Hereditary,recurrent corneal erosions

The review presents a clinical case of 40 years old female patient with bilateral recurrent corneal erosions (r.c.e.), without an obvious cause. It is relevant to mention the present of the r.c.e. in her family during 4 generations. The acute corneal lesions looked like a "map-dot-fingerprint" dystrophy and 3 weeks later cornea became normal.     

Phototherapeutic keratectomy for epithelial irregular astigmatism: an emphasis on map-dot-fingerprint degeneration

PURPOSE: To study the changes in irregular astigmatism in patients with recurrent corneal erosion syndrome due to map-dot-fingerprint degeneration and to demonstrate that both symptomatic and asymptomatic map-dot-fingerprint degeneration/recurrent corneal erosion syndrome may be related to irregular astigmatism, which can effectively be corrected by phototherapeutic keratectomy (PTK) or standard spherical photorefractive keratectomy (PRK). METHODS: Eleven eyes with irregular astigmatism related to map-dot-fingerprint degeneration/recurrent corneal erosion syndrome with and without changes at the slit-lamp microscope were treated with PTK or spherical PRK, which was performed with a VISX S2 or S4 excimer laser. All eyes underwent videokeratography performed pre- and postoperatively using either the Tomey TMS-2N Topographic Modeling System or EyeSys Technologies Corneal Analysis System 2000. RESULTS: Mean pre- and postoperative best spectacle-corrected visual acuity (BSCVA) was -0.18 +/- 0.14 logMAR and 0.04 +/- 0.04 logMAR, respectively. This improvement in BSCVA was statistically significant. The mean gain in Snellen lines was 2.4 +/- 2.1. Preoperative corneal videokeratography showed irregular astigmatism with an elevation pattern that was corrected in all eyes despite the fact that no astigmatic photorefractive correction was performed. CONCLUSIONS: Following PTK, eyes with irregular astigmatism related to map-dot-fingerprint degeneration showed significant improvement in BSCVA and correction of irregular astigmatism as revealed by videokeratography. This study shows that irregular astigmatism can be exclusively of epithelial origin and in some eyes abnormal corneal epithelium may create optical aberrations. This possibility should be taken into account when, for example, wavefront-guided stromal photoablation procedures are being planned.     

Long-term results of phototherapeutic keratectomy for corneal map-dot-fingerprint dystrophy (Cogan-Guerry)

PURPOSE: Patients with corneal map-dot-fingerprint dystrophy suffer typically from recurrent corneal erosion, disturbed vision, or both. The purpose of this study was to assess the morphologic and functional long-term results of minimal invasive subepithelial phototherapeutic keratectomy (PTK) for corneal map-dot-fingerprint dystrophy. METHODS: Of a total of 390 PTKs performed between October 1994 and January 2004, 15 PTKs on 15 eyes of 11 patients were included in this single-center study. All patients had symptoms of recurrent corneal erosion; in 12 eyes, reduced visual acuity was observed. The median duration of complaints was 18 months. Using 193-nm excimer laser (MEL 60/70; Carl Zeiss-Meditec), a manually guided spot profile was applied in 7 cases (pulse energy, 12 mJ; repetition rate, 2/s or 3/s; 189-425 pulses). In 8 cases, a scanning slit mode was chosen (intended ablation, 1 microm/scan; repetition rate, 20/s; 150-483 pulses). In each case, a broad deepithelialization of the Bowman layer was followed by application of defocused overlapping laser pulses. RESULTS: Complete epithelial closure was achieved after an average of 3.5 +/- 0.6 days (median, 3 days). The mean follow-up was 4.8 +/- 3.0 years, with a maximum of 9.3 years. Best corrected visual acuity increased from 0.7 +/- 0.26 preoperatively to 0.9 +/- 0.16 postoperatively. The keratometric central power remained constant (preoperatively, 43.0 +/- 1.6 D; postoperatively, 42.6 +/- 1.0 D). The average keratometric astigmatism remained constant (1.3 +/- 0.9 D, preoperatively; 1.0 +/- 0.5 D, postoperatively). In the early postoperative stage, subtle superficial corneal opacities ("haze") were observed in 6 eyes (40%), being completely reversible during the follow-up in 5 cases. No recurrence of corneal erosion was observed during the follow-up. Asymptomatic dystrophic signs in the midperiphery became visible in 2 eyes 3 and 5 years after PTK. CONCLUSION: For corneal map-dot-fingerprint dystrophy, PTK using an excimer laser with low pulse energy and low number of pulses can be considered an effective and minimal invasive treatment modality to achieve a fast and durable epithelial closure, to prevent recurrent corneal erosions, and to increase visual acuity in most patients.     

"With the rule" astigmatism is not the rule in the elderly. Reykjavik Eye Study: a population based study of refraction and visual acuity in citizens of Reykjavik 50 years and older. Iceland-Japan Co-Working Study Groups

PURPOSE: To study refractive state and visual acuity in citizens of Reykjavik 50 years and older. METHODS: 1700 persons were randomly selected from the national population census. 1379 could be located and qualified whereof 1045 participated. Evaluation of refraction was performed using Nidek ARK 900 autorefracto-keratometer. Visual acuity was tested on a Snellen chart. RESULTS: The prevalence of hypermetropia increases with age by 0.3 D in five years. The prevalence of "against the rule" astigmatism increased on average 5.3% and oblique 3.9% in five years. Analysis of corneal astigmatism measured by keratometer shows an "against the rule" change with age. CONCLUSIONS: Hypermetropia increases by age. The prevalence of astigmatism increases and the axis turns to "against the rule". The changes in total astigmatism and corneal astigmatism is almost parallel which might indicate that the "against the rule" change is related to changes in the cornea.     

Clinical and experimental studies concerning circulating antibodies to corneal epithelium antigens

Corneal epithelium antibodies were detected in patients with corneal melting disease (55%), uveitis (42%), corneal transplantation (42%) and marginal furrow disease (20%). These antibodies were not found in herpetic keratitis patients. In control groups, consisting of ocular surgery patients (glaucoma, retinal detachment and cataract) and persons without a history of ocular disease, approximately 4% of the subjects had these antibodies. To investigate the possible role of trauma to the cornea as an initiator of corneal epithelium antibodies, these antibodies were determined in rabbits after alkaline burns were made on the cornea. These antibodies were detected one week later and disappeared after six weeks.Serum from three patients with corneal melting disease and corneal transplantation containing a high antibody titre, against corneal epithelium were used to isolate corneal epithelium antigens.A 54 kD and a 17 kD corneal epithelium antigen were isolated. The incidence of autoantibodies directed against these antigens was investigated in patients with corneal melting disease, uveitis and corneal transplantation using an ELISA. 50% of the sera positive in the immunofluorescence test were positive in the ELISA.     

北京市西城区特定人群干眼症的患病率调查

目的 调查西城区一机关内在职及离退休人员干眼症的患病情况，以了解北京市社区正常人群干眼症的流行病学情况。方法 2003年9月1日～30日对北京西城区一机关内在职及离退休人员共1997人进行常规眼部检查，同时进行SchirmerⅠ试验和泪液镜（Tearscope Plus，Keeler公司）检测BUT，共有3994只眼。共中男性1072人，年龄20～90岁（59．53±15．86岁），女性925人，19～85岁（55．36±14．28岁）。角结膜干燥症的诊断标准为：SchirmerⅠ≤5mm、BUT≤10秒，伴随患者主诉眼干燥症状，所得数据采用SPSSforWindows统计软件进行分析。结果本次分层非匹配病例对照研究检查确诊为干眼症的病人共有122人。眼部疾病尤其是角膜疾病对病人的干眼症有影响，干眼症的发病机会随接触电脑时间的增多而增多，有线性关系。结论 多种因素影响患者的泪膜状态，干眼症的患病率是比较高的，应正确对待干眼症，不要滥用抗生素。     

Pathogenesis and treatment of recurrent erosion.

A series of recurrent corneal erosions secondary to map-dot-fingerprint dystrophy is presented. Erosions were closely related to the Hudson-Stahli line, and this may be a factor in pathogenesis. Traumatic abrasions did not demonstrate such localisation, evidence that trauma is not a primary cause. A trial of management with therapeutic contact lenses versus topical medication was performed. Therapeutic contact lenses were shown to be inferior and had a high complication rate. Recurrent erosion is often considered an indication for therapeutic contact lenses, but this is questioned and great caution recommended in such use.     

Phototherapeutic keratectomy for the treatment of Cogan's microcystic dystrophy

PURPOSE: This retrospective study was designed to investigate the therapeutic potential of phototherapeutic keratectomy (PTK) for the treatment of map-dot-fingerprint corneal dystrophy (MDFCD). PATIENTS AND METHODS: PTK was performed with the Excimed UV 200 and with the Nidek EC5000 on 38 patients (55 eyes). Mean patient age was 51 years (range, 28-86 years). The mean follow-up period was 19 months (range, 8-54 months). The treatment goal for each patient was to improve vision (17 eyes), to heal recurrent corneal erosions (28 eyes) or both (10 eyes). RESULTS: In 13 of 17 eyes (76.47%) with reduction in visual acuity, best corrected visual acuity (BCVA) improved by two lines or more. In 36 eyes of 38 (94.7%) with recurrent corneal erosions, there was no recurrence during the follow-up period. No decreased BCVA was noted. No recurrence of corneal dystrophic changes was seen in the ablation zone at the final follow-up. The mean hyperopic shift caused by tissue ablation was +O.55+/-1.35 after 1 year. CONCLUSION: Excimer laser PTK is a safe and effective outpatient treatment and should be used as initial treatment for MDFCD.     

Irregular astigmatism in patients with anterior membrane dystrophy (map-dot-fingerprint type)

Irregular astigmatism, the clinical manifestation of corneal warpage, was evaluated as a cause of decreased visual acuity in 24 patients with anterior membrane dystrophy (map-dot-fingerprint type). Qualitative ophthalmometry demonstrated corneal warpage in 20 of the patients. The best corrected visual acuity was improved with contact lenses over that achieved with spectacles in 14 of the 20. This appeared to confirm corneal warpage to be an important cause of decreased visual acuity in patients with anterior membrane dystrophy. Although the long-term use of contact lenses may be limited by these patients' tendency to corneal epithelial erosion, 18 of the 24 patients in this series had been wearing contact lenses and tolerating them well.     

Corneal topography in atypical pellucid marginal degeneration.

PURPOSE: Pellucid marginal degeneration (PMD) is commonly described as a noninflammatory corneal ectasia typically involving the inferior cornea. Although reports of superior corneal changes in PMD exist, the topographic patterns of these PMD variants are not well characterized. We describe corneal topographic alterations seen in eyes with atypical PMD. METHODS: Computer-assisted videokeratography was performed in 10 eyes of five patients with PMD. RESULTS: Eight of the eyes studied had changes involving the superior cornea, with or without changes in the inferior cornea. Atypical presentations of PMD included extension of inferior peripheral thinning above the horizontal meridian and occurrence of superior corneal thinning and ectasia with or without typical inferior changes. Atypical PMD reveals topographic patterns similar to classic PMD, but corneal changes also involve the nasal, temporal, or superior quadrants or a combination of these. The topographic maps reflect the location of peripheral corneal thinning and extent of corneal protrusion. CONCLUSIONS: Corneal topographic changes in atypical PMD are similar to those seen in typical PMD but can involve the superior cornea.     

RGP对早期圆锥角膜患者屈光状态改变的分析

目的：分析RGP与框架镜对圆锥角膜患者屈光系统的影响及RGP对不同类别(性别、年龄不同)患者的影响,进而探讨RGP的作用。

方法：确诊为早期圆锥角膜患者46例,其中27例为双眼,19例为单眼,共73眼。对患者配戴框架镜及RGP时,视力矫正幅度的差异进行比较; 对配戴RGP前、后分别进行的2次验光的差值进行比较,并针对RGP对不同性别、年龄人群矫正视力的改变值进行统计学分析,对其中44例患者69眼的角膜厚度与正常人进行比较。

结果：配戴框架镜与配戴RGP的患者视力矫正幅度的差异有统计学意义(P<0.05)。患者配戴RGP前2次验光结果的差值与配戴RGP时2次验光结果的差值相比较,差异有统计学意义(P<0.05)。男性与女性矫正视力幅度差异有统计学意义(P<0.05)。25岁以下与大于等于25岁患者矫正视力幅度差异无统计学意义(P>0.05)。确诊患者角膜厚490μm。

结论：(1)配戴RGP患者矫正视力优于配戴框架镜患者。(2)配戴RGP时验光误差小于不戴镜验光。(3)RGP矫正视力男性优于女性。(4)RGP矫正视力与年龄差异无关。(5)圆锥角膜患者角膜中央区厚度变薄。     

Recurrent erosions of the cornea: epidemiology and treatment

PURPOSE: To evaluate the epidemiologic characteristics of a large clinical population of patients with recurrent erosions of the cornea. The efficacy of different modalities of treatment was also evaluated. METHODS: A retrospective chart review of all patients with the diagnosis of recurrent corneal erosion treated between January 1990 and December 1998 was performed. RESULTS: Clinically confirmed recurrent erosions were identified in 104 patients. There were 36 males and 68 females. A history of trauma was present in 47 patients (45%), 30 patients (29%) had epithelial basement membrane dystrophy (EBMD), and 18 patients (17%) had both a history of trauma and evidence of EBMD. More than 87% of all erosions occurred on the inferior third of the cornea. Conservative therapy was used as the primary treatment in 52 patients with a recurrence rate of 6%. Corneal stromal micropuncture was performed on 38 patients with a recurrence rate of 40%. Eleven patients had epithelial debridement with a recurrence rate of 18%. Four patients had a superficial keratectomy with a diamond bur with a recurrence rate of 25%. Only one patient had an excimer phototherapeutic keratectomy, and she had a minor recurrence posttreatment. CONCLUSIONS: In our series, the distribution of trauma and EBMD in patients with recurrent erosions of the cornea is roughly equivalent. Conservative therapy was effective in approximately one half of the patients. All surgical treatment modalities were associated with recurrences. Those patients with both EBMD and trauma were more likely to have a recurrence after treatment. More effective treatment modalities for recurrent erosions of the cornea need to be investigated.     

Nonfamilial anterior corneal dystrophy.

A 31-year-old woman with subepithelial corneal opacification and numerous clear round or oval areas of epithelial edema confined to the palpebral fissure underwent a penetrating keratoplasty. The clinical appearance was similar to that of a severe Meesmann or Stocker-Holt dystrophy. The prominent histopathologic features were thickening and excrescences of the epithelial basement membrane, intense basal cell edema, but no intraepithelial cysts. The basement membrane changes are compatible with those seen in Meesmann, Stocker-Holt, and map-dot-fingerprint dystrophy, but the lack of intraepithelial cysts is not characteristic of these dystrophies. This case represents a variant of the known anterior corneal dystrophies which have an overproduction of epithelial basement membrane.     

Corneal thickness in nephropathic cystinosis.

Cystinosis is a rare autosomal recessive metabolic disorder in which non-protein cystine accumulates within cellular lysosomes owing to a defect in lysosomal cystine transport. The pathognomonic ocular manifestation of cystinosis is the deposition of distinctive iridescent crystals in the cornea, not associated with any inflammatory response or recognised change in corneal function. We measured corneal thickness in nine patients with infantile nephropathic cystinosis. We also studied a corneal button from one of these patients who underwent corneal transplantation. All nine patients had increased corneal thickness in comparison with an age matched control population. Electron microscopy analysis of the cystinotic button revealed structural changes of both epithelium and endothelium layers. Increased corneal thickness in patients with nephropathic cystinosis may reflect subclinical corneal oedema.     

Corneal topography in cataract surgery

Keratometry and corneal topography remain the most important means of evaluating induced corneal changes after surgery and have comparable sensitivities in the paracentral region of the cornea. However, keratometry gives no information about the peripheral cornea or about asymmetry of the cornea. Videokeratography should be performed after cataract surgery in cases in which best-corrected visual acuity is not adequate and there are no other obvious causes for poor vision to determine whether corneal irregularities are present. The recent literature on corneal topographic evaluation of induced astigmatism after cataract surgery suggests that in general, smaller, temporal incisions result in less astigmatism. Preoperatively, corneal topography can be used in the calculation of intraocular lens power as well as incision planning. Postoperatively, it can be used to detect tight sutures, torsion of the wound, internal wound gape, and irregular astigmatism, as well as to guide suture removal. In the future, corneal topography will become increasingly important in the determination of intraocular lens power in difficult cases such as patients undergoing combined cataract extraction and penetrating keratoplasty as well as patients with a history of radial keratotomy or photorefractive surgery.     

Recurrence of lattice corneal dystrophy type 1 in the corneal grafts of two siblings

Amyloid was identified by light and electron microscopy within corneal grafts of two male siblings from a family with lattice corneal dystrophy type 1. These deposits indicate a recurrent disease within the donor tissue, and we believe this reflects an infiltration of the grafts by genetically abnormal host corneal fibroblasts. One of the patients developed bilateral recurrent disease in the grafts eight and 13 years after penetrating keratoplasty. His brother required a regraft 16 years after a penetrating keratoplasty. Although both of these patients required regrafts because of impaired visual acuity, the decrease in visual acuity in one case was not solely the result of reaccumulation of amyloid, but was at least in part caused by a plaque of fibrous tissue behind the cornea.     

Recurrent corneal erosion, microcystic epithelial dystrophy, map configurations and fingerprint lines in the cornea

A 45-year-old lady presented with a recurrent corneal erosion. On closer examination, she was found to have epithelial microcysts and fingerprint dystrophy. Pathologic material from the affected cornea showed microcysts, protrusions of basement membrane and evidence of poor adhesion between the basement membrane and the epithelial cells, as well as between the epithelial cells themselves. The case demonstrated the clinical and pathologic features of recurrent corneal erosion, microcystic disease and fingerprint dystrophy of the cornea, together with the pathologic findings of map dystrophy. The authors suggest that these four diseases are various expressions of a clinicopathologic entity, epithelial basement membrane corneal dystrophy. A pathologic explanation for recurrent corneal erosion is apparent.     

Endothelial cells in dominant cornea guttata

A family with autosomal dominant cornea guttata in three generations is described. The investigated pedigree consisted of 25 healthy patients and 6 patients with cornea guttata. A few guttata were already present at an early age and very pronounced corneal endothelial guttata appeared in the middle-aged patients. No other ocular pathology was associated with this condition and the endothelial cell counts were not affected by guttata excrescences. The corneal thickness was normal in the affected and nonaffected members of the family while bullous keratopathy did not seem to be associated with dominant cornea guttata in this family.     

Double-blind method of using solcoseryl ophthalmic gel and 2,4% cysteine in ophthalmic gel in patients with chronic recurrent keratitis and keratitis sicca

A double blind study with Solcoseryl compounds in ophthalmic gel and with 2.4% cystein in ophthalmic gel was performed in the period 1985-1988; it was used in 18 cases: with chronic recurrent keratitis (11 patients) and sicca keratitis (7 patients). Observed was the influence of these compounds on the epithelialization of the cornea, on the improvement of corneal transparency, the diminutation of the troubles connected with dessication of the eye in sicca keratitis. In spite of the small number of cases the observations were interesting. In patients with a chronic recurrent keratitis the influence of both compounds showed to be beneficial for the condition of the cornea. In patients with keratitis sicca one could observe a better tolerance of the Solcoseryl ophthalmic gel which could be applied for a longer period in comparison with cystein in gel.     

Is endothelial microscopy necessary before cataract surgery?

Specular microscopy was performed before cataract surgery in approximately 3,500 patients. In 127 cases (approx. 4%), implantation of an intraocular lens (IOL) did not appear appropriate, in view of the condition of the cornea. Many of these patients had additional ocular pathology, such as retinal detachment, glaucoma, and intraocular inflammation. In 40 of the patients, IOLs were implanted in spite of the results of specular microscopy, while 48 patients underwent surgery without lens implantation. The incidence of postoperative corneal complications in the two groups was the same. The main problems were caused by cell polymorphism and severe cornea guttata. It is extremely difficult to assess cell polymorphism at the slitlamp. Especially in patients with other ocular diseases and corneal lesions visible at the slitlamp, it is recommended that specular microscopy be performed prior to cataract surgery with implantation of an IOL.