye and a history of bilateral LASIK performed 8 years before. Examination revealed dense opacities in the left cornea only, which were confirmed to be confined to the LASIK interface and adjacent corneal stromal tissue, as determined by AS-OCT. The patient underwent flap lift, interface debris removal, and stromal bed phototherapeutic keratectomy. Cytological analysis showed eosinophilic corneal stromal deposits that stained with trichrome stain and were congophilic on Congo red stain. Genetic testing was positive for heterozygous GCD2 transforming growth factor ß-induced gene ( TGFBI ), c.371G>A, p.R124H mutation. There were no opacities identifiable in the right eye on serial slit-lamp examination, Scheimpflug imaging, or OCT imaging at 4 or 8 years after bilateral LASIK. Literature review failed to identify any previous reports of unilateral GCD2. CONCLUSIONS: This is the first known reported case of unilateral granular corneal dystrophy type 2. LASIK is contraindicated in eyes with corneal stromal dystrophies related to mutations in TGFBI as both flap creation and laser ablation can exacerbate visually significant opacity formation. Scheimpflug and AS-OCT imaging are useful to identify opacities in GCD2.

Neurotrophic Keratopathy After Slow Coagulation Transscleral Cyclophotocoagulation.

PURPOSE: Decreased corneal sensation and subsequent neurotrophic keratopathy (NK) is an uncommon complication after transscleral cyclophotocoagulation (TSCPC). Post-TSCPC NK has been rarely reported in the literature, predominantly after traditional, "pop technique" continuous-wave TSCPC or micropulse CPC. The authors report the first case series of NK after slow-coagulation TSCPC (SC-TSCPC). METHODS: This was a respective chart review of patients who developed NK after SC-TSCPC. The collected data included demographic data, type of glaucoma, risk factors for corneal anesthesia in addition to the number of laser spots, and the extent of the treated area. RESULTS: Four eyes experienced NK after SC-TSCPC. The median time for the development of NK was 4 weeks. At the final visit, 2 patients had a resolution of NK, 1 had a persistent corneal ulcer, and 1 had worsening NK and corneal perforation. CONCLUSIONS: NK is a rare but a vision-threatening complication that can develop after SC-TSCPC in patients with risk factors for decreased corneal sensation. Early diagnosis and proper management are crucial to reducing the risk of vision loss and improving the prognosis of these cases.

Paraproteinemic Keratopathy in a Patient With Previous LASIK Procedure Mimicking Lattice Corneal Dystrophy.

PURPOSE: The aim of this study was to report a unique clinical presentation of paraproteinemic keratopathy after a myopic uneventful laser in situ keratomileusis (LASIK) procedure that led to the diagnosis of gammopathy of undetermined significance. METHODS: This was an interventional case report. A 55-year-old woman present with bilateral branching opacities limited to the optical zone of myopic LASIK. The patient's medical history was unremarkable. RESULTS: After ruling out a mutation in TGF-ß1 , a systemic workup was performed, revealing an IgG level of 12.8 mg/dL, lambda-free light chain of 12.8 mg/dL, and M-spike of 0.6 g/dL. Bone marrow aspiration was slightly hypercellular, without evidence of neoplastic infiltration by plasma cells. The patient underwent 3 cycles of systemic chemotherapy, with improvement in best-corrected visual acuity. CONCLUSIONS: Paraproteinemic keratopathy is a rare clinical presentation that may lead to a systemic diagnosis of hematologic malignancy. To the best of our knowledge, this is the first reported case of paraproteinemic keratopathy after LASIK.

Concomitant Bandage Contact Lens, Oral Nicergoline, and Topical Autologous Serum for Severe Neurotrophic Keratitis.

BACKGROUND: To report the outcomes of using the combination of oral nicergoline, autologous serum, and contact lens to enhance corneal epithelization in neurotrophic keratitis and to discuss the clinical potential of this management. METHODS: This was a prospective consecutive case series study of eight patients treated for neurotrophic keratitis at the "Conde de Valenciana" Institute of Ophthalmology. Oral nicergoline, autologous serum, and bandage contact lens were initiated at the same time, immediately after stage 3 diagnosis keratitis was confirmed clinically, and until corneal epithelialization was achieved or eminent corneal perforation was seen. In patients where diabetes was a cause, glycosylate hemoglobin was measured to asses metabolic control. Corneal esthesiometry and corrected distance visual acuity were assessed before and after treatment. RESULTS: This study included eight eyes of eight patients (5 men [62.5%], average age 57±17.9 years). All patients completed at least 1 month of follow-up after nicergoline and contact lens suspension. Of the eight eyes, no one had positive culture growth and complete epithelial healing was achieved in all cases. Half of patients had diabetes and had a poor metabolic control. Corneal sensitivity improved in all eyes almost 2 centimeters in Cochet-Bonnet esthesiometry ( P= 0.01). In addition, final visual acuity gains were obtained ( P= 0.100). CONCLUSIONS: The combination of oral nicergoline, autologous serum, and bandage contact lens simultaneously could be an alternative in the management of stage 3 neurotrophic keratitis when conventional medical treatment has no improvement of corneal epithelization.

Corneal Electrolysis for Granular Corneal Dystrophy Type 2 (Avellino Corneal Dystrophy) Exacerbation After LASIK.

PURPOSE: To report two cases in which exacerbation of granular corneal dystrophy type 2 (GCD2; Avellino corneal dystrophy) after laser in situ keratomileusis (LASIK) was successfully removed by corneal electrolysis. METHODS: This study involved a 66-year-old man and a 43-year-old man with GCD2 who had undergone bilateral LASIK for myopia 10 or more years prior to presentation. In both patients, GCD2 corneal opacity gradually developed postoperatively at the LASIK flap interface, thus resulting in a decrease of visual acuity. For treatment, the LASIK flaps in both patients were surgically lifted to directly remove the opacity. Corneal electrolysis was then applied to the back of each LASIK flap and stromal bed. RESULTS: Postoperatively, the ocular symptoms and corneal opacities related to GCD exacerbation disappeared, with improvement of corrected and uncorrected distance visual acuity and almost no change of refractive error. CONCLUSIONS: The findings reveal that corneal electrolysis is safe and effective for treating exacerbations of GCD2 following LASIK when applied to a surgically lifted flap, and that it successfully removes GCD2-related LASIK flap interface opacities with almost no change of refractive error postoperatively. [J Refract Surg. 2023;39(1):61-65.].

CHORIORETINAL ATROPHY IN VITREORETINAL LYMPHOMA: Risk Factors and Visual Outcomes.

PURPOSE: To investigate the frequency, risk factors, and functional prognosis of chorioretinal atrophy (CRA) in vitreoretinal lymphoma (VRL). METHODS: This was a retrospective cohort study of consecutive patients with vitreoretinal lymphoma. The demographic, clinical, and retinal features and the treatment modalities of each patient were collected. The charts and the multimodal imaging at each visit were reviewed. The risk factors associated with CRA were investigated with a mixed-model Cox regression. RESULTS: Of the 79 eyes of 40 patients included, 41 eyes (52%) had CRA; 27 and 14 eyes had focal and diffuse CRA, respectively. The rate of vitreoretinal lymphoma lesions in the macula was similar between focal and diffuse CRA (96% vs. 93%). The eyes with CRA had worse best-corrected visual acuity (P = 0.006) than eyes with no CRA; diffuse atrophy had the worst best-corrected visual acuity (P < 0.001). The presence of retinal infiltrates (hazard ratio = 3.75, 95% confidence interval [CI] 1.46-9.59, P = 0.006) and vertical hyperreflective lesions (hazard ratio= 4.13 95% CI 1.14-14.93, P = 0.03) on optical coherence tomography and macular involvement (hazard ratio = 6.59, 95% CI 1.41-30.53, P = 0.02) were associated with a higher risk of CRA. CONCLUSION: Vitreoretinal lymphoma presenting with retinal infiltrates and macular involvement carried a higher risk of CRA. Risk factors for CRA should be identified for the potential of severe visual loss. Prompt diagnosis of vitreoretinal lymphoma may allow better control of the disease.

Recurrence of Avellino Corneal Dystrophy Following Penetrating Keratoplasty: A Case Report.

Granular - lattice (Avellino) corneal dystrophy is inherited in an autosomal dominant fashion which affects stroma of the cornea with recurrent erosions and decreased vision due to clouding of cornea in later stage. We reported a case of 53-year old woman presented with pain and blurring of vision of left eye for 10 days with history of right eye deep anterior lamellar dystrophy and Left eye penetrating keratoplasty 5years back for Avellino dystrophy. On examination right eye graft was clear and left eye showed circular edges of irregular epithelium with patchy stains and epithelial defect suggestive of recurrence of dystrophy. A patient with recurrent corneal erosions and opacity in cornea has to be examined carefully so as not to overlook Avellino corneal dystrophy. Being a rare disorder this case has been reported to draw the attention of ophthalmologists about its recurrence following keratoplasty.

Exacerbation of Granular Corneal Dystrophy Type 2 After Small Incision Lenticule Extraction.

PURPOSE: To report the outcome of unilateral small incision lenticule extraction (SMILE) in a patient with granular corneal dystrophy type 2 (GCD2). METHODS: Slit-lamp photography and Fourier domain optical coherence tomography were used to document the clinical course and appearance of the corneas in a patient with genetically determined GCD2 who underwent unilateral SMILE in the right eye. RESULTS: Slit-lamp examination of a 23-year-old woman revealed 2 faint opacities at the surgical interface approximately 2 months after the SMILE procedure had been performed on her right eye. Nine and 3 typical GCD2 deposits located immediately beneath the Bowman layer were observed in the right and left corneas, respectively. Over time, the deposits at the interface increased in size, density, and number in the right eye. Fourier domain optical coherence tomography performed 33 months after the SMILE procedure revealed deposits at the SMILE interface that were distinct from those located immediately beneath the Bowman layer. The severity of disease exacerbation was less in this patient than what is typically observed in others who have undergone laser-assisted in situ keratomileusis or photorefractive keratectomy. CONCLUSIONS: SMILE is contraindicated in patients with GCD2, as are other corneal refractive surgical procedures. This case highlights the importance of genetic testing before the performance of refractive corneal procedures-especially for patients with corneal opacities on preoperative slit-lamp examination or a family history of corneal disease compatible with that of a corneal dystrophy.

Ophthalmic Complications in Pediatric Uveitis.

Purpose: We aim to look at the complications encountered by a cohort of pediatric uveitis patients from north India.Methods: Retrospectively, complications seen in patients younger than 16 years diagnosed with uveitis between January 2006 to March 2015 were noted.Results: Data of 104 children, with a mean follow-up of 3.40 ± 2.34 years was studied. Cataract (n = 42, 24.00%), band-shaped keratopathy (n = 32, 18.29%) and Glaucoma/OHT (n = 11, 6.29%) were most encountered complications at presentation. Glaucoma/OHT (29.71%; n = 52), cataract (18.86%; n = 33) and maculopathy (n=12;6.86%) were the most common complications at follow up. Maculopathy (35%) and Glaucoma/OHT (20%) were the most common causes of visual acuity ≤3/60.Conclusions: Cataract is the most critical complication in children with uveitis at presentation and raised intraocular pressure occurs at follow-up, perhaps attributed to the treatment. Maculopathy is the most common cause of blindness in these children.

Ethylenediaminetetraacetic Acid Chelation in Herpes Zoster Ophthalmicus Is Associated With a High Rate of Corneal Melt and Perforation.

PURPOSE: To examine the rate and risk factors for band keratopathy after herpes zoster ophthalmicus (HZO) and the outcomes of ethylenediaminetetraacetic acid (EDTA) treatment. METHODS: This is a retrospective review of all subjects with HZO seen at Auckland District Health Board between January 2006 and December 2016. RESULTS: A total of 869 subjects with HZO were included in the study. Median follow-up was 6.3 years (total 5504.4 patient-years). Band keratopathy developed in 13 subjects (1.5%). On multivariate analysis, older age at onset [hazard ratio (HR), 1.092; P = 0.034], intraocular pressure ≥30 mm Hg at presentation (HR, 5.548; P = 0.013), and number of recurrences (HR, 1.849; P < 0.001) were associated with increased risk for band keratopathy. Corneal melt occurred in 22 subjects (2.5%) during the follow-up period. On multivariate analysis, uveitis (HR, 8.618; P = 0.004) and disodium EDTA chelation (HR, 8.666; P < 0.001) were associated with increased risk for corneal melt. EDTA chelation was performed in 8 subjects. Corneal melt occurred after EDTA chelation in 4 subjects, and corneal perforation occurred in 2 subjects. One subject was eviscerated due to severe endophthalmitis after repeated corneal perforation and another required enucleation for recurrent corneal melt and microbial keratitis. CONCLUSIONS: Band keratopathy is an uncommon complication of HZO. Treatment with EDTA chelation might be associated with a significant risk for severe complications in these eyes and should be approached with caution.

Collagen Remodeling Plays a Pivotal Role in Endothelial Corneal Dystrophies.

Purpose: To elucidate the collagen structure in the Descemet membrane (DM) of the human cornea and to characterize its rearrangement in patients with endothelial corneal dystrophies. Methods: Corneas from nine human donors and dystrophic DMs removed from 16 affected eyes of 13 patients by endothelial keratoplasty (DMEK) were investigated using a correlative RT-qPCR and label-free two-channel multiphoton microscopy (MPM) setup. Although collagen formation was visualized by second harmonic generation, the cellular structure was determined by autofluorescence. Results: The DM of the human donor cornea was characterized by a consistent pattern of fine hexagonal collagen structures that form a supportive scaffold for the endothelial cells. Accordingly, network-forming collagens (8A1 and 8A2) but less fibrillar collagens (only 1A2) were expressed. DMEK resulted in significant (P < 0.0001) improvement of best-corrected visual acuity. In the removed dystrophic DMs, MPM analyses revealed collagen rearrangement in addition to loss of endothelial cells and the development of guttae. MPM analyses of the whole patient's DM demonstrated this collagen remodeling in its entirety and facilitated correlation to Scheimpflug corneal tomography. In most DMs a unique honeycomb collagen network was identified, with distinct bundles surrounding the guttae and correlating with expression of fibrillar collagens (1A1). Conversely, some DMs showed either reduced collagen on MPM and RT-qPCR analysis or diffuse thickening and storage of extracellular matrix. Conclusions: The collagen structure of the DM and its adaptive remodeling in endothelial corneal dystrophies has been characterized for the first time here and will facilitate individual therapeutic approaches.

Customized Ablation Area PTK as a Technique for Salzmann's Degeneration and Other Focal Stromal Pathologies.

PURPOSE: To introduce a customized ablation area photo-therapeutic keratectomy (PTK) technique that uses a preoperatively generated laser ablation profile to accurately match and ablate the area of the pathology. METHODS: A case of mid-peripheral Salzmann's nodular degeneration causing recurrent epithelial erosion is described. The white-to-white distance, on a slit-lamp image of the patient's eye, was measured by a Placido and dual-Scheimpflug analyzer and used as a scale on a Java-based image processing program to extrapolate the focal corneal pathology's vertical and horizontal dimensions on the corneal plane. The lesion's depth was measured by corneal optical coherence tomography (OCT). Customized ablation area transepithelial PTK, tailored to the exact dimensions of the pathology was then performed in one pass, regionally ablating the area of the pathology only. RESULTS: Complete epithelial healing was observed by the next day with unaltered visual acuity of 20/20. Corneal OCT performed at 1 and 3 months postoperatively showed near-complete resolution of the lesion. The patient was followed up for a total of 6 months with no reported symptoms of corneal erosions. CONCLUSIONS: The application of a customized laser ablation area in the treatment of Salzmann's nodular degeneration and other focal stromal pathologies avoids unnecessary epithelial and stromal ablation. This contributes to faster visual recovery and less refractive impact, especially for peripheral lesions. [J Refract Surg. 2020;36(5):340-344.].

DIFFUSE CHORIORETINAL ATROPHY IN CHINESE HIGH MYOPIA: The ZOC-BHVI High Myopia Cohort Study.

PURPOSE: To explore the associations between diffuse chorioretinal atrophy (DCA) and age, sex, axial length, spherical equivalent, and best-corrected visual acuity (BCVA) among highly myopic eyes. METHODS: This study included right eyes of 857 bilaterally highly myopic individuals from the ZOC-BHVI Cohort Study. Participants underwent examinations, including BCVA, ocular biometry, autorefraction, and color fundus photography. An Early Treatment Diabetic Retinopathy Study grid was applied on the fundus photographs to evaluate the location of DCA, which was graded into four categories (D0-D3). The characteristics and ocular biometry were compared between participants' eyes with and without DCA. RESULTS: Diffuse chorioretinal atrophy was found in 177 (20.6%) eyes. The proportion of participants with DCA in age groups of 7 to 11, 12 to 18, 19 to 39, and ≥ 40 years old was 20.9%, 9.2%, 23.1%, and 52.9%, respectively. The proportion of DCA significantly increased with longer axial length and worse myopic spherical equivalent. Eyes with DCA had poorer BCVA (Snellen visual acuity 20/36, logarithm of minimal angle of resolution 0.26 ± 0.25) than those without DCA (Snellen visual acuity 20/23, logarithm of minimal angle of resolution 0.06 ± 0.14) (P < 0.001). The BCVA gradually declined as the lesion got closer to the fovea (P for trend < 0.001). CONCLUSION: The proportion of DCA increased with older age, longer axial length, and more myopic spherical equivalent. Diffuse chorioretinal atrophy is a vision-threatening complication of high myopia where BCVA gradually worsens with foveal involvement.

Case Report of a 4-Year-Old Child with Complicated Vogt-Koyanagi-Harada at a Tertiary Eye Hospital.

Vogt-Koyanagi-Harada (VKH) disease is a chronic, bilateral, granulomatous panuveitis associated with cutaneous, neurologic, and auditory manifestations. We report a 4-year-old Saudi boy who developed severe ocular complications by 5 years of age. He presented to King Khalid Eye Specialist Hospital at the age of 4 years and was previously operated on elsewhere for cataract with intraocular lens implantation in his right eye at the age of 3 years. He consecutively had iris capture and membrane formation around the intraocular lens. Examination revealed band keratopathy, posterior synechiae, and fundus depigmentation in both eyes with cataract formation in his left eye. At the age of 5.5 years, he developed subretinal neovascular membrane formation in the left eye. To the best of our knowledge, this patient is youngest VKH case that manifested most of the major complications at a young age as 5 years old.

Band keratopathy in children previously treated with diode laser for type 1 retinopathy of prematurity.

Band keratopathy is a corneal degeneration caused by chronic inflammation, systemic abnormalities, or, rarely, a primary biallelic SLC4A4 mutation leading to calcium hydroxyapatite deposition in Bowman's layer. We report a series of 16 eyes of 10 children with a remote history of diode laser treated retinopathy of prematurity who developed late-onset band keratopathy without evidence of other prior risk factors. The majority of patients developed band keratopathy bilaterally. Five eyes had visually significant central band keratopathy that required treatment with disodium ethylenediaminetetracetic acid (EDTA) chelation or phototherapeutic keratectomy. Band keratopathy may be an underreported late ophthalmic complication of diode-laser treated retinopathy of prematurity.

Recurrent corneal erosion syndrome.

Recurrent corneal erosion syndrome (RCES) is a disorder characterised by a dysfunctional epithelial ecosystem. It often begins after trauma, or in the setting of epithelial basement membrane degeneration or dystrophy. Historically, RCES has been understood as a structural derangement of the anterior corneal architecture. More recently, studies have demonstrated the important role of neuropeptides in corneal homoeostasis. Thus, RCES may also be understood as a disorder of corneal epithelial cell biology. Management of RCES can be challenging, but newer therapies have demonstrated improved efficacy for this condition. This review examines the aetiology and pathogenesis of RCES, and provides an update on current and emerging treatment modalities for the management of this disorder.

[Paraproteinemic Keratopathy as a Clinical Sign of Monoclonal Gammopathy]. / Paraproteinämische Keratopathie als klinisches Zeichen einer monoklonalen Gammopathie.

Patients with monoclonal gammopathy can show paraproteinemic keratopathy (PPK) with an indication to treatment. PPK has to be differentiated from corneal dystrophies, systemic metabolic disorders with corneal involvement, as well as from immunologic and inflammatory corneal diseases.

Salzmann's nodular degeneration of cornea associated with thyroid eye disease.

Salzmann's nodular degeneration (SND) typically occurs in patients who are female, 50-60 years old, and have a history of corneal inflammation and irritation. Multiple case reports have documented associations between SND and trachoma, viral infections, trauma, contact lens wear, corneal surgeries and corneal exposure. The authors describe a patient with bilateral SND confirmed by anterior segment optical coherence tomography (OCT) imaging in the context of thyroid eye disease (TED) and history of LASIK. Treatment involved propylthiouracil (PTU), artificial tear use, loteprednol etabonate ophthalmic gel, eyelid taping and selenium supplementation and prospective superficial keratectomy with diamond burr polish.