Membrane Dystrophy Research Articles

False corneal ectasia in patients referred for corneal crosslinking, topography-guided photorefractive keratectomy, and intrastromal corneal rings

To determine the misdiagnosis of ectasia in patients referred for corneal crosslinking and possible topography-guided photorefractive keratectomy (PRK) or intrastromal corneal rings. Bochner Eye Institute, Toronto, Ontario, Canada. Retrospective data review. Chart review of consecutive cases referred for corneal crosslinking to determine the number of cases of misdiagnosis of ectasia. Examination findings were reviewed consisting of best-corrected spectacle distance acuity, slit lamp examination, and computerized tomography. The study analyzed 1000 consecutive records of patients referred with a presumed diagnosis of keratoconus, pellucid marginal degeneration, and ectasia after laser vision correction that were examined between January 1, 2010 and November 1, 2016. There were 26 eyes without ectasia detected in 20 patients. The etiology of these misdiagnoses was epithelial basement membrane dystrophy (9 eyes), superficial punctate keratitis (7 eyes), amblyopia secondary to high astigmatism (3 eyes), amiodarone keratopathy (2 eyes), corneal warpage from rigid gas permeable lenses (2 eyes), corneal scars (1 eye), and measurement or alignment error with topography (2 eyes). Analysis of data detected a misdiagnosis of ectasia in 20 patients (26 eyes), a finding of 2.0% (20 of 1000) of referred cases that did not satisfy the diagnostic criteria of corneal ectasia. These conditions are considered a contraindication to corneal crosslinking and there is usually no benefit to topography-guided PRK or intrastromal corneal rings. It is important that clinicians recognize the clinical findings of these conditions and differentiate from true keratoconus, pellucid marginal degeneration, or ectasia after laser vision correction.

Prevalence of ocular surface dysfunction in patients presenting for cataract surgery evaluation

To report the prevalence of ocular surface dysfunction in patients presenting for cataract surgery evaluation. Duke University Eye Center and Weill Cornell Ophthalmology, single-physician practices. Prospective case series. Consecutive patients presenting for cataract surgery evaluation were identified. Patient information including demographics, medical history, slitlamp findings, tear osmolarity, and tear matrix metalloproteinase-9 (MMP-9) levels were recorded. Patients were considered to have ocular surface dysfunction if any of the following outcomes were present: visually significant abnormal corneal surface examination, positive MMP-9 test, or abnormal osmolarity values (>307 mOsm/L or >7 mOsm/L intereye difference). Patient symptoms were recorded using the ocular surface disease index (OSDI) or Symptom Assessment iN Dry Eye questionnaires. There were 120 patients (69% women), mean age 69.5years±8.4 (SD). Abnormal osmolarity was found in 68 patients (56.7%), and abnormal MMP-9 in 76 patients (63.3%). Clinical findings showed that 47 patients (39.2%) had positive corneal staining on presentation, 9 patients (7.5%) had epithelial basement membrane dystrophy, and 2 patients (1.6%) had Salzmann nodules. Questionnaire data showed 54 (54.0%) of 100 patients reported symptoms suggestive of ocular surface dysfunction. In the asymptomatic group of 46 patients, 39 (85%) had at least 1 abnormal tear test (osmolarity or MMP-9) and 22 (48%) had both tests abnormal. Overall, 96 (80%) of 120 patients had at least 1 abnormal tear test result suggestive of ocular surface dysfunction and 48 patients (40%) had 2 abnormal results. Objective ocular surface dysfunction findings werecommon among patients presenting for cataract surgery, yet many presented undiagnosed. Clinicians should be aware ofthis high prevalence and consider screening with tear testing before surgery.

Recurrent Corneal Erosions in Epithelial Corneal Dystrophies

The corneal epithelium is the most important structure of the ocular optical system. Recurrent corneal erosions can result from inflammation, trauma, degeneration and dystrophies. Epithelial basement membrane dystrophy (EBMD), epithelial recurrent erosion dystrophy (ERED) and Francheschetti and Meesmann's epithelial corneal dystrophy (MECD) can all - besides other signs and symptoms - result in more or less frequent corneal erosions. The pathomechanisms involved however are different. In EBMD, corneal erosions are facultative and clinical signs are often subtle. Aberrant basement membrane structures are associated with thinning of the epithelium and can be clinically identified as maps or fingerprints. In ERED, recurrent corneal erosions are - predominantly in the first decades of life - always present. A defect in the COL17A1 gene results in a dysfunctional hemidesmosome. In MECD, punctate corneal erosions are less frequent and result from intraepithelial microcysts which open spontaneously onto the ocular surface. Usually lubricants, therapeutic contact lenses and sometimes epithelial debridement and phototherapeutic keratectomy are the mainstay for treating corneal erosions in these three dystrophies.

Superficial keratectomy: indications and outcomes

IntroductionTo evaluate the indications and outcomes of manual blade superficial keratectomy Study designRetrospective, nonrandomized, consecutive case series MethodsDatabase search of patients from 2012-2017 who underwent superficial keratectomy was conducted at a tertiary care hospital cornea clinic. Charts of 121 patients (156 eyes) were reviewed who had at least 4 weeks of follow-up and both preoperative and postoperative measurements of best-corrected visual acuity or corneal cylinder. Outcome measures included: patient demographics, surgical indication, prior ocular history, best-corrected visual acuity (BCVA) changes, corneal and refractive astigmatism changes, recurrence of pathology, additional treatments required, and intraoperative and postoperative complications. ResultsMean patient age at time of operation was 63.3 (±14.8), 39% were male. Indications included epithelial basement membrane dystrophy, recurrent corneal erosion syndrome, Salzmann nodular degeneration, band keratopathy, and suspected ocular surface neoplasia. In eyes with epithelial basement membrane dystrophy, mean BCVA (20/47 to 20/40, P = 0.033), refractive astigmatism (1.76 ± 1.83 D to 1.15 ± 1.08 D, P = 0.010), and corneal astigmatism (1.44 ± 0.88 D to 1.06 ± 0.88 D, P = 0.022) significantly improved. Twenty-four percent (5/21) of eyes with recurrent erosions had symptoms return at a mean 6.5 months follow-up. No intraoperative complications were noted. The epithelium healed completely in all 156 eyes at final follow-up. ConclusionSuperficial keratectomy is a simple, safe procedure that can be performed for a variety of conditions to improve visual acuity, reduce corneal astigmatism, and alleviate symptoms secondary to ocular surface pathology.

Corneal Dystrophy Adds to the Frustration of a Dry Eye Patient

PURPOSEThis case report highlights how epithelial basement membrane dystrophy (EBMD), coupled with dry eye, can contribute to symptoms of unstable vi-sion and discomfort. This report also reviews corneal dystrophies and offers eye care practitioners (ECPs) clinical pearls for identifying key features. CASE REPORTA 62-year-old Caucasian female presented for a dry eye evaluation due to fluctuating vision and longstanding ocular discomfort, despite ocular lubri-cation. Anterior segment examination revealed Meibomian gland dysfunc-tion (MGD), upper lid margin staining (ULMS) and anterior blepharitis. The patient was unaware of a pre-existing EBMD and this lack of knowl-edge contributed to her frustration concerning her unstable vision, which she had solely attributed to her glasses. Management included warm com-presses for MGD and targeted preservative-free artificial tears for ULMS and EBMD. Photographs were essential for educating the patient with respect to the irregularities of the ocular surface and its effect on vision. This provided a deeper understanding of the multifactorial nature of her symptoms.CONCLUSIONUnstable and/or poor vision is among the main reasons why patients con-sult ECPs and it can be difficult to identify contributory factors. This report highlights that additional chair time may be warranted to educate patients on the multifactorial nature of dry eye and the complexities of corneal dys-trophy.

Linker proteins restore basement membrane and correct LAMA2-related muscular dystrophy in mice.

LAMA2-related muscular dystrophy (LAMA2 MD or MDC1A) is the most frequent form of early-onset, fatal congenital muscular dystrophies. It is caused by mutations in LAMA2, the gene encoding laminin-α2, the long arm of the heterotrimeric (α2, β1, and γ1) basement membrane protein laminin-211 (Lm-211). We establish that despite compensatory expression of laminin-α4, giving rise to Lm-411 (α4, β1, and γ1), muscle basement membrane is labile in LAMA2 MD biopsies. Consistent with this deficit, recombinant Lm-411 polymerized and bound to cultured myotubes only weakly. Polymerization and cell binding of Lm-411 were enhanced by addition of two specifically designed linker proteins. One, called αLNNd, consists of the N-terminal part of laminin-α1 and the laminin-binding site of nidogen-1. The second, called mini-agrin (mag), contains binding sites for laminins and α-dystroglycan. Transgenic expression of mag and αLNNd in a mouse model for LAMA2 MD fully restored basement membrane stability, recovered muscle force and size, increased overall body weight, and extended life span more than five times to a maximum survival beyond 2 years. These findings provide a mechanistic understanding of LAMA2 MD and establish a strong basis for a potential treatment.

Internal limiting membrane dsytrophy: a case series study of a rarely reported retinal dystrophy

Purpose The purpose of this study was to describe the clinical features of 10 cases of a rarely reported internal limiting membrane dystrophy with glistening retinal reflex and peculiar fundus mottling. Patients and methods This is a retrospective review of 10 patients attending the outpatient clinic of Mansoura Ophthalmic Center in the period between 2000 and 2014. For all cases, measuring BCVA, fundus examination and fundus fluorescein angiography were done. Optical coherence tomography (OCT) was done for nine patients, with one patient missed (was not accessible for the investigation). Results The study included 10 unrelated patients − three female and seven male. The age ranged from 21 to 36 years (mean: 27±0.5 years). The mean visual acuity was 0.3±0.04. In all cases, fundus examination revealed the presence of a peculiar reddish mottling, with glistening retinal reflex and areas of epiretinal membranes and distortion of retinal blood vessels. Fundus fluorescein angiography revealed capillary dye leakage all over the retina with cystoid macular edema in five eyes. In one patient, abrupt cessation of retinal blood vessels was evident in the temporal retina. OCT revealed the presence of epiretinal membranes and schisis cavities in all retinal layers. Secondary choroidal neovascular membrane was evident in one eye. Conclusion Internal limiting membrane dystrophy is an extremely rare hereditary disorder that presents early in life (second-third decade) with early drop of vision. Clinically, it presents with glistening retinal surface, epimacular membranes and peculiar reddish mottling of the whole retina extending from the central retina to the far periphery. Fluorescein angiography shows capillary leakage all over the retina and cystoid macular edema. OCT shows schiscis cavities in all retinal layers. Some cases are complicated by choroidal neovascular membrane.

Phototherapeutic keratectomy for epithelial basement membrane dystrophy.

PurposeThe purpose of this study was to evaluate the long-term efficacy of phototherapeutic keratectomy (PTK) in treating epithelial basement membrane dystrophy (EBMD).MethodsPreoperative and postoperative records were reviewed for 58 eyes of 51 patients with >3 months follow-up (range 3–170 months) treated for EBMD with PTK after failure of conservative medical treatment at Byers Eye Institute of Stanford University. Symptoms, clinical findings, and corrected distance visual acuity (CDVA) were assessed. The primary outcome measure was symptomatic recurrence as measured by erosions or visual complaints >3 months after successful PTK.ResultsFor eyes with visual disturbances (n=30), preoperative CDVA waŝ20/32 (0.24 Log-MAR, SD 0.21) and postoperative CDVA was ~20/25 (0.07 LogMAR, SD 0.12; P<0.0001). Twenty-six eyes (86.7%) responded to treatment, with symptomatic recurrence in 6 eyes (23.1%) at an average of 37.7 months (SD 42.8). For eyes with painful erosions (n=29), preoperative CDVA was ~20/25 (0.12, SD 0.19) and postoperative CDVA was ~20/20 (0.05. SD 0.16; P=0.0785). Twenty-three eyes (79.3%) responded to treatment, with symptomatic recurrence in 3 eyes (13.0%) at an average of 9.7 months (SD 1.5). The probability of being recurrence free after a successful treatment for visual disturbances and erosions at 5 years postoperatively was estimated at 83.0% (95% confidence interval 68.7%–97.0%) and 88.0% (95% confidence interval 65.3%–96.6%), respectively.ConclusionThe majority of visual disturbances and painful erosions associated with EBMD respond to PTK. For those with a treatment response, symptomatic relief is maintained over long-term follow-up.

Genotype-Phenotype Correlation for TGFBI Corneal Dystrophies Identifies p.(G623D) as a Novel Cause of Epithelial Basement Membrane Dystrophy.

The majority of anterior corneal dystrophies are caused by dominant mutations in TGFBI (transforming growth factor β-induced) collectively known as the epithelial-stromal TGFBI dystrophies. Most cases of epithelial basement membrane dystrophy (EBMD) are thought to result from a degenerative (nongenetic) process; however, a minority of cases are associated with specific TGFBI mutations. We evaluated the spectrum of TGFBI mutations and associated phenotypes in a United Kingdom cohort with typical epithelial-stromal TGFBI dystrophies and an EBMD cohort. We recruited 68 probands with a clinical diagnosis of epithelial-stromal TGFBI dystrophy and 23 probands with bilateral EBMD. DNA was extracted from peripheral leukocytes, and TGFBI was bi-directly Sanger sequenced. Nine TGFBI mutations were identified. The most common occurred at the mutation hot-spot residues R124 and R555 in 61 probands; these individuals had a genotype-phenotype correlation consistent with prior reports. Four probands with lattice corneal dystrophy carried a mutation in exon 14: p.(A620D), p.(V625D), and p.(H626R). We identified a p.(G623D) mutation in five probands, including two probands from the EBMD cohort. These subjects typically had an onset of severe recurrent corneal epithelial erosion in the fourth decade with mild diffuse or geographic subepithelial corneal opacities and only small anterior stromal lattice structures in older individuals. Symptoms of painful epithelial erosion improved markedly following phototherapeutic keratectomy. There was a strong correlation between genotype and phenotype for the majority of TGFBI mutations. In this cohort, the p.(G623D) mutation caused a greater proportion of TGFBI-associated disease than anticipated, associated with variable phenotypes including individuals diagnosed with EBMD.

Confocal microscopy findings in corneal dytrophies

SummaryCorneal confocal microscopy in vivo is a diagnostic method that uses 670 nm laser beam to visualize the structures of the cornea with an accuracy of 1 μm and visualization of single cells with 63× magnification. Proper diagnosis of corneal diseases is often difficult. The use of confocal microscopy for the in vivo analysis of the microarchitecture of the cornea can provide rapid and highly specific differentiation of corneal pathologies.The aim of the course is to acquaint participants with the method – corneal confocal microscopy in vivo and to present its utility in diagnosis and management of corneal dystrophies. Course consists of description of epithelial‐basal membrane dystrophies, Bowman membrane dystrophies, stromal and endothelial dystrophies with respect to their typical histological features, differential diagnosis, evaluation and qualification for surgery and follow‐up with detection of possible recurrences and post‐surgical complications.

Uncovering the profile of mutations of transforming growth factor beta-induced gene in Chinese corneal dystrophy patients.

To uncover the mutations profile of transforming growth factor beta-induced (TGFBI) gene in Chinese corneal dystrophy patients and further investigate the characteristics of genotype-phenotype correlations. Forty-two subjects (6 unrelated families including 15 patients and 8 unaffected members, and 19 sporadic patients) of Chinese origin were subjected to phenotypic and genotypic characterization. The corneal phenotypes of patients were documented by slit lamp photography. Mutation screening of the coding regions of TGFBI was performed by direct sequencing. We detected four corneal dystrophy types. The most frequent phenotypes were granular corneal dystrophy (GCD) (including 3 families and 8 sporadic patients) and lattice corneal dystrophy (LCD) (including 2 families and 9 sporadic patients). The next phenotypes were corneal dystrophy of Bowman layer (CDB) (1 family and 1 sporadic patient) and epithelial basement membrane dystrophy (EBMD) (1 sporadic patient). Six distinct mutations responsible for TGFBI corneal dystrophies were identified in 30 individuals with corneal dystrophies. Those were, p.R124H mutation in 1 family and 2 sporadic patients with GCD, p.R555W mutation in 2 families and 3 sporadic patients with GCD, p.R124C mutation in 2 families and 7 sporadic patients with LCD, p.A620D mutation in 1 sporadic patient with LCD, p.H626R mutation in 1 sporadic patient with LCD, and p.R555Q in 1 family and 1 sporadic patient with CDB. No mutation was detected in the remaining 3 atypical GCD patients and 1 EBMD patient. GCD and LCD are the most frequent phenotypes in Chinese population. R555W was the most common mutation for GCD; R124C was the most common mutation for LCD. Our findings extend the mutational spectrum of TFGBI, and this is the extensively delineated TGFBI mutation profile associated with the various corneal dystrophies in the Chinese population.

Investigation of genotype‐phenotype correlation of TGFBI mutations reveals c.1868G&gt;A; p.(Gly623Asp) is associated with a variable clinical phenotype, including epithelial basement membrane dystrophy

PurposeAutosomal dominant mutations in TGFBI cause a range of clinically distinct corneal dystrophies. We investigated the TGFBI mutation spectrum in our cohort and correlated genotype with phenotype.MethodsTGFBI exons 4, 11, 12, 13, 14 and 16 were Sanger sequenced in 59 unrelated probands attending Moorfields Eye Hospital with a diagnosis of a potential TGBFI‐associated corneal dystrophy.ResultsThe majority of individuals, 86%, carried a mutation at one of the two known hotspot residues for TGFBI‐associated corneal dystrophies: Arg‐124 and Arg‐555. Mutations affecting either of these residues demonstrated genotype‐phenotype correlation. A c.1868G&gt;A; p.(Gly623Asp) mutation was identified in five unrelated probands; one with a clinical diagnosis of lattice corneal dystrophy, two with a Bowman's layer dystrophy (Reis‐Bϋcklers or Thiel‐Behnke corneal dystrophy) and two with epithelial basement membrane dystrophy (EBMD). The clinical variability associated with this mutation indicates that other genetic or environmental factors can influence phenotypic expression.ConclusionsThis is the first time the c.1868G&gt;A; p.(Gly623Asp) mutation has been associated with EBMD, although other mutations in TGFBI have previously been identified in a small number of EBMD patients. These results demonstrate that the c.1868G&gt;A; p.(Gly623Asp) mutation is responsible for a significant proportion of the disease burden for TGFBI‐associated corneal dystrophies in the UK and highlights the need for patients with EBMD to be screened for mutations in TGFBI.

Topical Mitomycin-C enhances subbasal nerve regeneration and reduces erosion frequency in the debridement wounded mouse cornea

Corneal epithelial basement membrane dystrophies and superficial injuries caused by scratches can lead to recurrent corneal erosion syndrome (RCES). Patients and animals with reduced corneal sensory nerve innervation can also develop recurrent erosions. Multiple wild-type mouse strains will spontaneously develop recurrent corneal erosions after single 1.5 mm debridement wounds. Here we show that this wound is accompanied by an increase in corneal epithelial cell proliferation after wound closure but without a commensurate increase in corneal epithelial thickness. We investigated whether excess corneal epithelial cell proliferation contributes to erosion formation. We found that topical application of Mitomycin C (MMC), a drug used clinically to improve healing after glaucoma and refractive surgery, reduces erosion frequency, enhances subbasal axon density to levels seen in unwounded corneas, and prevents excess epithelial cell proliferation after debridement wounding. These results suggest that topically applied MMC, which successfully reduces corneal haze and scarring after PRK, may also function to enhance subbasal nerve regeneration and epithelial adhesion when used to treat RCES.

Imagerie des dystrophies cornéennes : corrélations entre l’OCT en face de segment antérieur et la microscopie confocale in vivo

To evaluate the usefulness of en face Optical Coherence Tomography (OCT) for evaluation of corneal dystrophies and to describe correlations with in vivo confocal microscopy (IVCM). Thirty-two eyes of 16 patients with 4 types of corneal dystrophies (epithelial basement membrane dystrophy, Fuchs dystrophy, Reis-Bücklers corneal dystrophy and Crocodile Shagreen dystrophy) were enrolled in this study. Axial and reconstructed en face scans were acquired using OCT. Images were then correlated to IVCM findings. En face OCT provided new insights into the structure, size and depth of corneal tissue alterations in various corneal dystrophies. OCT en face images were well correlated with IVCM features. Despite lower resolution than IVCM, en face OCT offers the advantages of being non-invasive and allowing the analysis of larger corneal areas. En face OCT provides useful new information in corneal dystrophies. This imaging technique will probably increase in popularity in the near future for the assessment of various anterior segment diseases.

Fourier-Domain Optical Coherence Tomography Imaging in Corneal Epithelial Basement Membrane Dystrophy: A Structural Analysis

To investigate the features of corneal epithelial basement membrane dystrophy using spectral-domain optical coherence tomography (SD OCT) and to examine the reliability of SD OCT in distinguishing epithelial basement membrane dystrophy from the normal cornea. Diagnostic test study. Forty-five individuals with epithelial basement membrane dystrophy and 45 age- and sex-matched controls with normal corneas were examined, and SD OCT scans of their corneas were performed. Invivo confocal microscopy was performed to confirm or rule out the diagnosis of epithelial basement membrane dystrophy. The structural corneal changes occurring in eyes with epithelial basement membrane dystrophy based on SD OCT findings were described. Epithelial abnormalities were observed in 86 of 87 eyes with epithelial basement membrane dystrophy (45 patients) on SD OCT scans. The 2 main features were the presence of an irregular and thickened epithelial basement membrane duplicating or insinuating into the corneal epithelium layer, or both, and the presence of hyperreflective dots. In some cases, we detected hyporeflective spaces between the corneal epithelial layer and the Bowman layer similar to a corneal epithelial detachment. This corneal epithelial detachment sometimes was associated with a cleavage with a stair-step appearance within the corneal epithelial layer. We found a perfect correlation between invivo confocal microscopy and SD OCT findings in the diagnosis of epithelial basement membrane dystrophy (κ= 0.98). SD OCT provides an accurate assessment of the structural changes occurring in eyes with epithelial basement membrane dystrophy. These changes, visible on SD OCT scans, are easily detectable and permit an accurate diagnosis, especially in patients with no biomicroscopically visible corneal changes.

Visual and subjective outcomes of phototherapeutic keratectomy after Descemet's stripping endothelial keratoplasty.

BackgroundPatients with endothelial disease also often have scarring or surface corneal disease. This study examined the outcomes of phototherapeutic keratectomy (PTK) performed in patients with prior Descemet’s stripping endothelial keratoplasty (DSEK).MethodsThis retrospective case series examined all patients undergoing PTK after DSEK in a single private practice. The primary outcome was best-corrected visual acuity, with secondary outcomes of change in spherical equivalent versus targeted change, change in endothelial cell count, improvement in corneal topography, and subjective satisfaction with vision.ResultsFor the ten patients who met the inclusion criteria, mean best-corrected visual acuity improved from logarithm of the minimum angle of resolution (logMAR) 0.43 to 0.36 (P=0.24) at the most recent postoperative visit. PTK treatments that included a refractive component showed good predictability of 1.13 diopters spherical equivalent/diopter attempted. There was no evidence of a decrease in endothelial cell count from PTK, even with the use of mitomycin C (P=0.95). Subjectively, 80% of patients noted improvement in their vision.ConclusionPTK after DSEK has the potential to improve patients’ vision with high levels of patient satisfaction, even though many patients had significant ocular comorbidities. Surgeons should consider using PTK to treat anterior corneal pathology following DSEK.

Pathologically Reduced Subbasal Nerve Density in Epithelial Basement Membrane Dystrophy Is Unaltered by Phototherapeutic Keratectomy Treatment

To investigate the effect of phototherapeutic keratectomy (PTK) treatment on corneal epithelial wing cell and corneal subbasal nerve density in epithelial basement membrane dystrophy (EBMD). A total of 39 patients with EBMD who underwent PTK treatment, 40 healthy volunteers, and 24 untreated eyes with EBMD were examined with laser-scanning in vivo confocal microscopy (IVCM). Corneal subbasal nerves and epithelial wing cells were manually quantified from IVCM images by two observers, while epithelial wing cells were additionally quantified by a fully automated method. Subbasal nerve density was significantly reduced in untreated (10,164 ± 4139 μm/mm(2); n = 24) and PTK-treated (10,624 ± 4479 μm/mm(2); n = 39) EBMD eyes, relative to healthy controls (18,241 ± 4479 μm/mm(2); n = 40) (P < 0.001). Subbasal nerve density in PTK-treated and untreated eyes did not differ (P > 0.05). Epithelial wing cell density did not differ between PTK-treated and untreated EBMD eyes, by either manual or automated analysis; however, epithelial wing cell density in PTK-treated EBMD corneas was significantly reduced (P = 0.008) relative to healthy corneas, by automated cell counting. Subbasal nerve density in EBMD is reduced by 45% and recovers only to the reduced level in the long term after PTK treatment, whereas epithelial wing cell density in EBMD is not affected by PTK in the long term. Fully automated cell analysis from IVCM images could provide an objective, standardized means to quantify and compare corneal cell densities in future studies.

Demographic Patterns and Treatment Outcomes of Patients With Recurrent Corneal Erosions Related to Trauma and Epithelial and Bowman Layer Disorders

To evaluate demographics and outcomes of patients with recurrent corneal erosions related to trauma and epithelial and Bowman layer disorders. Retrospective case series. setting: Wills Eye Institute, Philadelphia, Pennsylvania. study population: Two hundred seventy-two eyes (230 patients) presenting between 2008 and 2011 with recurrent corneal erosions related to trauma and epithelial and Bowman layer disorders. procedure: Conservative management, diamond burr polishing, excimer laser phototherapeutic keratectomy (PTK), and epithelial debridement alone. main outcome measure: Recurrence of erosions, more than 6weeks after commencing treatment. Mean age was 46.5 ± 14.6 (2-85) years. Female subjects accounted for 53% of patients and 18.3% had bilateral involvement. Etiology included epithelial basement membrane dystrophy in 52.9% (144/272) and trauma in 25.7% (70/272). One hundred sixty-six eyes with more than a single visit were initially managed conservatively, 68 of 166 (40.9%) had diamond burr polishing, 7 of 166 (4.2%) had PTK, and 4 of 166 (2.4%) had epithelial debridement alone. Many eyes had >6weeks follow-up: 120 of 166 (72.3%) after conservative management, 54 of 68 (79.4%) after diamond burr polishing, 7 of 7 (100%) after PTK, and 4 of 4 (100%) after epithelial debridement. Percentage of eyes with recurrence was 56.7% (68/120) after conservative management, 14.8% (8/54) after diamond burr polishing, and 28.6% (2/7) after PTK. Rate of recurrence (eye-years) was 0.74, 0.19, and 0.23 with conservative management, diamond burr polishing, and PTK, respectively. Mild haze was seen in 22.2% of eyes (12 eyes) after diamond burr polishing and 28.6% (2 eyes) after PTK. Recurrent corneal erosions are most commonly associated with epithelial basement membrane dystrophy. Patients are generally initially managed conservatively, with recurrence in more than half of eyes. Diamond burr polishing is the most common surgical intervention and is effective in a majority of eyes.

In Vivo Confocal Microscopic Findings in Posterior Polymorphous Corneal Dystrophy

To describe the corneal findings in posterior polymorphous corneal dystrophy (PPCD) as imaged with laser scanning in vivo confocal microscopy (IVCM). IVCM images of 7 subjects with PPCD who had typical slit-lamp biomicroscopic findings of endothelial vesicular, band, and/or placoid lesions were evaluated. Five women and 2 men aged 7 to 64 years were included in this study. Laser scanning IVCM (Heidelberg Retina Tomograph II, Rostock Cornea Module) revealed hyporeflective, round, vesicular lesions with diameters ranging between 20 and 200 µm in 3 subjects, combined vesicular and curvilinear hyperreflective band-like lesions in 3 subjects, and combined vesicular and placoid hyperreflective lesions in 1 subject at the level of Descemet membrane (DM), endothelial cell layer, and posterior stroma adjacent to DM. One subject had coassociated epithelial basement membrane dystrophy. Additional findings included posterior stromal keratocytes with elongated spindle-like nucleus, giant and nucleated endothelial cells, endothelial deposits, and guttae-like dark spots. The mean endothelial cell density was 1485.7 ± 486.3 cells per square millimeter (range, 990-2365 cells/mm). The mean central corneal thickness was 585.3 ± 37.17 μm (range, 534-643 μm). Laser scanning IVCM is able to highlight the characteristic microstructural alterations at the level of endothelium and DM in the setting of PPCD and may have diagnostic utility in equivocal cases with borderline biomicroscopic findings. The possible association of PPCD with epithelial basement membrane dystrophy warrants further investigation.

Cocaine-Assisted Epithelial Debridement for the Treatment of Anterior Basement Membrane Dystrophy

To describe a method of epithelial debridement with the adjunctive use of topical cocaine for the treatment of symptomatic anterior basement membrane dystrophy (ABMD). Retrospective consecutive chart review. Symptomatic patients with ABMD were treated with 4% topical cocaine followed by epithelial debridement. Preoperative and postoperative best-corrected visual acuities (BCVA), topography, subjective symptoms, complications, and evidence of disease recurrence were recorded. Thirty-three eyes of 27 patients were included. The average follow-up duration was 5 years (range, 1.3 months to 8 years). Fourteen patients (17 eyes) presented with recurrent erosion symptoms (group 1) and 13 patients (16 eyes) presented with reduced visual acuity from irregular astigmatism (group 2). Only 1 patient from group 1 experienced symptomatic recurrence of the corneal erosion. In this group, BCVA improved from a mean of 20/45 preoperatively to 20/38 at the last follow-up visit. In group 2, BCVA improved from a mean of 20/63 preoperatively to 20/32 at the last follow-up visit. No major recurrences were noted at the last follow-up visit in this group. No residual effect from the application of cocaine was noted in any of the eyes. Significant postoperative corneal haze occurred in 1 eye. Topical cocaine-assisted epithelial debridement offers a simple and inexpensive method for treating patients with ABMD who experience recurrent corneal erosions and/or irregular astigmatism resulting in decreased visual acuity.