Orsola Caporossi

Corneal Healing After Riboflavin Ultraviolet-A Collagen Cross-Linking Determined by Confocal Laser Scanning Microscopy In Vivo: Early and Late Modifications

PURPOSE To assess early and late micromorphological modifications of cross-linked corneas in vivo by means of Heidelberg Retinal Tomography (HRT) II confocal microscopy. DESIGN Prospective nonrandomized open trial. METHODS Micromorphological examination of 44 cross-linked keratoconic corneas was performed in vivo by HRT II confocal laser scanning microscopy. Riboflavin ultraviolet (UV)-A-induced corneal collagen cross-linking (CXL) was performed according to the Siena protocol: pilocarpin 1% drops 30 minutes before, topical anesthesia with lidocaine 4% drops 15 minutes before irradiation, mechanical scraping of epithelium (9-mm-diameter area), preirradiation soaking for 10 minutes in riboflavin solution 0.1% (Ricrolin, Sooft, Italy) applied every 2.5 minutes for 30 minutes, 30 minutes exposure to solid-state UVA illuminator (Caporossi; Baiocchi; Mazzotta, X-linker, CSO, Italy), 8-mm-diameter irradiated area, energy delivered 3 mW/cm(2). All patients were examined by confocal scans preoperatively and at the following times after treatment: one, three, and six months, and one, two, and three years. RESULTS No damage to the limbal region was observed. Epithelial regrowth was complete after four days of soft contact lens bandage. The anatomy of the subepithelial plexus was restored one year after the operation with full corneal sensitivity. Increased density of extracellular matrix in late postoperative period indicated cross-linked collagen to a depth of 340 microm expressed by a late demarcation line. CONCLUSION In vivo confocal microscopy showed early and late modification of corneal microstructure after the treatment. The three-year stability of CXL recorded could be related to increased cross-links formation, synthesis of well-structured collagen and new lamellar interconnections.

Confocal microscopy identification of keratoconus associated with posterior polymorphous corneal dystrophy

We present a case of keratoconus in which the association with posterior polymorphous corneal dystrophy was established by confocal microscopy. Confocal microscopy enable us to assess the differential diagnosis between posterior corneal dystrophies, essential for a prognosis and therapeutic decision.

Long-Term Results of Photodynamic Therapy for Choroidal Neovascularization in Pediatric Patients with Best Vitelliform Macular Dystrophy

Abstract Purpose: To report long-term results of photodynamic therapy (PDT) in young patients affected by Best vitelliform macular dystrophy (VMD) complicated by choroidal neovascularization (CNV). Methods: We evaluated a group of 30 VMD patients with confirmed mutations in the BEST1 gene. Five of these patients had been diagnosed with CNV when younger than 15 years of age and three of them were treated by PDT. After the treatment they were followed for an average period of 77 months (range 62–99). Results: In all the treated eyes visual acuity was stable during the first year of follow-up and then slowly improved even some years after the treatment. The improvement in visual acuity was associated with the development of fibrous tissue in the macula. Conclusions: PDT was a safe procedure in our series of pediatric patients with VMD complicated by CNV. It was followed by a CNV regression and a consequent improvement in visual acuity which continued to progress even several years after the treatment.

EDI-OCT evaluation of choroidal thickness in retinitis pigmentosa

Purpose: To evaluate choroidal thickness (CT) in retinitis pigmentosa (RP) using enhanced depth imaging (EDI) optical coherence tomography (OCT). Methods: A retrospective analysis of a group of patients with RP who underwent EDI-OCT was performed. Choroidal thickness measurements were compared with those of age- and sex-matched healthy subjects. In the RP group, the possible association between subfoveal CT and some clinical parameters (visual acuity, age, age at disease onset, duration of the disease, macular thickness, visual field loss, electroretinography [ERG]) was evaluated. Results: The study recruited 39 patients with RP with an average age of 43.3 ± 11.3 years while the control group consisted of 73 healthy subjects with an average age of 42.9 ± 12.10 years. On average, CT was significantly thinner in the RP group compared to the controls (p<0.0001). In the RP group, we could not find any significant association between CT and the considered clinical parameters even if there was a trend for decreasing CT with increasing age (r = −0.23, p = 0.096). In the control group, subfoveal CT showed a slightly significant correlation with age (r = −0.21, p = 0.04) but not with macular thickness and visual acuity. Conclusions: In our series, CT was significantly lower in the RP group in comparison with the controls, as measured by EDI-OCT, but did not correlate with age, age at onset, duration of the disease, macular thickness, visual acuity, visual field loss, or ERG responses. Although the clinical implications of choroidal changes in RP have not yet been clearly determined, the evaluation of choroidal features may provide information that could be useful to clarify the pathophysiology of the disease.

EDI OCT evaluation of choroidal thickness in stargardt disease

Purpose Choroidal thickness (CT) evaluation with EDI-OCT in Stargardt Disease (STGD), considering its possible association with some clinical features of the disease. Methods CT was evaluated in 41 STGD patients and in 70 controls. Measurements were performed in the subfoveal position and at 1000 μm nasally and temporally. CT average values in STGD and in the control group were first compared by means of Student’s T test. Then, the possible association between CT and some clinical features was evaluated by means of linear regression analysis. Considered clinical parameters were: age, age on onset, duration of the disease, visual acuity, foveal thickness, Fishman clinical phenotype, visual field loss and ERG response. Results Average CT was not significantly different between controls and STGD patients. In the STGD group the correlation between CT and age (r = 0.22, p = 0.033) and age of onset (r = 0.05, p = 0.424) was modest, while that of CT with disease duration (r = 0.30, p<0.001) was moderate. CT and foveal thickness were also significantly but modestly correlated (r = 0.15, p = 0.033). Conclusion In our series average CT is not significantly changed in STGD in comparison with the controls. Nevertheless a choroidal thinning may be identified in the more advanced stages of the disease.

CFH Y402H polymorphism in Italian patients with age-related macular degeneration, retinitis pigmentosa, and Stargardt disease

ABSTRACT Background: The complement system has been implicated in the pathogenesis of age-related macular degeneration (AMD) and the CFH Y402H polymorphism has been suggested as a major risk factor for AMD. Recent evidences supported the role of inflammation in the pathogenesis of some retinal dystrophies. Aim of this study was to evaluate the prevalence of CFHY402H polymorphism in a group of Italian patients affected by atrophic AMD, Stargardt disease (STGD), or retinitis pigmentosa(RP). Materials and Methods: Our case–control association study included 116 patients with atrophic AMD, 77 with RP, 86 with STGD, and 100 healthy controls. All the patients were evaluated by a standard ophthalmologic examination and OCT. ERG was performed on STGD and RP patients. All the subjects underwent a blood drawing for genetic testing and the CFHY402H polymorphism was genotyped with the TaqMan real-time polymerase chain reaction single nucleotide polymorphism assay. Results: The prevalence of the risk genotype C/C was higher in the AMD group than in controls (p < 0.001). The risk allele C was more frequent in the AMD group than in controls (p < 0.001). The prevalence of the risk genotype was higher in the RP patients than in controls (p < 0.001) and similarly the risk allele C was more frequent in the RP group (p = 0.008). The CFHY402H genotype distribution was not different between patients with STGD and the controls, for the biallelic (p = 0.531) and for the monoallelic (p = 0.318) evaluation. Conclusions: In our series of Italian patients, the CFHY402H genotype is associated with atrophic AMD and RP, but not with STGD. This result may support the hypothesis of a complement system dysregulation in the pathogenesis of AMD and RP

An in vivo confocal microscopy study of corneal changes in pseudoexfoliation syndrome

Purpose: To evaluate, through the in vivo confocal microscopy, the pathological changes of each corneal layer in eyes affected by pseudoexfoliation syndrome. Methods: We studied 40 eyes of 40 patients with diagnosis of unilateral senile cataract associated with pseudoexfoliation syndrome and 40 eyes of 40 control subjects with senile cataract without pseudoexfoliation syndrome. All patients underwent a complete ophthalmic examination including best corrected visual acuity, slit-lamp examination, corneal sensitivity measurement using a Cochet-Bonnet nylon thread esthesiometer, and anterior segment optical coherence tomography (Visante OCT, Carl Zeiss Meditec AG, Germany); in vivo confocal microscopy of corneal sections (endothelium, stroma, sub-basal nerve plexus, and superficial and basal epithelium) was performed with the ConfoScan 4.0 (Nidek, Japan). Results: In pseudoexfoliation syndrome group, the mean corneal sensitivity was 44.1 ± 1.3 mm and in the control group was 55.6 ± 4.7 mm. The corneas of the eyes with pseudoexfoliation syndrome were significantly less sensitive than those of control group eyes (p < 0.001). Pseudoexfoliation syndrome eyes had a lower nerve density and less nerve beadings and a higher degree of tortuosity in sub-basal plexus compared to the control group. The cell density of epithelial and endothelial layers was significantly lower in pseudoexfoliation syndrome eyes than controls. In 80% of pseudoexfoliation syndrome eyes, we found activated keratocytes and inflammatory cells in the anterior stroma. Conclusion: Our study demonstrates the morpho-structural corneal alterations in eyes affected by pseudoexfoliation syndrome, using corneal in vivo confocal microscopy as a non-invasive and high-reproducible technique to evaluate pathophysiology of each corneal layer; the sub-basal nerve plexus alterations are correlated with the lower corneal sensitivity.

COMPUTER ASSISTED RETINAL VESSEL TORTUOSITY EVALUATION IN NOVEL MUTATION FABRY DISEASE: Towards New Prognostic Markers

Purpose: Fabry disease is a rare lysosomal storage disorder with systemic involvement. The authors report on a large Fabry family with GLA p.M187R mutation and exhaustive ophthalmologic assessment. Methods: Comprehensive systemic evaluation and genetic diagnosis were performed. Ophthalmologic evaluation included intraocular pressure/visual acuity measurement, refractometry, slit lamp examination, retinography, and optical coherence tomography. Three parameters quantified retinal vessel tortuosity: sum of angle metrics, product of angle distance, and triangular index. Calculations were semiautomatized using dedicated software. Results: Ten individuals (2 males and 8 females) were described. Seventy-five percent had retinal vessel tortuosity. One hundred percent had cornea verticillata. Perimacular vessels were predominantly involved. The correlation between the right and left eye tortuosity measurements was very tight. A significant correlation between retinal vessel tortuosity and systemic severity measured by general Mainz Severity Score Index (MSSI), renal MSSI, and neurological MSSI but no cardiac MSSI was observed. Right sum of angle metrics value was an independent statistical predictor of the general-MSSI score in presence of age. Conclusion: p.M187R mutation causes a severe systemic and ophthalmologic phenotype, in both male and female patients. Semiautomatic assessment of retinal vessel tortuosity is an objective and reproducible tool. All three parameters of tortuosity are closely associated with Fabry severity scores. Studies of larger series are being awaited to establish the role of retinal vessel tortuosity as a noninvasive marker of disease progression.

Corneal Cross-linking in Children

This chapter brings together all the current evidence in children for a now well-established procedure for keratoconus (KC) in children. With the aid of research from Caporossi and his group, most recent results of which will be presented in this chapter, the authors will present the similarities and differences found in KC in children.

Visual performance, reading ability and patient satisfaction after implantation of a diffractive trifocal intraocular lens

Purpose To evaluate visual outcome, reading performance, contrast sensitivity, and patient satisfaction after cataract surgery with implantation of a diffractive trifocal intraocular lens (IOL). Patients and methods A total of 42 eyes (21 patients) underwent cataract surgery with implantation of the trifocal IOL AT LISA tri 839MP. Visual acuity, contrast sensitivity, and patient satisfaction were evaluated 3 months postoperatively. Reading performance was evaluated at 3 months postoperatively with the MNREAD charts. Results All eyes achieved a 3-month postoperative monocular uncorrected distance visual acuity of 0.10 logMAR or better (Snellen 20/25). Likewise, 97.62% and 85.71% of eyes achieved a postoperative monocular uncorrected intermediate, and near visual acuity of 0.20 logMAR (Snellen 20/30) or better. All patients achieved postoperative binocular uncorrected distance visual acuity, uncorrected intermediate, and uncorrected near visual acuity of 0.20 logMAR (Snellen 20/30) or better. Mean photopic reading acuity and speed were 0.24±0.07 logMAR and 177.61±20.67 words per minute, respectively. Postoperative contrast sensitivity values were within the ranges of normality for all spatial frequencies evaluated. Postoperative spectacle independence and patient satisfaction was very high, with most of the patients reporting a good or very good visual quality at far, intermediate, and near distances. All patients would choose the same lens again. Conclusion The evaluated trifocal IOL provides an effective restoration of the visual function after cataract surgery, with high levels of distance, intermediate, and near visual acuity, strong reading performance, and patient satisfaction.