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Corneal dystrophies may develop gradually from early onset in childhood and persist for years to decades. Visual impairment or repeated ocular irritations caused by corneal dystrophies severely affect the quality of life. Although various surgical treatment options are indicated for adult patients, the treatment plan for pediatric patients remains unclear. Herein we describe clinical observations of phototherapeutic keratectomy for corneal epithelial-stromal dystrophies in three pediatric patients (five eyes). Corneal opacities were successfully removed, and visual acuity was greatly improved in all operated eyes. The procedure of phototherapeutic keratectomy seems to be feasible in the treatment of corneal epithelial-stromal dystrophies in c hildren, with advantages of minimal invasion and good visual outcomes., Competing Interests: Declarations. Conflict of interest: The authors have no conflicts of interest to declare., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

In May 2023, a 36-year-old carpenter complained of a sudden decrease in visual acuity in both eyes after his fourth COVID-19 vaccination. He underwent extensive evaluation by ophthalmological, neurological, and internal medicine specialists elsewhere, which was unremarkable, except for a computed tomography scan of his brain showing minor occipital calcifications. In 2021, he had been diagnosed with anterior basement membrane dystrophy and treated with phototherapeutic keratectomy (PTK) of the left eye, leading to significant postoperative haze. On referral in July 2023, slitlamp examination showed significant anterior basement membrane dystrophy in the right eye, whereas the left eye had an extensive central scar in the anterior stroma, measuring up to 6 mm in width and 140 µm in depth (Figures 1-3JOURNAL/jcrs/04.03/02158034-202410000-00017/figure1/v/2024-09-18T130724Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202410000-00017/figure2/v/2024-09-18T130724Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202410000-00017/figure3/v/2024-09-18T130724Z/r/image-tiff). His corrected distance visual acuity was 20/80 in his right eye and 20/200 in his left eye, with a manifest refraction of -0.50 -3.75 × 170 and +0.00 -4.75 × 180, respectively. Corneal Scheimpflug topography showed regular corneal astigmatism of 3.3 diopters (D) and 5.5 D in the right and left eyes, respectively, with a corneal thickness of 550 and 566 µm (Figure 4JOURNAL/jcrs/04.03/02158034-202410000-00017/figure4/v/2024-09-18T130724Z/r/image-tiff). The Schirmer tear test was 20-20 mm. Fundoscopy, electrophysiological testing, and retinal optical coherence tomography (OCT) showed no abnormalities. The patient mentioned he had Crohn disease, managed with ustekinumab (a monoclonal antibody inhibitor of cytokines interleukin [IL]-12 and IL-23), but he had discontinued it because of the apparent remission of the disease. Attempts to improve visual acuity involved a soft bandage contact lens in the right eye, which was discontinued after 2 days because of the occurrence of a small corneal infiltrate that healed with a short course of topical antibiotics. Unfortunately, because of his vision, he cannot perform his tasks as a carpenter anymore. Which is your recommended treatment for both eyes?, (Copyright © 2024 Published by Wolters Kluwer on behalf of ASCRS and ESCRS.)

Purpose: To evaluate the recurrence characteristics on optical coherence tomography and clinical outcomes after phototherapeutic keratectomy (PTK) or penetrating keratoplasty (PKP) in patients with Reis-Bücklers corneal dystrophy (RBCD)., Design: Retrospective interventional case series., Methods: Seventeen patients with RBCD (31 eyes, including 6 surgery-naïve eyes and 25 surgical eyes) received 44 surgical interventions from 1996 through 2022. PTK or PKP was performed as the initial surgical procedure. Significant recurrence was determined when best spectacle-corrected visual acuity decreased at least 2 lines with increased opacity in the superficial cornea. Repeated PTK or PTK on the corneal graft (CG-PTK) was considered if patients could not endure poor vision due to significant recurrence. Recurrence depth and annual increase in thickness of the central cornea and subepithelial deposits were assessed by anterior segment optical coherence tomography., Results: The mean follow-up time was 12.8 ± 8.5 years (range, 2.0-25.5 years). The mean logMAR best spectacle-corrected visual acuity improved from 1.24 ± 0.48 preoperatively to 0.27 ± 0.09 postoperatively in the initial PTK group (13 eyes, P < .001), from 1.84 ± 0.69 to 0.40 ± 0.13 in the PKP group (12 eyes, P < .001), from 1.04 ± 0.46 to 0.30 ± 0.07 in the repeated PTK group (12 times in 7 eyes, P < .001), and from 1.29 ± 0.43 to 0.39 ± 0.11 in the CG-PTK group (7 times in 5 eyes, P = .001). The median significant recurrence time was 27 months (95% confidence interval 23.9-30.1), 96 months (84.1-107.9), 31 months (28.8-33.1), and 24 months (19.8-28.2), respectively (P < .001). The depth of superficial deposits located between the epithelium and the anterior stroma was approximately 115 µm (85-159 µm). The annual thickening of subepithelial deposits was 14 ± 2 µm after initial PTK, 7 ± 3 µm after PKP, 14 ± 3 µm after repeated PTK, and 30 ± 11 µm after CG-PTK, compared to 4 ± 2 µm in surgery-naïve eyes (P = .002, .515, .002, <.001). The thickness of the central cornea increased by 15 ± 2 µm, 7 ± 2 µm, 15 ± 3 µm, and 31 ± 10 µm per year in the 4 surgery groups, respectively, compared to 5 ± 2 µm in surgery-naïve eyes (P = .001, .469, .001, <.001)., Conclusions: Better visual acuity can be achieved after PTK than PKP for treatment of RBCD. The annual thickening of subepithelial deposits may approximate an increase in central corneal thickness. The superficial distribution of subepithelial deposits makes it feasible to perform repeated PTK, even on the corneal allograft, for recurrent RBCD., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: To evaluate the efficacy and safety of trans-epithelial phototherapeutic keratectomy (TE-PTK) as a treatment for recurrent corneal erosion syndrome (RCES) in patients with symptoms refractory to conventional treatments., Methods: All patients who received TE-PTK treatment for RCES had failed 3 or more conventional treatments and were reviewed, and if met criteria, approved by healthcare workers of the British Columbia public health authority (Medical Services Plan (MSP). A retrospective chart review and telephone survey were conducted at the Pacific Laser Eye Centre (PLEC). Exclusion criteria were ocular co-morbidities potentially affecting treatment efficacy., Results: This study included 593 eyes of 555 patients (46.2% male; 50.9 ± 14.2 years old) who underwent TE-PTK. The leading identified causes of RCES were trauma (45.7%) and anterior basement membrane dystrophy (44.2%). The most common pre-PTK interventions were ocular lubricants (90.9%), hypertonic solutions (77.9%), and bandage contact lenses (50.9%). Thirty-six eyes had undergone surgical interventions such as stromal puncture, epithelial debridement, or diamond burr polishing. Post-PTK, 78% of patients did not require any subsequent therapies and 20% required ongoing drops. Six patients (1.1%) reported no symptom improvement and required repeat TE-PTK for ongoing RCES symptoms after initial TE-PTK. All 6 eyes were successfully retreated with TE-PTK (average time to retreatment was 11.3 ± 14.9 months). There was no significant difference in best corrected visual acuity pre- vs. post-operatively. The mean post-operative follow-up was 60.5 months (range: 5-127 months)., Conclusion: TE-PTK has a good efficacy and safety profile for treatment-resistant RCES. The third-party public health-reviewed nature of this study, the low recurrence rate of RCES, and the low PTK retreatment rate suggest that TE-PTK might be considered for wider use in the management of RCES., (© 2024. The Author(s).)

Purpose: To report a case of severe verrucous posterior polymorphous corneal dystrophy (PPCD) and cataract, which was treated with Descemet membrane endothelial keratoplasty (DMEK) and simultaneous cataract surgery as a triple procedure (Triple-DMEK)., Methods: A 62-year-old female patient presented to our department for co-evaluation of advanced PPCD with cataract and progressive light sensitivity in both eyes. The clinical examination demonstrated unusual clinical findings with prominent verrucous lesions on the posterior surface of the cornea without corneal decompensation. We performed a Triple-DMEK in case of simultaneous cataract. The corneal tissue was examined by light and transmission electron microscopy., Results: Intraoperatively, it was difficult to remove the verrucous structures completely after classical descemetorhexis. Light microscopic examination demonstrated epithelium-like transformation of the corneal endothelium by immunostaining (cytokeratin AE1/3 staining). Transmission electron microscopy revealed thickening of Descemet's membrane (18.5 to 30.0 µm). The anterior banded layer had a normal structure and was slightly thickened (3.5 to 5.5 µm). A normal posterior non-banded layer (PNBL) was observed but thinned (2.5 to 4.0 µm) or missing. It was followed by an altered PNBL with abnormal fibrillary inclusions, which was strongly and variably thickened (11.0 to 24.5 µm). The corneal endothelium was degenerated, partially absent, and epithelial-like altered. The nodular lesions were found to consist of a few degenerated cells that were embedded in an amorphous extracellular matrix interspersed with collagen fibers, which were not arranged in regular lamellae, forming the corneal stroma. The occurrence of pigment granules among the cellular debris suggested that the cells were endothelial cells. The corrected distance visual acuity improved from 20/50 to 20/30 in the right eye (+ 0.00/- 1.75/157°) and from 20/60 to 20/30 in the left eye (+ 0.00/- 1.75/33°), with significant improvement in light sensitivity., Conclusion: The clinical and ultrastructural findings seem to be an unusual variant of the typical characteristic appearance of a PPCD. This case demonstrates that Triple-DMEK is feasible even in very advanced dystrophic changes of the posterior corneal surface, with good morphological and functional results., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Rationale: There are reports of safe cataract surgery in eyes with posterior polymorphous corneal dystrophy (PPCD); however, to our knowledge, there are no reports of minimally invasive glaucoma surgery (MIGS) in eyes with PPCD. Herein, we report a case of poor intraoperative visibility with gonioscopy, postoperative corneal edema, and corneal astigmatism in eyes with PPCD treated with trabecular micro-bypass stent combined with cataract surgery., Patient Concerns/diagnosis: A 78-year-old man was referred to our hospital for MIGS. He presented with bilateral corneal endothelial vesicular changes and band lesions. Endothelial cell density was 2983/2871 cells/mm2 (right/left eye), central corneal thickness was 581 μm/572 μm, best-corrected visual acuity values (Snellen equivalent) were 20/32 (right) and 20/100 (left), and corneal astigmatism was -2.7D in the right eye and -2.5D in the left eye., Intervensions/ Outcoms: After phacoemulsification and aspiration with intraocular lens implantation with a 2.4-mm corneal incision in both eyes, trabecular micro-bypass stents were inserted successfully despite the poor intraoperative visibility with gonioscopy. One week after surgery, the central corneal thickness was 614 μm/609 μm, and Descemet's membrane folds and mild corneal edema were observed. Best-corrected decimal visual acuity was 20/40 for the right eye and 20/50 for the left eye. In the left eye, total corneal astigmatism increased from -2.5D to -5.5D. Corneal astigmatism and edema showed gradual improvement., Lessons: Although reports have shown that cataract surgery can be safely performed in eyes with PPCD, MIGS in eyes with PPCD may require caution regarding intraoperative visibility with gonioscopy and visual function in the early postoperative period., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: To describe the outcomes of five Salzmann's Nodular Degeneration (SND) cases treated with a combination of alcohol delamination, superficial keratectomy and amniotic membrane patch with fibrin glue., Methods: Five patients affected by SND with significant discomfort and decreased visual acuity were evaluated with refraction, best corrected visual acuity, Break-up time test, corneal topography, Anterior Segment of Optical Coherence Tomography and images of anterior segment with a full slit lamp examination. All the patients underwent alcohol delamination of the corneal epithelium followed by superficial keratectomy to remove the subepithelial nodules. A patch of amniotic membrane was applied to all of them with a ring shape and a banana shape in two patients due to the location of the disease. A bandage contact lens was inserted. The patch was kept in place for 14 days. Another bandage contact lens was inserted for 21 days until the corneal epithelium had completely healed., Results: The surgical procedure was successful in removing the nodules in all patients. Vision was restored in all cases with a significant regularization of the corneal surface and reduction of ocular discomfort. After a 12-month follow-up, none of the patients showed recurrence of the disease., Conclusions: Combining alcohol delamination with superficial keratectomy and amniotic membrane patch with fibrin glue is an effective procedure for the management of SND to regularize the corneal surface and avoid the recurrence of subsequent nodules. This report is the first description of the outcomes of SND cases treated with a combination of the aforementioned techniques., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: This study aimed to describe the optical and topographic changes after manual superficial keratectomy (MSK) for Salzmann nodular degeneration., Methods: This was a descriptive, retrospective study. All patients with a clinical diagnosis of Salzmann nodular degeneration were examined at the Cornea Service of the Instituto Clínico Quirúrgico de Oftalmología (ICQO), Bilbao, Spain, and treated with MSK after presenting ocular discomfort and/or decreased visual acuity. The clinical characteristics (including anterior segment optical coherence tomography and Pentacam topography), treatment regimens, surgical procedures, and outcomes were recorded. Descriptive statistics were constructed using mean ± SD, minimum, maximum, and median. The Shapiro-Wilk normality test was used. The Student t test was used to determine significance., Results: Ten eyes of 9 patients were included in this study: 6 patients (66.6%) were female and 3 were male (33.3%). The mean age was 62.2 years, and the follow-up time was between 5 and 21 months. The best-corrected visual acuity before MSK was 0.20 LogMAR (median) and improved to 0.10 after the surgical procedure. The mean spherical equivalent was reduced from -0.23 ± 3.39 D preoperatively to -1.3 ± 3.0 D postoperatively. Astigmatism decreased between 0.5 and 3.75 D. Topographic irregularity normalized total root mean square from 11,596.4 ± 6854.01 to 4817.2 ± 2725.68 μm., Conclusions: MSK is an effective and safe technique for the treatment of Salzmann nodular degeneration when the Bowman layer is preserved. Anterior segment optical coherence tomography and corneal topography are essential tools for the surgical plan and for the detection of corneal aberrations., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To determine the clinical outcomes of keratorefractive lenticule extraction (KLEx) in eyes with epithelial basement membrane dystrophy (EBMD) that developed after surgery., Setting: Onnuri Smile Eye Clinic, Seoul, South Korea., Design: Retrospective single-center study., Methods: This study reviewed the medical records of 26 eyes of 16 patients who exhibited signs of EBMD after KLEx. Postoperatively, corneal findings and visual outcomes were evaluated. Statistical analyses were also performed on eyes without corneal complications after KLEx., Results: Signs of EBMD were first observed within 1 week postoperatively in 22 eyes (84.6%): 6 eyes (23.1%) showed epithelial defects, 10 eyes (38.5%) showed subepithelial corneal opacity, 5 eyes (19.2%) showed diffuse lamellar keratitis, and 1 eye (3.9%) showed epithelial ingrowth. Symptoms such as pain, while corneal lesions were present, occurred in 21 eyes (80.8%). At 3 months postoperatively, 21 eyes (80.8%) showed no specific findings on slitlamp microscopy after medical treatment, 2 eyes (7.7%) showed subepithelial corneal opacity, 2 eyes (7.7%) showed corneal scar, and 1 eye (3.9%) showed epithelial ingrowth. Uncorrected distance visual acuity was 20/25 or better in 24 eyes (92.3%), and spherical equivalent was within ±1.0 D in 20 eyes (76.9%). The efficacy index of the EBMD group did not significantly differ from the control group, while the safety index was significantly lower., Conclusions: Manifestations of EBMD can occur after KLEx and can affect clinical outcomes. Most cases showed favorable results with appropriate nonsurgical treatment; however, some cases had long-term complications such as corneal scarring., (Copyright © 2024 Published by Wolters Kluwer on behalf of ASCRS and ESCRS.)

Purpose: The purpose of this study was to highlight characteristic clinical and microscopic findings and report the long-term follow-up of pediatric excimer laser-assisted penetrating keratoplasty (excimer-PKP) for congenital stromal corneal dystrophy (CSCD)., Methods: A 2-year-old Greek child presented with CSCD at our department. Clinical examination showed bilateral flake-like whitish corneal opacities affecting the entire corneal stroma up to the limbus. Genetic testing identified a mutation of the decorin gene (c.962delA). The variant was not present in the parents and represented a de novo mutation. The uncorrected visual acuity was 20/100 in both eyes. Excimer-PKP (8.0/8.1 mm) was performed on the right eye at the age of 2.5 years and on the left eye at the age of 3 years. Postoperatively, alternating occlusion treatment was performed., Results: The light microscopic examination demonstrated a disorganized extracellular matrix of the corneal stroma characterized by a prominent irregular arrangement of stromal collagen lamellae with large interlamellar clefts containing ground substance, highlighted by periodic acid-Schiff- and Alcian blue-positive reaction detecting acid mucopolysaccharides. Electron microscopy showed disorganization and caliber variation of collagen lamellae and thin filaments within an electron-lucent ground substance. The postoperative course was unremarkable. Both grafts remained completely clear 14 years postoperatively. Corneal tomography showed moderate regular astigmatism with normal corneal thickness. The corrected distance visual acuity was 20/25 in both eyes., Conclusions: Excimer-PKP for CSCD might be associated with excellent long-term results and a good prognosis, particularly when the primary surgery is performed at a very young age. However, this requires close postoperative follow-up examinations by an experienced pediatric ophthalmologist to avoid severe amblyopia., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

A young a presented with painless, progressive diminution of vision in both eyes (BE). Slit lamp examination revealed the presence of a single central corneal opacity in the right eye and multiple corneal opacities of varying sizes in the left eye (LE), limited to the anterior-mid corneal stroma. Microcornea with reduced central corneal thickness and complete inferonasal iris coloboma along with inferior fundal coloboma, sparing both the disc and macula, were noted in BE. A diagnosis of BE macular corneal dystrophy (MCD) and iridofundal coloboma (IFC) was made. The patient underwent LE sutureless anterior lamellar therapeutic keratoplasty. On histopathological examination, the excised corneal tissue revealed stromal lamellar disarray with positive colloidal iron staining, strongly suggestive of MCD. Whole-exome sequencing revealed the presence of a likely pathogenic carbohydrate sulfotransferase 6 ( CHST6 ) mutation, confirming the diagnosis of MCD. This concurrent presence of IFC with a corneal stromal dystrophy is previously unreported in the literature, to the best of our knowledge., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe four Finnish families with epithelial recurrent erosion dystrophy (ERED) caused by the pathogenic variant c.3156C>T in collagen type XVII alpha 1 chain gene (COL17A1)., Methods: Eleven affected and two unaffected individuals underwent clinical ophthalmological examination, anterior segment photography, and corneal topography. Two of them underwent phototherapeutic keratectomy (PTK). Genetic analysis included both next-generation and Sanger sequencing. Specimens from the manual keratectomy of one patient were available for ophthalmic pathologic examination, including immunohistochemistry., Results: The common splice-site altering synonymous variant c.3156C > T, p.(Gly1052=) in COL17A1 was confirmed in 15 individuals with ERED from the four families. Subepithelial corneal scarring grades varied and increased with age, leading to decreased best-corrected visual acuity. PTK improved vision in 58- and 67-year-old individuals without reactivating the disease. The keratectomy specimens showed an uneven epithelium and a spectrum of basement membrane abnormalities, including breaks, fragmentation, multiplication and entrapment within the subepithelial scar, reflecting recurrent erosions. The stromal cells consisted of varying proportions of bland and activated fibroblasts and myofibroblasts, reflecting different ages of scars. The family with the largest number of known affected generations originated from Southern Sweden., Conclusion: The phenotype in the Finnish ERED families is consistent with earlier reports of the c.3156C > T variant, although the severity has varied between reports. The phenotype may be modulated by other genes. This study suggests a likely founder effect of the variant in both Finnish and Swedish populations due to their shared population histories. If vision is compromised, PTK can be considered especially in older patients., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: This study aimed to report cases of bilateral corneal Bowman layer deposits in 4 patients with a history of keratorefractive surgery. To our knowledge, this condition has not previously been reported and should be distinguished from granular corneal dystrophy type 2 and other corneal dystrophies., Methods: We reviewed all available medical records that were collected between January 2010 and December 2021 at a tertiary referral center and performed whole-exome sequencing to provide diagnostic information., Results: Four patients exhibited similar bilateral corneal deposits that were observed more than 10 years after keratorefractive surgery. The patients' ages ranged from 36 to 53 years; 3 of the 4 patients were female. Three patients received laser in situ keratomileusis surgery, and 1 received radial keratotomy. All 4 patients denied having a family history of ocular diseases and reported an uneventful postoperative course. On examination, the best-corrected visual acuity ranged from 6/10 to 6/6 in all 4 patients. Slit-lamp examination revealed bilateral superficial corneal deposits involving the central cornea, and anterior segment optical coherence tomography revealed hyperreflective deposits located in the Bowman layer. Such unique manifestations suggested corneal dystrophy; thus, whole-exome sequencing was performed on all 4 patients. Only 1 patient exhibited a missense mutation in TGFBI . We further analyzed common de novo mutations to explore possible candidate genes associated with this presentation., Conclusions: We report a rare entity of presumed corneal dystrophy with deposits located in the Bowman layer in 4 patients who had received keratorefractive surgery. Clarifying the underlying pathophysiology and genetic predisposition of this disease may aid in diagnosing and preventing potential complications after keratorefractive surgery., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purposes: This study aims to assess and compare the postoperative visual and topographic outcomes, complications, and graft survival rates following deep anterior lamellar keratoplasty and penetrating keratoplasty in patients with macular corneal dystrophy., Methods: In this study we enrolled 59 patients (23 male; and 36 female) with macular corneal dystrophy comprising 81 eyes. Out of these, 64 eyes underwent penetrating keratoplasty, while 17 eyes underwent deep anterior lamellar keratoplasty. The two groups were analyzed and compared based on best-corrected visual acuity, corneal tomography parameters, pachymetry, complication rates, and graft survival rates., Results: After 12 months, 70.6% of the patients who underwent deep anterior lamellar keratoplasty (DALK) and 75% of those who had penetrating keratoplasty (PK) achieved a best-corrected visual acuity of 20/40 or better (p=0.712). Following surgery, DALK group showed lower front Kmean (p=0.037), and Q values (p<0.01) compared to the PK group. Postoperative interface opacity was observed in seven eyes (41.2%) in the DALK group. Other topography values and other complications (graft rejection, graft failure, cataract, glaucoma, microbial keratitis, optic atrophy) did not show significant differences between the two groups. The need for regrafting was 9.4% and 11.8% in the PK and DALK groups, respectively (p=0.769). Graft survival rates were 87.5% and 88.2% for PK and DALK; respectively (p=0.88 by Log-rank test)., Conclusion: Both PK and DALK are equally effective in treating macular corneal dystrophy, showing similar visual, topographic, and survival outcomes. Although interface opacity occurs more frequently after DALK the visual results were comparable in both groups. Therefore, DALK emerges as a viable surgical choice for patients with macular corneal dystrophy without Descemet membrane involvement is absent.

Background: Familial calcific band-shaped keratopathy (BSK) is a very rare disease, with no underlying cause. There is no underlying disease in this form of the disease. This article introduces a family with seven children, three of whom were diagnosed with familial primary calcific BSK. One of them developed a systemic disease 38 years after ocular manifestation., Case Presentation: In this case report, three Iranian siblings from a family with familial calcific band-shaped keratopathy (BSK) are introduced. Systemic and ocular examinations performed on these patients indicated the occurrence of chronic kidney disease in the older child, a 41-year-old woman, 38 years after ocular manifestation. The examinations conducted on the other two siblings revealed no pathological findings. The 41-year-old sister and 37-year-old brother underwent unilateral deep anterior lamellar keratoplasty (DALK), while the 33-year-old sister underwent bilateral superficial keratectomy (SK)., Conclusion: Considering the late onset of systemic disease in one of the siblings diagnosed with familial calcific band-shaped keratopathy (BSK), it is crucial to emphasize the necessity of long-term follow-up for these patients and their families., (© 2024. The Author(s).)

Purpose: To describe a case of a patient treated for neurotrophic keratopathy (NK) with direct corneal neurotization (CN), where a modification to the CN technique allowed for semiscleral contact lens use postoperatively., Observation: Our patient had successful CN with improved corneal sensation. During the procedure, a 1.0 mm gutter was created between the limbus and nerve graft to allow for semiscleral contact lens fitting., Conclusions: With the use of preoperative planning and a limbal gutter during CN, a semiscleral contact lens can serve as a well-tolerated postoperative management option to improve visual acuity and protect the corneal surface in patients with NK., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Contact Lens Association of Ophthalmologists.)

Background: Endothelial Keratoplasty (EK) is now considered as the standard treatment for Congenital Hereditary Endothelial Dystrophy (CHED) by many surgeons. We present the 12-year clinical outcome of the youngest operated patient with CHED in which we successfully performed a bilateral EK procedure without removing the recipient endothelium-Descemet complex., Case Presentation: In November 2010 we performed EK without Descemet Stripping in a 3-month female newborn, thinking that the lower manipulation obtained by leaving the recipient endothelium-Descemet complex could be the key factor for the success of our surgery. Such a particular technique was new in newborns. The surgery was a success, but the long-term visual result was not predictable at that time. We followed the patient at 4 months, and then yearly. At the latest visit in October 2022 the visual, cognitive, and motorial developments were normal, with Best-corrected Distance Visual Acuity of 0.4 LogMAR with - 0.75 D sf + 2.75 D cyl @ 105° in the right eye (RE) and 0.4 LogMAR with + 1.50 D sf + 2.50 D cyl @ 60° in the left eye (LE). The endothelial microscope showed an unexpected healthy endothelium, with a cell count of 2383 cells/mm 2 in the RE and of 2547 cells/mm 2 in the LE from a starting donor count of 2900 cells/mm 2 . No secondary procedures were performed during the 12-year follow-up., Conclusion: EK without Descemet stripping has proved to be a successful procedure over time in our newborn. The unexpected healthy endothelium suggests a role of the Descemet membrane in CHED., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Purpose: To report indications and clinical outcomes of corneal grafts ≤ 5.5 mm in diameter ("mini-KP") in a German tertiary referral center., Methods: Patients who had undergone mini-KP to treat corneal ulcers with or without perforation between 2011 and 2018 at the Department of Ophthalmology, University of Düsseldorf, Germany, were identified from the local keratoplasty registry. All patient records were reviewed for age, gender, laterality, systemic and ophthalmological diseases, etiology of the corneal ulcerative disease, pre- and postoperative visual acuity over a follow-up time of up to 12 months, graft size, postoperative complications and the need for and timing of further corneal interventions., Results: 37 eyes of 37 patients (male: n = 20; female: n = 17) with a mean age (± standard deviation) at presentation of 70 ± 18.8 years (range: 22-92 years) were identified. Most common etiologies were neurotrophic keratopathy (n = 15), dysfunctional tear syndrome (n = 9) and atopic keratoconjunctivitis (9). Mean graft diameter was 4.51 ± 0.63 mm (range: 3-5.5 mm). 23/37 eyes (62%) required no further intervention in the acute phase. 14/37 patients (38%) required secondary corneal intervention, due to complications. One-year graft survival was 78.4%. One eye had to be eviscerated due to recurrent corneal ulceration and endophthalmitis. 36 of 37 eyes were preserved. We found a highly significant correlation between type 2 diabetes and the development of postoperative complications (r = .46; p = .005). Corrected distance visual acuity (CDVA) improved from 1.42 ± 0.75 logMAR to 0.9 ± 0.65 logMAR postoperatively (t (23) = 5.76; p < .001)., Conclusion: Mini-KP can be used successfully in eyes with advanced corneal ulcers due to various infectious and noninfectious etiologies to restore tectonic stability in the long-term and with moderate visual gains., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Purpose: The aim of this study was to define, following the IC3D template format, the clinical and histopathologic phenotype of the p.(His626Arg) missense variant lattice corneal dystrophy (LCDV-H626R), the most common variant lattice dystrophy, and to record long-term outcome of corneal transplantation in this dystrophy., Methods: A database search and a meta-analysis of published data on LCDV-H626R were conducted. A patient diagnosed with LCDV-H626R who underwent bilateral lamellar keratoplasty followed by rekeratoplasty of 1 eye is described, including histopathologic examination of the 3 keratoplasty specimens., Results: One hundred forty-five patients from at least 61 families and 11 countries diagnosed with LCDV-H626R were found. This dystrophy is characterized by recurrent erosions, asymmetric progression, and thick lattice lines that extend to corneal periphery. The median age is 37 (range, 25-59) years at the onset of symptoms, 45 (range, 26-62) years at the time of diagnosis, and 50 (range, 41-78) years at the time of the first keratoplasty, suggesting a median interval from the first symptoms to diagnosis and to keratoplasty of 7 and 12 years, respectively. Clinically unaffected carriers have been of age 6 to 45 years. Central anterior stromal haze and centrally thick, peripherally thinner branching lattice lines in the anterior to midstroma of the cornea were noted preoperatively. Histopathology of the host anterior corneal lamella showed a subepithelial fibrous pannus, a destroyed Bowman layer, and amyloid deposits extending to the deep stroma. In the rekeratoplasty specimen, amyloid localized to scarring along the Bowman membrane and to the margins of the graft., Conclusions: The IC3D-type template for LCDV-H626R should help diagnose and manage variant carriers. The histopathologic spectrum of findings is broader and more nuanced than what has been reported., Competing Interests: T.T.K. received lecture fees from Santen Finland outside submitted work. A.M.J. declares no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To determine the effect of the formulation of topical medications on the healing of corneal epithelial cells after phototherapeutic keratectomy (PTK)., Study Design: Retrospective cohort study., Methods: We studied 271 eyes of 189 consecutive patients (aged 67.6 ± 11.8 years) who had undergone PTK for granular corneal dystrophy (n = 140), band keratopathy (n = 47), or lattice corneal dystrophy (n = 2). Postoperatively, generic or brand-named levofloxacin, 0.1% betamethasone, or 0.1% bromfenac sodium hydrate was applied topically. Patients were examined on postoperative days 1, 2, and 5 and weekly thereafter. The time to re-epithelialization was assessed by use of Kaplan-Meier and Cox proportional hazards analyses., Results: The time to re-epithelialization was significantly longer with generic 0.5% levofloxacin, at 8.2 ± 3.5 days, than with 0.5% Cravit (levofloxacin), at 6.7 ± 3.5 days (P = 0.018), or with 1.5% Cravit, at 6.3 ± 2.6 days (P = 0.000). In addition, the time to re-epithelialization was significantly longer with generic 0.1% betamethasone (Sanbetason), at 7.3 ± 3.4 days, than with brand-name 0.1% betamethasone (Rinderon), at 6.1 ± 2.5 days (P = 0.0002). The Cox proportional hazards model indicated that the use of generic formulations for levofloxacin eye drops and 0.1% betamethasone was a significant factor that delayed corneal re-epithelialization (hazard ratio [HR] = 0.72, P = 0.002 and HR = 0.77, P = 0.006, after adjustment for age). Re-epithelialization was significantly shorter in band keratopathy than in corneal dystrophy (HR = 1.56, P = 0.004). No other factors, including age, bandage contact lens, and diabetes mellitus, were significantly associated with time to re-epithelialization., Conclusion: Corneal epithelial healing can be significantly affected by different antibacterial or steroid eye drops. Clinicians need to be aware that a generic formulation may affect corneal epithelial healing., (© 2023. Japanese Ophthalmological Society.)

A male patient in his 30s, with a history of bilateral microkeratome-assisted myopic laser-assisted in situ keratomileusis (LASIK) 8 years ago at another centre, presented to us with gradually progressive diminution of vision and glare in both eyes for the last 4 years. On presentation, uncorrected distance visual acuity (UDVA) was 6/24 and 6/15 in the right eye and left eye, respectively, with normal intraocular pressures. Slit-lamp examination and anterior segment optical coherence tomography revealed well-defined white deposits, limited to an area within the LASIK flap. The deposits were confluent, at the level of the LASIK flap interface, and few discrete opacities were present in the posterior stroma. His father also had a similar clinical picture in both eyes. A diagnosis of both eyes post-LASIK exacerbation of granular corneal dystrophy with epithelial ingrowth was made. He underwent right eye femtosecond laser-assisted sutureless superficial anterior lamellar keratoplasty. At 6-month follow-up, UDVA improved to 6/12 with graft clarity of 4+ and coexistent grade 1 epithelial ingrowth., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To compare outcomes of phototherapeutic keratectomy (PTK) versus epithelial debridement combined with anterior stromal puncture (ASP) or diamond burr for the treatment of recurrent corneal erosions (RCES) in a large tertiary centre., Methods: Patients with a diagnosis of RCES secondary to trauma or epithelial basement membrane dystrophy who underwent a surgical procedure between 2009 and 2019 were included in the study. The following data were collected: demographics, ocular history, prior medical treatment, ocular surgeries, intervention, complete epithelialization at postoperative week 1, recurrences, and complications. Recurrence was defined as either an objective finding of a corneal epithelial defect or symptoms suggestive of recurrent epithelial erosion on history. Recurrence rate and time to epithelialization were compared between groups., Results: A total of 97 eyes (73 patients) were included in the study. Mean patient age was 51 ± 16.1 years, and mean follow-up was 474 days. RCES was secondary to epithelial basement membrane dystrophy in 80% (n = 78 of 97), trauma (15%, n = 15 of 97), or idiopathic (4%, n = 4 of 97). Epithelial debridement with ASP was performed in 34 eyes (35%), diamond burr in 33 eyes (33%), and PTK in 30 eyes (31%). Compared with epithelial debridement with ASP (recurrence 29.4%), the recurrence rate was significantly lower for both the diamond burr (9.1%, p = 0.031) and PTK groups (10%, p = 0.048). The diamond burr and PTK groups also had a significantly higher rate of complete epithelialization at 1 week (p < 0.05)., Conclusion: Compared with epithelial debridement with ASP, diamond burr and PTK have significantly lower rates of recurrence and time to epithelialization and may be considered first for surgical management of RCES., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the effectiveness of sequential custom phototherapeutic keratectomy (SCTK) for granular corneal dystrophy type 1 (GCD1)., Methods: Thirty-seven eyes of 21 patients with GCD1 were treated with SCTK to remove superficial opacifications, regularize the corneal surface, and decrease optical aberrations. SCTK is a sequence of custom therapeutic excimer laser keratectomies with step-by-step intraoperative corneal topography monitoring of results. Six eyes of 5 patients previously treated with penetrating keratoplasty received SCTK for disease recurrence. Pre-operative and postoperative corrected distance visual acuity (CDVA), refractive values, mean pupillary keratometry, and pachymetry were retrospectively analyzed. The mean follow-up period was 41.3 months., Results: SCTK provided significant decimal CDVA improvement, from 0.33 ± 0.22 to 0.63 ± 0.24 ( P < .0001) at the last available follow-up visit. One eye, initially treated with penetrating keratoplasty, showed visually significant disease 8 years after the first SCTK and was re-treated. Mean corneal pachymetry difference between preoperative and final follow-up values was 78.42 ± 62.26 µm. Mean corneal curvature and the spherical component did not show a statistically significant change or hyperopic shift. Astigmatism and higher order aberration reduction were statistically significant., Conclusions: SCTK is a powerful tool for the treatment of anterior corneal pathologies hindering vision and quality of life, such as GCD1. SCTK is less invasive and fosters more rapid visual recovery than penetrating keratoplasty or deep anterior lamellar keratoplasty. Providing significant visual improvement, SCTK can be the preferred initial treatment in eyes with GCD1. [ J Refract Surg . 2023;39(6):422-429.] .

Purpose: To evaluate the effectiveness of repeat deep anterior lamellar keratoplasty (DALK) in patients of previous failed DALK., Methods: : A retrospective analysis of records of seven patients who had undergone repeat DALK following the failure of the primary DALK was done. The indications for repeat surgery, time elapsed since the first surgery, and pre- & postoperative best-corrected visual acuity (BCVA) were noted for all the patients., Results: The follow-up period ranged between one- to four-year post repeat DALK. The indication of primary DALK was keratoconus with vernal keratoconjunctivitis (VKC) (n = 3), corneal amyloidosis (n = 2), Salzman nodular keratopathy (n = 1), and healed keratitis (n = 1). The need for repeat surgery arose when the BSCVA dropped to less than 20/200. The time interval elapsed since the first surgery ranged from two months to four years. Postoperatively, the BSCVA improved from 20/120 to 20/30 at the end of one-year post repeat DALK in all except one patient. All regrafts were clear at the most recent examination, performed after a mean period of 18 months after the secondary graft. No complication was encountered during the resurgery. The dissection of the host bed was easier in the second surgery owing to weaker adhesions., Conclusion: The prognosis for repeat DALK for failed DALK is excellent, and the outcomes of secondary grafts were comparable to those of primary DALK grafts. Re DALK offers the advantage of an easier dissection and lower chances of graft rejection compared to penetrating keratoplasty., Competing Interests: None

Purpose: The aim of this study was to evaluate the etiology-specific clinical outcomes and complications of repeat deep anterior lamellar keratoplasty (DALK) after failed DALK., Methods: This retrospective case study included 32 eyes of 27 patients who underwent repeat DALK of 450 cases of DALK performed for optical indications between 1997 and 2013. The patients were divided into 4 etiology-specific subgroups (the corneal dystrophy, ocular surface disease, stromal scar, and others) or those with or without limbal stem cell deficiency (LSCD). The clinical outcomes evaluated were graft survival, best-corrected visual acuity, endothelial cell density, and complications., Results: The mean postoperative follow-up duration was 69.6 ± 54.8 months. The 1-, 3-, and 5-year overall graft survival rate were 76.7%, 57.5%, and 38.8% respectively. The graft survival rate was the highest in the corneal dystrophy group ( P = 0.0014) and was significantly ( P = 0.0010) higher in eyes without LSCD than in eyes with LSCD. There were no significant differences in the graft survival rates between the previous and current DALK groups. The postoperative best-corrected visual acuity of all subjects improved significantly. The postoperative endothelial cell density did not decrease after repeat DALK. There were no significant differences in the incidence of complications between patients with and without LSCD, except the incidence of persistent epithelial defects., Conclusions: Repeat DALK had favorable outcomes in all etiology-specific groups, whereas eyes with LSCD required careful assessment of the ocular surface to avoid graft failure due to persistent epithelial defects., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

BACKGROUND: This report presents a unique case that illustrates the importance of ocular history and photo documentation of ophthalmologic pathology when waivers are granted. CASE REPORT: A United States Navy pilot applicant was granted a waiver for a corneal scar of unknown etiology. He chose not to pursue Navy pilot training and reenrolled as a United States Marine Corps pilot applicant. He did not mention the previous waiver or subsequent civilian surgical corneal treatment for Salzmann's nodular degeneration and was diagnosed with gelatinous drop-like corneal dystrophy. Eventually all information was disclosed, and the diagnosis was changed to postoperative changes from previous Salzmann's nodule removal, which is disqualifying for Marine Corps pilot applicants. DISCUSSION: Corneal dystrophy and degeneration are disqualifying conditions for military pilot applicants. A detailed history, to include surgical history, must be disclosed by the applicant. Photo documentation and appropriate topographic studies should also be completed and reviewed when waivers for corneal pathology are considered. Thorgrimson JL, Hessert DD. Salzmann's nodular degeneration in a pilot applicant . Aerosp Med Hum Perform. 2023; 94(5):400-403.

Competing Interests: The authors declare that they have no conflict of interest.

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-β1 , 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., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: The purpose of this study was to report the use of bilateral corneal neurotization for neurotrophic keratitis in the setting of Ramos-Arroyo syndrome., Methods: The case report and surgical technique are described in detail in this article, as well as a review of the literature on corneal neurotization for congenital corneal anesthesia., Results: We report a 17-year-old patient who underwent bilateral corneal neurotization for neurotrophic keratitis secondary to corneal anesthesia in Ramos-Arroyo syndrome. Corneal neurotization was performed with great auricular nerve transfers extended by sural nerve autografts., Conclusions: We present the treatment of neurotrophic keratopathy with corneal neurotization in a patient with Ramos-Arroyo syndrome. We describe how bilateral corneal neurotization using the great auricular nerve technique is a safe and effective procedure for patients with congenital/developmental corneal anesthesia., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Congenital hereditary endothelial dystrophy (CHED) is a rare, autosomal recessive, monogenic corneal condition with variable expressivity. Often presents in bilateral symmetrical progressive corneal cloudiness that starts in the early infancy. It is characterized by increased corneal thickness, profound corneal edema, and thickening of the Descemet membrane due to endothelial dysfunction. The published literature lacks uniform guidelines for grading corneal cloudiness and management algorithm for CHED cases. This article focuses on applying newer investigational modalities to fine-tune surgical outcomes and more recent CHED management strategies., Methods: This comprehensive literature review was performed based on a search on the PubMed database of relevant CHED articles focusing on those published in the last 7 years. A total of around 70 articles were reviewed, and 17 of them were included in this review. These include systemic reviews, randomized controlled clinical trials, cohort studies, case-controlled studies, and case series., Results: Corneal cloudiness grading in CHED using subjective and objective methods using Anterior Segment Optical Coherence Tomography (AS-OCT) and densitometry using Scheimpflug imaging can help select appropriate management plan for CHED cases. DSAEK outscores penetrating keratoplasty with much fewer complications and expedites visual recovery, which helps mitigate amblyopia., Conclusion: Managing cases of CHED has been a challenge due to the dilemma in timing and appropriate surgical method selection and lack of definitive medical or other conservative approaches. Currently, DSAEK has shown favorable results in cases of CHED. However, appropriate staging of CHED and selecting the appropriate management approach appears to play a critical role in managing such cases. Besides these, novel treatment modalities such as nonsteroidal anti-inflammatory agents (NSAIDS) that target restoring water-flux activity in subtype of CHED and gene editing using CRISPR-Cas9 are promising paradigm treatment modalities.

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.] .

Neurotrophic keratopathy (NK) is a rare degenerative disease in which damage to the corneal nerves leads to corneal hypoesthesia or anesthesia. Neurotrophic corneal ulcers are notoriously difficult to treat and can lead to blindness. Corneal neurotization (CN) is a recent surgical technique aimed at restoring corneal sensation and may offer a definitive treatment in the wake of NK. Herein, we review the surgical techniques utilized in direct and indirect CN. Technical considerations, outcomes, current limitations and future perspectives are also discussed. This article highlights the key points of this promising procedure and biological aspects that will help provide the best treatment options for patients with severe NK., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Neurotrophic keratopathy (NK), or neurotrophic keratitis, is a degenerative condition that results from decreased innervation to the cornea. The cornea is innervated by the ophthalmic branch of the trigeminal nerve. Neurotrophic keratopathy is most commonly caused by herpes keratitis however, any condition that disrupts the normal corneal innervation can cause NK. Neurotrophic keratopathy is a clinical diagnosis and is classified into three stages based on the disease severity. Stage 1 has mild epithelial defects, such as punctate keratopathy, stage 2 disease has persistent epithelial defects, and stage 3 is defined by the presence of ulcers. Current treatment modalities consist of medical and surgical options. Stage 1 is treated with lubrication through artificial tears, eyelid taping, and punctal plug/cautery. Stage 2 treatment can involve therapeutic contact lenses, topical autologous or allogenic serum, tarsorrhaphy, botulinum toxin injections, and possibly anti-inflammatory medications. Stage 3 disease may require human nerve growth factor, amniotic membrane transplantation, conjunctival flap, or corneal neurotization. New therapies, such as matrix regenerating therapy, plasma rich in growth factors, Thymosin β4, Substance P/Insulin like growth factor-1, and nicergoline represent exciting future options.KEY MESSAGESNeurotrophic keratopathy is a rare degenerative disease defined by decreased innervation to the cornea that is associated with significant morbidity.Treatment options range from lubrication alone to various medical and surgical treatments.Matrix regenerating therapy, plasma rich in growth factors, Thymosin β4, Substance P/Insulin like growth factor-1, and nicergoline are exciting novel therapies that will influence how neurotrophic keratopathy is treated in the future.

Purpose: The purpose of this study was to compare the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) and penetrating keratoplasty (PKP) in patients with congenital hereditary endothelial dystrophy (CHED)., Methods: This was a retrospective, comparative study of all the patients with a histopathological diagnosis of CHED who underwent PKP or DSAEK between January 1, 1990, and December 31, 2016. All the cases were included except those patients who had clear grafts but did not complete 2 years of postoperative follow-up. The main outcome measure was graft clarity 2 years after surgery., Results: There were 111 eyes of 63 patients. Seventy-six eyes underwent PKP, and 35 eyes underwent DSAEK. The median age at surgery was 6.8 years in the PKP group and 10.32 years in the DSAEK group. At 2 years postoperatively, clear grafts were noted in 66 of 76 (86.8%) eyes in the PKP group and 30 of 35 (85.7%) eyes in the DSAEK group. At the last follow-up, 80.3% of PKP grafts and 82.8% of DSAEK grafts were clear ( P =0.5). The type and timing of complications differed between the 2 groups. The PKP group had a statistically significant higher rate of graft rejection (19.5%) versus the DSAEK group (0%) ( P =0.01). DSAEK complications were mainly lenticule detachment that developed within one month postoperatively. There was no statistically significant difference in the visual outcomes at the last follow-up between the groups., Conclusion: Endothelial keratoplasty is a safe alternative to conventional PKP in CHED. The visual outcome and survival rates were comparable, but DSAEK had a lower rejection rate and fewer suture-related complications., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Contact Lens Association of Ophthalmologists.)

Congenital hereditary endothelial dystrophy affects the Descemet membrane and endothelium, resulting in corneal decompensation. Penetrating keratoplasty (PKP) has been the gold-standard surgical management until recently; however, at present, endothelial keratoplasty (DSEK/DSAEK/n-DSEK: Descemet-stripping or non-Descemet stripping endothelial keratoplasty and DMEK/n-DMEK: Descemet membrane endothelial keratoplasty) is being preferred due to lesser intraoperative and postoperative complications, early visual recovery, and comparable visual outcomes. Endothelial keratoplasty (EK) can be challenging, especially in pediatric eyes with CHED due to smaller eyeballs, shallow anterior chambers, phakic status, and poor intraoperative visibility due to thick and hazy corneas. A total of 198 articles matched our search strategy. After screening for duplication and going through the titles and abstracts, 12 relevant original articles, one case series, and six case reports were included in this review. Various surgical modifications have to be adopted in comparison to adult eyes to overcome the aforementioned difficulties. Regardless, studies have shown favorable visual outcomes with better graft survival and fewer complications in eyes that underwent EK compared to PKP. Hence, timely surgical intervention and strict amblyopia management can result in better final visual outcomes. The purpose of this review is to summarize various intraoperative difficulties and the surgical modifications required, different surgical techniques, visual and graft-related outcomes, and various complications of EK in CHED eyes., Competing Interests: None

Purpose: To highlight the typical histological and ultrastructural features of severe infectious crystalline keratopathy (ICK) in a corneal graft, which required excimer laser-assisted repeat penetrating keratoplasty (PKP) and to present the challenging treatment conditions associated with ICK., Methods: An 85-year-old female patient underwent PKP for secondary graft failure after Descemet membrane endothelial keratoplasty (DMEK) for Fuchs' endothelial corneal dystrophy in the left eye. One year later, white branched opacities were observed in the superficial corneal stroma of the graft without surrounding inflammation in the left eye. The patient underwent excimer laser-assisted repeat PKP (8.0/8.1 mm) in the left eye after prolonged refractory topical anti-infectious treatment for 1 month. The corneal explant was further examined by light and transmission electron microscopy (TEM)., Results: The light microscopic examination of the corneal explant demonstrated aggregates of coccoid bacteria in the superficial and mid-stromal region that were positive for periodic acid-Schiff (PAS) and Gram stain. The bacterial aggregates extended into the interlamellar spaces, showed a spindle-shaped appearance, and were not surrounded by an inflammatory cellular reaction. TEM demonstrated lamellae separation within the anterior corneal stroma with spindle-shaped aggregates of bacteria, which were embedded in an extracellular amorphous matrix with incipient calcification, being consistent with a biofilm. No inflammatory cellular reaction was evident by TEM. At discharge from hospital, the corrected visual acuity was 20/80 in the left eye., Conclusion: ICK is often challenging due to the difficult diagnosis and treatment conditions. The refractory courses are mainly attributed to a biofilm formation, which inhibits effective topical anti-infectious treatment. In such cases, (repeat) PKP may be necessary to completely remove the pathology, prevent recurrences, and improve vision., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Competing Interests: None

Purpose: Anterior segment abnormalities associated with Noonan syndrome are rare. We report our experience with 2 patients who developed keratopathy with significant visual sequelae., Methods: case series., Results: The first patient is a 9-year-old boy with genetically confirmed Noonan syndrome. At presentation, he was noted to have diffuse inferior epitheliopathy with vascularization and bilateral mild ptosis. Over 1 year, he developed focal areas of scarring with deterioration of vision and underwent superficial keratectomy in the left eye. However, over the following 2 years, he experienced recurrent corneal scarring and vascularization. The second patient is a 7-year-old boy with phenotypic Noonan syndrome. At presentation, he had an anterior subepithelial corneal scar inferiorly with epithelial defects in both eyes. He also had bilateral moderate ptosis and lagophthalmos. Despite lubrication, he developed recurrent bilateral corneal erosions with focal areas of scarring associated with vascularization and underwent superficial keratectomy for both eyes. Despite this, there was worsening corneal scarring and vascularization over time, eventually requiring deep anterior lamellar keratoplasty in the left eye. The host cornea showed a disturbed Bowman layer and an acellular mass of fibrous collagenous tissue between epithelium and stroma., Conclusions: Noonan syndrome may be associated with visually significant keratopathy, manifesting as focal corneal scarring with vascularization. These changes may due to an excessive fibrotic response in Noonan syndrome. Early recognition and treatment can help to delay the progression of keratopathy and need for surgical intervention., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Limbo-keratoplasty enables visual improvement and limbal stem cell transplantation at the same. During follow-up, most grafts show vascularization of the limbus. However, it is unclear whether vascularization is harmful due to immunologic effects or helpful to nourish the limbal stem cells and is therefore necessary for a clear graft. The aim of our study is to analyze the influence of graft vascularization on graft survival following homologous limbo-keratoplasty., Methods: In this retrospective study, we assessed all consecutive limbo-keratoplasties performed in our hospital. All eyes with suitable photo-documentation were included and divided into two groups (limbal stem cell deficiency and corneal dystrophy). We categorized the grade of vascularization (0, 1, 2, 3, 3b) and analyzed clear graft survival, recurrence of the underlying disease and the endothelial cell density (ECD) with regard to the reason for the graft. Event rates were estimated with the Kaplan-Meier method., Results: A total of 79 eyes with limbal stem cell deficiency and 15 with corneal dystrophies were analyzed. A high degree of graft vascularization had a tendency for better graft survival in limbal stem cell deficiency, whereas in corneal dystrophies, grafts with no vascularization had preferable outcomes. Recurrence-free graft survival was only seen in grade 1 and 3 vascularization in corneal dystrophies., Conclusion: Vascularization of the limbus seems to have an impact on the long-term outcome of limbo-keratoplasty. The effect seems to be favorable in limbal stem cell deficiency and on recurrence rates in corneal dystrophies. However, the latter might be overshadowed by an unfavorable immunologic effect in corneal dystrophies where the baseline immunologic risk profile is commonly more favorable than in limbal stem cell deficiency., (© 2022. The Author(s).)

Background: Phototherapeutic keratectomy (PTK) is an established treatment method for patients suffering from either genetic corneal dystrophy or recurrent corneal erosion (RCE) without underlying basement membrane dystrophy, often caused by trauma., Objective: This study aimed to describe the changes in manifest subjective refraction and pachymetry after PTK treatment in patients suffering from epithelial basement membrane dystrophy (EBMD) and traumatic or atraumatic RCE without underlying EBMD., Material and Methods: This was a retrospective, single-center study performed at the Department of Ophthalmology of the Ludwig-Maximilians University (Munich). Patient data were retrospective collected using the smart-eye database of the Department of Ophthalmology including diagnostic data from an autorefractometer and from the Pentacam HR. All laser treatments were performed with an ablation depth of 10 μm for EBMD patients and 6 μm for RCE patients without EBMD., Results: Both collectives showed a decrease in pachymetry larger than the calculated ablation depths after a follow-up interval of 126 days (95% CI 104-147 days). While the EBMD collective receiving an ablation of 10 μm showed a decrease of 25.8 μm (N = 74; 95% CI 21.2-30.3; p < 0.001), the non-EBMD collective receiving an ablation of 6 μm showed a decrease of 12.3 μm (N = 44, 95% CI 7.0-17.7; p < 0.001). Both, total corneal refractive power (TCRP) as well as spherical equivalent (SE) offered no significant change in preoperative and postoperative comparison for the EBMD collective. On the other hand, patients without underlying EBMD showed a significant myopic decrease in SE of 0.4 dpt (±0.7 dpt SD, p < 0.05). The mean follow-up interval was 126 days (95% CI 104-147 days)., Conclusion: PTK treatment offers an effective method for patients suffering from either EBMD dystrophy or RCE syndrome without underlying EBMD. The final ablation based on pachymetry at the apex can be estimated at 2.3 to 2.6 times higher compared to the original ablation depth. The reasons for this are on the one hand the laser ablation itself and the influence of the reactive wound healing of the corneal epithelium., (© 2022. The Author(s).)

Purpose: To analyze the complications in patients managed with deep anterior lamellar keratoplasty (DALK) for diseases of the anterior corneal stroma., Methods: This is a retrospective analysis of all the patients who underwent DALK in a tertiary care center in South India from 2010 to 2020. A total of 474 eyes in 373 patients were included in the study. Patients who underwent DALK for advanced keratoconus, keratoconus with Bowman's membrane scar, healed hydrops, macular corneal opacity, macular corneal dystrophy, granular corneal dystrophy, spheroidal degeneration, pellucid marginal degeneration, post-laser-assisted in situ keratomileusis ectasia, descematocele, post-collagen cross-linking aborted melt and dense scar, and post-radial keratotomy were included in the study. The patients were followed up for 17.2 +/- 9.2 months (1-9 years)., Results: Complications noted in the surgery were intra-operatively Descemet's membrane perforation in 31 eyes (6.54%), post-operatively secondary glaucoma in 16 eyes (3.37%), cataract in seven eyes (1.47%), suture-related complications in five eyes (1.05%), graft rejection in three eyes (0.63%), traumatic dehiscence in two eyes (0.42%), filamentary keratitis in two eyes (0.42%), interface infiltrate in one eye (0.21%), and recurrence of disease in four eyes (7.14%) out of 57 eyes with corneal dystrophy., Conclusion: DALK as an alternative to penetrating keratoplasty for anterior corneal stromal diseases. It has become an automatic choice for diseases of the anterior cornea requiring keratoplasty. Complications can occur at any stage of surgery; however, if identified and managed early, they can result in optimal outcome., Competing Interests: None

Objective: To observe the restriction effect of silicone oil retention sutures and their protective effect on the corneal endothelium. Methods: Retrospective case series study. The clinical data of 6 patients (6 eyes) with lens-iris damage after severe ocular trauma were collected from March 2017 to November 2021 in Beijing Tongren Hospital. Their was combined retinal injury in each eye, so silicone oil retention sutures and silicone oil filling were performed. The silicone oil position was observed by slit lamp and ultrasound biomicroscopy. The corneal endothelium was examined by specular microscopy during the follow-up. Results: All patients were male, with an average age of 47 years (range, 26 to 73 years). The diseased eyes included 2 left eyes and 4 right eyes. Five eyes had ocular rupture, and 1 eye had ocular contusion. The follow-up time ranged from 5 months to 51 months, with an average of 18.5 months. In all 6 eyes, the silicone oil was completely located in the posterior segment and never touched the corneal endothelium. Four eyes maintained healthy and had a clear cornea during the follow-up, while 2 eyes developed band keratopathy at the 11 month and 16 month separately during follow-up, 1 of which was found to suffer corneal decompensation at the last follow-up (51 month). Both band keratopathy and corneal decompensation were caused by severe anterior segment trauma and were not related to the silicone oil contact with the cornea. There were no complaints about occlusion or glare due to silicone oil retention sutures. All patients did not need to keep the prone position postoperatively. Silicone oil emulsification occurred in 1 eye and ocular hypertension occurred in this eye after silicone oil emulsification. Conclusion: Silicone oil retention sutures can effectively prevent silicone oil from migrating to the anterior chamber or contacting the corneal endothelium.

Introduction: This study aimed to establish which worldwide population cohorts have a genetic make-up closest to that of a large sample of the Maltese population with regard to corneal dystrophy (CD) genes., Methods: Single nucleotide polymorphisms (SNPs) in the Maltese cohort were compared with worldwide cohorts. Fixation index (F ST ) values were calculated to evaluate population differentiation. The genetic prevalence of CD subtypes in worldwide and Maltese cohorts were calculated, and single nucleotide missense mutations present in the Maltese cohort were evaluated for potential pathogenicity., Results: F ST values showed that CD-related genes differ substantially among the studied cohorts. F ST values for each SNP showed greatest differentiation between the Maltese and African cohorts and least differentiation with the Puerto Rican, Mexican, and Colombian cohorts. One TGFBI casual CD mutation, 502V, which causes a Bowman's layer CD/atypical Thiel-Behnke CD was identified in the Maltese cohort. The KRT3 NC&#95;000012.11:g.53186088G>C mutation was potentially deleterious., Conclusion: Identifying populations with least genetic differentiation can facilitate and help guide future diagnostic and treatment strategies for Maltese individuals with CDs in the absence of comparable Maltese data. Analysing the previously unknown CD genetic pool present in a large Maltese cohort adds to the global genetic bank that researchers rely on for medical progress., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Background: Excimer laser-assisted phototherapeutic keratectomy (PTK) has become established as the gold standard in treatment of epithelial basement membrane dystrophy (EBMD), commonly also known as map-dot-fingerprint dystrophy (MDF). At the Department of Ophthalmology, Saarland University Medical Center in Homburg/Saar, systems from Zeiss Meditec and Schwind have been used. The outcomes of both were compared in this study., Patients and Methods: The retrospective study included patients who underwent PTK with a diagnosis of MDF between 2007 and 2017. A total of 170 operations were performed using Zeiss Meditec MEL-70 (Carl Zeiss Meditec AG, Jena, Germany) and 98 using a Schwind eye-tech-solutions Amaris 750S laser (Schwind eye-tech-solutions GmbH, Kleinostheim, Germany). Preoperative and postoperative data for visual acuity, refraction and astigmatism as well as curvature data from the Pentacam and endothelial cell count were collected. The follow-up period averaged 8 months., Results: In both groups visual acuity postoperatively was significantly better (Zeiss: p < 0.001, Schwind p < 0.004). The improvement in the Schwind group was less than in the Zeiss group, which is the reason why there was a significant difference between the laser systems postoperatively (p < 0.017). There were no significant changes regarding the spherical equivalent after PTK. Regarding astigmatism, there was a significant decrease in the Zeiss group (p < 0.042), while it did not change significantly in patients treated with Schwind laser (p < 0.217). Overall, this led to a significant postoperative difference between both laser systems (p < 0.014)., Conclusion: The PTK can be recommended as an effective treatment method for patients with EBMD, regardless of the laser systems used. Patients benefit from long relief from recurrences with improved or constant visual acuity and stable refraction., (© 2022. The Author(s).)

Purpose: To report on the preliminary outcomes of mitomycin C (MMC) intravascular chemoembolization (MICE) for corneal neovascularization (NV)., Methods: This is a retrospective case series of three consecutive eyes that underwent MICE for progressive corneal NV with sight threatening lipid keratopathy. A 1.0 cc syringe was partially filled with MMC (0.4 mg/mL) and attached to a 33-gauge needle used to cannulate the vessels. The MMC (0.01-0.05 ml) was injected with enough retrograde hydrostatic force to fill efferent and afferent vessels. Follow-up ranged from 4 months to 1 year., Results: Three eyes of three patients aged 59, 73 and 33 years were included. There were no intraoperative or postoperative complications associated with the MICE procedure. Patient 1 presented with progressive corneal NV and lipid keratopathy secondary to herpes zoster ophthalmicus (HZO) and a best-corrected spectacle visual acuity (BSCVA) of 20/100 Snellen. At one-year post-MICE, there was no recurrence (BSCVA was 20/20 Snellen). Patient 2 presented with idiopathic lipid keratopathy (BSCVA 20/50 Snellen). At four months post-MICE, there were no signs of recurrence (BSCVA 20/20 Snellen). Patient 3 presented with corneal NV and lipid keratopathy secondary to HZO (BSCVA 20/30 Snellen). At four months following two MICE treatments, resolution of the lipid keratopathy was noted (BSCVA 20/20 Snellen)., Conclusions: Preliminary findings suggest that MICE may be an additional modality for treating progressive corneal NV with lipid keratopathy. Larger comparative studies with longer follow-up are warranted., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: The purpose of this study was to describe a case with recurrent corneal erosions who was treated with a Bowman layer (BL) onlay graft., Method: BL onlay transplantation was performed., Results: In a 79-year-old female patient who presented with bilateral map-dot-fingerprint dystrophy and a history of recurrent painful corneal erosions, BL onlay grafting was performed to restore the corneal surface. At 1 month postoperatively, the epithelium was smooth over the graft, and until 1.5 years postoperatively, the patients had no complaints and no recurrence of the epithelial corneal erosion., Conclusions: In the described case, the transplantation of an isolated BL graft as an onlay proved to be an effective treatment for painful chronic recurrent erosions in the context of map-dot-fingerprint dystrophy in a patient who had undergone numerous unsuccessful previous treatments., Competing Interests: G. R. J. Melles is a consultant for DORC International and SurgiCube International. J. S. Parker is a consultant for DORC International and Ziemer Ophthalmic Systems. The remaining authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: The purpose of this study was to describe the genotypic and phenotypic characteristics of an infant with a SLC4A11 mutation associated with bilateral corneal edema, hearing loss, and hydronephrosis present since birth., Methods: This was a case report. Ophthalmic and systemic examination of the proband, histopathologic and ultrastructural characteristics of bilateral corneal discs, and molecular genetic evaluation by whole-exome sequencing are described., Results: A male infant was born with bilateral corneal opacities, sensorineural hearing loss, and hydronephrosis to healthy parents after an uneventful pregnancy. Penetrating keratoplasty of the left eye at age 10 months demonstrated minimal corneal edema with normal thickness Descemet membrane and cellular endothelium with intracytoplasmic vacuoles and degenerative changes in rare cells. Penetrating keratoplasty of the right eye 6 months later disclosed prominent corneal edema with a thickened posterior banded layer of Descemet membrane and severe endothelial atrophy. Whole-exome sequencing of the proband and parents' blood demonstrated a homozygous mutation in SLC4A11 gene (c.1735&#95;1737delCTC,p.Leu579del). The combined clinical, histopathologic, and molecular genetic findings raised consideration of an unusual phenotype of Harboyan syndrome manifesting as congenital hereditary endothelial dystrophy with a prelingual rather than, as previously described, postlingual hearing loss., Conclusions: We report a novel homozygous SLC4A11 variant with a previously undocumented phenotype of CHED in association with prelingual sensorineural hearing loss and hydronephrosis, thus broadening our understanding of the spectrum of genotypic and phenotypic findings of Harboyan syndrome., Competing Interests: Conflicts of interest statement: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

A young man in his first decade of life presented with insidious onset, gradually progressive diminution of vision in right eye since 2 months associated with foreign body sensation. Slit-lamp examination revealed superior arcuate band of corneal stromal thinning extending from 9-3 o' clock. There was associated corneal vascularisation and presence of lipid deposition at the leading edge of the furrow formation with intact epithelium without any apparent signs of inflammation. There was no previous history of ocular or systemic disease, trauma, ocular surgery, collagen vascular disease or contact lens wear. Serological tests for rheumatic diseases did not show any abnormalities. Scheimpflug imaging showed high against the rule astigmatism with a 'reverse crab claw' pattern. A provisional diagnosis of right eye Terrien's marginal corneal degeneration was made. On a 1-month follow-up visit, there was significant symptomatic relief with lubricants alone and with spectacles the visual acuity improved to 20/60 in the right eye., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)