Monitoring the transition from corneal ulceration to healed scar using a Scheimpflug tomography-based densitometry.

PURPOSE: To compare corneal haze between active ulcer and healed scarring using a Scheimpflug densitometry. MATERIALS AND METHODS: A prospective longitudinal study enrolled 30 patients (30 eyes) with ulcerative keratitis (UK). Each subject's corneal optical density (COD) was measured with a Scheimpflug corneal densitometry, Pentacam® AXL (Oculus GmbH, Wetzlar, Germany), at the active ulcerative and complete scarring stage. The COD data were analyzed through distinct methods (inbuilt, sorted annular partitions, and ulcer-matching densitometric maps). We compared different CODs to select the better index for clinically monitoring the transition from corneal ulceration to healed scar. RESULTS: The CODs of the periphery (P = 0.0024) and outside of the active ulcer (P = 0.0002) significantly decreased after scarring. Partitioning the cornea into different depths and annular zones, the anterior layer, center layer, and the 2-6 mm annular zone had a more remarkable COD decrease after scar formation. The 3rd-sorted COD in the anterior layer revealed the highest area under the receiver-operating characteristic curves (0.709), in which 90% of subjects had COD reduction during the ulcer-to-scar transition. CONCLUSIONS: Aside from subjective judgment based on clinical signs, the Scheimpflug tomography-based densitometry could provide objective and efficient monitoring of the corneal opacity evolution in UK patients. Because the 3rd-sorted annular COD is a better index than the inbuilt or mapping CODs in differentiating active ulcers from healed scars, this COD could be a clinically promising parameter to monitor the progression of UK patients.

Recurrent De Novo NAHR Reciprocal Duplications in the ATAD3 Gene Cluster Cause a Neurogenetic Trait with Perturbed Cholesterol and Mitochondrial Metabolism.

Recent studies have identified both recessive and dominant forms of mitochondrial disease that result from ATAD3A variants. The recessive form includes subjects with biallelic deletions mediated by non-allelic homologous recombination. We report five unrelated neonates with a lethal metabolic disorder characterized by cardiomyopathy, corneal opacities, encephalopathy, hypotonia, and seizures in whom a monoallelic reciprocal duplication at the ATAD3 locus was identified. Analysis of the breakpoint junction fragment indicated that these 67 kb heterozygous duplications were likely mediated by non-allelic homologous recombination at regions of high sequence identity in ATAD3A exon 11 and ATAD3C exon 7. At the recombinant junction, the duplication allele produces a fusion gene derived from ATAD3A and ATAD3C, the protein product of which lacks key functional residues. Analysis of fibroblasts derived from two affected individuals shows that the fusion gene product is expressed and stable. These cells display perturbed cholesterol and mitochondrial DNA organization similar to that observed for individuals with severe ATAD3A deficiency. We hypothesize that the fusion protein acts through a dominant-negative mechanism to cause this fatal mitochondrial disorder. Our data delineate a molecular diagnosis for this disorder, extend the clinical spectrum associated with structural variation at the ATAD3 locus, and identify a third mutational mechanism for ATAD3 gene cluster variants. These results further affirm structural variant mutagenesis mechanisms in sporadic disease traits, emphasize the importance of copy number analysis in molecular genomic diagnosis, and highlight some of the challenges of detecting and interpreting clinically relevant rare gene rearrangements from next-generation sequencing data.

Cornea opacity, uveitis with iris atrophy and lens damage following cosmetic high-intensity ultrasound of the eyelid: a case report.

BACKGROUND: High-intensity focused ultrasound (HIFU) is a cosmetic procedure that aims to tone the skin through thermal collagen coagulation. The energy is delivered in the deep layers of the skin, and because of these characteristics, the risks of severe damage to adjacent tissue and the ocular surface may be underestimated. Previous reports have demonstrated superficial corneal opacities, cataracts, increased intraocular pressure, or ocular refractive changes in different patients following HIFU. In this case, we report deep stromal opacities associated with anterior uveitis, iris atrophy and lens opacity formation following a single HIFU superior eyelid application. CASE PRESENTATION: A 47-year-old female presented to the ophthalmic emergency department complaining of pain, hyperemia and photophobia in the right eye following a HIFU application to the superior right eyelid. A slit lamp examination showed three temporal-inferior corneal infiltrates with edema and severe anterior uveitis. The patient was treated with topical corticosteroids, and six months later, there was residual corneal opacity, iris atrophy and peripherical cataract formation. No surgical procedure was needed, and the final vision was Snellen 20/20 (1.0). CONCLUSION: The risk of severe impairment to the ocular surface and ocular tissues may be underestimated. Cosmetic surgeons and ophthalmologists must be aware of the complications, and the long-term follow-up of these changes needs further investigation and discussion. Safety protocols of the HIFU intensity threshold for thermal lesions in the eye and the use of protective eye devices should be better evaluated.

[PETERS ANOMALY AND PETERS PLUS SYNDROME].

INTRODUCTION: Peters anomaly is characterized by a defect in the development of the anterior segment of the eye during fetal development (Anterior segment dysgenesis). This anomaly presents a broad clinical presentation ranging from minimal peripheral corneal opacity to extensive adhesions of the iris and lens with dense central corneal opacity that impairs vision. Peters Plus Syndrome is a recessive autosomal syndrome manifested by Peters anomaly, along with systemic disorders such as brachydactyly (short fingers and toes), short stature, a developmental delay, dysmorphic facial features, and may accompanied with heart and genitourinary malformations. The most common sign of Peters' anomaly is corneal opacity that appears at birth. This opacity can cause blockage of the central visual axis and cause the development of a deprivational amblyopia. In addition, the patient may suffer from glaucoma due to malformations in the angle structures as well as a shallow anterior chamber. Treatments are aimed at clearing the central visual axis as soon as possible in order to allow the visual system to mature and to avoid the development of amblyopia. Full-thickness corneal transplantation combined with Cataract surgery if necessary is the current standard of care. Optical iridoplasty is a milder surgical alternative in cases where the corneal opacity is not significant.

Anterior synechiae after penetrating keratoplasty in infants and children with Peters' anomaly.

BACKGROUND: To evaluate anterior synechiae after penetrating keratoplasty (PK) in patients with Peters' anomaly using anterior segment optical coherence tomography (OCT). METHODS: A retrospective cross-sectional study was performed. The medical records of patients diagnosed with Peters' anomaly who underwent PK between 2013 and 2018 were reviewed. In addition to basic ophthalmic examinations, images of anterior segment structures were obtained via spectral-domain OCT at baseline and during the postoperative follow-up period. The profiles of postoperative anterior synechiae and multiple potential risk factors were analyzed. RESULTS: Seventy-one eyes of 58 patients, aged 5 to 23 months, were included. Various extent of postoperative anterior synechiae was observed in 59 eyes (83.1%). OCT findings revealed graft-host junction synechiae, peripheral anterior synechiae, and a combination of both. Disease severity and malposition of the internal graft-host junction were significantly associated with the formation of postoperative synechiae. Multivariate regression analysis found that preexisting iridocorneal adhesion [odds ratio (OR) = 16.639, 95% confidence interval (CI) 1.494-185.294, p = 0.022] was positively correlated with postoperative anterior synechiae, whereas anterior chamber depth (OR = 0.009, 95% CI 0.000-0.360, p = 0.012) and graft size (OR = 0.016, 95% CI 0.000-0.529, p = 0.020) were negatively correlated with postoperative synechiae. In addition, quadrants of preexisting iridocorneal adhesion and width of the host corneal bed were identified as risk factors for increased postoperative anterior synechiae. CONCLUSIONS: Anterior synechiae following PK is a relatively common occurrence in Peters' anomaly patients and is found to be associated with preexisting iridocorneal adhesion, a shallow anterior chamber, small graft size, graft-host junction malposition, and graft closer to the corneal limbus. These data indicate the need for careful consideration when performing PK on these patients.

Thygeson Superficial Punctate Keratitis: A Clinical and Immunologic Review.

ABSTRACT: Thygeson superficial punctate keratitis (TSPK) is clinically characterized by exacerbations and remissions of gray-white opacities within the corneal epithelium, most often bilateral but may be asymmetric. Symptoms typically include photophobia, tearing, blurring, and eye irritation. Although disease progression and prognosis are well described, the exact cause is unknown. Hypotheses exist implicating virus-mediated immunity as the cause of TSPK following cases of viral keratitis; however, several polymerase chain reaction studies refute the infectious process concurrently with symptomatic TSPK. This is further supported by the consistent lack of response to antiviral and antibacterial treatment. A subset of dendritic cells known as Langerhans cells (LC) found within the corneal epithelium has been positively correlated with exacerbations of TSPK. Langerhans cells proliferate to protect and mitigate the cornea's inflammatory response, but the inflammatory triggers and relapses associated with TSPK are not well understood. Several topical drugs exist to treat inflammation related to TSPK; however, drug delivery is a major barrier to treatment because of the tear film and epithelial barrier. Drug-eluting contact lenses that target intermediates of inflammation could serve as a more effective treatment modality because of the increased bioavailability of the drugs. This review is an in-depth survey of the literature regarding the relationship between the origin and pathophysiology of LC and TSPK at the immunologic level. We also discuss potential pharmacotherapeutic interventions for TSPK prevention and treatment.

Extracellular matrix changes in corneal opacification vary depending on etiology.

Purpose: The purpose of this study is to examine the expression of tenascin-C and matrilin-2 in three different disorders, which frequently require corneal transplantation. These pathological conditions include bullous keratopathy (BK), Fuchs' endothelial corneal dystrophy (FECD), and corneal scarring in herpetic keratitis. Methods: Histological sections of corneal buttons removed during keratoplasty were analyzed in BK (n = 20), FECD (n = 9), herpetic keratitis (n = 12), and cadaveric control (n = 10) groups with light microscopy following chromogenic immunohistochemistry. The sections were evaluated by three investigators, and semiquantitative scoring (0 to 3+) was applied according to standardized methods at 400X magnification. Each layer of the cornea was investigated; moreover, the stroma was subdivided into subepithelial, middle, and pre-Descemet's membrane areas for more detailed analysis. Results: Excessive epithelial and stromal expression of tenascin-C was identified in all investigated conditions; the results were most pronounced in the pre-Descemet's membrane. Regarding matrilin-2, when examined in BK, there was increased labeling intensity in the epithelium (p<0.001) and stromal layers (p<0.05), and a decrease in the endothelium (p<0.001). In the other investigated conditions, only a low degree of stromal localization (p<0.05) of matrilin-2 was detected. Conclusions: The expression of tenascin-C and matrilin-2 differs when examined in various corneal pathologies resulting in opacification. Both molecules seem to be involved in regeneration and wound healing of the corneal matrix in these diseases.

An immune response to the avascular lens following wounding of the cornea involves ciliary zonule fibrils.

The lens and central cornea are avascular. It was assumed that the adult lens had no source of immune cells and that the basement membrane capsule surrounding the lens was a barrier to immune cell migration. Yet, microfibril-associated protein-1 (MAGP1)-rich ciliary zonules that originate from the vasculature-rich ciliary body and extend along the surface of the lens capsule, form a potential conduit for immune cells to the lens. In response to cornea debridement wounding, we find increased expression of MAGP1 throughout the central corneal stroma. The immune cells that populate this typically avascular region after wounding closely associate with this MAGP1-rich matrix. These results suggest that MAGP1-rich microfibrils support immune cell migration post-injury. Using this cornea wound model, we investigated whether there is an immune response to the lens following cornea injury involving the lens-associated MAGP1-rich ciliary zonules. Our results provide the first evidence that following corneal wounding immune cells are activated to travel along zonule fibers that extend anteriorly along the equatorial surface of the lens, from where they migrate across the anterior lens capsule. These results demonstrate that lens-associated ciliary zonules are directly involved in the lens immune response and suggest the ciliary body as a source of immune cells to the avascular lens.

Loss of Down Syndrome Critical Region-1 Mediated-Hypercholesterolemia Accelerates Corneal Opacity Via Pathological Neovessel Formation.

OBJECTIVE: The calcineurin-NFAT (nuclear factor for activated T cells)-DSCR (Down syndrome critical region)-1 pathway plays a crucial role as the downstream effector of VEGF (vascular endothelial growth factor)-mediated tumor angiogenesis in endothelial cells. A role for DSCR-1 in different organ microenvironment such as the cornea and its role in ocular diseases is not well understood. Corneal changes can be indicators of various disease states and are easily detected through ocular examinations. Approach and Results: The presentation of a corneal arcus or a corneal opacity due to lipid deposition in the cornea often indicates hyperlipidemia and in most cases, hypercholesterolemia. Although the loss of Apo (apolipoprotein) E has been well characterized and is known to lead to elevated serum cholesterol levels, there are few corneal changes observed in ApoE-/- mice. In this study, we show that the combined loss of ApoE and DSCR-1 leads to a dramatic increase in serum cholesterol levels and severe corneal opacity with complete penetrance. The cornea is normally maintained in an avascular state; however, loss of Dscr-1 is sufficient to induce hyper-inflammatory and -oxidative condition, increased corneal neovascularization, and lymphangiogenesis. Furthermore, immunohistological analysis and genome-wide screening revealed that loss of Dscr-1 in mice triggers increased immune cell infiltration and upregulation of SDF (stromal derived factor)-1 and its receptor, CXCR4 (C-X-C motif chemokine ligand receptor-4), potentiating this signaling axis in the cornea, thereby contributing to pathological corneal angiogenesis and opacity. CONCLUSIONS: This study is the first demonstration of the critical role for the endogenous inhibitor of calcineurin, DSCR-1, and pathological corneal angiogenesis in hypercholesterolemia induced corneal opacity.

Two-step keratoplasty for deep vascularized corneal opacity with severe peripheral thinning following Acanthamoeba keratitis.

PURPOSE: In cases of corneal opacity with vascularization and peripheral thinning, traditional keratoplasty techniques have several risks and drawbacks. We report the results of a two-step surgical strategy consisting in performing a large diameter tectonic lamellar keratoplasty (TLK) to restore appropriate corneal thickness and an avascular recipient bed, followed by central optical PK within the lamellar graft at a later date. METHODS: This single-institution study analyzes the results of 7 eyes of 7 patients who received PK after large diameter TLK. All patients were affected by deep post-infectious corneal opacity with persistent stromal vascularization and peripheral thinning. The main outcomes measured were graft survival, visual acuity, refraction, and endothelial cell density. RESULTS: TLK was performed in all cases with 10/10.1-mm diameters. After a mean interval of 14 months, central PK was performed with a median host-graft diameter of 7.75/8.25 mm. Mean follow-up after PK was 52 months. At last follow-up, 6/7 (86%) grafts were clear. Endothelial rejection occurred in 5/7 (71%) eyes, with one patient having multiple episodes and subsequent graft failure. At 2 years, all patients had a visual acuity ≥ 20/40, with an average refractive astigmatism of 3.75 diopters. CONCLUSION: Optical PK within a previous TLK is a safe and efficient technique for treating deep corneal opacity associated with extensive vascularization and peripheral thinning. Extended follow-up period is necessary to assess endothelial cell loss and long-term efficacy of the procedure.

Transgenic Mice Overexpressing PG1 Display Corneal Opacity and Severe Inflammation in the Eye.

Antimicrobial peptides (AMPs) are of interest as alternatives to antibiotics or immunomodulators. We generated and characterized the phenotypes of transgenic mice overexpressing protegrin 1 (PG1), a potent porcine cathelicidin. No obvious differences were observed between PG1 transgenic and wild-type mice in terms of growth, development, general behaviour, and the major immune cell population. However, PG1 transgenic mice intranasally infected with Staphylococcus aureus resulted in a reduction in microscopic pulmonary injury, improved clearance of bacteria, and lower proinflammatory cytokine secretion, compared to those of wild-type mice. On the other hand, approximately 25% of PG1 transgenic mice (n = 54/215) showed corneal opacity and developed inflammation in the eye, resulting ultimately in phthisis bulbi. Immunohistochemical analyses revealed that PG1 and its activator, neutrophil elastase, localized to the basal cells of the cornea and glands in eyelids, respectively. In addition, apoptosis indicated by a Terminal deoxynucleotidyl transferase dUTP nick end labelling (TUNEL)-positive signal was detected from flat cells of the cornea. Our study suggests that the expression regulation or localization of AMPs such as PG1 is important to prevent their adverse effects. However, our results also showed that the cytotoxic effects of PG1 on cells could be tolerated in animals, except for the eyes.

Visual rehabilitation by using corneal wavefront-guided transepithelial photorefractive keratectomy for corneal opacities after epidemic keratoconjunctivitis.

PURPOSE: To evaluate long-term visual and refractive outcomes of corneal wavefront-guided transepithelial photorefractive keratectomy (t-PRK) with mitomycin C for the treatment of corneal opacities secondary to adenoviral epidemic keratoconjunctivitis. METHODS: Records of patients who underwent corneal wavefront-guided t-PRK with excimer laser from January 2012 to December 2018 were retrospectively reviewed. Preoperative and postoperative uncorrected visual acuity, best-spectacle corrected visual acuity, slit-lamp biomicroscopic examination findings, manifest refraction, and corneal aberrations and fundus examination findings were evaluated. RESULTS: Twenty-two eyes of 22 patients comprising 12 male (55%) and 10 female (45%) were treated. The mean age was 34.5 ± 10.8 years (range 19-55). The mean follow-up time was 34.4 ± 17.50 months (range 13-61 months). There was a statistically significant improvement in UCVA and BSCVA (p < 0.001 and p = 0.02), and there was a significant decrease in total higher-order aberrations, spherical, coma and trefoil aberration at postoperative first year (p < 0.001 in each). In two eyes of two patients, minimal haze formation was observed after the procedure, and both eyes were treated with topical steroid. No recurrence was observed in subepithelial infiltrates in any patient during long-term follow-up. CONCLUSION: In long-term clinical follow-up, corneal wavefront-guided t-PRK treatment is an effective and reliable treatment method for rehabilitation of visual impairment due to corneal scars following adenoviral infections, in properly selected patients.

Biological effects of mitomycin C on late corneal haze stromal fibrosis following PRK.

This review details the current understanding of the mechanism of action and corneal effects of mitomycin C (MMC) for prophylactic prevention of stromal fibrosis after photorefractive keratectomy (PRK), and includes discussion of available information on dosage and exposure time recommended for MMC during PRK. MMC is an alkylating agent, with DNA-crosslinking activity, that inhibits DNA replication and cellular proliferation. It acts as a pro-drug and requires reduction in the tissue to be converted to an active agent capable of DNA alkylation. Although MMC augments the early keratocyte apoptosis wave in the anterior corneal stroma, its most important effect responsible for inhibition of fibrosis in surface ablation procedures such as PRK is via the inhibition of mitosis of myofibroblast precursor cells during the first few weeks after PRK. MMC use is especially useful when treating eyes with higher levels of myopia (≥approximately 6 D), which have shown higher risk of developing fibrosis (also clinically termed late haze). Studies have supported the use of MMC at a concentration of 0.02%, rather than lower doses (such as 0.01% or 0.002%), for optimal reduction of fibrosis after PRK. Exposure times for 0.02% MMC longer than 40 s may be beneficial for moderate to high myopia (≥6D), but shorter exposures times appear to be equally effective for lower levels of myopia. Although MMC treatment may also be beneficial in preventing fibrosis after PRK treatments for hyperopia and astigmatism, more studies are needed. Thus, despite the clinical use of MMC after PRK for nearly twenty years-with limited evidence of harmful effects in the cornea-many decades of experience will be needed to exclude late long-term effects that could be noted after MMC treatment.

Femtosecond laser-assisted cataract surgery in a patient with traumatic cataract and corneal opacity after LASIK: a case report.

BACKGROUND: Femtosecond laser-assisted cataract surgery (FLACS) has been reported to reduce phacoemulsification time and energy compared to the manual phacoemulsification technique. This technique has been used in several complex cases such as zonular weakness, subluxated lens and traumatic cataracts because it causes less damage to weakened zonules. However, corneal opacity is considered a relative contraindication to FLACS, as it may interfere with laser beam delivery, thus causing unpredictable capsulorhexis and lens fragmentation/liquefaction. CASE PRESENTATION: We present here a case with traumatic cataract and corneal opacity after laser-assisted in situ keratomileusis (LASIK). The patient was successfully treated using FLACS, capsular tension ring and intraocular lens (IOL) implantation. Posterior capsule rupture and vitreous loss were noted during the operation. However, the intraocular lens was successfully captured because of a complete capsulorhexis performed by FLACS. CONCLUSION: This case report demonstrates that FLACS is a useful tool in selected patients with concurrent corneal opacity and traumatic cataract.

The 3-year outcomes of corneal tattooing for severely disfigured corneas.

BACKGROUND: To evaluate the efficacy of corneal tattooing for various clinical applications. METHODS: The medical charts of 62 eyes of 62 patients who underwent corneal tattooing between March 2016 and August 2017 were retrospectively reviewed. The causes of opacity and various methods of corneal tattooing were analyzed. RESULTS: Among our 62 patients, 38 were males and 24 were females. Average age was 48.47 ± 15.30 (range, 12-74) years old. The mean follow-up period was 40.19 ± 2.34 (range, 36-43) months. The most common causes of corneal opacity were ocular trauma (33 eyes, 53.2%), prior retinal surgery (9 eyes, 14.5%), congenital etiologies (8 eyes, 12.9%) and measles (5 eyes, 8.0%). The most common associated ocular findings were strabismus (23 eyes, 37.0%), phthisis bulbi (17 eyes, 27.4%) and band keratopathy (13 eyes, 20.9%). Cosmetic outcomes improved without serious complications in all cases. CONCLUSIONS: Corneal tattooing is a viable option with an expanding set of indications, such as discolored previous corneal tattoos, white pupil due to inoperable cataract with clear cornea and dense corneal opacities in blind eyes. Elective corneal tattooing seems to be a viable and convenient method to improve cosmesis with minimal complications and high patient satisfaction.

Ophthalmological findings in Gaucher disease.

Gaucher disease is an autosomal recessive lysosomal storage disorder caused by mutations in the gene GBA1, which encodes the lysosomal protein glucocerebrosidase. Patients with Gaucher disease generally have a variety of clinical manifestations ranging from visceral to neurological involvement and some develop ocular involvement. The most commonly affected organs include the spleen, liver, and bone. Moreover, patients often have hepatosplenomegaly, thrombocytopenia, anemia, and bone involvement related to deficient glucocerebrosidase and the subsequent accumulation of glucosylceramide and glucosylsphingosine in cells. A subset of patients develops neurological manifestations, including seizures, myoclonic epilepsy, and progressive neurodegeneration. Eye involvement tends to be less common and presents with diverse clinical findings. These rare and variable ocular manifestations, involving the vitreous, retina, cornea, uvea, conjunctiva and eye movements, can pose a diagnostic challenge for clinicians, especially those not familiar with the disorder. In this review, we explore the different ophthalmologic findings reported in patients with Gaucher disease, aiming to facilitate diagnosis and expedite treatment for patients presenting with ocular manifestations of this rare disorder.

Desmin deficiency is not sufficient to prevent corneal fibrosis.

The type III intermediate filament (IF) proteins vimentin and desmin are sequentially overexpressed in stromal myofibroblasts over the period when fibrosis sets in after corneal injury. Prior findings have revealed vimentin-deficient mice are significantly protected from corneal fibrosis after alkali injury, which has implicated this IF protein as an important regulator of corneal fibrosis. It has remained as yet unproven whether desmin contributes in any significant manner to corneal fibrosis. Here we have employed desmin-deficient (Des KO) mice in the corneal alkali injury model and show that injured Des KO mice develop fibrosis and show similar levels of corneal opacity at 14 days post-injury as wild type (WT) mice and retain this phenotype even at 30d post injury. Des KO corneas from injured mice show upregulation of vimentin and alpha-smooth muscle actin expression to equivalent levels as WT corneas, illuminating that desmin deficiency does not interfere with myofibrobast differentiation. Employing the small molecule withaferin A (WFA), an inhibitor of vimentin, we show that WFA treatment causes the decrease in steady state levels of vimentin and serine 38 phosphorylated vimentin, the latter a biomarker associated with corneal fibrosis, and improved corneal clarity through blockade of myofibroblast differentiation. To investigate further the mechanism of fibrosis in desmin deficiency, we examined keratin 8 expression in the epithelium, and found reduced levels of this cytokeratin in injured Des KO corneas compared to WT corneas. This finding also corroborates the decrease of cell proliferation in injured Des KO corneas compared to that in WT corneas. The fibrotic phenotype of Des KO corneas also features abundant vascularization, further exemplifying the magnitude of corneal pathology. Together, these findings illuminate that desmin does not contribute significantly to corneal fibrosis in this injury model.

Short-term results of acellular porcine corneal stroma keratoplasty for herpes simplex keratitis.

BACKGROUND: Corneal transplantation is a common surgical intervention for restoring vision loss due to corneal damages. However, for cultural reasons, there is a huge shortage of donor corneas in China. Acellular porcine corneal stromas (APCSs) can be used as corneal substitutes in lamellar keratoplasty for corneal ulcers. This study was conducted to analyze the results of APCS use for herpes simplex keratitis (HSK). METHODS: The study involved HSK patients who underwent keratoplasty with APCSs from February 2016 to October 2017 in the second affiliated hospital of Zhejiang University. Patient data were collected at 7 days, 1 month, 3 months, 6 months, and at the last follow-up (7-25 months) postoperative. The corneal transparency, neovascularization, visual acuity, and graft stability were observed. RESULTS: Thirteen patients with HSK including five patients with corneal perforation were included in this study, nine patients underwent deep anterior lamellar keratoplasty (DALK) and five perforation patients underwent double lamellar keratoplasty. There were nine men and four women with an average age of 62.5 ± 5.6 years old (ranging from 52 to 70 years old). The mean postoperative follow-up duration was 15.1 ± 5.8 months (ranging from 7 to 25 months). At the last visit, visual acuity improved in nine patients (69.2%) compared with preoperative (P = 0.008).The grafts of seven individuals (53.8%) were completely transparent or slightly opaque; their corneal transparency score had improved significantly compared with before the surgery (P = 0.010). Various degrees of neovascularization were present in 11 of the 13 patients (84.6%), most neovascularization gradually stabilized. Graft dissolution occurred in three eyes (23.1%) during the observation period, two underwent regrafting, the other one became stable after treatment. Three patients underwent second allograft transplantation, two of which encountered APCS graft dissolution and one of the patients requested a human donor allograft transplantation due to transparency issues despite the absence of adverse issues. CONCLUSION: Acellular porcine corneal stroma seems to be effective in the treatment of HSK and can be used in HSK with corneal perforation by using double lamellar keratoplasty in an emergency.

Case Report: Management of Corneal Clouding from Lecithin: Cholesterol Acyltransferase Deficiency.

SIGNIFICANCE: Given that there are few reported cases of lecithin:cholesterol acyltransferase (LCAT) deficiency, recognition of the condition with proper management is notable. Long-term follow-up and contact lens fitting after penetrating keratoplasty provide best possible outcomes. PURPOSE: The purpose of this study was to report a case of LCAT deficiency successfully treated with penetrating keratoplasty and longer-term follow-up with contact lens fitting. CASE REPORT: A 43-year-old white woman of Italian descent presented with corneal clouding and trouble with night vision. The patient had a history of LCAT deficiency, irritable bowel syndrome, gastroesophageal reflux disease, osteoporosis, and hemolytic anemia. Slit-lamp examination demonstrated corneal haze throughout the corneal layers. The corneas had normal pachymetry. Given the opacity of each cornea (right greater than left) and decreased night vision, penetrating keratoplasty was performed on the right eye. At post-operative month 16, the corneal graft remained clear. The patient was able to achieve a best-corrected visual acuity of 20/30+ with a scleral lens. CONCLUSIONS: Penetrating keratoplasty may be necessary to provide better quality of vision in LCAT deficiency patients, specifically to enhance one's contrast sensitivity, despite relatively good Snellen visual acuity.

Central Toxic Keratopathy After Contact Lens Wear and Mechanical Debridement: Clinical Characteristics, and Visual and Corneal Tomographic Outcomes.

OBJECTIVES: To highlight the clinical characteristics, and visual and corneal tomographic outcomes of central toxic keratopathy (CTK) after contact lens (CL) wear and mechanical debridement. METHODS: A retrospective observational case series with literature review. RESULTS: Four patients (4 eyes) were included in this study; 3 (75%) females, mean age 29.3±8.1 years. The mean follow-up was 13.5±7.5 months. Early central or paracentral stromal opacification, assuming an inverse dome-shaped pattern observed under anterior-segment optical coherence tomography, with corneal flattening (Kmean 40.4±1.3 D) and thinning (mean thinnest pachymetry=404.8±29.4 microns) were observed in all cases. All patients had a recent use of CL wear, with three after mechanical debridement for recurrent corneal erosion syndrome. None of them had any previous laser refractive surgery (LRS). The mean corrected distance visual acuity improved from 20/40 (ranged 20/25-20/50) initially to 20/30 (ranged 20/20-20/40) at final follow-up, and the outcome was not influenced by the use of topical steroids. A mean improvement of corneal flattening (+Kmean 1.2±1.2 D), thinning (+123.5±23.8 microns), and astigmatism (-3.0±2.7 D), via epithelial and stromal remodeling, was observed up to 15 months after CTK. Persisting reduced corneal sensation was noted in all patients at the final follow-up. CONCLUSIONS: Central toxic keratopathy is not an exclusive complication of LRS, and it may occur after CL wear and mechanical debridement. Our findings are similar to those of LRS-related CTK and toxic peripheral keratopathy. Awareness of the clinical associations and understanding of the clinical course and tomographic characteristics of CTK helps obviate unnecessary investigation and overtreatment. Further studies are required to elucidate the underlying pathogenesis of this rare clinical entity.