准分子激光双面式切削原位角膜磨镶术的研究进展

准分子激光双面式切削原位角膜磨镶术(Both-sided LASIK,BSL)是准分子激光原位角膜磨镶术(laser in situ keratomileusis, LASIK)的改良,BSL将部分激光切削分布在角膜瓣基质面,因而减少了对角膜基质床的切削,最大限度的保留了角膜基质床的剩余厚度,为降低术后角膜膨出提供可能,对屈光度相对偏高和/或角膜相对偏薄的患者,尽量增加手术的安全性,并为LASIK术后屈光回退的增强手术提供了一种新的方法。本文对近年BSL的研究进展作一综述。     

Evolving concepts on the pathogenic mechanisms of aniridia related keratopathy

Heterozygosity for PAX6 deficiency (PAX6+/-) results in aniridia. Corneal changes in aniridia-related keratopathy (ARK) include corneal vascular pannus formation, conjunctival invasion of the corneal surface, corneal epithelial erosions and epithelial abnormalities, which eventually result in corneal opacity and contribute to visual loss. Corneal changes in aniridia have been attributed to congenital deficiency of corneal limbal stem cells. The aim of this paper is to review the potential mechanisms that may underlie the pathogenesis of aniridia related keratopathy. Current evidence, based on clinical observations and an animal model of aniridia suggest that the proliferative potential of the corneal limbal stem cells may not primarily be impaired. The corneal changes in aniridia may be related to an abnormality within the limbal stem cell niche. The mechanisms underlying progressive corneal pathology in aniridia appear multi-factorial and include: (1) abnormal corneal healing responses secondary to anomalous extracellular matrix metabolism; (2) abnormal corneal epithelial differentiation leading to fragility of epithelial cells; (3) reduction in cell adhesion molecules in the PAX6 heterozygous state, rendering the cells susceptible to natural shearing forces; and (4) conjunctival and corneal changes leading to the presence of cells derived from conjunctiva on the corneal surface.     

Canine mycotic keratoconjuntivitis caused by Acremonium kiliense

Mycotic keratoconjuntivitis caused by Acremonium kiliense in a German Shepherd bitch was diagnosed with the aid of laboratory tests. The dog presented with photophobia, tearing, corneal edema and reduction of the visual capacity. A thick white layer partially covered the right eye. The left eye showed irritation and small brown stains which were diagnosed as pigmentary keratitis. The initial treatment consisted of 2% yellow mercury oxide. Natamycin was used as final treatment. Seven days later, the natural brightness of the eye as well as the visual capacity were restored.     

Predicting AD Conversion: Comparison between Prodromal AD Guidelines and Computer Assisted PredictAD Tool

Purpose

To compare the accuracies of predicting AD conversion by using a decision support system (PredictAD tool) and current research criteria of prodromal AD as identified by combinations of episodic memory impairment of hippocampal type and visual assessment of medial temporal lobe atrophy (MTA) on MRI and CSF biomarkers.

Methods

Altogether 391 MCI cases (158 AD converters) were selected from the ADNI cohort. All the cases had baseline cognitive tests, MRI and/or CSF levels of Aβ1–42 and Tau. Using baseline data, the status of MCI patients (AD or MCI) three years later was predicted using current diagnostic research guidelines and the PredictAD software tool designed for supporting clinical diagnostics. The data used were 1) clinical criteria for episodic memory loss of the hippocampal type, 2) visual MTA, 3) positive CSF markers, 4) their combinations, and 5) when the PredictAD tool was applied, automatically computed MRI measures were used instead of the visual MTA results. The accuracies of diagnosis were evaluated with the diagnosis made 3 years later.

Results

The PredictAD tool achieved the overall accuracy of 72% (sensitivity 73%, specificity 71%) in predicting the AD diagnosis. The corresponding number for a clinician’s prediction with the assistance of the PredictAD tool was 71% (sensitivity 75%, specificity 68%). Diagnosis with the PredictAD tool was significantly better than diagnosis by biomarkers alone or the combinations of clinical diagnosis of hippocampal pattern for the memory loss and biomarkers (p≤0.037).

Conclusion

With the assistance of PredictAD tool, the clinician can predict AD conversion more accurately than the current diagnostic criteria.     

Hypertrophic cardiomyopathy in daily practice: an introduction on diagnosis,prognosis and treatment

Hypertrophic cardiomyopathy (HCM) is a complex, inherited cardiac disease that has been subject to intense investigation since it was first described in 1957. Over the past 40 years, understanding has evolved regarding the diagnosis, prognosis and treatment of HCM. Analyses of HCM populations from nonreferral centres have refined the insights into the natural history and the occurrence of sudden cardiac death, which is the most devastating component of its natural history. Therapeutic strategies are diverse and may vary during the course of the disease. Optimal therapy depends on symptoms, haemodynamic findings and the presence of risk factors for sudden cardiac death. At present, invasive therapy for patients with obstructive HCM and drug-refractory symptoms includes surgery or percutaneous transluminal septal myocardial ablation.This report summarises the diagnostic criteria, clinical course and therapeutic management of HCM. Attention is also paid to certain issues of special interest in this disease.     

An Alkali-burn Injury Model of Corneal Neovascularization in the Mouse

Under normal conditions, the cornea is avascular, and this transparency is essential for maintaining good visual acuity. Neovascularization (NV) of the cornea, which can be caused by trauma, keratoplasty or infectious disease, breaks down the so called ‘angiogenic privilege'' of the cornea and forms the basis of multiple visual pathologies that may even lead to blindness. Although there are several treatment options available, the fundamental medical need presented by corneal neovascular pathologies remains unmet. In order to develop safe, effective, and targeted therapies, a reliable model of corneal NV and pharmacological intervention is required. Here, we describe an alkali-burn injury corneal neovascularization model in the mouse. This protocol provides a method for the application of a controlled alkali-burn injury to the cornea, administration of a pharmacological compound of interest, and visualization of the result. This method could prove instrumental for studying the mechanisms and opportunities for intervention in corneal NV and other neovascular disorders.     

角膜内皮细胞的检测、受损因素及治疗新进展

摘要：角膜内皮细胞(corneal endothelial cells,CECs)通过其屏障作用及离子泵功能维持角膜透明,但年龄、疾病及创伤等因素会使其功能有所降低,导致角膜失代偿、角膜水肿、大泡性角膜病变,甚至视力的丧失。因此,CECs可谓是角膜的"生命"。近年来,CECs的检测方法逐渐新增,并且不同检测指标具有不同意义;导致CECs受损的因素也有所增加,同时CECs损伤后的治疗也逐渐成为近年来的研究热点。早期发现CECs受损、明确病因并进行干预或治疗会减少CECs的损伤,对指导临床工作有重要的意义。本文主要对CECs检测方法及指标、损伤因素及其治疗的最新进展进行了综述。     

Methanol Intoxication Victims are Suitable Donors for Corneal Transplantation

Purpose. To evaluate the outcome of corneal transplantation and the feasibility of tissue donation from donors with methanol intoxication. Methods. Four corneas from two methanol intoxication victims were procured and transplanted in four patients at our medical center. The outcome and graft survival were evaluated. Results. The recipients were between 12 and 49 years of age. Indications for transplantation were keratoconus (two patients), post-traumatic corneal perforation (one patient) and alkaline burn (one patient). The follow-up period ranged between 12 and 60 months. All grafts remained clear, and the postoperative visual acuity was 20/30 in keratoconus patients and 20/60 in others. Conclusions. The methanol intoxication victims appear to be suitable donors for corneal transplantation. To the best of our knowledge, this is the first study to report successful corneal transplantation from donors with methanol intoxication.     

The Relationship between CPAP Usage and Corneal Thickness

The purpose of this study was to determine whether there is a correlation between CPAP usage and corneal thickness in patients with sleep disordered breathing. Full-night polysomnography (PSG) recordings were collected. Ten patients had undergone PSG recordings with continuous positive airway pressure (CPAP), and seven patients had undergone PSG recordings without CPAP. We measured corneal thickness by ultrasonic pachymeter before sleep and ten minutes after waking. We also measured visual acuity with a routine ophthalmologic eye chart before and after sleep. We asked patients to fill out a post-sleep questionnaire to get their subjective opinions. In the without-CPAP group, corneal thickness increased significantly during sleep in both eyes (left, p = 0.0025; right, p<0.0001). In the with-CPAP group, corneal thickness did not increase significantly (p>0.05 for both left and right cornea). There was no significant difference in visual acuity tests (p>0.05 for both left and right eye) between the two groups. According to our results, there is a significant increase in corneal thickness in the without-CPAP group. Our data show that a low percentage of Rapid Eye Movement (REM) sleep may cause an increase in corneal thickness, which can indicate poor corneal oxygenation. In fact, many sleep-disordered breathing (SDB) patients have low REM. Since a contact lens may cause low corneal oxygenation, SDB patients with contact lenses should be monitored carefully for their corneal thickness.     

Long-term result of autologous cultivated oral mucosal epithelial transplantation for severe ocular surface disease

The present study aimed to investigate the clinical outcomes of autologous cultivated oral mucosal epithelial transplantation (COMET) on human amniotic membrane (AM) for corneal limbal stem cell deficiency (LSCD). In this prospective, noncomparative case series, 20 eyes (18 patients) with bilateral severe ocular surface disease were chosen to undergo COMET on human AM. The primary outcome was clinical success, and the secondary outcomes were the best-corrected visual acuity difference, corneal opacification, symblepharon formation, and complications. The mean patient age was 48.2 ± 15.5 years. The mean follow-up time was 31.9 ± 12.1 months (range 8–50 months). All except one eye exhibited complete epithelialization within the first postoperative week. A successful clinical outcome, defined as a stable ocular surface without epithelial defects, a clear cornea without fibrovascular tissue invasion at the pupillary area, and no or mild ocular surface inflammation, was obtained in 15 of 20 eyes (75 %). The clinical success rate at 1 year was 79.3 %, and that at 4 years (end of follow-up) was 70.5 %. Fourteen of 20 (70 %) eyes exhibited improvement in visual acuity after COMET, and some required subsequent cataract surgery (2 eyes), penetrating keratoplasty (3 eyes), or keratoprosthesis implantation (1 eye). Preoperative symblepharon was eliminated in most eyes (8 of 13, 61.5 %) after COMET combined with eyelid reconstruction when needed. The only complication was corneal perforation (1 eye) induced by a severe eyelid abnormality; treatment with a tectonic corneal graft was successful. COMET can successfully restore ocular surface damage in most eyes with corneal LSCD.     

Convergence of specialised behaviour, eye movements and visual optics in the sandlance (Teleostei) and the chameleon (Reptilia)

Chameleons have a number of unusual, highly specialised visual features, including telescopic visual optics with a reduced lens power, wide separation of the eye's nodal point from the axis of rotation, a deep-pit fovea, rapid pre-calculated strikes for prey based on monocular depth judgements (including focus), and a complex pattern of partially independent alternating eye movements. The same set of features has been acquired independently by a teleost, the sandlance Limnichthyes fasciatus. Despite its underwater lifestyle, this fish displays visual behaviour and rapid strikes for prey that are remarkably similar to those of the chameleon [1]. In a direct comparison of the two species, we have revealed other, previously unsuspected, similarities, such as corneal accommodation, which was unknown in teleosts, as well as bringing together, for the first time, data collected from both species. The sandlance is the only teleost, among thousands studied, that has corneal refraction, corneal accommodation and reduced lens power, as well as sharing the other specialised optical features seen in chameleons. The independent eye movement pattern in the sandlance is also unusual and similar to that of the chameleon. The selection pressures that have produced this remarkable example of convergence may relate to common visual constraints in the life styles of these two phylogenetically disparate species.     

Pediatric idiopathic intracranial hypertension: clinical and demographic features

Idiopathic intracranial hypertension (IIH) is a clinical condition characterized by elevated intracranial pressure and absence of clinical, laboratory or radiographic evidence of central nervous system infection, vascular malformation, intracranial space occupying lesion or hydrocephalus. In the last years the raising understanding of pediatric IIH, especially concerning its demographics and epidemiology, has brought up to a redefinition of diagnostic criteria and reevaluation of pathogenesis and treatment. The authors reviewed the records of nineteen consecutive children with newly diagnosed IIH in order to compare demographic characteristics, clinical pictures and ophthalmologic aspects as optic disc evaluation and visual field evaluation, as well as treatment modalities and follow up. Beside obesity and female gender, potential alternative risk factors remains to be investigated, which need a good collaboration between neuro-ophthalmologists and pediatric neurologists.     

Clinical findings of a probable case of MM2-cortical-type sporadic Creutzfeldt-Jakob disease with antibodies to anti-N-terminus of α-enolase

We report the case of a 76-year-old woman presenting with 47-month history of progressive dementia and cortical blindness with no family history. Antibodies against thyroid glands and the N-terminus of α-enolase (NAE) were detected in her serum. Neurological examination revealed progressive dementia, frontal signs, visual disturbance, and exaggerated bilateral tendon reflexes in both legs. Diffusion MRI showed cortical hyper-intensities in the bilateral occipital and parietal, and the left frontal and temporal cortices. ^99mTc-ethyl cysteinate dimer-single photon emission computed tomography indicated decreased regional cerebral blood flow throughout the bilateral parietal lobes and partially in the left frontal and temporal lobes. PRNP gene analysis showed no mutations with methionine homozygosity at codon 129 in peripheral blood. Cerebrospinal fluid examination, including 14-3-3 and total tau protein detection, revealed normal levels; however, prion proteins were amplified by the real-time quaking-induced conversion method. Hashimoto's encephalopathy was excluded on the basis of unresponsiveness to corticosteroids. The symptoms progressed slowly. Periodic sharp-wave complexes were observed on electroencephalogram 36 months after the onset of symptoms; the patient reached a state of akinetic mutism at 47 months. This was a probable case of MM2-cortical-type sCJD with anti-NAE antibodies based on the World Health Organization (WHO) diagnostic criteria for sCJD, genetic information, and the slowly progressive course. However, this case did not meet with the probable WHO diagnostic criteria until 3 years after symptom onset, highlighting the difficulty of diagnosing a living case of the MM2-type of sCJD. Therefore, establishment of clinical diagnostic criteria for MM2-type of sCJD is required.     

Defining non-pathologic inferior corneal steepening

Computer-assisted videokeratography (corneal topography) has the ability to identify inferior corneal steepening in patients without biomicroscopic evidence of keratoconus. This may be an early form of keratoconus, an abortive form of keratoconus, a variable expression of the condition, or an unrelated diagnostic entity. Differentiating non-pathological inferior corneal steepening from keratoconus is essential for appropriate patient education and management and their candidacy for refractive surgery. Three cases are presented with this topographical finding and the relationship to keratoconus is discussed.     

Effect of Intraocular Forward Scattering and Corneal Higher-Order Aberrations on Visual Acuity after Descemet’s Stripping Automated Endothelial Keratoplasty

Purpose

To assess the relationship of intraocular forward scattering and corneal higher-order aberrations (HOAs) with best spectacle corrected visual acuity (BSCVA) after Descemet’s stripping automated endothelial keratoplasty (DSAEK), and to compare these parameters between DSAEK and non-Descemet’s stripping automated endothelial keratoplasty (n-DSAEK) groups.

Methods

This retrospective study enrolled thirty eyes of 30 consecutive patients who underwent standard DSAEK, and who underwent successful phacoemulsification with intraocular lens implantation before DSAEK. The mean age at the time of surgery was 71.7 ± 10.4 years. We quantitatively evaluated the objective scattering index (OSI) using the double-pass instrument (OQAS II, Visiometrics) and corneal HOAs using Hartmann-Shack aberrometry (KR-9000PW, Topcon) 3 months postoperatively.

Results

The mean OSI, corneal HOAs, and logMAR BSCVA 3 months after DSAEK were 7.91 ± 3.58, 0.43 ± 0.27 μm, and 0.32 ± 0.25, respectively. We found a significant correlation between the OSI and logMAR BSCVA (Spearman correlation coefficient r=0.714, p<0.001), but no significant association between corneal HOAs and logMAR BSCVA 3 months postoperatively (r=0.209, p=0.267). We found no significant differences in any postoperative parameters between the DSAEK and n-DSAEK groups (p>0.05).

Conclusions

Our pilot study demonstrated that the postoperative corrected visual acuity was significantly correlated with intraocular forward scattering, but not with corneal HOAs in post-DSAEK eyes, suggesting that intraocular forward scattering plays a more essential role in postoperative visual performance than corneal aberrations after DSAEK. The detailed visual performance, such as HOAs and intraocular scattering, after n-DSAEK appears to be essentially equivalent to that after DSAEK.     

A Native-Like Corneal Construct Using Donor Corneal Stroma for Tissue Engineering

Tissue engineering holds great promise for corneal transplantation to treat blinding diseases. This study was to explore the use of natural corneal stroma as an optimal substrate to construct a native like corneal equivalent. Human corneal epithelium was cultivated from donor limbal explants on corneal stromal discs prepared by FDA approved Horizon Epikeratome system. The morphology, phenotype, regenerative capacity and transplantation potential were evaluated by hematoxylin eosin and immunofluorescent staining, a wound healing model, and the xeno-transplantation of the corneal constructs to nude mice. An optically transparent and stratified epithelium was rapidly generated on donor corneal stromal substrate and displayed native-like morphology and structure. The cells were polygonal in the basal layer and became flattened in superficial layers. The epithelium displayed a phenotype similar to human corneal epithelium in vivo. The differentiation markers, keratin 3, involucrin and connexin 43, were expressed in full or superficial layers. Interestingly, certain basal cells were immunopositive to antibodies against limbal stem/progenitor cell markers ABCG2 and p63, which are usually negative in corneal epithelium in vivo. It suggests that this bioengineered corneal epithelium shared some characteristics of human limbal epithelium in vivo. This engineered epithelium was able to regenerate in 4 days following from a 4mm-diameter wound created by a filter paper soaked with 1 N NaOH. This corneal construct survived well after xeno-transplantation to the back of a nude mouse. The transplanted epithelium remained multilayer and became thicker with a phenotype similar to human corneal epithelium. Our findings demonstrate that natural corneal stroma is an optimal substrate for tissue bioengineering, and a native-like corneal construct has been created with epithelium containing limbal stem cells. This construct may have great potential for clinical use in corneal reconstruction.     

Comparative analysis of the size and shape of the lizard eye

Lizards occupy both scotopic (light-limited) and photopic (light-rich) environments, thereby making this clade ideal for analyses of eye morphology adaptations. This study examines how in lizards the morphology of the eye varies according to activity in these different light environments. Measurements were collected on corneal diameters and axial lengths of the eye for 239 specimens of 116 lizard species (including Sphenodon) that include both species with scotopic and photopic visual adaptations. I show that the light level available to a lizard for vision has a significant effect on eye shape and size. Scotopic lizards have eye shapes that are optimized for visual sensitivity, with larger corneal diameters relative to axial lengths. However, photopic lizards do not exhibit absolutely larger axial lengths than do scotopic lizards, and the groups have the same absolute axial lengths of the eye. Results also indicate that the light level the lizard functions under is a more significant influence on eye shape, as defined by the relationship between corneal diameter and axial length of the eye, than is phylogeny.     

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy presenting with severe keratopathy in an Egyptian patient with a homozygous R139X mutation

OBJECTIVE: To report a patient with an unusual presentation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) and severe keratopathy. CASE HISTORY: An Egyptian male sustained an injury to the left eye at 13 years of age and was found to have corneal damage which was attributed to the injury. Subsequently, however, he continued to have sore eyes with photophobia. A year later he became weak with pigmentation and episodes of collapse, and investigation showed that he had Addison's disease together with mucocutaneous candidiasis. At 15 years of age he developed carpo-pedal spasm and was found to have hypoparathyroidism with intracranial calcification. At 20 years of age the ophthalmic diagnosis was revised to keratopathy by which time the patient had corneal opacity and problems with visual acuity, especially in the right eye. Investigation at 22 years of age showed that he was homozygous for an R139X mutation in the gene encoding the AIRE protein, a mutation which to date has only been found in Sardinian patients. CONCLUSIONS: Keratopathy can be an early and severe manifestation of APECED, requiring expert ophthalmic care. Its presence should prompt a search for other components of APECED, some of which are life-threatening.     

Aerial visual acuity in harbor seals (<Emphasis Type="Italic">Phoca vitulina</Emphasis>) as a function of luminance

In this study, we measured aerial visual acuity in harbor seals. As a first approach to the hypothesis that harbor seals can obtain acute aerial visual acuity mediated by the interaction of the vertical slit-shaped pupil and the corneal flattening although refractive measurements had revealed aerial myopia, visual acuity was tested as a function of luminance and pupil dilation. We analyzed aerial visual acuity (minimal resolvable stripe width) in three harbor seals in a two-alternative-forced-choice discrimination experiment. Our results further support the hypothesis that harbor seals possess an aerial visual acuity comparable to the acuity in clear waters if the vertical slit pupil does not exceed the zone of corneal flattening in bright light. When the pupil dilates with decreasing luminance, visual acuity decreases which might be due to deflected light from the stronger curved peripheral cornea.     

Corneal accommodation in chick and pigeon

Summary We have investigated the role of changes in corneal radius of curvature in effecting accommodation in the bird's eye. It was found that in natural accommodation (measured by IR photoretinoscopy) changes of corneal radius of curvature (measured by IR photokeratometry) play an important role in both the chick and the pigeon. In the adult pigeon the cornea is indeed responsible for the largest part of natural accommodation (up to approx. 9 D). In this animal the corneal diameter (as seen from the optical axis of the eye) decreases in accommodation which can be taken to explain the change of corneal radius of curvature. In the chicken, corneal accommodation is combined with other mechanisms (total accommodative range 15–17 D, corneal accommodation about 8 D). The chicken's cornea is aspherical within the pupil area leading to large measurement variation in photokeratometry if the Purkinje images are not symmetrical to the pupillary axis.Abbreviation IR infrared