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Background: Corneal perforation is an ophthalmic emergency. The conventional management of corneal perforation can be associated with severe complications especially in patients with ocular surface disease. Endothelial keratoplasty has been suggested as an alternative surgical technique for the management of corneal perforations. We present a case series of nine patients with corneal perforation and ocular surface disease managed with secondary patch endothelial keratoplasty., Methods: This is a retrospective case series of nine patch endothelial keratoplasties performed between 2016 and 2022 at a quaternary eye hospital in Australia. The surgical technique is similar to conventional endothelial keratoplasty except descemetorhexis was not performed., Results: A total of 9 cases were treated during the review period. Eight of the nine cases had an improvement in visual acuity. One case failed to achieve corneal tectonic objective., Conclusion: Patch endothelial keratoplasty is a safe secondary procedure for the management of corneal perforations in patients with ocular surface disease., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Selective keratoplasty involves replacing the affected layers of the cornea with similar donor tissue. In case of pathological changes in the middle and posterior stroma, deep anterior lamellar keratoplasty (DALK) is performed. Chronic corneal edema caused by endothelial dysfunction is an indication for endothelial keratoplasty - Descemet membrane endothelial keratoplasty (DMEK) or Descemet Stripping Endothelial Keratoplasty (DSAEK). Compared to penetrating keratoplasty (PK), these operations are characterized by a low risk of damage to intraocular structures and a relatively short rehabilitation period. Complications of selective keratoplasty include the formation of a false chamber between the lamellar graft and the recipient's cornea, ocular hypertension during anterior chamber air tamponade. Persistent epithelial defect can be a sign of primary graft failure in DALK, DSAEK and DMEK. Selective keratoplasty is characterized by a lower incidence of immune rejection than PK. In some cases, DALK can be complicated by corneal changes related to suture fixation of the graft. Long-term postoperative use of topical glucocorticoids can cause ocular hypertension and cataracts.

Purpose: We aimed to determine whether Descemet's membrane (DM) scrolling occurs primarily along the vertical or horizontal axis and establish whether oval trephination along the axis of least scrolling can reduce the grade of the scroll., Methods: The longest limbus-to-limbus axis on 28 sclerocorneal discs was taken as the horizontal axis. The horizontal (n = 7) or (right angles to it) vertical (n = 6) axis was marked on DM before peeling it off. The direction and grade of scrolling was observed. Narrow strips (3-4 mm wide) were then cut along the two axes (n = 4 each) and the scrolling pattern was observed. Ellipses (7 × 9 mm) of DM were punched along the two axes (n = 6 each) and the scrolls graded. Immunofluorescent staining for elastin on horizontal and vertical tissue sections from three DM samples was performed. The intensity and thickness of elastin staining were measured., Results: Twenty-four (85.72%) DM samples showed scrolling along the horizontal axis, none showed scrolling along the vertical axis, and four (14.28%) samples showed a spiral scroll, regardless of which axis was marked (grade 3.7 and 3.6). Vertically oval discs showed significantly reduced scrolling (grade 1.2) compared to horizontally oval discs (grade 3.5). Narrow strips of DM showed a similar scrolling pattern. Immunohistology showed no difference in any of the parameters examined along the two axes or from the center to the periphery., Conclusion: DM scrolls primarily along the horizontal axis. Vertically oval DM samples show minimal scrolling, which can be an advantage in DMEK. Differential scrolling is not determined by the distribution of elastin., Competing Interests: None

Aim: To report the clinical characteristics of six patients with corneal allograft endothelial rejection after COVID-19 vaccination with Sinopharm and to review the literature., Methods: This is a prospective case series describing corneal allograft rejection among subjects having received Sinopharm (BBIBP-CorV) vaccine, coming to cornea clinic at a university-based hospital (Rassoul Akram Hospital, Tehran, Iran) from September 2021 to March 2022 for regular follow-up examinations. Data on demographics, vaccination (based on vaccine card), and graft condition (based on recent examination and previous medical documents) were recorded., Results: Out of 54 eyes (46 patients), 6 eyes (6 patients) had corneal allograft endothelial rejection after 3 to 117 days, post-vaccination. Three out of six rejections occurred within two weeks following vaccination. All of them were male with the mean age of 53.00 ± 19.66 years. The graft type of all patients was penetrating keratoplasty (PKP). The adverse event developed on average at 40.67 ± 34.33 months after surgery. Four patients were under maintenance treatment by topical steroid at the time of vaccination. One also received systemic immunomodulatory medication. Four grafts ended up with partial or complete graft failure. One case had received two doses of vaccine before undergoing the second corneal graft transplantation., Conclusion: COVID-19 vaccination with Sinopharm may trigger corneal allograft endothelial rejection even in individuals with low-risk graft and under maintenance topical and/or systemic immunomodulatory medications.

Purpose: To find preoperative simple geometric parameters to predict the outcome of lamellar keratoscleroplasty in patients with corneal limbal dermoids., Methods: We retrospectively analyzed the data of 30 patients with limbal dermoids who underwent lamellar keratoscleroplasty and were followed up for more than 6 months. Seven geometric parameters were used to analyze the relationship with postoperative visual acuity, astigmatism, and scar formation and investigated for their influence on postoperative outcome. These seven parameters included the cornea-invaded area of dermoid, dermoid area, the cornea-invaded length of dermoid, the corneal limbus invaded length of dermoid, dermoid size, bed size, graft size. Furthermore, we divided patients with corneal limbal dermoids into amblyopia group and non-amblyopia group, and analyzed the clinical characteristics of the amblyopia group., Results: The mean age of the patients at surgery was 10.6 ± 5.83 years. The 7 geometric parameters, preoperative and postoperative astigmatism of the amblyopia group were higher than those of the non-amblyopia group (all P < 0.05). Among the geometric parameters analyzed, the r coefficients between the cornea-invaded length of dermoid and postoperative visual acuity and astigmatism were the highest (r = 0.854, r = 0.714). The r coefficient between the corneal limbus invaded length of dermoid and the postoperative scar was the highest (r = 0.375). The r coefficient between age and postoperative steroid-induced high intraocular pressure was the highest (r = - 0.416). In univariate regression analysis, the cornea-invaded length of dermoid was significantly correlated with postoperative visual acuity (β = 0.270, P < 0.001) and postoperative astigmatism (β = 1.362, P < 0.001). Among the geometric parameters analyzed, the cornea-invaded length of dermoid had best stratified patients in grouping with or without amblyopia (cutoff > 2.24)., Conclusion: The cornea-invaded length of dermoid was the most important related factor of postoperative visual acuity, astigmatism, and amblyopia. The corneal limbus invaded length of dermoid was the most important related factor of postoperative scar formation. The geometric parameters and astigmatism in patients with amblyopia were larger than those in patients without amblyopia., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Corneal melt is a sight-threatening complication of Boston type 1 keratoprosthesis (KPro). Severe corneal melt may result in hypotony, choroidal hemorrhage, and even spontaneous extrusion of the KPro, which may lead to a poor visual prognosis. Lamellar keratoplasty is one surgical option for the management of mild corneal melt, especially when a new KPro is not available. Herein, we present a new surgical technique application, intra-operative optical coherence tomography (iOCT) for the management of cornea graft melt after Boston type 1 KPro implantation. The visual acuity and the intra-ocular maintained stable at 6 months post-operatively, and the KPro remained in place without corneal melting, epithelial ingrowth, or infection. iOCT may prove to be a real-time, non-invasive, and accurate treatment for corneal lamellar dissection and suturing beneath the anterior plate of the KPro, which can effectively help the surgeon to make surgical decisions and reduce post-operative complications., Competing Interests: None

Purpose: The following is a comparative analysis on the treatment outcomes of corneal perforations using amniotic membrane transplantation (AMT) or penetrating keratoplasty (PK)., Methods: This monocentric retrospective study was performed at the Department of Ophthalmology, University Hospital Ulm, Germany. A total of 78 eyes of 78 patients were included. Thirty-nine eyes received an AMT, and 39 patients were treated with a PK. Primary outcome was recurrence of perforation. Secondary outcomes were patient mortality and visual acuity., Results: No statistically significant difference was observed with regard to a recurrence of perforation between the two groups (26% in AMT vs 23% in PK, p > 0.99). The time of recurrences was within the first two years and did not differ statistically (p = 0.97). In addition, a proportional hazards model with cox regression regarding recurrent perforation showed no significant differences (p = 0.5). After AMT, 41% and after KP, 28% of the patients died during follow-up (p = 0.2), respectively. The Charlson Comorbidity Index (p < 0.0001) and the age at the time of surgery (p = 0.0002) were statistically significantly higher in those who were deceased. A mean follow-up of 485 ± 517 days was recorded., Conclusion: Both surgical methods show good results and no statistically significant difference regarding recurrent perforation rate. About a third of the patients died during the follow-up period. The decision regarding the appropriate method should therefore be based on a combination of all factors., (© 2023. The Author(s).)

Purpose: This study aims to analyze the pattern of follow-ups and the reasons for follow-up loss in keratoplasty cases in a tertiary eye care center., Methods: This is a single-center retrospective cross-sectional study. During the study period, 165 eyes underwent corneal transplantation. The data on the demographic features of the recipients and the indications of keratoplasty, including visual acuity before and after surgery, duration of follow-up, and the condition of the graft at the last follow-up, were collected. The primary outcome was to determine the factors causing lost-to-follow-up (LTFU) among graft recipients. LTFU was defined when a patient failed to adhere to any of the following seven follow-up visits since the surgery: 4 ± 2 weeks, 3 ± 1 months, 6 ± 1 months, 12 ± 2 months, 18 ± 2 months, 24 ± 3 months, and 36 ± 6 months. The secondary outcome was to analyze the best-corrected visual acuity (BCVA) among patients available for the final follow-up., Results: The recipient follow-up rates at 6, 12, 18, 24, and 36 months were 68.5%, 57.6%, 47.9%, 42.4%, and 35.2%, respectively. Old age and distance to the center were significant factors for lost-to-follow-up. A failed graft as an indication for transplantation and those undergoing penetrating keratoplasty for optical purposes were significant factors for completing follow-up., Conclusion: The inability to follow-up after corneal transplantation is a common challenge. Elderly patients and those living in remote areas must be prioritized for follow-ups., Competing Interests: None

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

Purpose: The purpose of this study is to study the frequency, surgical transplantation technique and outcome in patients with aniridia-related keratopathy (ARK) with two-year follow-up period., Methods: A retrospective registry-study including all ARK cases performed in Sweden and Denmark between 2001 and 2016 and registered in the Swedish Cornea Transplant Registry., Results: A total of 36 eyes of 26 patients were subjected to corneal transplantation due to ARK during 2001 to 2016. Penetrating keratoplasty (PK) was the procedure of choice in 58.3% (n = 21) of the eyes, followed by a combination of PK and limbal stem cell transplantation in 13.9% (n = 5) and keratolimbal allograft in 13.9% (n = 5). Boston keratoprosthesis was used in 8.3% (n = 3), and anterior lamellar keratoplasty in 5.6% (n = 2). Thirteen of the procedures (36.1%) were retransplantations. Two years after surgery 26 cases were available to follow-up of which 16 of the grafts were functioning (61.5%). The median visual acuity showed a trend of improvement from hand motion to counting fingers., Conclusions: A majority of the ARK cases (61.5%) had a graft providing useful vision for the patient 2 years after corneal transplantation, but the visual gain was modest at best. Longer follow-up time is required to evaluate functional graft outcomes. Despite the introduction of limbal stem cell transplantation as a suitable treatment, PK was the most common surgical method in the present study., (© 2022 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

The article presents the surgical technique of penetrating keratoplasty (PK) and deep anterior lamellar keratoplasty (DALK) with femtosecond-laser assistance involving simultaneous implantation of an intracorneal continuous ring (ICCR) or an intracorneal ring segment (ICRS) into the graft. Surgical technique no. 1 - keratoplasty with simultaneous implantation of ICRS. Intrastromal circular tunnel is formed in the central zone of donor cornea using femtosecond laser. Then penetrating trepanation 8.1 mm in diameter is performed symmetrically to the formed tunnel. After preparing penetrating or lamellar recipient bed, suture fixation is placed in the corneal transplant, then the ICRS is implanted into the graft. Surgical technique no. 2 - keratoplasty with simultaneous implantation of ICCR. The donor cornea is dissected from periphery to center using femtosecond laser. Central zone remains untouched. A large diameter full-thickness trepanation is performed and the donor cornea is divided into the anterior and posterior layers. The ICCR is put on the donor cornea while holding the posterior layer with forceps. Penetrating or lamellar recipient bed is prepared, then the corneal graft is fixed with sutures. Transparent corneal graft acceptance does not guarantee high visual acuity due to post-keratoplasty astigmatism. Surgical correction of astigmatism is performed in the long term post-operatively and isn't effective enough. We proposed this new surgical technique of keratoplasty with simultaneous implantation of ICCR and ICRS into the graft as close as possible to the visual axis of the eye, which can help make the postoperative astigmatism minimal both immediately after surgery and in the long term. The study proposes a new approach to intraoperative prevention of post-keratoplasty astigmatism. The technique is simple, safe and effective. Analysis of long-term outcomes is required before recommending this method for widespread use in clinical practice.

Competing Interests: None

Background: This video demonstrates a useful technique of keratoplasty which can be routinely undertaken by all surgeons when imaging modalities such as anterior segment optical coherence tomography are not available and prior patient history is not forthcoming., Purpose: To demonstrate a technique of lamellar separation and layer by layer removal of host cornea when dealing with keratoplasty in perforated corneal ulcers, adherent leucomas, dense corneal opacities, which obscure visualization of the iris and anterior chamber details., Synopsis: In this video, we demonstrate penetrating keratoplasty in a failed opacified graft with iridocorneal adhesions, with no visualization of anterior chamber details. Lamellar dissection of the host cornea is done starting at its periphery and moving centrally, with gentle peeling of the superficial layers, the epithelium and bulk of stroma, following which, the deeper portion of the cornea is dissected and separated from underlying adherent iris tissue. Layer by layer separation allows better visualization through the remaining thin layers of the cornea. This permits fine dissection and layered removal of the cornea, thereby avoiding injury to iris and lens. Debulking of the host cornea decreases the force that is needed to be applied to separate adherent iris tissue from the host cornea, and reduces the chances of sudden entry into the anterior chamber and subsequent damage to the iris or lens. This also reduces the chance of iris tears, iridodialysis and bleeding from the iris and helps maintain iris integrity, which is essential intraoperatively for protection of lens and anterior chamber formation, and to avoid glare and photophobia postoperatively. Preventing iris damage also reduces the chances of formation of peripheral anterior synechiae (PAS), which can predispose to graft rejection, graft failure and secondary glaucoma., Highlights: Layer by layer corneal separation beginning inside the graft host junction, careful separation of iridocorneal adhesions and PAS is a helpful technique to optimally preserve the anterior segment anatomy during difficult cases of penetrating keratoplasty., Online Video Link: https://youtu.be/ZmQQhuOnAh4., Competing Interests: None

Mucopolysaccharidosis (MPS) is a group of genetic disorders with seven types and 13 subgroups which are characterized by an inherent deficiency of the enzymes responsible for the degradation of glycosaminoglycans (GAGs). Defective breakdown of GAG products leads to their widespread accumulation within the lysosomes of various organs involving the eye, central nervous system, skeletal, ocular, nervous, respiratory, cardiac, and the gastrointestinal systems. Clinical spectrum varies from mild systemic and ocular abnormalities with a normal life span to severe phenotype, fatal in the first few months of life. Visual disability due to corneal clouding, retinopathy, and optic nerve involvement causes additional impairment of physical and cognitive functions. Treatment modalities such as bone marrow transplantation and enzyme replacement therapies help in increasing the life span as well as the quality of life of the affected patients. For patients with significant corneal clouding, keratoplasty is the answer. The decision to proceed with keratoplasty is governed by various factors such as the motivation of the patient and his family, other systemic affections and anesthesia concerns. A detailed preoperative counseling should be done regarding the expected visual outcomes in the presence of other ocular comorbidities and the postoperative complication such as graft re-opacification, rejection and glaucoma. Future treatment options such as targeted gene therapy and substrate reduction therapy hold promise to reverse corneal clouding, thereby obviating the need for corneal transplantation. These treatment therapies are still in the experimental stages and human trials are needed to validate their outcomes., Competing Interests: None

Penetrating keratoplasty is a tissue transplant commonly performed around the world. For over a century, penetrating keratoplasty has been the standard method of treatment for many corneal diseases causing visual impairment. Recently, lamellar anterior keratoplasty (LAK) or lamellar endothelial (LEK) have become preferable options, resulting in fewer complications and faster functional rehabilitation. While penetrating keratoplasty is less frequently indicated, in favor of these lamellar grafts, it has not necessarily become obsolete, and its use remains appropriate for its chosen indications. Functional results are good if the indication for surgery is properly assessed, therapeutic precautions are taken in view of complications, and follow-up aims to improve initial refractive results as needed., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Purpose: To describe the changes in corneal epithelial thickness and reflectivity following simple limbal epithelial transplantation (SLET) using anterior segment optical coherence tomography (ASOCT)., Methods: This was a prospective imaging study of 31 eyes of 29 patients who had undergone autologous or allogeneic SLET for limbal stem cell deficiency and had stable, avascular surfaces postoperatively. ASOCT scans of all four quadrants were taken preoperatively and at 1-week, 1-month, 3-month, 6-month, and 1-year postoperative time points in the eyes undergoing SLET. Baseline scans were also taken from the normal eye in unilateral cases. The following parameters were obtained from the scans: (i) epithelial thickness (ET), (ii) stromal thickness, (iii) total corneal thickness, (iv) epithelial reflectivity (ER), (v) stromal reflectivity, and (vi) epithelial/stromal (ES) reflectivity ratio., Results: Chemical injury (24 eyes, 77.4%) was the most common indication for surgery. There was a significant improvement in the ET (184.8 ± 117.1 vs 60.3 ± 10 µm, p < .0001) and ER (144.5 ± 26.4 vs 120.9 ± 28.9, p < .0001) within the initial postoperative period following SLET, which remained stable at the end of 1 year follow-up. There was no difference in the ET of the post-SLET and normal eyes after the 3-month timepoint and this normalization was maintained until the end of the first year. A significant normalization was also noted in the ES reflectivity ratio at the end of 1 year (2.1 ± 0.8 vs 1 ± 0.2, p < .001). A significant correlation was found between the final visual acuity and the total corneal thickness (r = 0.942, p = .005)., Conclusion: There is a significant improvement in the epithelial thickness and reflectivity of the cornea in eyes undergoing SLET. ASOCT provides a reliable objective measure of these changes and can be used to monitor the outcomes in these eyes postoperatively.

Purpose: This study evaluated the safety and efficacy of Bowman's membrane electrocautery in blind painful eyes with bullous keratopathy not amenable to corneal transplantation., Methods: Eleven eyes of 11 subjects with painful bullous keratopathy and poor visual potential who underwent electrocautery of Bowman's membrane at a tertiary referral ophthalmology clinic were reviewed retrospectively. Subject demographics and preoperative and postoperative data were collected, including description of pain, slit lamp biomicroscopy, best corrected visual acuity, topical medication use, and complications. Efficacy of the procedure on pain reduction, bullae resolution, and topical medication use were assessed at post-operative visits. Safety was also evaluated based on any complications., Results: Bowman's membrane electrocautery effectively resolved bullae in all eyes examined up to 6 months postoperatively; however, 2 eyes had recurrence by 1 year. Mean age at the time of surgery was 69.8 years and mean duration of follow-up was 15.4 months. Pain reduction was achieved in all eyes at 1 month, but 1 subject had pain recurrence by 6 months and another by 1 year. The median number of drops per day decreased from 6 preoperatively to 1.7 at 6 months. Two subjects who had underlying advanced ophthalmic disease had a mild reduction in vision., Conclusion: Bowman's membrane electrocautery is a safe and minimally invasive procedure for the management of painful bullous keratopathy in eyes with low vision potential and not amenable to corneal transplantation. Duration of effect appears to last at least 6 months and up to 3 years post-procedure., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Penetrating keratoplasty used to be the only surgical technique for the treatment of end-stage corneal endothelial diseases. Improvements in surgical techniques over the past decade have now firmly established endothelial keratoplasty as a safe and effective modality for the treatment of corneal endothelial diseases. However, there is a worldwide shortage of corneal tissue, with more than 50% of the world having no access to cadaveric tissue. Cell injection therapy and tissue-engineered endothelial keratoplasty may potentially offer comparable results as endothelial keratoplasty while maximizing the use of cadaveric donor corneal tissue. Descemet stripping only, Descemet membrane transplantation, and selective endothelial removal are novel therapeutic modalities that take this a step further by relying on endogenous corneal endothelial cell regeneration, instead of allogenic corneal endothelial cell transfer. Gene therapy modalities, including antisense oligonucleotides and clustered regularly interspaced short palindromic repeats-based gene editing, offer the holy grail of potentially suppressing the phenotypic expression of genetically determined corneal endothelial diseases at the asymptomatic stage. We now stand at the crossroads of exciting developments in medical technologies that will likely revolutionize the way we treat corneal endothelial diseases over the next 2 decades., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To describe a case of intracorneal epithelial cyst and present a surgical technique successfully used to treat this patient., Methods: This interventional case report presents a 4-year-old boy with a progressive intrastromal corneal opacity and decreased vision in his right eye. The patient had no previous history of trauma or ocular surgery. After clinical examination and corneal optical coherence tomography, a presumptive diagnosis of a developmental epithelial cyst was made, and surgery was indicated. Four years later, when the patient was 8 year old, he returned after having performed drainage of the cyst in another service but still complaining of low vision. The biomicroscopy showed an intrastromal recurrent cyst. At this time, we recommended the surgical retreatment. The cyst was drained, a lamellar keratosclerectomy was performed at the site of the limbus where the epithelial nest was placed, and distilled water was used to wash the corneal cyst - to eliminate the epithelial cells. A donor cornea button was cut manually and was transplanted to cover the area where the corneoscleral tissue was removed., Results: The surgical technique described resulted in nearly complete clearing of the opacity and improved vision, and no recurrence was documented up to 19 months of follow-up. The best-corrected visual acuity improved from 20/100 before surgery to 20/30 after surgery., Conclusions: This surgical technique may be a good alternative to other previously described treatments for intracorneal epithelial cysts aiming to avoid recurrence and without the need for central corneal transplantation., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To characterize trends in procedures, and indications for corneal transplantation over 27 years in a single referral center in Japan., Study Design: Retrospective study., Methods: Data from a consecutive series of corneal transplant surgery cases performed from 1991 to 2017at Ichikawa General Hospital were collected. The surgical procedures, indications, and causes of corneal endothelial dysfunction were evaluated., Results: Among 6,025 transplantations, a total of 3,991 cases underwent penetrating keratoplasty (PKP), 1009 underwent (deep) anterior lamellar keratoplasty, 746 underwent endothelial keratoplasty, and 309 underwent epithelial transplantation. Over that period the frequency of PKP decreased, whereas the frequency of lamellar keratoplasty increased. The four major surgical indications were bullous keratoplasty (BK 30.0%), regraft (24.2%), post-keratitis scars (17.1%), and keratoconus (11.4%). After dividing the study period into four time periods, we found that BK and regraft increased (from 20.3% to 34.5% and 18.2% to 31.1%, respectively), whereas post-keratitis scars and keratoconus decreased (from 26.0% to 9.5% and 14.1% to 7.2%, respectively) between the first and last period. Pseudophakic or aphakic BK were the major causes of corneal endothelial dysfunction until 2010. Cases of laser-iridotomy-induced BK peaked in 35 eyes (34.3% of BK) in 2000 and declined after 2013. Cases of Fuchs endothelial corneal dystrophy increased from 0 in 1991, to 15 eyes (18.5% of BK) in 2017., Conclusion: Lamellar keratoplasty has replaced PKP in our hospital. BK and regraft have increased, whereas post-keratitis scars and keratoconus have decreased. Laser-iridotomy-induced BK has decreased during the past decade, whereas Fuchs endothelial corneal dystrophy has increased., (© 2021. Japanese Ophthalmological Society.)

The purpose of this report is to highlight the early clinical signs and management of ocular surface neoplasia following penetrating keratoplasty in xeroderma pigmentosa. A 14-year-old girl reported increased conjunctival hyperemia, tearing, photophobia in the right eye at 6 months after penetrating keratoplasty that worsened rapidly over the subsequent 2 weeks. Slit lamp examination showed increased vascularity, epithelial haze and raised whitish lesions at the graft-host junction. Alcohol keratoepithelectomy was performed for confirming the diagnosis. Histology of the sample revealed severe dysplasia. The lesion resolved after surface plaque brachytherapy. The graft clarity was restored and maintained until the last follow-up of 15 months following brachytherapy. Exacerbated symptoms and clinical signs of increased vascularisation and epithelial lesions should arouse the suspicion of ocular surface neoplasia in grafted eyes. Early diagnosis and management can salvage the graft clarity., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

There is an international shortage of donor corneas for transplantation to treat the 1.5-2.0 million new cases of blindness secondary to corneal disease. Research has therefore been directed towards the development of artificial corneas using alternative materials such as collagen. The biocompatibility of an acellular collagen-based scaffold for anterior lamellar keratoplasty was investigated in vivo in a rabbit model. This scaffold has previously shown promise as a corneal substitute in vitro. Slit-lamp and Optical Coherence Tomography examinations were carried out at 2 weeks, 1, 2, 3, and 6 months post-operatively. Graft-host integration was investigated using immunohistochemistry of the cornea at 6 months. Results showed that the graft was biocompatible, supported corneal re-epithelialisation, and showed no signs of rejection. Migration of stromal cells into areas of the graft was observed, however this was accompanied by extensive graft digestion. Whilst the scaffold was biocompatible, further modifications to the material or supplementation with matrix metalloproteinase inhibitors are required to bring us closer to a stable and fully integrated corneal substitute., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The authors report a case of a bilateral limbal dermoid in a 4-year-old girl with no extraocular anomalies or systemic associations. The child's best corrected visual acuity was 20/50 in the right eye and 20/40 in the left eye. There was a small limbal dermoid located on the inferotemporal side in the right eye and a large elevated limbal lesion that extended over the visual axis in the left eye. Limbal dermoid excision and lamellar keratoplasty were performed in the left eye and followed by a simple excision of the limbal dermoid in the right eye. Histopathological examination confirmed the clinical diagnosis of a dermoid. At the 2-year follow-up visit, the child's best corrected visual acuity had improved to 20/30 in both eyes. Tumors of the corneal limbus are extremely rare. Simple excision is a viable method of treatment for smaller, superficial lesions, although larger lesions can obstruct the visual axis and produce irregular astigmatism and amblyopia. In this patient, surgical intervention resulted in a reasonable cosmetic and final visual outcome. [ J Pediatr Ophthalmol Strabismus . 2021;58(4):e27-e29.] .

Purpose: Lamellar corneal grafts have revolutionized the management of corneal blindness by replacing only the disease specific corneal layers. To the best of our knowledge, there is no study in literature describing the outcomes of bilateral lamellar keratoplasty in the Indian population. The aim of this work was to study the demographic profile, surgical outcomes, and quality of life in patients who underwent bilateral lamellar keratoplasty and to assess the correlation between these three., Methods: An observational cross-sectional study was conducted on 47 patients who underwent bilateral deep anterior lamellar keratoplasty (DALK) (n = 31) or descemet stripping automated endothelial keratoplasty (DSAEK) (n = 16) with a minimum follow-up of 6 months after the second surgery. Demographic parameters were collected by interview, surgical outcomes by clinical examination, and quality of life by a questionnaire., Results: A total of 47 patients were evaluated, women 42.56%, (n = 20) and men 57.44% (n = 27) with 38.3% being in 18-30 years age group and 23.4% above 60 years; 17.02% patients had a good socioeconomic status (score >60) and 61.70% had poorer socioeconomic status (score <50). Quality of life score (VR-QoL) was ≥50 in 82.9% patients and ≥70 in 14.89%. No significant association existed between VR-QoL scores and demographic factors. However, statistically significant association existed between VR-QoL and age of patient (P < 0.05), postoperative vision (P ≤ 0.05), and contrast sensitivity (P ≤ 0.01)., Conclusion: Bilateral lamellar corneal grafts provide satisfactory visual outcomes compatible with day-to-day functioning. VR-QoL has a direct correlation to the age, visual acuity, contrast sensitivity, and inverse correlation with lenticule thickness in DSAEK and residual bed thickness in DALK., Competing Interests: None

Purpose: Although acellular porcine corneal stroma (APCS) is a promising alternative to the human donor cornea for lamellar keratoplasty, here, we report 2 patients who exhibited persistent epithelial defects and sterile keratolysis after APCS transplantation to treat peripheral corneal diseases., Methods: Two patients with different peripheral corneal diseases underwent lamellar keratoplasty by using D-shaped lamellar APCS as graft materials. Standard keratoplasty postoperative treatments, including topical antibiotic-corticosteroid, tacrolimus, and artificial tears, were applied., Results: Patient 1 was a 7-year-old boy with limbal dermoid, and patient 2 was a 50-year-old man suffered from simultaneous Mooren ulcer with pterygium. Both patients developed persistent graft epithelial defects postoperatively, which were refractory to conventional nonsurgical therapies. The APCS grafts were noted to start sterile keratolysis at approximately 1 month after implantation and were completely dissolved within 3 months, leaving vascularized scars in the previously grafted area., Conclusions: These 2 cases demonstrated that given the high risk of postoperative persistent epithelial defect and sterile keratolysis, the application of APCS in peripheral keratoplasty may need further evaluation.

Integrated intraoperative anterior segment optical coherence tomography is an extremely valuable tool that can be used by corneal surgeons when performing pediatric lamellar corneal transplant surgery. This chapter will demonstrate its use in infants and children with a variety of corneal diseases. These diseases include congenital corneal opacities such as Peters' anomaly (both types I and II), surgical management of limbal dermoids, and its use in selective corneal transplant surgery such as DALK (deep anterior lamellar keratoplasty) or DSEK (Descemet stripping endothelial keratoplasty). These techniques could be used in children with anterior corneal scarring or storage disorders, such as mucopolysaccharidoses, or posterior endothelial disease, such as congenital hereditary endothelial dystrophy or posterior polymorphous dystrophy. This chapter will demonstrate the author's experience using this device during anterior segment corneal surgery., (© 2021 S. Karger AG, Basel.)

A corneal descemetocele, the anterior herniation of an intact Descemet membrane through an overlying stromal defect, is a rare, but serious outcome of progressive corneal ulceration and mandates urgent intervention owing to the imminent risk of perforation. Various ocular and systemic abnormalities that can lead to the formation of descemetocele include microbial keratitis, neurotrophic keratopathy, dry eye disorders, and corneal inflammation associated with immune-mediated disorders. The primary aim of management of a descemetocele remains prompt restoration of ocular integrity to prevent the rupture of the Descemet membrane and further complications. Medical therapy is instituted immediately while deciding on the most suitable operative modality for an individual case. Commonly available treatment options include therapeutic bandage contact lenses, tissue adhesives, amniotic membrane transplantation, corneal patch grafts, penetrating or lamellar keratoplasty, and conjunctival flaps. Infrequently, platelet-rich fibrin membrane grafting and umbilical cord patch transplantation have also been tried with success. The surgical strategy and the outcome are commonly determined by the size, location, and etiology of descemetoceles. Despite the availability of all these treatment options, ambiguity remains about management. We review the available literature on pathogenesis, causes, presentation, differential diagnoses, and management of this disorder and also discuss our experience., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Persistent corneal graft erosion or persistent epithelial corneal defect is a frequent complication of penetrating keratoplasty. Its development can be contributed by the dry eye syndrome, rare blinking, lagophthalmos, symblepharon, viral infection, autoimmune aggression, and the use of epithelial-toxic eye drops. The article presents three clinical observations of patients who developed persistent corneal graft erosion after penetrating keratoplasty. Due to the ineffectiveness of local conservative therapy for more than 3 weeks, anterior stromal corneal micropuncture was performed. After the procedure, there was a gradual epithelial proliferation, complete healing of the corneal surface was observed 10-16 days after the manipulation, the follow-up period was at least 1 year. The mechanism of action of stromal micropuncture is associated with the creation of a porous surface with better adhesion properties, as well as with the activation of the production of extracellular matrix glycoproteins such as fibronectin, type IV collagen and laminin, which are necessary for stable adhesion of the epithelium. The use of stromal micropuncture of the donor flap in the treatment of post-keratoplasty persistent corneal epithelial defect was proposed for the first time.

Background: Hurler syndrome-associated keratopathy is an exceedingly rare corneal disorder that requires corneal transplantation in advanced stages. Precise assessment of the corneal condition is necessary for deciding which type of keratoplasty (i.e., deep anterior lamellar or penetrating) should be proposed. We aimed to confront the results of multimodal imaging with those of histology in a case of Hurler syndrome-associated keratopathy., Case Presentation: A 16-year-old patient with Hurler's syndrome treated with hematopoietic stem cell transplantation was referred for decreased vision related to advanced keratopathy. The patient was treated with deep anterior lamellar keratoplasty (DALK) in both eyes with uncomplicated outcome. Visual acuity improved from 0.1 (20/200) preoperatively to 0.32 (20/63) and 0.63 (20/32) after transplantation. The corneal endothelial cell density was 2400 cells/mm 2 in both eyes 3 years after transplantation. In vivo confocal microscopy (IVCM) and spectral domain optical coherence tomography (SD-OCT) were performed preoperatively. The corneal buttons retrieved during keratoplasty were processed for histology. In SD-OCT scans, corneal opacities appeared as diffuse stromal hyperreflectivity associated with increased corneal thickness. IVCM showed diffuse cytoplasmic granular hyperreflectivity and rounded/ellipsoid aspects of keratocytes, presence of small intracellular vacuoles, and hyperreflective epithelial intercellular spaces. Bowman's layer was thin and irregular. The corneal endothelium was poorly visualized but no endothelial damage was observed. Histology showed irregular orientation and organization of stromal lamellae, with the presence of macrophages whose cytoplasm appeared clear and granular. A perinuclear clear halo was visible within the epithelial basal cells. Bowman's layer featured breaks and irregularities., Conclusions: The observed corneal multimodal imaging features in mucopolysaccharidosis-related keratopathy were concordant with histology. Compared with standard histology, multimodal imaging allowed additional keratocyte features to be observed. It revealed both morphological and structural changes of all corneal layers but the endothelium. This information is essential for therapeutic management which should include DALK as the first-choice treatment in case of impaired visual acuity.

Purpose: To compare the long-term outcomes of living-related conjunctival limbal allograft (lr-CLAL) with keratolimbal allograft (KLAL) in patients with limbal stem cell deficiency., Methods: A retrospective, comparative, interventional cohort of patients with bilateral total limbal stem cell deficiency who underwent surgical treatment with a KLAL or lr-CLAL procedure alone (not combined with any other ocular surface stem cell transplantation procedures) with a minimum follow-up of 1 year and who received systemic immunosuppression. Ocular surface stability, best-corrected visual acuity (BCVA), and postoperative complications at the last follow-up were the main outcome measures., Results: There were 224 eyes that underwent KLAL alone and 63 eyes that underwent lr-CLAL alone, with a mean follow-up time for all eyes of 7.2 years (range 1.0-16.0 years). For lr-CLAL eyes, 82.5% maintained a stable ocular surface compared with 64.7% of KLAL eyes at the last follow-up. Only 6.3% of lr-CLAL eyes demonstrated a failed ocular surface compared with 15.6% of KLAL eyes. The mean BCVA was 20/158 for KLAL eyes compared with 20/100 for lr-CLAL eyes at the last follow-up. A smaller proportion of lr-CLAL eyes (30.2% compared with 43.3%) developed an episode of acute rejection, and a higher proportion of these episodes resolved with treatment in the lr-CLAL group (79.0% compared with 53.6%)., Conclusions: lr-CLAL demonstrates lower rejection rates, improved graft survival, and better BCVA compared with KLAL. Both careful preoperative donor selection and triple-agent systemic immunosuppression (including tapered systemic corticosteroids) are critical to optimizing the ocular surface stem cell transplantation outcomes.

Introduction: Penetrating keratoplasty has become one of the most commonly performed transplant procedures where full thickness diseased host corneal tissue is replaced with healthy donor corneal tissue. Aims of penetrating keratoplasty include improvement in visual acuity, removal of infectious load, alleviate pain or even to simply save the anatomy of the eye. Visual improvement and removal of infection is the prime objective of majority of corneal grafts. The objective of the study was to evaluate various current indications of penetrating keratoplasty in central Nepal and its difference from the developed world., Material and Methods: A hospital based study of 36 patients who underwent penetrating keratoplasty between January 2014 and June 2015 was carried out at B.P. Koirala Centre for ophthalmic studies. Following clinical examination and investigations, surgical intervention was done when required. The patient data were recorded and followed up. Indication for surgery, investigations and demographic profile were assessed., Results: Mean recipient age was 46.56 years (+/- 16.02 years). The male: female ratio was 1.57:1. The most common indication for penetrating keratoplasty was infectious keratitis (44.44%), followed by corneal opacity or scarring (30.56 %), re-grafts (13.89%),bullous keratopathy (2.78%), keratoconus (2.78%) and others (2.78%) Introduction: Penetrating keratoplasty has become one of the most commonly performed transplant procedures where full thickness diseased host corneal tissue is replaced with healthy donor corneal tissue. Aims of penetrating keratoplasty include improvement in visual acuity, removal of infectious load, alleviate pain or even to simply save the anatomy of the eye. Visual improvement and removal of infection is the prime objective of majority of corneal grafts. The objective of the study was to evaluate various current indications of penetrating keratoplasty in central Nepal and its difference from the developed world., Material and Methods: A hospital based study of 36 patients who underwent penetrating keratoplasty between January 2014 and June 2015 was carried out at B.P. Koirala Centre for ophthalmic studies. Following clinical examination and investigations, surgical intervention was done when required. The patient data were recorded and followed up. Indication for surgery, investigations and demographic profile were assessed., Results: Mean recipient age was 46.56 years (+/- 16.02 years). The male: female ratio was 1.57:1. The most common indication for penetrating keratoplasty was infectious keratitis (44.44%), followed by corneal opacity or scarring (30.56 %), re-grafts (13.89%),bullous keratopathy (2.78%), keratoconus (2.78%) and others (2.78%) Conclusion: Though corneal diseases and indications for transplant surgery in developing countries are different from those in the western world, penetrating keratoplasty has emerged as one of the most successful way to reduce corneal blindness and to restore vision., Conclusion: Though corneal diseases and indications for transplant surgery in developing countries are different from those in the western world, penetrating keratoplasty has emerged as one of the most successful way to reduce corneal blindness and to restore vision., (© NEPjOPH.)

Purpose: To report the outcomes of corneal transplantation utilizing corneas retrieved from donors with chronic kidney disease (CKD)., Methods: Outcomes of corneal transplantation (optical PK and EK) performed from Jan 2018 to Dec 2018 utilizing donor corneas retrieved from CKD patients was performed retrospectively., Results: Of the total of 233 donor corneas retrieved from CKD, 135 (57.9%) were utilized for transplantation after the routine screening protocol of the eye bank. Mean age of the donors was 56.2 ± 13.5 years. The mean endothelial cell density on specular microscopy of the donor corneas used for optical PK was 2685.7 ± 377.6 cells/mm 2 (range, 2028-3448 cells/mm 2 ) and for EK was 2731.7 ± 189.1 cells/mm 2 (range, 2380-3194 cells/mm 2 ). The overall primary graft failure rate was 5.1%. All grafts except 1, cleared in the PK group. In the EK group (6 DMEK and 16 DSAEK), 1 patient had a complete graft detachment and another 1 had a primary graft failure after DMEK., Conclusion: The donor corneas retrieved from chronic kidney disease patients are safe and suitable for optical keratoplasty provided they meet the criteria for transplantation., Competing Interests: None

A 3-month-old baby presented with a whitish lesion over the right cornea since birth. Due to the clinical and ultrasonic characteristics of the lesion, a manual anterior lamellar keratoplasty was performed. Histopathological examination showed it to be a simple grade II epibulbar choristoma. Although dermoid tumours are usually considered as benign, some of them, depending on their location, can seriously affect the vision of a paediatric patient. Due to the high probability of amblyopia in these cases, a surgical procedure is mandatory. Anterior lamellar keratoplasty is recommended when the visual axis is compromised without intraocular implications., (Copyright © 2019. Publicado por Elsevier España, S.L.U.)

Dysfunction of the corneal endothelium reduces the transparency of the cornea and can cause blindness. Because corneal endothelial cells have an extremely limited proliferative ability in vivo, treatment for corneal endothelial dysfunction involves the transplantation of donor corneal tissue. Corneal endothelium can also be restored via intraocular injection of endothelial cells in suspension after their expansion in vitro. Yet, because quality assessment during the expansion of the cells is a destructive process, a substantial number of the cultured cells are lost. Here, we show that the 'spring constant' of the effective interaction potential between endothelial cells in a confluent monolayer serves as a biomarker of the quality of corneal endothelial cells in vitro and of the long-term prognosis of corneal restoration in patients treated with culture-expanded endothelial cells or with transplanted corneas. The biomarker can be measured from phase contrast imaging in vitro and from specular microscopy in vivo, and may enable a shift from passive monitoring to pre-emptive intervention in patients with severe corneal disorders.

Competing Interests: There are no conflicts of interest.

Background: To investigate the transparency, biocompatibility, and safety of human-derived acellular dermal matrix for application in corneal stromal transplantation., Methods: Twenty-four patients (24 eyes) with pellucid marginal corneal degeneration were enrolled, and intrastromal keratoplasty was performed with human-derived acellular dermal matrix. The ocular symptoms and signs as well as graft characteristics were evaluated at baseline and at 1 day, 1 week, and 1, 3, and 6 months postoperatively. Photography by a slit lamp, topography by Pentacam, anterior segment-optical coherence tomography, and corneal confocal microscopy were conducted at baseline and during the follow-up period., Results: Postoperative discomfort was relieved during the follow-up period. No abnormal ocular signs were observed at 6 months, indicating the safety of the procedure. Desirable and improved transparency of the grafts was demonstrated, and all the grafts healed without dissolution or fall at 6 months postoperatively. Reepithelization was completed, and confocal microscopy revealed that keratocytes and nerves repopulated in all the grafts at 6 months postoperatively. The thinning of the marginal corneal stroma was eliminated following the transplantation, and the curvature and corneal regularity remained stable at 6 months compared with baseline., Conclusions: The present study demonstrated the transparency, biocompatibility, and safety of human-derived acellular dermis matrix in intrastromal keratoplasty. With further improvements, human-derived acellular dermis matrix could be applied in central lamellar keratoplasty and ultimately solve the shortage of donor grafts.

Purpose: Corneal melting with perforation is a severe ophthalmic complication of autoimmune disorders such as rheumatoid arthritis. It requires urgent medical management in order to maintain the integrity of the globe and preserve vision. Treating this complication by penetrating keratoplasty is problematic due to the high rate of recurrence of corneal melting as well as other complications. We describe the use of a tectonic fresh-tissue corneolimbal covering graft., Methods: An interventional case series including three patients that presented to our tertiary center between 2000 and 2015 with corneal melting and perforation, secondary to rheumatoid arthritis. Emergency surgery included suturing of a 13.00- to 13.50-mm full-thickness fresh-tissue corneolimbal covering graft to the patient's posterior limbal zone., Results: The corneolimbal graft maintained the integrity of the cornea in all cases, by sealing the perforation and promoting the creation of a fibrovascular scar at the area of corneal melting. There were no complications, recurrences of host corneal melting, or perforation during the follow-up period., Conclusion: Fresh-tissue full-thickness corneolimbal grafts may be used to cover emergency corneal melting and perforations secondary to rheumatoid arthritis.

In France, endothelial dysfunction represents approximately one half of the indications for corneal transplants performed each year. However, the use of endothelial keratoplasty is limited by the technical difficulty of the procedure, a shortage of available grafts, and the potential for graft failure or rejection. These limitations are driving researchers to develop new, less invasive, and more effective therapies. Corneal endothelial cell therapy is being explored as a potential therapeutic measure, to avoid the uncertainty associated with grafting. The human cornea is an ideal tissue for cell therapy. Due to its avascular and immunologically privileged characteristics, transplanted cells are better tolerated compared with other vascularized tissues and organs. Advances in the field of stem cell engineering, particularly the development of corneal epithelial stem cell therapy for the treatment of severe ocular surface disease, have aroused a massive interest in adapting cell therapy techniques to corneal endothelial cells. This chapter, based on a review of the literature, aims at educating the reader on the latest research in the field of corneal endothelial cell therapy., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Purpose: To evaluate the biomechanical properties of the cornea and their impact on intraocular pressure (IOP) measurement after lamellar keratoplasty, compared to healthy eyes, using a non-contact tonometer with a Scheimpflug camera., Methods: This study, from 2014 to 2015, included 22 primary DSAEK, 5 DALK, 6 DSAEK after PK, and 50 control eyes. Using a non-contact tonometer with a high-speed Scheimpflug camera (CORVIS ST, Oculus Optikgeräte GmbH, Wetzlar, Germany), several biomechanical parameters were recorded, including radius at highest concavity (R hc ) and defomation amplitude (DA). Central corneal thickness (CCT) and uncorrected IOP, were also recorded. For the control eyes only, a corrected IOP was calculated, based on age, central corneal thickness, and biomechanical parameters., Results: R hc was significantly lower after DALK (R hc =5.54±0.71, P=0.007) and DSAEK (R hc =6.26±0.77, P=0.042) compared to control eyes (R hc =6.82±0.76). DA was higher after DALK and DSAEK, but not significantly (respectively 1.24±0.09 P=0.41 and 1.22±0.15, P=0.923) compared to normal eyes (1.18±0.15). Uncorrected IOP was not significantly different between post-keratoplasty and control eyes. In control eyes, the corrected IOP (15.23±1.88) was lower than the uncorrected IOP (16.10±2.34); a statistically significant positive correlation between R hc and CCT (R 2 =0.6020, P<0001), and a significant negative correlation between DA and CCT (R 2 =-0.641, P<0.0001) were found., Conclusion: Our study showed that, after lamellar keratoplasty, corneal biomechanics are altered. Corneas with higher ocular rigidity will show a lower DA and a higher R hc ., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)