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Purpose To report the occurrence of large intraoperative epithelial sloughing during laser in situ keratomileusis (LASIK) as a first diagnostic sign for silent epithelial basement membrane dystrophy (EBMD). Setting Refractive Surgery and Cornea Unit, Alicante Institute of Ophthalmology, Alicante, Spain. Methods In this retrospective case series, the medical records of all patients with large corneal epithelial sloughing/defects during LASIK from January 1995 to December 2004 were reviewed. All patients who presented normal corneas before LASIK and EBMD changes after LASIK were included in this study. The surgical procedures and postoperative course were recorded. The follow-up period was 12 months for all patients. Results Eleven eyes of 6 patients were included. The mean age was 35 years ± 6.1 (SD). Mean preoperative uncorrected visual acuity (UCVA) was 20/500 (0.04 ± 0.02); 12 months after surgery, mean UCVA increased to 20/27 (0.74 ± 0.21). There was a significant decrease in best spectacle-corrected visual acuity 1 month after surgery. One year postoperatively, 10 of 11 eyes (91%) were within ±1.00 diopter of emmetropia. Postoperatively, clinical manifestations of EBMD were observed in all eyes and complications were common. In the early postoperative period, diffuse lamellar keratitis was observed in 6 of 11 eyes (54.5%) and flap microfolds were noted in 2 of 11 (18.2%). One year after surgery, epithelial ingrowth was present in 8 of 11 eyes (72.7%) and flap melting was noted in 4 of 11 (36.4%). Conclusion Occurrence of large intraoperative epithelium sloughing/defects during LASIK might be a diagnostic sign for subclinical EBMD. These patients are predisposed to multiple postoperative complications. Because of the high risk for epithelial sloughing in the second eye, LASIK should not be performed.

Epithelial ingrowth at the edge of the flap is one of the most significant complications of LASIK. Reports of the incidence of epithelial ingrowth have ranged from 0 to 20 % [1–4]. It usually presents in the early postoperative period and is known to be associated with loose epithelium, epithelial defects at the time of surgery, hyperopic LASIK correction, enhancement surgeries, flap instability, and corneal epithelial basement membrane dystrophy [1, 3–5]. While not always serious, it can progress to induce irregular astigmatism or melting of the overlying flap and threaten the central vision.

Epithelial basement membrane dystrophy (EBMD) is the most common corneal dystrophy, with an estimated prevalence of 5 %. However, it is often undiagnosed because it has nonspecific symptoms [1]. The pathogenesis of EBMD involves primary synthesis of an abnormal epithelial basement membrane with intraepithelial extensions. The absence of anchoring fibrils in patients with EBMD leads to a poorly adherent epithelium and increased risk for recurrent corneal erosion (RCE). It also increases risk of a perforated flap during cutting and of diffuse lamellar keratitis (DLK) [2].

In pursuance of the IC3D classification the group of the anterior corneal dystrophies include: Epithelial and Subepithelial Dystrophies: (( Epithelial basement membrane dystrophy (EBMD)—majority degenerative, some C1; Epithelial recurrent erosion dystrophy (ERED) C4, (Smolandiensis variant) C3;Subepithelial mucinous corneal dystrophy (SMCD) C4; Mutation in keratin genes: Meesmann corneal dystrophy (MECD) C1; Lisch epithelial corneal dystrophy (LECD) C2; Gelatinous drop-like corneal dystrophy (GDLD) C10 and Bowman Layer Dystrophies: (( Reis–Bucklers corneal dystrophy (RBCD)—Granular corneal dystrophy type 3 C1;Thiel–Behnke corneal dystrophy (TBCD) C1, potential variant C2; Grayson –Wilbrandt corneal dystrophy (GWCD) C4). Foregoing course summarizes genetic background, invasive examination (histological, confocal) and non invasive modern visualization techniques such as AS SOCT. Above lecture will also assume clinical approach.

Background. Epithelial basement membrane dystrophy (EBMD) is a common disease of the anterior cornea that can lead to problems with vision and/or painful recurrent erosions of the corneal epitheliu ...

Epithelial basement membrane dystrophy, the most common hereditary anterior corneal dystrophy and considered a "category 1" dystrophy in some cases, encompasses microcystic dystrophy and other conditions affecting the epithelial basement membrane. The management of symptomatic epithelial basement membrane dystrophy includes alleviating blurred vision, treating recurrent corneal erosion, or both. Treatment of distorted vision may be as simple as prescribing lubricating drops and/or ointment, and posttrauma corneal erosion is often a limited problem that disappears over time and does not require laser or surgical treatment. This article describes treatment for more severe cases of corneal erosion, which includes mechanical debridement of the loosened epithelium.

PURPOSE: To report sloughing of corneal epithelium during laser in situ keratomileusis and subsequent wound healing complications in patients with epithelial basement membrane dystrophy. METHODS: In a retrospective study, the surgical procedures, postoperative course, and visual acuities of 16 eyes of nine patients with epithelial basement membrane dystrophy who underwent laser in situ keratomileusis complicated with epithelial sloughing at three centers were reviewed. The mean follow-up period was 23 weeks (range, 4 to 52 weeks). RESULTS: In 13 (81%) of 16 eyes with epithelial basement membrane dystrophy, epithelial sloughing occurred during laser in situ keratomileusis. In eight of the 13 eyes, epithelial growth beneath the flap was observed. The flap was lifted and the interface epithelium scraped in six eyes. Flap melt or keratolysis occurred in four eyes. At the last follow-up visit, 13 of 16 eyes had an uncorrected visual acuity of 20/30 or better, and all eyes had a best-corrected visual acuity of 20/30 or better. CONCLUSIONS: Patients with epithelial basement membrane dystrophy have poorly adherent corneal epithelium and are predisposed to epithelial sloughing during the microkeratome pass of laser in situ keratomileusis. This may lead to flap distortion, interface epithelial growth, flap keratolysis, and corneal scarring. It is not recommended that laser in situ keratomileusis be performed in patients with classic, symptomatic epithelial basement membrane dystrophy. In patients who present with mild and asymptomatic epithelial basement membrane dystrophy, laser in situ keratomileusis should be performed with caution, or photorefractive keratectomy may be the preferred refractive procedure.

Objective To characterize morphologic changes in corneas of patients with recurrent erosion syndrome or epithelial basement membrane dystrophy using in vivo confocal microscopy. Design Observational case series Participants Fourteen eyes of eight patients with diagnosed epithelial basement membrane dystrophy and 13 eyes of seven patients with recurrent erosion syndrome were examined. Methods Slit-lamp examination and in vivo confocal microscopy. The pathologic findings are presented as digitized images obtained from video tape recorded during the confocal microscopy. Main outcome measures The morphology of corneal surface epithelial cells, basal epithelial cells, subbasal nerve plexus, Bowman's layer, stromal keratocytes, and endothelium was analyzed. Results The surface epithelium was intact in all but two eyes. One cornea (a basement membrane disorder with clinically visible dots) had multinucleate surface epithelial cells, and one eye with recurrent corneal erosions showed a freely floating surface epithelium sheet in the tear fluid. Patients in both groups showed islets of highly reflective cells with presumed intracellular deposits surrounded by normal cells in the basal epithelial cell layer. The basal epithelial cell area also showed other pathologic changes, including drop-shaped configurations, streaks, or ridges. Folding of the Bowman's layer was also observed in both groups. Anterior keratocytes showed signs of activation (highly reflective nuclei with visible processes) in some of the patients regardless of the clinical diagnosis, and in recurrent erosions even increased deposition of abnormal extracellular matrix in the anterior stroma was suspected. Posterior corneal keratocytes and endothelium appeared normal when examined. The subbasal nerve plexus showed various pathologic changes, such as short or strangely shaped nerve fiber bundles, decreased numbers of long nerve fiber bundles, only faintly visible long nerve fiber bundles (instead of the normally observed long parallel running interconnected bundles), or increased amounts of Langerhans cells, but only one patient (with recurrent erosion syndrome) lacked the subbasal nerve plexus. Conclusions In vivo confocal microscopy of corneas with recurrent erosions or epithelial basement membrane dystrophy showed deposits in basal epithelial cells, subbasal microfolds and streaks, damaged subbasal nerves, or altered morphology of the anterior stroma. Confocal microscopy cannot replace biomicroscopy in making a specific diagnosis, but it sometimes helps the diagnosis in corneas that appear normal under a biomicroscope.

Purpose: To report a case of excimer laser photorefractive keratectomy (PRK) in a patient with epithelial basement membrane dystrophy (EBMD). Methods: Interventional case report. Results: A 36-year-old male with clinical manifestations of EBMD underwent bilateral, simultaneous PRK for low myopia correction. A delay (up to 17 days) in the epithelial healing process with unstable epithelium was observed. During the first three postoperative months a visually significant subepithelial haze was developed in both eyes. One year postoperative, an improvement in haze formation and visual acuity in both eyes was found. Conclusions: PRK in patients with EBMD could result in favorable refractive and clinical outcome.

Objective This study aimed to describe the morphology of cystic disorders of the corneal epithelium by confocal microscopy. Design The study design was a prospective evaluation of confocal microscopic images of patients with cystic corneal disorders. Participants Thirteen patients (19 eyes) were included. The corneal disorders included four patients with corneal decompensation (Fuchs’ dystrophy), five patients with epithelial basement membrane dystrophy (e.g., Cogan’s microcystic and map-dot dystrophies), one patient with Meesmann’s dystrophy, and three patients with recurrent erosion syndrome of unknown etiology. Confocal images of diseased comeas were compared with those of ten normal control eyes (ten subjects). Intervention All patients were examined by slit-lamp biomicroscopic analysis and confocal microscopic analysis (Tomey, Erlangen-Temmenlohe, Germany). Image analysis was used to identify the corneal epithelial structures correlated with the corresponding pathology. Main outcomes measures Confocal microscopy was used to assess the size, shape, light scatter, and reflection of the cysts. Results Slit-lamp examination results showed corneal epithelial cystic lesions in all cases. Confocal microscopy was able to identify cystic lesions in 9 (69.2%) of 13 patients. Of the four patients in whom lesions could not be found by confocal microscopy, three had recurrent erosion syndrome and the other one had epithelial basement membrane dystrophy. The confocal images were compatible with the clinical and histologic pictures of the disease. Normal control eyes did not show any epithelial lesion, either by biomicroscopy or confocal microscopy. Conclusions Confocal microscopy provides an in vivo evaluation of cystic epithelial corneal lesions. This study shows that confocal microscopy is suitable for examining cystic lesions of the corneal epithelium. Nevertheless, it is not as sensitive as biomicroscopy in detecting cystic lesions in certain corneal conditions.

Background. Epithelial basement membrane dystrophy (EBMD) is a common disease of the anterior cornea that can lead to problems with vision and/or painful recurrent erosions of the corneal epithelium. Several treatment options have been used, but recurrence of EBMD after treatment is a problem. Excimer laser phototherapeutic keratectomy (PTK) has become an increasingly popular surgical option in recent years due to its accuracy, reproducibility, and good clinical outcomes. When treating EBMD with PTK, the anterior corneal structures including the epithelium, Bowman´s layer (BL), and subbasal nerves are disrupted or removed completely. Little is known about how BL, nerves, and the stroma recover after PTK treatment, or how they could influence recurrence of EBMD symptoms. Additionally, very little is known about the properties and actual thickness of BL in-vivo.Aims. To improve the understanding and management of EBMD by investigating the clinical diagnosis and treatment of EBMD and its relationship to Bowman´s layer.Method. An excimer laser was used to treat EBMD patients at the Department of Ophthalmology during the period 2001-2010. IVCM was used to perform pre- and postoperative examinations. In particular, images of anterior corneal structures, cells, and nerves in high-resolution were obtained. Additionally, a group of over 100 healthy volunteers underwent a full ophthalmic examination including IVCM. Other subjects examined in this work included a group of 17 patients who underwent full-thickness transplantation of the cornea.Results and conclusions. Clinical follow-up revealed that PTK is an effective method of alleviating the clinical symptoms of EBMD, but the dystrophy can recur with time. Recurrence can be divided into clinical and morphologic types, and may depend upon treatment parameters including the type and depth of ablation. IVCM was found to be a useful screening tool pre- and postoperatively, and could prevent patients with symptoms, but no visible signs of EBMD on slit lamp examination, to go undiagnosed and untreated. BL was found to play a role in regenerative wound healing after PTK, and was also found to be important regarding the treatment and recurrence of EBMD. BL may present a physical barrier that protects the subepithelial nerve plexus thereby facilitating sensory recovery, and BL may also serve as a barrier that prevents direct traumatic contact with the corneal stroma, avoiding a stromal wound healing response. To aid in accurate assessment of BL in patients, an in vivo method for determining BL thickness was developed. This method could be an important tool to aid in clinical assessment and planned treatments of the anterior cornea. Using this tool, a large inter-individual variability in BL thickness and a strong negative correlation of BL thickness with age were found in a healthy population. Using IVCM, it was also found that subbasal nerves are pathologically reduced in EBMD compared to a healthy population, and that this nerve deficit does not improve in the long term after PTK treatment.

One hundred and seventeen patients with a history of recurrent corneal erosion were recruited at initial hospital presentation. Seventy-five cases had a history of shallow corneal injury, 23 had epithelial basement membrane dystrophy (EBMD), 8 had both and 11 had neither. Mean age at presentation was 38 years and follow-up ranged from 6 to 16 months (mean 10.6 months). Sixty-one patients presented with a first acute corneal erosion, 21 with a subsequent acute corneal erosion and 35 with chronic symptoms. Patients with EBMD or a trauma-related focal epithelial basement membrane abnormality were more likely to present with chronic recurrent symptoms than trauma-related cases with no abnormality on examination. Both EBMD and trauma-related cases typically recurred in the lower half of the cornea, frequently in the midline (z = 7.3, p < 0.0001), suggesting an intrinsic or acquired abnormality of the epithelial basement membrane at this site. Only four of 82 acute episodes did not resolve by 5 days with simple patching, cycloplegia and topical antibiotic ointment. In the vast majority of patients presenting with an acute erosion, simple management measures only are required. Of 117 cases started on prophylactic ointment at night, further therapy due to prophylaxis failure was required in only 5. EBMD was a risk factor for failure (relative risk 10.77). There was no difference in efficacy between once daily prophylactic paraffin and hypertonic saline ointments (p = 0.17), suggesting they both have only a lubricant action.

PURPOSE To report the confocal microscopic appearance of bleb-like disorder in epithelial basement membrane dystrophy (EBMD). METHODS Two subjects with bleb-like disorder in EBMD were examined with slit-lamp biomicroscopy and in vivo confocal microscopy using the Heidelberg Retina Tomograph II/Rostock Cornea Module. A sample obtained from epithelial debridement from 1 subject provided histologic correlation. RESULTS Confocal microscopy showed areas of circular and irregular hyporeflectivity ranging from 30 to 150 μm in diameter at the level of the basal epithelium and Bowman layer, with apparent fragmentation of the subbasal nerve plexus. The stroma, Descemet membrane, and corneal endothelium appeared normal. The epithelial biopsy results revealed subepithelial deposits of material anterior to the Bowman layer that stained positively with periodic acid-Schiff (PAS). CONCLUSIONS This clinicopathologic correlation of bleb-like disorder in EBMD using confocal microscopy demonstrates the distinctive morphology of this disorder. It provides additional information to help with the interpretation of confocal images.

To report the utility of epithelial debridement and diamond burr polishing of Bowman's layer (ED + DBP) in the management of recurrent corneal erosions and visually significant epithelial irregularity associated with epithelial basement membrane dystrophy (EBMD).Retrospective interventional consecutive case series.All patients who underwent ED + DBP by a single surgeon between November 1, 2002 and November 1, 2008.Data were collected regarding the frequency and severity of symptoms associated with EBMD as well as previous treatments. Details regarding the procedure and the postoperative course were recorded as well. The significance of the improvement in visual acuity after treatment was determined using Wilcoxon signed rank test.Change in visual acuity and recurrent corneal erosions after treatment.ED + DBP was performed on 56 eyes (42 patients) during the 72-month period under review. Of the 56 eyes, 37 (66%) were treated for recurrent corneal erosions and 22 (39%) were treated for visually significant epithelial irregularity (3 eyes were treated for both conditions). EBMD was diagnosed in 46 eyes (82%), and a history of corneal trauma was elicited in 9 eyes (16%). Visual acuity improved significantly (P = 0.016), and recurrent corneal erosions resolved after treatment in 24 (96%) of the 25 eyes with a history of corneal erosions before treatment with more than 3 months of follow-up (average, 18.9 months; range, 3.5-66.5 months). Visual acuity improved significantly (P = 0.004), and visual aberrations related to epithelial irregularity resolved in all 14 eyes treated for visually significant EBMD with more than 3 months of follow-up (average, 14.2 months; range, 3.4-50.8 months). Mild, central subepithelial corneal haze developed in 12 (26%) of the 47 eyes that did not demonstrate subepithelial haze before ED + DBP, although it was not associated with decreased vision at the last follow-up visit in any patient.ED + DBP is a safe and effective technique in the management of recurrent corneal erosions and visually significant epithelial irregularity associated with EBMD.

PURPOSE. To evaluate the corneal characteristics of patients with epithelial basement membrane dystrophy (EBMD) using an in vivo laser confocal microscope, Heidelberg Retina Tomograph II, Rostock Cornea Module (HRT II RCM). METHODS. Sixteen women and 13 men who were diagnosed with or suspected to have EBMD were included in the study. The mean age of the patients was 56.4±17.2 years within a range of 25 to 81 years. Nine patients (31%) had a history of recurrent corneal erosions and 3 patients had been misdiagnosed with herpetic epithelial keratitis. The remaining 17 patients were asymptomatic and EBMD was diagnosed incidentally during routine eye examinations. RESULTS. Confocal microscopy revealed highly reflective tissue in various configurations within the intermediate and basal epithelial cell layers corresponding to the abnormal basement membrane extending into the epithelium. There were thin, parallel hyperreflective lines and high contrast round lesions in sizes ranging between 10 and 250 µm within the epithelium. Basal epithelial cells around the abnormal basement membrane and cysts seemed to be highly distorted. In two subjects with bleb-like disorder, the authors observed circular or oval hyporeflective areas with a diameter ranging between 40 and 100 µm at the level of basal epithelium and the Bowman layer, accompanied by hyperreflective, linear structures extending into the epithelium. CONCLUSIONS. EBMD is most commonly asymptomatic and undiagnosed; however, it might be associated with recurrent corneal erosions and lead to severe complications after LASIK surgery. The confocal images are highly characteristic for EBMD; therefore, confocal microscopy seems to be a valuable tool in the diagnosis of EBMD. (Eur J Ophthalmol 2009; 19: 348-54)

Background: The recent availability of genetic analyses has demonstrated the shortcomings of the current phenotypic method of corneal dystrophy classification. Abnormalities in different genes call Cause a single phenotype, whereas different defects in a single gene can Cause different phenotypes. Sonic disorders termed corneal dystrophies do not appear to have a genetic basis. Purpose: The purpose Of this Study was to develop a new classification system for corneal dystrophies, integrating up-to-date information on phenotypic description, pathologic examination, and genetic analysis. Methods: The International Committee for Classification of Corneal Dystrophies (IC3D) was created to devise a current and accurate nomenclature. Results: This anatomic classification continues to Organize dystrophies according to the level chiefly affected. Each dystrophy has a template summarizing genetic, clinical, and pathologic information. A category number from I through 4 is assigned, reflecting the level of evidence supporting the existence of a given dystrophy. The most defined dystrophies belong to category 1 (a well-defined corneal dystrophy in which a gene has been mapped and identified and specific imitations are known) and the least defined belong to category 4 (a suspected dystrophy where the clinical and genetic evidence is not yet convincing). The nomenclature may be updated over time as new information regarding the dystrophies becomes available. Conclusions: The IC3D Classification of Corneal Dystrophies is a new classification system that incorporates many aspects of the traditional definitions of corneal dystrophies with new genetic, clinical, and pathologic information. Standardized templates provide key information that includes a level of evidence for there being a corneal dystrophy. The system is User-friendly and upgradeable and call be retrieved on the website www.corneasociety.org/ic3d.

Purpose 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. Design Diagnostic test study. Methods 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. In vivo 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. Results 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 in vivo confocal microscopy and SD OCT findings in the diagnosis of epithelial basement membrane dystrophy (κ = 0.98). Conclusions 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.

Purpose To compare the efficacy of phototherapeutic keratectomy (PTK) to epithelial debridement and polishing of Bowman's membrane using a diamond burr (DB) in the treatment of recurrent corneal erosions associated with anterior basement membrane (ABM) dystrophy. Design Retrospective nonrandomized comparative trial. Methods Medical records of 39 patients (42 eyes) who underwent a PTK or DB procedure for recurrent corneal erosions associated with ABM dystrophy between March 1992 and June 2000 were reviewed. History of injury, prior treatment received, and the corneal slit-lamp findings were noted in all patients. In both procedures, all loose epithelium was completely removed. In PTK patients, 5 μm of Bowman's membrane was ablated with the excimer laser. In patients who underwent DB treatment, a hand-held battery-driven diamond dusted burr was used to gently and uniformly polish Bowman's membrane in the area of the epithelial defect. Main outcome measures Symptomatic improvement, recurrence of painful erosions, development of haze, and change in the visual acuity. Statistical analysis was performed for comparison of data between the two groups. Results Fifteen eyes of 14 patients underwent PTK, and 27 eyes of 25 patients underwent epithelial debridement and DB treatment. In the PTK group, mild haze was seen in five (35.7%) eyes. Recurrence of painful erosions was seen in four eyes (26.7%). The mean follow-up was 17.6 ± 5.7 months (range, 0.7–81.9 months). Best-corrected visual acuity was better in five eyes (35.7%) after the procedure and the same in nine eyes (64.3%). In the DB group, mild haze was seen in seven eyes (25.9%). Recurrence of painful erosions was seen in three eyes (11.1%). The mean duration of follow-up was 6.7 months (range, 1–24.2 months). Best-corrected visual acuity was better in 3 eyes (14.3%), the same in 17 eyes (81%), and worse in 1 eye (4.8%) after the procedure. Final visual acuity was not available for one eye in the PTK group and 6 eyes in the DB group. There was no statistically significant difference in haze (Fisher's exact test, P = 0.38), recurrence of erosions (Kaplan-Meier analysis with log rank, P = 0.73), and vision being better or the same (Fisher's exact test, P = 0.6) between the PTK and DB groups. Conclusions Both PTK and DB treatment are effective methods of treating recurrent corneal erosions associated with ABM dystrophy. Diamond burr treatment, being a simpler, less expensive office procedure with a tendency toward lesser incidence of haze and recurrence in this study, seems to have advantages over PTK in the treatment of recurrent corneal erosions. Further prospective studies are required to confirm the long-term efficacy of DB treatment in the management of recurrent corneal erosions associated with ABM dystrophy.

Recurrent corneal erosion (RCE) is a common disorder that usually arises following specific trauma to the cornea, or epithelial basement membrane dystrophy (EBMD).1,2 Several studies on patients with RCE have been carried out light, electron or in vivo confocal microscopy.3–5 However, the tear film condition over abnormal elevated epithelium in RCE or EBMD patients is little understood.2 The present study evaluated the correlation between tear film and diseased corneal epithelium in patients with RCE or EBMD.

Background. Epithelial basement membrane dystrophy (EBMD) is a common disease of the anterior cornea that can lead to problems with vision and/or painful recurrent erosions of the corneal epithelium. Several treatment options have been used, but recurrence of EBMD after treatment is a problem. Excimer laser phototherapeutic keratectomy (PTK) has become an increasingly popular surgical option in recent years due to its accuracy, reproducibility, and good clinical outcomes. When treating EBMD with PTK, the anterior corneal structures including the epithelium, Bowman´s layer (BL), and subbasal nerves are disrupted or removed completely. Little is known about how BL, nerves, and the stroma recover after PTK treatment, or how they could influence recurrence of EBMD symptoms. Additionally, very little is known about the properties and actual thickness of BL in-vivo.Aims. To improve the understanding and management of EBMD by investigating the clinical diagnosis and treatment of EBMD and its relationship to Bowman´s layer.Method. An excimer laser was used to treat EBMD patients at the Department of Ophthalmology during the period 2001-2010. IVCM was used to perform pre- and postoperative examinations. In particular, images of anterior corneal structures, cells, and nerves in high-resolution were obtained. Additionally, a group of over 100 healthy volunteers underwent a full ophthalmic examination including IVCM. Other subjects examined in this work included a group of 17 patients who underwent full-thickness transplantation of the cornea.Results and conclusions. Clinical follow-up revealed that PTK is an effective method of alleviating the clinical symptoms of EBMD, but the dystrophy can recur with time. Recurrence can be divided into clinical and morphologic types, and may depend upon treatment parameters including the type and depth of ablation. IVCM was found to be a useful screening tool pre- and postoperatively, and could prevent patients with symptoms, but no visible signs of EBMD on slit lamp examination, to go undiagnosed and untreated. BL was found to play a role in regenerative wound healing after PTK, and was also found to be important regarding the treatment and recurrence of EBMD. BL may present a physical barrier that protects the subepithelial nerve plexus thereby facilitating sensory recovery, and BL may also serve as a barrier that prevents direct traumatic contact with the corneal stroma, avoiding a stromal wound healing response. To aid in accurate assessment of BL in patients, an in vivo method for determining BL thickness was developed. This method could be an important tool to aid in clinical assessment and planned treatments of the anterior cornea. Using this tool, a large inter-individual variability in BL thickness and a strong negative correlation of BL thickness with age were found in a healthy population. Using IVCM, it was also found that subbasal nerves are pathologically reduced in EBMD compared to a healthy population, and that this nerve deficit does not improve in the long term after PTK treatment.

PURPOSE: To report the incidence and risk factors for clinically significant epithelial ingrowth after laser in situ keratomileusis as well as the recurrence rate and visual outcomes after its treatment. METHODS: We defined clinically significant epithelial ingrowth as that which required surgical removal. From a cohort of 3,786 eyes that underwent primary laser in situ keratomileusis from February 1996 to August 1998 and its derivative of 480 eyes that later underwent enhancement laser in situ keratomileusis by one surgeon (R.K.M.), we identified all eyes with clinically significant epithelial ingrowth. RESULTS: The incidence of significant epithelial ingrowth after primary treatment was 35 (0.92%) of 3,786 eyes. The incidence after enhancement treatment was eight (1.7%) of 480 eyes (p = NS). Fourteen of 43 eyes had a postoperative epithelial defect associated with subsequent development of epithelial ingrowth. Six of 43 eyes had loose epithelium intraoperatively, suggesting epithelial basement membrane dystrophy. Epithelial ingrowth was treated by lifting the flap, scraping the bed and the posterior surface of the flap, and replacing the flap without the use of caustic agents. In 42 of 43 eyes, the epithelial ingrowth under the flap was continuous with the surface epithelium. Clinically significant ingrowth recurred in 10 of 43 eyes after the initial surgical removal. CONCLUSIONS: Clinically significant epithelial ingrowth is an infrequent complication of laser in situ keratomileusis. We hypothesize that epithelial ingrowth is secondary to postoperative invasion under the flap by surface epithelial cells rather than intraoperative implantation of epithelial cells. Treatment should consist of complete mechanical removal of epithelium from the posterior surface of the corneal flap and keratectomy bed and ensuring tight apposition of the flap with the bed.

Editor,—I read with interest the article by Heyworth and associates on the natural history of recurrent corneal erosion syndrome,1 published in the January 1998 issue of BJO . However, this article raises certain issues of concern. Firstly, the title “A 4 year review of 117 patients” is misleading, since 94 patients were studied. Furthermore, the authors also state in the first sentence that epithelial basement membrane dystrophy (EBMD) is an anterior stromal dystrophy. The two are distinct clinical and pathological entities. A further point of concern is that none of these patients was examined …

Background. Epithelial basement membrane dystrophy (EBMD) is a common disease of the anterior cornea that can lead to problems with vision and/or painful recurrent erosions of the corneal epithelium. Several treatment options have been used, but recurrence of EBMD after treatment is a problem. Excimer laser phototherapeutic keratectomy (PTK) has become an increasingly popular surgical option in recent years due to its accuracy, reproducibility, and good clinical outcomes. When treating EBMD with PTK, the anterior corneal structures including the epithelium, Bowman´s layer (BL), and subbasal nerves are disrupted or removed completely. Little is known about how BL, nerves, and the stroma recover after PTK treatment, or how they could influence recurrence of EBMD symptoms. Additionally, very little is known about the properties and actual thickness of BL in-vivo.Aims. To improve the understanding and management of EBMD by investigating the clinical diagnosis and treatment of EBMD and its relationship to Bowman´s layer.Method. An excimer laser was used to treat EBMD patients at the Department of Ophthalmology during the period 2001-2010. IVCM was used to perform pre- and postoperative examinations. In particular, images of anterior corneal structures, cells, and nerves in high-resolution were obtained. Additionally, a group of over 100 healthy volunteers underwent a full ophthalmic examination including IVCM. Other subjects examined in this work included a group of 17 patients who underwent full-thickness transplantation of the cornea.Results and conclusions. Clinical follow-up revealed that PTK is an effective method of alleviating the clinical symptoms of EBMD, but the dystrophy can recur with time. Recurrence can be divided into clinical and morphologic types, and may depend upon treatment parameters including the type and depth of ablation. IVCM was found to be a useful screening tool pre- and postoperatively, and could prevent patients with symptoms, but no visible signs of EBMD on slit lamp examination, to go undiagnosed and untreated. BL was found to play a role in regenerative wound healing after PTK, and was also found to be important regarding the treatment and recurrence of EBMD. BL may present a physical barrier that protects the subepithelial nerve plexus thereby facilitating sensory recovery, and BL may also serve as a barrier that prevents direct traumatic contact with the corneal stroma, avoiding a stromal wound healing response. To aid in accurate assessment of BL in patients, an in vivo method for determining BL thickness was developed. This method could be an important tool to aid in clinical assessment and planned treatments of the anterior cornea. Using this tool, a large inter-individual variability in BL thickness and a strong negative correlation of BL thickness with age were found in a healthy population. Using IVCM, it was also found that subbasal nerves are pathologically reduced in EBMD compared to a healthy population, and that this nerve deficit does not improve in the long term after PTK treatment.

Altogether, 80 patients aged between 24 and 73 years with recurrent erosion of the cornea have been studied and compared with a control group of 200. The patients' erosions were divisible into macroform and microform types. The macroform occurred in 10%, the microform in 56%, and both types in the same patients in 31%. The macroform was more commonly related to trauma than the microform. However, many (40%) were spontaneous in origin. The most common cause of the initial trauma was a finger nail. The recurrences occurred at around the time of waking, either just before or just after. Difficulty in opening the eye occurred in 10%. There was little evidence of precipitating factors, but eye rubbing was admitted by 10% and barbiturates were implicated in 3%. The corneae were examined in the healed state, when a high incidence (59%) were found to have superficial corneal dystrophies of the fingerprint lines, bleb, and Bietti's lacunar (map-like) types. These are considered individually, particular attention being paid to the distinction between the various types of line resembling the fingerprint line. Epithelial microcysts were also a common finding (59%) and were sometimes of the Cogan type. In only 11% of patients were there no corneal signs in the healed state. The need for careful examination of the cornea by retroillumination, using both the iris and the fundus, is stressed. The control group, in contrast, showed a very low incidence of dystrophies and cysts. Treatment was given initially with either drops or ointment and no differences in healing were found. Debridement was performed in 12 eyes as an initial treatment and also in four eyes which were not healing on medical treatment. Debridement assisted healing, but did not prevent recurrence. One eye was treated with debridement and scarification and seven with carbolization. These procedures appeared to reduce the recurrence rate. Sodium chloride ointment 5% was found useful as a prophylactic taken at bedtime, and the recurrence rate increased when it was withdrawn.

Thirty eyes with symptomatic epithelial basement membrane dystrophy that were unresponsive to current standard modalities of therapy were treated with a total superficial epithelial keratectomy. Follow-up ranged from two months to 89 months (average, 37 mo). One hundred percent of patients were relieved of their preoperative symptoms, and only 3% have shown any evidence of recurrence of their dystrophic process.

Subendothelial blebs were seen in cases of superficial keratopathy of varied etiology: superficial punctate keratopathy (42 patients); keratoconjunctivitis sicca (11 patients); epithelial basement membrane dystrophy (9 patients); mild superficial keratopathy due to exposure (9 patients), trichiasis (6 patients), or chemical contact (4 patients); and in contact-lens wearers (29 patients). The blebs were probably due to intercellular edema, separating endothelial cells from Descemet's membrane, and were reversible after resolution of the keratopathy. If the condition is chronic, changes in endothelial morphology may occur, with pleomorphism and polymegathism of the endothelial cells. These cases show that conditions which cause fine disruption of the corneal epithelium affect the integrity of the corneal endothelium. Recognition of resulting long-term changes may be important before intraocular surgery or if the cornea is to be used for grafting.

Seventeen consecutive eyes with corneal epithelial basement membrane with symptoms of recurrent erosion and/or decreased vision were treated with corneal epithelial basement membranectomy. Younger patients tended to have recurrent erosion, whereas older patients had decreased vision. All patients' symptoms improved following surgery. Electron microscopy revealed reduplication of the corneal epithelial basal lamina.

A series of 35 patients manifested various configurations of the anterior cornea. Previous reports have consisted of less than ten patients, and have implied that this condition is relatively rare and innocuous. This report suggests that, because of its subtle appearance, the condition may be easily overlooked. Hence it may be more common than supposed. It may also cause considerable morbidity as a result of poor epithelial adherence and corneal surface irregularities. Preliminary therapeutic trials suggest that hypertonic agents like sodium chloride as well as mechanical debridement are effective in controlling recurrent corneal erosions.

Map-dot-fingerprint basement-membrane abnormalities of the cornea are common in the general population, affecting as many as 76% of persons over age 50 and 42% of persons of all ages. The prevalence of this condition in the general population is not significantly different from that found in families of patients with recurrent corneal erosions and map-dot-fingerprint corneal changes. Despite this extremely high prevalence of basement-membrane changes the incidence of recurrent erosive symptoms in total groups of patients with basement-membrane changes is quite rare, suggesting that these 2 entities are possibly not related. Although previous observers have suggested an autosomal dominant mode of inheritance of these basement-membrane changes, our data raise the possibility that map-dot-fingerprint basement-membrane changes represent an age-dependent, degenerative condition of the cornea. We were unable, however, to prove either hypothesis.

Corneal epithelial opacities similar to those seen in corneal epithelial basement membrane dystrophy were seen in 33 of 71 eyes following radial keratotomy. These changes were clinically indistinguishable from those seen in map-fingerprint-dot dystrophy with map type changes seen most often. Basement membrane changes tended to be transient (persisting less than 3 months in 75.3% of eyes) and not usually visually significant. However, three eyes had changes which persisted for 12 months. Most eyes with basement membrane changes had involvement of less than one-half quadrant of the corneal (68%). Involvement of two or more quadrants occurred in 9.4% of eyes with these changes. One eye had visually significant changes which persisted for 3 months. There were no episodes of recurrent erosion.

Refractile changes in corneal transparency that are associated with epithelial basement membrane dystrophy have been detected in contact lens wearers who show a wide range of contact lens tolerance. In some of these cases, contact lens wear precipitates an episode of recurrent epithelial erosion. In other cases excellent contact lens tolerance is observed. The cases described indicate that pure bleb forms of epithelial basement membrane dystrophy may not be of benign significance when contact lenses are worn.

Experimental and pathologic studies implicated the role of the epithelial basement membrane and its associated hemidesmosomes and anchoring fibrils in mediating tight adhesion between corneal epithelium and stroma. We evaluated the ultrastructural quality of basement membrane complexes in patients with Reis-Bucklers dystrophy, Cogan's microcystic dystrophy, and nontraumatic recurrent erosion who presented with clinically significant erosive symptoms. Despite the variable clinical and histopathologic appearances among these dystrophies, incompleteness of basement membrane complexes apparently was a common fault shared by these erosive disorders. Such basic incompetence at the basement membrane level can both provoke and sustain erosions, while the more individual pathologic manifestations of a given anterior dystrophy may be of less primary significance.

• Thirteen eyes with symptomatic basement membrane dystrophy were treated with total superficial keratectomy and soft bandage lenses. During a follow-up of six weeks to 48 months, 84.7% (11/13) were relieved of symptoms. A low incidence of recurrent basement membrane abnormalities was observed.

A case of fingerprint corneal dystrophy with epithelial cysts has been studied histologically, using ultrathin sections for light and electron microscopy. Fingerprint lines that are ensheathed in normal basement membrane consist of a fibrillogranular protein apparently a product of the corneal epithelium. They are continuous with a subepithelial seam of similar, friable material thus accounting for the ready dehiscence and recurrent breakdown of the epithelium. Moribund cells stimulate fusion of epithelial cells to form multinucleate giant cells. These seclude degenerating cells that thus sequestered are taken by centripetal cell movement toward the tear film where they are discharged. The histologic evidence suggests that fingerprint dystrophy, the microcystic dystrophy of Cogan, and the recurrent erosion syndrome are closely related disorders.