Juvenile idiopathic arthritis-associated uveitis in the era of biological therapy: how the disease changed in more than 20 years of observation in a tertiary referral center in Rome (Italy).

OBJECTIVES: To describe the ophthalmological characteristics in a Juvenile idiopathic arthritis (JIA) cohort and to evaluate how therapeutic advances have changed the course of the uveitis. METHODS: Analysis of a retrospective cohort study of consecutive JIA pediatric patients including JIA-associated uveitis (JIA-U) and comparison with a previous study in the same uveitis center assessed before the wide-spread of biological therapy. RESULTS: The total of 49 JIA patients were analyzed, of whom 18 JIA-U, compared with a JIA-U past cohort of 66 patients. Systemic corticosteroids were used significantly less in the current JIA-U group (p = 0.008) than in the past one. JIA-U present cohort was on therapy more frequently with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) than the past group (p = 0.039), mostly treated with methotrexate (93.3%). Furthermore, a larger use of biologic disease-modifying anti-rheumatic drugs (bDMARDs) was described in the current JIA-U group (p = 0.005) also associated with csDMARDs (p = 0.003). Adalimumab was used more (72.7%) in the present JIA-U cohort compared to a larger treatment with infliximab (61.5%) in the past (p = 0.005). Higher number of uveitis recurrences was observed in the previous cohort compared to the current one (p = 0.005). Fewer complications were described in this study than in the previous: posterior synechiae (p = 0.007), cataract (p < 0.001), band keratopathy (p < 0.001), and elevated intraocular pressure (IOP) (p = 0.047). CONCLUSION: Current therapies reduced the uveitis recurrences and ocular complications including cataract due also to the lower use of corticosteroids. The new close collaboration with the pediatric rheumatologic center in the same University has contributed to the care improvement and decrease of uveitis complications.

Improving keratoconus management with central cor neal regularization and corneal collagen cross-linking protocol treatment.

Purpose: To evaluate safety and efficacy of customized central corneal regularization (CCR), together with simultaneous accelerated corneal collagen cross-linking (A-CXL) - CCR-CXL protocol, to treat keratoconus-related corneal ectasia. Design: Retrospective, comparative observational case series. Methods: Patients that had undergone combined CCR-CXL protocol. Main inclusion criteria were keratoconus visual acuity deterioration and contact lens intolerance. All patients underwent complete ophthalmological evaluation, corrected distance visual acuity (CDVA) and Scheimpflug-corneal tomography. Central corneal regularization was performed by ablation using flying spot laser. Subsequently, the stroma was saturated with 0.17% riboflavin-5-phosphate added every 2 minutes, followed by A-CXL 9 mW/cm2 for 10 minutes. CDVA, medium keratometry value (Kmed), and total corneal morphological irregularity index (CMI) of patients were analyzed before surgery and after 1, 3 and 12 months. A P value of .05 or less was considered statistically significant. Results: 46 eyes of 39 keratoconus patients were treated. At 1 month, the mean CDVA (LogMar) increased from 0.19 ± 0.02 to 0.12 ± 0.02 (P < .05), and the difference remained stable at month 12. Kmax decrease was statistically significant from 57.02 ± 5.65 to 50.21 ± 4.48 (P < .05). CMI decreased significantly from 47.8 ± 2.84 to 30.1 ± 2.4 (P < .01). Conclusions: CCR-CXL protocol is safe and effective in arresting keratectasia progression and increasing corneal optic regularity in keratoconus. These findings showed a significant improvement in CDVA, keratometry values and corneal optical aberrations after being treated with the CCR-CXL protocol.

A new method to assess foveal morphology with optical coherence tomography.

PURPOSE: To measure foveal morphology using optical coherence tomography (OCT). METHODS: Three examiners classified normal subjects and patients with macular edema and epiretinal membranes from a normative database (299 OCT scans) and a study sample (80 OCT scans) on the basis of the foveal depression appearance (reference classification). The following parameters were measured for each OCT scan: Central Foveal Thickness (CFT), Central Subfield Thickness (CST), Central Subfield Volume (CSV), Centrofoveal Index (CI), Parafoveal Index (PI), and Foveal Indices (FI1, FI2, FI3). Foveal depression of OCT scans in the study sample were classified based on the values of OCT parameters using Bayesian statistics (test classifications). RESULTS: Agreement between reference and test classifications was of 70% for CFT, 58.7% for CST, 60% for CSV, 80% for CI, 72.5% for PI, 88.7% for FI1, 96.2% for FI2 and 97.5% for FI3. CONCLUSIONS: IF3 seems to provide a reliable measure of the foveal morphology.

Fixation Improvement through Biofeedback Rehabilitation in Stargardt Disease.

Stargardt disease is the most common hereditary macular degeneration in juveniles. It is characterized by macular dystrophy associated with loss of central vision in the first or second decade of life, a "beaten-metal" appearance in the fovea or parafoveal region, yellowish flecks around the macula or in posterior area of the retina, progressive atrophy of the bilateral foveal retinal pigment epithelium, and the "dark choroid" sign on fundus fluorescein angiography in most cases. We report a case of Stargardt disease in a 26-year-old Caucasian female submitted to rehabilitative training with microperimetry MP-1 to find a new preferred retinal locus (PRL) and to train her to better her quality of life. Best corrected visual acuity, mean retinal sensitivity, fixation, bivariate contour ellipse area, and speed reading were evaluated before and after the training and results were discussed.

Macular hole in retinitis pigmentosa patients: microincision vitrectomy with polydimethylsiloxane as possible treatment.

PURPOSE: To investigate long-term retinal changes after microincision pars plana vitrectomy surgery (MIVS) for macular hole (MH) in retinitis pigmentosa (RP) patients-retrospective and observational study. METHODS: Three RP patients suffering from MH were evaluated by means of best corrected visual acuity, anterior and posterior binocular examination, spectralis high-resolution optical coherence tomography, MP-1 microperimetry (MP-1), and full-field electroretinogram (ERG), before MIVS and during the 36-month follow-up. Patients underwent simultaneous MIVS and microincision cataract surgery; IOL was positioned in capsular bag. Patients were hospitalised for 2 days after the surgery. Surgical procedure was performed according the following schedule: surgical removal of crystalline lens, MIVS with 23-gauge sutureless system trocars, core vitreous body removal, peeling of the inner limiting membrane, and balanced sterile saline solution-air-micro-structured polydimethylsiloxane (PDMS) exchange. PDMS tamponade, after 6 months starting from MIVS, was removed. RESULTS: In all patients visual acuity increased after vitrectomy as a consequence of complete MH closure and restoration of retinal architecture. None of the patients developed ocular hypertension, or re-opening of MH during the 3-year follow-up. MP-1 bivariate contour ellipse area was reduced in its dimensions and improved in all patients demonstrating a better fixation. CONCLUSIONS: MIVS could be an effective treatment in RP patients with MH if medical therapy is not applicable or not sufficient. Finally more studies will be needed to improve knowledge about this genetic disease.

Four techniques for retreatment after excimer laser photorefractive keratectomy.

BACKGROUND: Retreatment should be considered for patients unsatisfied because of undercorrection and/or haze following excimer laser photorefractive keratectomy (PRK) for myopia. It is unclear if any specific type of retreatment is superior to another, especially in terms of post-treatment myopic shift and haze. METHODS: After a mean follow-up of 12.4 +/- 5.5 months, we retreated 10 eyes by four different techniques following failed PRK: only PRK, PRK with phototherapeutic keratectomy (PTK), only PTK, and mechanical scraping. RESULTS: After a mean follow-up of 11.4 +/- 7.8 months (range, 6 to 28 mos), the final mean refraction was 2.40 +/- 2.00 D (range, 0 to -6.00 D). Corneal clarity improved in 70% of eyes. The mean uncorrected visual acuity improved from 20/200 to 20/40. Of the four retreatment techniques, only scraping proved ineffective, due to unstable outcome. CONCLUSIONS: PRK alone, PRK with PTK, and PTK alone appeared safe and effective methods of retreatment, with no worsening of corneal clarity.

Role of the corneal epithelium in refractive changes following laser in situ keratomileusis for high myopia.

PURPOSE: Epithelial hyperplasia is one of the factors that plays a role in myopic regression after photorefractive keratectomy (PRK). We evaluated the role of the epithelium following excimer laser in situ keratomileusis (LASIK) performed on highly myopic eyes. METHODS: Fifty eyes of 32 patients (18 females and 14 males; mean age, 31.7+/-6.5 years) were treated with LASIK for myopia with an attempted correction ranging between -8.50 and -12.25 D (mean, -10.48+/-1.43 D). No sutures were placed. The thickness of the central epithelium was evaluated either with a 50-MHz ultrasonic pachymeter or an ultrasound biomicroscope. Postoperatively, topical corticosteroid drops were administered for at least 1 month. RESULTS: Follow-up was at least 12 months. Epithelium measurements were thicker compared to preoperative measurements from the first week postoperatively (mean, +1.9 microm, +2.77%; P<.05). Epithelial thickness peaked at the third month after LASIK (mean, +6.5 microm, +9.5%; P<.05) and remained stable through 12 months. A negative correlation between epithelium thickness and manifest refraction from the first month postoperatively to the end of follow-up was present. CONCLUSION: The epithelium seemed to have a role in the regression of refractive effect after LASIK to correct high myopia.

Histological, immunohistochemical, and ultrastructural findings in human corneas after photorefractive keratectomy.

BACKGROUND: Four human corneas that had undergone photorefractive keratectomy (PRK) and subsequent penetrating keratoplasty were examined by means of light and electron microscopy in an attempt to detect possible causes for complications after PRK. METHODS: Four eyes with a central corneal leukoma resulting from a previous PRK treatment underwent penetrating keratoplasty respectively 3 days, 3 months, 5 months, and 13 months after the refractive procedure. Different excimer laser instruments (Meditec MEL 50, Summit UV200, and VISX 20/20) had been used. The corneal buttons removed were submitted for light microscopy, electron microscopy and immunohistochemistry. RESULTS: An immature (one to two layers, basement membrane anomalies) but continuous epithelium was present even in the 3-day specimen; between six and eight epithelial layers could be seen in the 13-month specimen, which still presented an undulated aspect of the otherwise normal basement membrane. A continuous acellular collagen layer underlying the epithelium of the ablated area was detected in the superficial stroma of all examined corneas. No Descemet's membrane or endothelial alterations could be seen. CONCLUSIONS: Despite recovery of a continuous epithelial layer as early as 3 days after PRK, abnormalities of both epithelium and superficial stroma could be detected in all specimens, including the one obtained 13 months after the refractive procedure.

Effect of myopic excimer laser photorefractive keratectomy on the electrophysiologic function of the retina and optic nerve.

PURPOSE: To assess by electrophysiologic testing the effect of photorefractive keratectomy (PRK) on the retina and optic nerve. SETTING: Eye Clinic, S. Salvatore Hospital, L'Aquila University, Italy. METHODS: Standard pattern electroretinograms (P-ERGs) and standard pattern visual evoked potentials (P-VEPs) were done in 25 eyes of 25 patients who had myopic PRK for an attempted correction between 5.00 and 15.00 diopters (D) (mean 8.00 D). Testing was done preoperatively and 3, 6, 12, and 18 months postoperatively. The contralateral eyes served as controls. During the follow-up, 3 patients (12%) developed steroid-induced elevated intraocular pressure (IOP) that resolved after corticosteroid therapy was discontinued. RESULTS: No statistically significant differences were seen between treated and control eyes nor between treated eyes preoperatively and postoperatively. CONCLUSION: Myopic excimer laser PRK did not seem to affect the posterior segment. The transient steroid-induced IOP rise did not seem to cause functional impairment.

Videokeratographic changes after laser in situ keratomileusis to correct high myopia.

PURPOSE: To evaluate the videokeratographic changes and patterns after laser in situ keratomileusis (LASIK) in high myopia using computerized videokeratography. SETTING: San Salvatore Hospital, Chair of Ophthalmology, University of L'Aquila, L'Aquila, Italy. METHODS: Forty-one eyes of 26 patients treated with no-suture nasal-hinged flap LASIK for an attempted mean spherical equivalent correction of -13.9 diopters (D) +/- 4.1 (SD) (range -8.0 to -22.0 D) were evaluated. Mean patient age was 35.2 +/- 9.8 years. Mean follow-up was 15.1 +/- 6.7 months (range 6 to 26 months). RESULTS: Thirty-five eyes (85.4%) were within +/- 2.0 D of the planned correction, with a refractive error greater than 3.0 D in the 7.3% of eyes. Mean central corneal power decreased from 43.2 +/- 1.8 D (range 38.70 to 47.40 D) to 35.8 +/- 3.8 D (range 30.3 to 42.6 D). Mean preoperative corneal astigmatism decreased from 1.2 +/- 0.9 D (range 0.2 to 4.2 D) to 0.9 +/- 0.9 D (range 0.2 to 3.3 D). Videokeratographic qualitative analysis showed corneal shape changes within 15 days after surgery. Corneal patterns then stabilized, and 37 eyes (90.3%) had no significant modifications in corneal refractive power after 1 month. CONCLUSION: After LASIK in highly myopic eyes, videokeratographic maps showed corneal power stabilized within 1 month in most eyes and there was no irregular astigmatism if the treatment was well centered.

Specular microscopy of the corneal endothelium after excimer laser photorefractive keratectomy.

PURPOSE: To evaluate endothelial cell morphology and density after excimer laser photorefractive keratectomy (PRK). METHODS: We used a noncontact specular microscope to examine the central corneal endothelium of 50 eyes of 50 patients who had PRK for an attempted correction between -2.5 and -17.0 diopters (D) (mean -7.8 D) beginning 18 to 24 hours postoperatively. RESULTS: After a follow-up of 11.4 +/- 6.1 months (mean +/- standard deviation), mean endothelial cell density was 2577.6 +/- 402.0 cells/mm2 with rare signs of polymegathism and pleomorphism. Preoperative and untreated fellow eye endothelial cell density values were used as a control. Paired Student's t-test and analysis of variance results were not significant (P > .05). CONCLUSION: Excimer laser PRK did not significantly change cell density and morphology.

PERG and P-VEP after surgical trabeculectomy in primary open-angle glaucoma.

Pattern-reversal electroretinograms (PERG) and visual evoked potentials (P-VEP) were measured for 10 eyes from 10 rigorously selected patients with bilateral advanced primary open angle glaucoma (POAG), before and after surgical trabeculectomy. The aim was to establish whether electrofunctional examinations improved after major IOP reduction. Only one eye at random was operated, and the fellow eye was used as control. The aim of this study, using electrofunctional examinations, was to clarify whether ganglion cell damage was reversible after marked reduction of IOP by surgery. The results indicate that glaucomatous damage seems to be irreversible.

Malignant conjunctival epibulbar fibrous histiocytoma with orbital invasion.

The Authors report on a rare case of malignant conjunctival epibulbar fibrous histiocytoma with orbital invasion. Fibrous histiocytoma is a tumour of mesenchymal origin, which, although among the most common adult age soft tumours, appears very rarely at the conjunctival level. In fact the most frequent site is the orbit. So far only 15 cases concerning conjunctiva have been described in the Literature only 4 of those have been reported as malignant. We observed a male patient, who 6 years ago, at the age of 53, noticed a neoformation on the temporal portion of the bulbar conjunctiva. In June 1988, after three successive operations, with a histological diagnosis of inflammatory granuloma, he came to our Clinic, where, because of the characteristics of the orbit infiltrations, only a partial excision was carried out for a biopsy. The histological examination, associated with immunohistochemical techniques, gave the result of malignant fibrous histiocytoma. Consequently in, July 1988, the patient underwent an exenteratio orbitae. To date, the patient enjoys good health without a trace of recurrence. Besides the clinical presentation of the case, histopathological and immunohistochemical findings concerning this type of lesion are presented and discussed, with a comparison of our findings with those reported in the literature.

Electro-oculographic abnormality in eyes with uveal melanoma.

PURPOSE: To seek out correlations between preoperative electro-oculogram (EOG) recordings with different types of uveal melanomas, after surgery. METHODS: We analysed the EOG recordings of 120 patients with uveal melanomas, histologically verified, 100 in the choroid and 20 in the iris and ciliary body. The EOG data were correlated with the site, size and histological type of the tumor. RESULTS: In 100 eyes with choroidal melanoma the Arden Index (AI) was less than in fellow eyes (mean 126.6, SD +/- 23.8 and 202.9, SD +/- 47.0; p=0.01). The EOG values were not different with respect to the histological type, site and size of tumor. In cases with iris and ciliary body melanomas the AI were not significantly different from the fellow eyes (mean 180.6, SD +/- 23.6 and 203.2, SD +/- 38.7; p=0.07). CONCLUSIONS: Since the EOG is abnormal in eyes with choroidal melanoma, it can be considered a powerful auxiliary for diagnosing these tumors.

Local excision of retinal metastasis from cutaneous melanoma.

The incidence of cutaneous melanoma is constantly rising. Ocular involvement is rare and the choroid is the most accessible structure to metastatic emboli. The case described is noteworthy on account of several particular features: 1) the missed diagnosis of the primary malignancy. This might be because either cutaneous melanoma or cutaneous nevi show several common features; 2) the rarity of the retinal metastatic site. Unlike the choroid, the retina is an unusual metastatic site but, regardless of organ blood flow, the metastatic efficiency may be related to interactions between tumor cells and host tissue; 3) the treatment of the retinal metastasis, excised conservatively. As far as we know, this is the first report of a retinal metastasis being treated by local resection.

Effectiveness of LASIK to correct refractive error after penetrating keratoplasty.

OBJECTIVE: Refractive errors may invalidate the good results of penetrating keratoplasty (PK). The Authors evaluate the effectiveness of excimer laser in situ keratomileusis (LASIK) in the correction of refractive error after PK. MATERIALS AND METHODS: Four patients, a 26-year-old woman, a 54-year-old man, a 19-year-old man, and a 51-year-old woman, showed refractive errors: -11 = -4.5 x 85 ; -8, -4.5 = -11 x 95 ; and -4.5 = -4 x = 1200, with a clear graft at least 20 months after penetrating keratoplasty secondary to keratoconus. However, they underwent the LASIK procedure with a nasal-hinged flap of 160 um. No sutures were placed. RESULTS: At follow-up, 24, 18, 12, and 12 months, respectively, the graft remained clear and the endothelial cells were unchanged. The uncorrected visual acuities were 20/50, 20/25, 20/50, and 20/25, respectively with an unchanged best corrected visual acuity (20/20) for all patients. No significant complications were observed. CONCLUSIONS: LASIK procedure seems to be an effective technique to correct refractive error after successful penetrating keratoplasty.

Long-term results of excimer laser photorefractive keratectomy in high myopia: a preliminary report.

BACKGROUND AND OBJECTIVE: To evaluate the results of 193-nm excimer laser photorefractive keratectomy (PRK) performed on highly myopic eyes with a follow-up of at least 2 years. PATIENTS AND METHODS: Fifty-three eyes of 41 patients were treated with a myopic PRK for an attempted correction ranging between -8 and -17 D (mean -10.9 +/- 2.8 SD). RESULTS: The follow-up ranged from 24 to 60 months (mean 34.1 months +/- 10 SD). Fifty-three percent of the eyes achieved a refraction within +/- 2 D of the planned correction, with a refractive error greater than 4 D in 21% of the treated eyes. The uncorrected visual acuity was 20/40 or better in 45% of the eyes. There were no significant opacities in 85% of the eyes examined. A second treatment was performed on 5 eyes. CONCLUSION: The results suggest that excimer laser PRK is a safe and relatively effective alternative for treating highly myopic eyes, and its effects are stable over a long period of time.

Penetrating keratoplasty with donor and recipient corneas of the same diameter.

BACKGROUND AND OBJECTIVE: A retrospective study was conducted to investigate the effect of penetrating keratoplasty (PK) with donor and recipient corneas of equal diameter on astigmatism, corneal curvature, and spherical equivalent. PATIENTS AND METHODS: A total of 89 eyes of 86 consecutive patients who underwent PK with donor and recipient corneas of equal diameter were studied. The surgical techniques were performed using the Hanna suction punch block (endothelial cut) with the trephine system, and a single running 16-bite 10-0 nylon suture. Sixteen eyes underwent a triple procedure (PK, extracapsular cataract extraction, and intraocular lens [IOL] insertion) and 14 eyes underwent PK with sulcus fixation of a posterior chamber IOL. Follow-up ranged from 12 to 30 months (mean +/- SD 17.5 +/- 4.7). The sutures were removed at approximately 12 months postoperatively. RESULTS: The postoperative astigmatism ranged from 0.4 to 10.8 D (mean +/- SD 4.5 +/- 2.8), as evaluated by a computer-assisted videokeratograph topography unit. Twenty-seven eyes (30.3%) achieved a refractive error (spherical equivalent) within +/- 1.5 D of emmetropia. The mean postoperative refractive error (spherical equivalent) was -0.1 +/- 3.5 D (range -6.75 to + 7.25). No wound dehiscences or glaucoma was noted during the follow-up. CONCLUSION: The Hanna suction system creates a sharp, deep, and perpendicular cut on both button cornea and host cornea, making it possible to use donor and recipient corneas of the same diameter in PK with good clinical and refractive results, particularly for myopic (keratoconus) patients.

[Electro-functional changes in pseudo-exfoliative syndrome]. / Modification des examens électrofonctionnels dans le syndrome pseudo-exfoliatif.

PURPOSE: Up to date the pathogenetic aspects of exfoliation syndrome which are not well agreed upon even though the vascular damage plays a major role. METHODS: In order to evaluate the electrophysiological changes occurring in patients affected by exfoliation syndrome a case-control study with pattern electrofunctional examinations and oscillatory potentials from standard flash-ERG in 80 eyes affected by exfoliation syndrome with or without ocular hypertension was undertaken. RESULTS: Some statistically significant PERG, P-PEV and OP abnormalities were found in eyes affected by exfoliation syndrome with or without ocular hypertension (p < 0.001). There is no statistically significant difference between affected and control eyes in monolateral syndrome (p > 0.05). The electrofunctional examination did not mark any statistically significant difference between eyes with and without ocular hypertension (p > 0.05). CONCLUSION: The results stressed by pattern and oscillatory potentials examinations may support the hypothesis of a primitive change of the optic nerve.

Treatment of vascular retinopathies with Pycnogenol.

The aim of our study was to investigate the effects of Pycnogenol on the progression of diabetic retinopathy and other vascular retinal disorders. The study consisted of a double-blind phase in which 20 patients were recruited and randomly treated with placebo or Pycnogenol (50 mg x 3/day for 2 months) and an open phase in which another 20 patients were treated with Pycnogenol at the same dose schedule. In total, 40 patients with diabetes, atherosclerosis and other vascular diseases involving the retina were enrolled; 30 of them were treated with Pycnogenol and 10 with placebo. The results demonstrated a beneficial effect of Pycnogenol on the progression of retinopathy. Without any treatment (placebo) the retinopathy progressively worsened during the trial and the visual acuity significantly decreased; on the contrary, the Pycnogenol-treated patients showed no deterioration of retinal function and a significant recovery of visual acuity was also obtained. The fluorangiography showed an improvement of retinal vascularization and a reduced endothelial permeability and leakage in the Pycnogenol, but not in the placebo-treated, patients. The ophthalmoscopy and the electroretinogram (ERG) also confirmed the beneficial effects of Pycnogenol. The mechanism of action of Pycnogenol may be related to its free radical (FR) scavenging, anti-inflammatory and capillary protective activities. It has been suggested that Pycnogenol may bind to the blood vessel wall proteins and mucopolysaccharides and produce a capillary 'sealing' effect, leading to a reduced capillary permeability and oedema formation.