Unilateral Granular Type 2 Corneal Dystrophy With Exacerbation After LASIK.

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

[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.

Surgical Technique to Treat Presbyopic Inlay-Associated Corneal Haze With Sequential Excimer Photoablation: A Case Series.

PURPOSE: To describe an approach using sequential excimer laser ablation of the stromal surface of the corneal flap with or without subsequent excimer ablation to the stromal bed to reduce presbyopic inlay-associated corneal haze. METHODS: Twelve patients who underwent KAMRA inlay (Acufocus) explantation due to corneal haze were included. The mean interval between explantation and the primary surgery (phototherapeutic keratotomy [PTK] to corneal flap) was 16.2 ± 29.7 months (range = 1 to 83 months). The corneal flap was lifted and laid on an evisceration spoon and an excimer laser was used to ablate the flap stroma by 30 to 40 µm depth. Subsequently, an excimer laser was used to ablate and treat the stromal bed following a second flap lift according to the manifest refraction, leaving a minimal residual stromal bed thickness of greater than 300 µm. For both procedures, mitomycin C 0.02% was applied to the stromal bed before the flap was replaced and a bandage contact lens applied. RESULTS: Reductions in corneal haze were observed, following PTK to the corneal flap with or without photorefractive keratectomy (PRK) to the stromal bed, both clinically and on imaging. No significant changes in uncorrected distance visual acuity (P = .442) and corrected distance visual acuity (P = .565) were observed. Improvements were observed for both spherical equivalent refractive errors (P = .036) and corneal light backscatter (P = .019). There were significant improvements in spherical aberrations (P = .014) but no changes in total lower and higher order aberrations. CONCLUSIONS: PTK to the corneal flap with or without subsequent stromal bed PRK is an effective technique in treating corneal haze following presbyopic inlay explantation. [J Refract Surg. 2023;39(9):639-646.].

Mini-Review: Clinical Features and Management of Granular Corneal Dystrophy Type 2.

Granular corneal dystrophy type 2 (GCD2) is an autosomal dominant corneal stromal dystrophy that is caused by p.Arg124His mutation of transforming growth factor ß induced (TGFBI) gene. It is characterized by well demarcated granular shaped opacities in central anterior stroma and as the disease progresses, extrusion of the deposits results in ocular pain due to corneal epithelial erosion. Also, diffuse corneal haze which appears late, causes decrease in visual acuity. The prevalence of GCD2 is high in East Asia including Korea. Homozygous patients show a severe phenotype from an early age, and the heterozygote phenotype varies among patients, depending on several types of compound heterozygous TGFBI mutations. In the initial stage, conservative treatments such as artificial tears, antibiotic eye drops, and bandage contact lenses are used to treat corneal erosion. Different surgical methods are used depending on the depth and extent of the stromal deposits. Phototherapeutic keratectomy removes anterior opacities and is advantageous in terms of its applicability and repeatability. For deeper lesions, deep anterior lamellar keratoplasty can be used as the endothelial layer is not always affected. Recurrence following these treatments are reported within a wide range of rates in different studies due to varying definition of recurrence and follow-up period. In patients who have undergone corneal laser vision-correction surgeries such as photorefractive keratectomy, LASEK, or LASIK including SMILE surgery, corneal opacity exacerbates rapidly with severe deterioration of visual acuity. Further investigations on new treatments of GCD2 are necessary.

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.

Changes in Corneal Clouding Over Time in Patients With Mucopolysaccharidosis.

PURPOSE: Mucopolysaccharidoses (MPSs) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems, including the eye. Visual loss occurs in MPS predominantly due to corneal clouding. Despite the success of enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) in improving many systemic manifestations of MPS, less is known about their effect on corneal clouding. This study prospectively analyses the effect of both ERT and HSCT on corneal clouding using objective measures over time. METHODS: This is a prospective longitudinal observational study. Corneal clouding was assessed in each participant using slitlamp, digital slit-lamp photographs, and an iris camera (Corneal Opacification Measure [COM] and the Pentacam system). RESULTS: Data were collected for 65 participants: 39 MPS I (Hurler), 5 MPS II (Hunter), 12 MPS IV (Morquio), and 9 MPS VI (Maroteaux-Lamy). Follow-up data are available for 45 participants (29 MPS I, 3 MPS II, 6 MPS IV, and 7 MPS VI). CONCLUSIONS: This study found corneal clouding to be stable in most participants with MPS I, II, IV, and VI over a follow-up period of 5 to 75 months (median of 30 months) when measured with clinical corneal grading systems, graded digital slit-lamp images, and iris camera COMs. For those with Pentacam densitometry measures, there was a progression of corneal clouding, on average, in those with MPS I and MPS VI. There was no apparent difference in progression of corneal clouding between patients who were on ERT, HSCT, or no treatment.

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

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

Topical glycerine for corneal clouding during retinal laser photocoagulation for retinopathy of prematurity.

AIM: To document the use of topical glycerine to reduce corneal edema in cases of retinopathy of prematurity (ROP) undergoing laser photocoagulation (PHC). METHODS: Thirty-two eyes of 16 babies (9 males) with a mean gestational age of 30 weeks, mean gestational weight of 1242 grams underwent PHC for Type 1 (zone 1 disease) retinopathy of prematurity. All babies received a single PHC session. Twenty eyes of 10 babies received intravitreal anti-VEGF injection, 1-3 weeks before PHC session. All patients received a single drop of glycerine during the PHC session to clear the corneal clouding. All patients underwent PHC to the avascular area right up to the ora serrata. Patients were seen at one week and one month to assess the adequacy of laser PHC. RESULTS: We were able to complete the PHC for all babies in a single session without any ocular or systemic adverse events. We did not find any skip lesions at follow-up, and the second session of laser PHC was not required in any eyes. CONCLUSION: Topical glycerine is safe and effective to clear corneal clouding in eyes undergoing laser PHC for retinopathy of prematurity.

Subjective and objective evaluation of corneal haze after accelerated corneal crosslinking for corneal ectasias.

PURPOSE: To evaluate the relationship between subjective (slit lamp examination [SLE]) and objective (densitometry) measurements of corneal haze after accelerated corneal crosslinking (aCXL), assess the relationship between densitometry and corrected distance visual acuity (CDVA), and determine the effect of baseline characteristics on densitometry after aCXL in eyes with progressive keratoconus and other ectasias. SETTING: Kensington Eye Institute and Bochner Eye Institute, Toronto, Canada. DESIGN: Retrospective analysis of a prospective interventional cohort study. METHODS: Scheimpflug-derived corneal densitometry, CDVA, maximum keratometry (Kmax ), and central corneal thickness were measured preoperatively and up to 1 year after aCXL, and post-operative haze was estimated with SLE (n = 483 eyes). A random effect model was used to examine the relationship between post-operative subjective haze with SLE and densitometry. Linear mixed models were used to examine the relationship between densitometry, pre-operative baseline characteristics, and CDVA. RESULTS: There was a significant association between subjective haze with SLE and densitometry (p < 0.001). There was a significant relationship between CDVA and densitometry: for every 10 GSUs of increased densitometry in the 0-2 mm zone, CDVA worsened by approximately half a Snellen line (p < 0.001). Age and pre-operative Kmax were significant predictors of densitometry. For every 10 years of age, densitometry increased by 0.68 GSUs (95% CI [0.27 to 1.07], p < 0.001). For every 10 D of increased preoperative Kmax , densitometry increased by 0.69 GSUs (95% CI [0.41 to 0.98], p < 0.001). CONCLUSIONS: Subjective haze after aCXL estimated with SLE, is significantly associated with densitometry. Increased densitometry after aCXL is associated with a reduction in CDVA.

Progeroid syndrome of De Barsy - a case report and review of ophthalmic literature.

BACKGROUND: This report describes a very rare case of progeroid syndrome of De Barsy (Cutis laxa-corneal clouding syndrome). MATERIALS AND METHODS: A 2 year-old child presented to the pediatric ophthalmology outpatients with bilateral congenital corneal opacification along with dysmorphic facial features, including loose wrinkled skin, progeroid appearance, delayed milestones, short stature, multiple hyper-extensible joints, muscular hypotonia, pectus excavatum and congenital dislocation of the hip joint. The child underwent a detailed ophthalmic work up and systemic evaluation by a clinical geneticist. RESULTS: Ophthalmic management in the form of bilateral sequential penetrating keratoplasties and a left eye trabeculectomy for medically uncontrolled angle-closure glaucoma was performed. Visual rehabilitation with glasses and amblyopia therapy is ongoing. Histopathology of the corneal button revealed loss of the bowman's layer which was replaced by a fibrous pannus while the stroma showed loss of stromal lamellar architecture with anterior and mid stroma showing vascularization. Genetic testing confirmed a mutation in the PYCR1 gene for a homozygous autosomal recessive cutis laxa type IIB. CONCLUSIONS: Although rare, De Barsy syndrome is an important cause of corneal opacification at birth with multiple systemic abnormalities that requires intervention.

Despigmentação precoce em caso de ceratopigmentação / Early depigmentation in case of keratopigmentation

RESUMO A ceratopigmentação teve seu primeiro registro pelo filósofo Galeno há muitos séculos como uma estratégia utilizada para o tratamento estético de pacientes com leucomas. As córneas com leucoma são patológicas e, muitas vezes, intolerantes a lentes de contato cosméticas ou próteses oculares, sendo comum a queixa de desconforto excessivo, proporcionado pela superfície corneana irregular. Assim, a ceratopigmentação é uma alternativa para a melhora estética de pacientes com opacidades corneanas. Descrevemos o caso de um paciente do sexo masculino, 39 anos, que apresentou despigmentação precoce em caso de ceratopigmentação associado a quadro de ceratite herpética necrotizante. O paciente foi submetido ao tratamento com aciclovir 2g ao dia e doxiciclina 200mg ao dia, evoluindo com melhora do quadro clínico, apesar da má adesão medicamentosa.

ABSTRACT Keratopigmentation was first recorded many centuries ago by the philosopher Galeno, as a strategy used for the aesthetic treatment of patients with leukomas. Corneas with leucoma are pathological and often intolerant of cosmetic contact lenses or ocular prostheses, with complaints of excessive discomfort provided by the irregular corneal surface being common. Therefore, keratopigmentation is an alternative for the aesthetic improvement of patients with corneal opacities. We describe the case of a 39-year old male patient, who presented early depigmentation in a case of keratopigmentation associated with necrotizing herpetic keratitis. The patient was treated with Acyclovir 2g/day and Doxycycline 200mg/day, evolving with clinical improvement, despite poor medication adherence.

Cumulative dissipated energy in eyes with and without corneal opacity.

Objective: To compare ultrasonic energy delivered into the eye [cumulative dissipated energy, (CDE)] and frequencies of required auxiliary surgical methods during phacoemulsification surgery in eyes with and without corneal opacity. Methods: The study was designed as a retrospective comparative observational study. The study group [Corneal Opacity Group, (COG)] was comprised of 31 eyes of 31 cataract patients with corneal opacity. Only nebular and macular corneal opacities (according to slit-lamp based classification of Agrawal) were included in the study. The control group (CG) was comprised of 40 eyes of 40 cataract patients without corneal opacity. The CDE values were obtained using the Centurion system (Alcon, Fort Worth, TX) and the patients were followed-up postoperatively for a period of one month. Results: The mean age of the subjects was 71.46 ± 8.86 years (52-89) in COG and 66.12 ± 5.96 years (55-80) in CG (p >0.05). In COG, the most common etiologic factors were trauma, keratitis, and degenerative diseases. The mean CDE value was 15.16 ± 8.71 (2.20-42.65) in COG and 10.04 ± 6.28 (3.77-31.80) in CG and it was found as significantly higher in COG (p=0.003). Some auxiliary surgical methods including posterior synechiolysis and anterior capsule staining were more commonly performed in COG (p=0.044 and p=0.040, respectively). No intraoperative or postoperative complication was observed. Conclusion: More ultrasonic energy is delivered into the eye and more auxiliary surgical methods are needed in cataract patients with corneal opacity who underwent phacoemulsification. Abbreviations: CDE = Cumulative dissipated energy, COG = Corneal Opacity Group, CG = Control group, IOL = Intraocular lens, LOCS = Lens Opacities Classification System, BCVA = best-corrected visual acuity, SRK/T = Sanders, Retzlaff, and Kraff theoretical, OVD = ophthalmic viscosurgical device, SPSS = Statistical Package for the Social Sciences.

Topical Losartan for Treating Corneal Fibrosis (Haze): First Clinical Experience.

PURPOSE: To report the first clinical experience with topical losartan for treating a case of severe corneal haze after complicated laser in situ keratomileusis (LASIK). METHODS: A 36-year-old woman presented with corneal haze in the left eye after femtosecond laser-assisted LASIK. The left eye had flap dislocation and significant striae, which had been re-lifted. Uncorrected distance visual acuity (UDVA) was 20/200 and corrected distance visual acuity was 20/30 in the left eye at the first presentation, 52 days after the first procedure. A dense layer of subepithelial opacity (haze) was noted in the left cornea. The patient elected to start the off-label treatment with topical losartan 0.8 mg/mL six times per day. RESULTS: Four and one-half months after initiating topical losartan, UDVA improved to 20/30 and CDVA improved to 20/25 in the left eye. A significant reduction of corneal haze was observed at the slit lamp and using Scheimpflug corneal tomography (Pentacam AXL; Oculus Optikgeräte GmbH) and anterior segment optical coherence tomography (Revo NX 130; Optopol). CONCLUSIONS: Losartan is an inhibitor of transforming growth factor-ß signaling. Topical treatment is promising to treat corneal haze formation after corneal injuries, chemical burns, and surgeries. Further clinical studies are needed to optimize losartan dosages and treatment durations. [J Refract Surg. 2022;38(11):741-746.].

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.

[Optical rehabilitation and pediatric ophthalmological care following keratoplasty for childhood corneal opacities]. / Optische Rehabilitation und kinderophthalmologische Betreuung nach Keratoplastik bei kindlichen Hornhauttrübungen.

BACKGROUND: The younger the children are at the time of corneal transplantation, the worse the survival prognosis of the graft. PREOPERATIVE CONSIDERATIONS: Preoperative considerations are significant in terms of accurate parental education, ensuring adherence to treatment and choosing the appropriate surgical time frame (amblyopia versus graft failure, compliance of the patient). Parental education must include the reduced visual prognosis in young children, exceptions being later acquired corneal pathologies such as inflammatory corneal scars (herpes) and keratoconus. POSTOPERATIVE CARE: A distinction must be made between morphological care after transplantation and refractive correction as well as treatment of amblyopia. The younger the children, the less favorable the prognosis for the transplant and the more often multiple examinations with anesthesia are necessary in order to detect complications, such as infiltrates or suture loosening at an early stage. Especially unilateral congenital pathologies often do not lead to a sufficient improvement of amblyopia (refractory amblyopia, poor compliance). CONCLUSION: The prognosis after keratoplasty in childhood is already partly decided by the careful evaluation of indications (no surgery of a sclerocornea) and the detailed and realistic clarification for the parents (cooperation with long-term ocular and systemic treatment even if the child has poor compliance, frequent check-ups, reduced chances of amblyopia treatment). The younger the child is at the time of transplantation, the more frequent are graft failure and the development of complications. Later manifesting diseases in older children (herpetic corneal scars, keratoconus) have a better prognosis.

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.

Suppression of lipopolysaccharide-induced corneal opacity by hepatocyte growth factor.

Keratitis induced by bacterial toxins, including lipopolysaccharide (LPS), is a major cause of corneal opacity and vision loss. Our previous study demonstrates hepatocyte growth factor (HGF) promotes epithelial wound healing following mechanical corneal injury. Here, we investigated whether HGF has the capacity to suppress infectious inflammatory corneal opacity using a new model of LPS-induced keratitis. Keratitis, induced by two intrastromal injections of LPS on day 1 and 4 in C57BL/6 mice, resulted in significant corneal opacity for up to day 10. Following keratitis induction, corneas were topically treated with 0.1% HGF or PBS thrice daily for 5 days. HGF-treated mice showed a significantly smaller area of corneal opacity compared to PBS-treated mice, thus improving corneal transparency. Moreover, HGF treatment resulted in suppression of α-SMA expression, compared to PBS treatment. HGF-treated corneas showed normalized corneal structure and reduced expression of pro-inflammatory cytokine, demonstrating that HGF restores corneal architecture and immune quiescence in corneas with LPS-induced keratitis. These findings offer novel insight into the potential application of HGF-based therapies for the prevention and treatment of infection-induced corneal opacity.

Corneal Opacity: Cell Biological Determinants of the Transition From Transparency to Transient Haze to Scarring Fibrosis, and Resolution, After Injury.

Purpose: To highlight the cellular, matrix, and hydration changes associated with opacity that occurs in the corneal stroma after injury. Methods: Review of the literature. Results: The regulated transition of keratocytes to corneal fibroblasts and myofibroblasts, and of bone marrow-derived fibrocytes to myofibroblasts, is in large part modulated by transforming growth factor beta (TGFß) entry into the stroma after injury to the epithelial basement membrane (EBM) and/or Descemet's membrane. The composition, stoichiometry, and organization of the stromal extracellular matrix components and water is altered by corneal fibroblast and myofibroblast production of large amounts of collagen type I and other extracellular matrix components-resulting in varying levels of stromal opacity, depending on the intensity of the healing response. Regeneration of EBM and/or Descemet's membrane, and stromal cell production of non-EBM collagen type IV, reestablishes control of TGFß entry and activity, and triggers TGFß-dependent myofibroblast apoptosis. Eventually, corneal fibroblasts also disappear, and repopulating keratocytes reorganize the disordered extracellular matrix to reestablish transparency. Conclusions: Injuries to the cornea produce varying amounts of corneal opacity depending on the magnitude of cellular and molecular responses to injury. The EBM and Descemet's membrane are key regulators of stromal cellularity through their modulation of TGFß. After injury to the cornea, depending on the severity of the insult, and possibly genetic factors, trace opacity to severe scarring fibrosis develops. Stromal cellularity, and the functions of different cell types, are the major determinants of the level of the stromal opacity.

Clinical Dilemma of Corneal Opacity, Very Low High-density Lipoprotein, and Nephrotic Syndrome: Mystery Revealed.

Lecithin-cholesterol acyltransferase (LCAT) is a liver enzyme necessary for the formation of cholesteryl esters in plasma from free cholesterol. The rare autosomal recessive disease resulting from familial deficiency of this enzyme can lead to nephropathy with kidney involvement generally being the most common cause of death. In addition, the disease process can engender corneal opacity, very low high-density lipoprotein, normochromic anemia, and nephropathy. We present this case of a 35-year-old male who initially visited for a second opinion for renal failure and nephrotic range proteinuria. He underwent renal biopsy which displayed focal segmental glomerulosclerosis-type injury pattern and was started on futile high-dose steroid therapy. A second renal biopsy coincided with the development of corneal opacity leading to a confirmatory testing of LCAT deficiency through biochemistry panel.