Outcome of cataract surgery in children affected by malignancies other than retinoblastoma with eye-lens radiation exposure.

PURPOSE: To describe, retrospectively, the visual outcome, feasibility, and safety of cataract surgery in a pediatric population affected by iatrogenic cataract, secondary to systemic oncological treatment for malignancies other than retinoblastoma. METHODS: Young patients, affected by radiation-induced cataract, who were referred to the San Paolo Ophthalmic Center in Padova between 2010 and 2017, were included in the study. All patients had previously received radiotherapy and/or chemotherapy treatment for malignancies, between 2004 and 2013. All medical records of infants who underwent cataract surgery were accurately reviewed. RESULTS: Eighteen eyes out of 11 patients included in the study underwent cataract surgery. The mean age at surgery was 9.7 ± 3.6 years. The interval between tumor diagnosis and cataract development was around 3 years. Mean follow-up after surgery was 15.4 ± 6.3 months. All eyes underwent posterior chamber intraocular lens implantation, posterior capsulotomy, and anterior vitrectomy in one time surgery. No intraoperative complications were shown. Post-operatively, only one eye received laser capsulotomy due to posterior capsule opacification. At the end of follow up, best-corrected visual acuity was 20/20 (LogMAR 0) in all eyes and significantly improved (p < 0.01) compared to baseline. CONCLUSIONS: Iatrogenic-cataract surgery in pediatric oncological patients is a safe and effective way to improve visual acuity. Posterior capsulotomy and anterior vitrectomy at the time of surgery reduce the rate of posterior lens opacification and guarantee an excellent visual acuity in these patients.

Bilateral Isolated Spherophakia in Two Young East European Siblings: A Case Report.

A 19-year-old Caucasian woman was referred to the emergency room and thereafter to the department of ophthalmology complaining for bilateral decrease of visual acuity and severe pain. A complete ophthalmological evaluation was performed. Best-corrected visual acuity (BCVA) was LogMAR 0.3 in the right eye (RE) and LogMAR 0.5 in the left eye (LE). Intraocular pressure (IOP) was 28 and 38 mm Hg in the RE and LE, respectively. The patient showed a shallow anterior chamber and spherical equivalent refractive error -29.0 diopters (D) in the RE and -30.0 D in the LE. The diagnosis of bilateral angle closure glaucoma, secondary to highly myopic, forward dislocated lens was made, in the setting of spherophakia. The ultra-sound biomicroscopy images confirmed the diagnosis. Clear lens extraction was promptly performed with resolution of ocular hypertension and restoration of BCVA. In view of the frequent systemic association, family members also underwent ophthalmological evaluation. The 13-year-old sibling showed mild myopia and borderline IOP. He was administered topical ß-blockers and observation. Genetic counseling did not reveal mutations usually associated with spherophakia or systemic conditions. This case report highlights the variable spectrum of clinical expression in spherophakia; therefore, ophthalmological treatment should be tailored according to clinical presentation. Systemic evaluation and genetic counseling are also recommended in the suspicion of spherophakia.

Prevalence of diabetes and diabetic macular edema in patients undergoing senile cataract surgery in Italy: The DIabetes and CATaract study.

BACKGROUND: The purpose of this study is to determine the prevalence of diabetes and diabetic macular edema in patients undergoing senile cataract surgery in Italy. METHODS: It is a prospective, multicenter, cross-sectional study. Thirteen ophthalmic units equally distributed across the Italian territory have been involved in the study. For a period of 3 months, all subjects undergoing phacoemulsification received an Optical Coherence Tompgraphy (OCT) scan and were screened for the anamnestic presence of diabetes. In addition, five selected units collected blood samples from all their patients to measure glycated hemoglobin (HbA1c) levels and detect the presence of occult diabetes (HbA1c > 6.5%). In diabetic patients, levels of retinopathy were measured and diabetic macular edema was considered significant (clinically significant macular edema) when foveal thickness was above 30% of normal levels. RESULTS: A total number of 3657 subjects have been screened. Among them, 20.4% were diabetics. Prevalence of diabetes was significantly higher in males (24.7%) than in females (17%). Levels of HbA1c were tested in a representative sample of 1216 consecutive subjects, and occult diabetes was diagnosed in 4.8% of cases. No significant differences were observed between age groups or different geographic areas. Among diabetic patients, diabetic macular edema of any kind was present in 27.5% (clinically significant macular edema (6.6%)). No significant differences were seen in the prevalence of diabetic macular edema between males and females or between age groups. Among the 745 diabetic patients, no signs of retinopathy were seen in 537 subjects (76.3%), while 101 patients (14.3%) had nonproliferative retinopathy, 13 (1.7%) had nontreated proliferative diabetic retinopathy, and 53 (7.5%) had laser-treated retinopathy. In the entire sample of 3657 subjects, a normal macula was present in 90.9% of cases, diabetic macular edema of any kind in 5.4%, and other maculopathies in 3.4%. CONCLUSION: In this large cohort study on patients undergoing cataract surgery, more than one-fourth were diabetics and more than one-fourth of these had diabetic macular edema. These high prevalences suggest the opportunity to plan an adequate preoperative assessment in all patients in order to reduce the risk of postoperative development or worsening of a sight-threatening complication such as chronic diabetic macular edema.

The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 (VA LV VFQ-48): Performance of the Italian version.

PURPOSE: The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 is among the most validated tools to collect patient-reported outcomes in a low-vision population. We have aimed to conduct a pilot validation of the Italian version of the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48. METHODS: The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 was translated using a standardized procedure and then administered to consecutive low-vision patients attending rehabilitation services in three centers. Patients were interviewed by a trained psychologist regarding the individual items of the tool. RESULTS: We included 131 patients with a mean visual acuity of 0.91 logMAR (standard deviation: 0.42 logMAR), mostly affected by age-related macular degeneration. The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 showed high internal consistency (Cronbach's alpha: 0.98) and good item-test and item-rest correlation (median: 0.73 and 0.71, respectively). Both the overall score and the subscale (reading, visual motor, mobility and visual information) scores significantly correlated with visual acuity, reading acuity and speed. Reading speed achieved the best absolute correlation with the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 scores (Spearman r: 0.39-0.49). CONCLUSION: The Italian version of the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 is a valid tool to assess patients attending low-vision services. Revising a few items may further improve the tool.

Rapid response to dexamethasone intravitreal implant in diabetic macular edema.

PURPOSE: To evaluate the early effects of dexamethasone (DEX) intravitreal implants in patients with diabetic macular edema (DME). METHODS: This was a prospective, single-arm, interventional clinical series. Eighteen patients (18 eyes) with chronic/recalcitrant or naive DME were included. Patients underwent single DEX intravitreal implant. Clinical assessments, including ophthalmologic examination, central retinal thickness (CRT) measurement by spectral-domain optical coherence tomography (SD-OCT) scan, best-corrected visual acuity (BCVA), and intraocular pressure (IOP) were carried out at baseline, 1-3 hours, and then 3, 7, and 30 days after treatment. The main outcome was change in CRT on SD-OCT, while secondary outcome measures included visual acuity (VA) and changes in IOP following implant. RESULTS: Mean CRT significantly decreased from 565 ± 171 µm at baseline to 310 ± 89 µm at end of follow-up (p<0.001), with reduction becoming evident 1-3 hours after injection. Mean BCVA also significantly improved 7 days and 30 days after treatment up to 0.14 logMAR (p<0.05). All patients had a controlled IOP after the injection with only 1/18 eyes having a transient increase in IOP during follow-up. CONCLUSIONS: This is the first study showing very early effects of DEX implants on CRT reduction and VA improvement in DME.

Optical coherence tomography angiography of circumscribed choroidal hemangioma treated with photodynamic therapy.

We describe features of a circumscribed choroidal hemangioma (CCH) treated with photodynamic therapy (PDT) by means of optical coherence tomography angiography (OCT-A). A 40-year-old man had a complaint of decreasing visual acuity in his left eye for 10 days. A diagnosis of CCH was confirmed by fundus examination, fluorescein angiography, and indocyanine green angiography. An OCT-A study of choroidal lesion before and after PDT was carried out. Segmented en face OCT-A of CCH showed multiple irregular connected vascular channels with hyperflow and a capillary-like lesion. Absent and/or reduced intrachannel flow in most of the intratumoral vessels was shown by OCT-A soon after treatment (2 days), with normalization of vascular flow 7 days and 1 month after PDT. OCT-A was able to noninvasively visualize intrinsic vasculature of CCH showing vascular remodeling after PDT.

Partial-thickness corneal tissue restoration after a chemical burn.

PURPOSE: We describe a case of full-thickness corneal restoration after an acute corneal burn with an acid agent. METHODS: A 32-year-old male reported painful discomfort, redness, photophobia, and a decrease in visual acuity in the left eye after a unilateral burn with an acid agent. Slit-lamp examination revealed massive corneal melting involving necrotic sequestrum of the entire corneal surface. Surgical approach was carried out in order to preserve residual ocular tissues. RESULTS: Extensive corneal-conjunctival layer curettage of the necrotic tissue was performed showing perfectly clear undamaged deep lamellar corneal layers. The patient underwent multilayered amniotic membrane transplantation and total capsular-conjunctival flap in order to preserve ocular tissue from further melting or corneal perforation. A complete and spontaneous "restitutio ad integrum" of the corneal layers was shown during the follow-up. The cornea was perfectly clear with restored normal anatomical architecture. CONCLUSION: In this case, a spontaneous full-thickness corneal tissue restoration occurred after an acute chemical burn. Studies about the mechanisms whereby different cells interact and replicate within the stroma may unveil the biology behind corneal regeneration and transparency.

Effect of 60 kHz and 150 kHz Femtosecond Lasers on Corneal Stromal Bed Surfaces: A Comparative Study.

Purpose. To compare the effect of 60 kHz and 150 kHz femtosecond (FS) laser on the corneal stromal bed surfaces (SBS). Methods. Sixteen human donor corneal tissues unsuitable for transplantation were used. Anterior and posterior lamella was obtained using 60 kHz and 150 kHz FS laser. A standard depth of 400 µ m was set for anterior lamellar keratoplasty (ALK) and endothelial lamellar keratoplasty (ELK). The quality and smoothness of the SBS post-FS laser dissection were graded for statistics. Results. No intraoperative complications were found. The side cuts were straight, and the SBS appeared smoother in cuts obtained using 150 kHz. The average values of the SBS quality of the anterior lamellar cut were found to be 2.25 (±0.28) for 60 kHz and 3.125 (±0.25) for 150 kHz (P = 0.0039). Whereas, 2 (±0.4) for 60 kHz and 2.75 (±0.28) for 150 kHz (P = 0.0273) was the quality observed in endothelial cuts. No significant difference was found between anterior and posterior cuts performed using the same FS laser (60 kHz or 150 kHz) (P > 0.05). Conclusions. The 60 kHz and 150 kHz FS lasers are equally effective in performing lamellar dissection for ALK and ELK. 150 kHz FS laser allows a tighter spot and layer separation which creates a slightly smoother SBS.

Outcomes of 195 consecutive patients undergoing 2-port pars plana vitrectomy with slit-lamp illumination system for posterior segment disease: a retrospective study.

PURPOSE: The purpose of this study was to evaluate the safety and efficacy of 2-port 20-gauge pars plana vitrectomy with a slit-lamp illumination system in different vitreoretinal pathologies. DESIGN: : Retrospective, consecutive, interventional case series. METHODS: One hundred and ninety-five consecutive eyes of 195 patients underwent 20-gauge 2-port pars plana vitrectomy with a combination of slit-lamp illumination and a plano-concave contact lens, at the San Paolo Ophthalmology Center, from September 2005 through November 2007. Postoperative visual acuity at baseline and at 1, 3, and 6 months; intraoperative and postoperative complication rate; and surgical time were evaluated. RESULTS: All patients completed 6 months of follow-up. The mean overall visual acuity was 0.74 ± 0.03 (mean ± SD) at baseline, improving to 0.56 ± 0.03 (P < 0.0001), 0.48 ± 0.03 (P < 0.0001), and 0.43 ± 0.03 (P < 0.0001) at 1, 3, and 6 months, respectively. No intraoperative complications occurred. Postoperative complications included retinal detachment in three patients, epiretinal membrane recurrence in three eyes, persistent macular hole in four eyes, and phthisis in one eye. Cataract formation was observed in 25 eyes. The total mean surgical time was 28.3 ± 10.1 minutes. No one had hypotony after the surgical procedure, and no cases of endophthalmitis were observed. Conversion to standard three-port vitrectomy was not necessary in any of the cases. CONCLUSION: Two-port 20-gauge pars plana vitrectomy with slit-lamp illumination is a safe and effective procedure for posterior segment surgeries.

A psychological perspective of eye floaters.

Patients experiencing flashes and spots in their perceptive fields often resort to consulting an ophthalmologist without finding an answer, mainly because these symptoms are not necessarily associated with pathology of the eye. The purpose of our research study was to understand the experience of these patients, differentiate among them, and propose different ways of treating them. We carried out an eye examination and echography, individual semistructured interviews, and dependency grids with 11 patients at the ophthalmology department of a public hospital in northern Italy. We found that individuals' ways of experiencing and reacting to eye floaters might be different and might depend on the perception of the disease, the personal explanation, the solutions tried, the trust placed in medicine, self-construction, and the dispersion of dependency. Understanding the experiences of patients suffering from eye floaters might help health care professionals to personalize their approaches to these patients.

Health status and patient satisfaction after corneal graft: results from the corneal transplant epidemiological study.

Purpose. To evaluate effects of corneal transplantation on the health-related quality of life and patients' satisfaction. Methods. Patients scheduled for elective penetrating or anterior lamellar keratoplasty completed by telephone interview the SF-12 Health Survey, before and one year after surgery, and a 6-item questionnaire on the satisfaction for graft outcomes. Results. The two questionnaires were answered by 1,223 patients. Transplantation did not influence the PCS-12 in males (ES = -0.01) and had a negative effect in females (ES = -0.18). Both sexes improved their MCS-12 (ES = 0.18 and 0.23, resp.). The majority of patients (83.1%) were satisfied by the outcome of the graft. Conclusions. This is the first report on the use of the SF-12 and one of the few that assess quality of life in patients after corneal transplantation. We showed that grafting improves patients' health-related quality of life results of patients, influencing mental health (i.e., psychological attitude, social interaction, and emotions) with minor effects on physical health (limitation, pain, and vitality).

Good epidemiologic practice in retinitis pigmentosa: from phenotyping to biobanking.

Inherited retinal dystrophies, such as retinitis pigmentosa (RP), include a group of relatively rare hereditary diseases caused by mutations in genes that code for proteins involved in the maintenance and function of the photoreceptor cells (cones and rods). The different forms of RP consist of progressive neurodegenerative disorders which are generally related to various and severe limitations of visual performances. In the course of typical RP (rod-cone dystrophy), the affected individuals first experience night-blindness and/or visual field constriction (secondary to rod dysfunctions), followed by variable alterations of the central vision (due to cone damages). On the other hand, during the atypical form of RP (cone-rod dystrophy), the cone's functionalities are prevalently disrupted in comparison with the rod's ones. The basic diagnosis of RP relies upon the documentation of unremitting loss in photoreceptor activity by electroretinogram and/or visual field testing. The prevalence of all RP typologies is variably reported in about one case for each 3000-5000 individuals, with a total of about two millions of affected persons worldwide. The inherited retinal dystrophies are sometimes the epiphenomenon of a complex framework (syndromic RP), but more often they represent an isolated disorder (about 85-90 % of cases). Although 200 causative RP mutations have been hitherto detected in more than 100 different genes, the molecular defect is identifiable in just about the 50% of the analyzed patients with RP. Not only the RP genotypes are very heterogeneous, but also the patients with the same mutation can be affected by different phenotypic manifestations. RP can be inherited as autosomal dominant, autosomal recessive or X-linked trait, and many sporadic forms are diagnosed in patients with no affected relatives. Dissecting the clinico-genetic complexity of RP has become an attainable objective by means of large-scale research projects, in which the collaboration between ophthalmologists, geneticists, and epidemiologists becomes a crucial aspect. In the present review, the main issues regarding clinical phenotyping and epidemiologic criticisms of RP are focused, especially highlighting the importance of both standardization of the diagnostic protocols and appropriateness of the disease's registration systems.

Large-spot subthreshold transpupillary thermotherapy for chronic serous macular detachment.

PURPOSE: To report the effect of subthreshold transpupillary thermotherapy (TTT) in treating serous detachment of the neurosensory retina secondary to chronic central serous chorioretinopathy (CCSC). METHODS: Seven eyes from five patients with CCSC, persistent serous detachment of the neurosensory retina and a clinical course of between 12 and 60 months were treated. All eyes received large-spot TTT guided by indocyanine green angiography (ICGA). Subthreshold TTT was performed using an 810 nm diode laser with a spot size of 3.0 mm (power was set at 350 mW). Treatment was applied for 60 seconds to the areas of choroidal hyperfluorescence on ICGA. RESULTS: The mean number of TTT sessions was 1.4 ± 0.5. All eyes were followed up for at least 6 months (mean 9.6 ± 3.2 standard deviation; range 6-12 months). The mean logarithm of the minimum angle of resolution best-corrected visual acuity was significantly better compared with baseline. All TTT-treated eyes had stable or improved vision (P < 0.001). Mean optical coherence tomography (OCT) central foveal thickness was significantly lower in all patients (P < 0.001) compared with pretreatment OCT, with a reduction in subretinal fluid and resolution of serous detachment associated with anatomical fovea restoration. No patient had any treatment-related side effects. CONCLUSION: Modified subthreshold TTT appears to have a beneficial effect in treating patients with CCSC and persistent neurosensory detachment. The encouraging results and lack of visually significant complications suggest that further investigation is warranted.

Fungal keratitis following deep lamellar keratoplasty.

A young man affected from keratoconus was submitted to deep lamellar keratoplasty (DLK). The day after, the presence of pseudochamber between the donor and the recipient cornea was observed by the slit-lamp and the patient was submitted to the injection of an air bubble into the anterior chamber. Approximately six days later, multiple, whitish patches mostly located in the centre of the lamellar interface were noticed. Medical treatment was started immediately but no improvement was observed and penetrating keratoplasty was performed. Although this organism has been described as a microbial pathogen in blepharitis, conjunctivitis, keratitis, canaliculitis, dacryocystitis, and endophthalmitis, to the best of our knowledge, this is the first case report of keratitis after DLK caused by Actinomyces species.

Stromal support for Descemet's membrane endothelial keratoplasty.

PURPOSE: To evaluate the use of a peripheral stromal support to facilitate and improve the outcomes of Descemet's membrane endothelial keratoplasty (DMEK). DESIGN: Prospective case series. PARTICIPANTS: Ten patients with Fuchs' endothelial dystrophy. INTERVENTION: Pneumatic dissection was used to detach the central part of the Descemet's membrane and endothelium from the deep stroma. Endothelial grafts including a peripheral stromal support were obtained by eccentric punching of donor tissue and used to perform DMEK surgery in 10 patients. MAIN OUTCOME MEASURES: Operative time, graft attachment rate, best spectacle-corrected visual acuity (BSCVA), endothelial cell loss, refraction, and complications. RESULTS: In all cases the surgical time was ≤ 1 hour. The postoperative course was uneventful in all but 2 cases, which required rebubbling owing to early graft detachment. Final attachment rate was 100%. The average follow-up was 8.4 months (range, 6-12). Postoperative BSCVA was ≥ 20/40 in all cases and no substantial change in refraction was recorded. Postoperative endothelial cell loss averaged 24.1% (range, 8%-34.9%). CONCLUSIONS: Stromal support facilitates surgery, reduces complications, and appears to maintain the favorable outcomes of DMEK.

Phosphodiesterase-5 inhibitor tadalafil acts on endothelial progenitor cells by CXCR4 signalling.

It has been proposed that endothelial progenitor cells (EPC), originating from the bone marrow contribute to neo-angiogenesis in vivo by forming endothelial cells. Once released in the bloodstream, EPC home at the site of vascular damage where they participate in endothelium regeneration. In this process CXCR4 plays a key role. Recently we demonstrated that a prolonged therapy with phosphodiesterase-5 (PDE5) inhibitors does improve endothelial function and increases circulating EPC, suggesting a role of PDE5 in EPC physiology. Here we tested the expression of PDE5 and CXCR4 on cultured, circulating, and bone marrow resident EPC, and we studied the effect in vivo and in vitro of PDE5 inhibition after administration of a PDE5 inhibitor (tadalafil) on EPC, in term of CXCR4 expression. We documented that in vivo and in vitro EPC express both PDE5 and CXCR4, and that tadalafil administration induced a significant increase in EPC number and the relative CXCR4 expression. This effect is inhibited by selective CXCR4 antagonist. We thus demonstrated that PDE5 inhibition acts on CXCR4 signalling in EPC and we can suppose an involvement of cGMP second messenger system in both EPC release from the bone marrow and EPC-mediated peripheral re-endothelization.

Unusual presentation of giant cell angiofibroma of the eyelids.

PURPOSE: To describe a case of bilateral eyelid-confined giant cell angiofibroma (GCAF) in a patient with a slowly progressive bilateral eyelid swelling. METHODS: A 40-year-old man with a 5-year history of slowly progressive bilateral eyelid swelling, severe functional impairment, and bilateral cosmetic deformity was studied. An extensive ophthalmologic evaluation, laboratory examinations, and orbital magnetic resonance imaging were carried out. RESULTS: Clinical examination showed nonpitting lymphedema affecting both upper and lower eyelids, with orange peel skin. Orbital magnetic resonance imaging revealed diffuse thickening of the preseptal structures in the eyelids without extension to the orbit. Histologic specimen revealed the presence of spindle and multinucleated giant cells surrounding pseudovascular spaces strongly positive to CD34 and vimentin. A diagnosis of GCAF was made and radiation therapy was performed 3 weeks after surgical debulking with partial recovery of visual and anatomic function. CONCLUSIONS: Giant cell angiofibroma involving the eyelid is rare and can represent a diagnostic and therapeutic challenge to the ophthalmologist.

Association of age-related macular degeneration with polymorphisms in vascular endothelial growth factor and its receptor.

OBJECTIVE: To analyze the association between age-related macular degeneration (AMD) and polymorphisms in vascular endothelial growth factor (VEGF) and VEGF receptor KDR gene polymorphisms. A complex, multifactorial disease in which genetic and environmental factors interact, AMD is the leading cause of blindness in the elderly. Vascular endothelial growth factor (VEGF), a key regulator of angiogenesis, is considered an important factor for the pathogenetic processes of AMD. Previous studies investigated the possible association between VEGF-A gene polymorphisms and AMD, with contrasting data. No study examined the possible role of VEGF receptor KDR gene polymorphisms. DESIGN: Case-control study. PARTICIPANTS AND CONTROLS: We enrolled 226 AMD cases and 248 controls from an ophthalmology hospital center. METHODS: Genotypying for 16 polymorphic markers (single nucleotide polymorphisms [SNPs]) in VEGF-A and KDR genes. MAIN OUTCOME MEASURES: Distribution of genotypes in AMD cases and controls. RESULTS: Two polymorphisms (rs833069 in intron 2 of the VEGF-A gene, rs2071559 in the promoter of the KDR gene) were significantly associated with risk of AMD. In particular, for VEGF-A rs833069 the AMD risk was increased >5-fold for G homozygotes compared with homozygous carriage of the A allele. For KDR rs2071559 the AMD risk was increased >3-fold for T homozygotes compared with homozygous carriage of the C allele. Carriers of risk alleles for both markers have a >6-fold increased risk of AMD with respect to carriers of non-risk alleles. CONCLUSIONS: We expand previous data on the association of AMD with VEGF-A gene variations and identify for the first time an association with variations in the KDR gene. Because the SNP-604T-bearing KDR promoter has higher transcription activity, our findings further support the role of the VEGF pathway in the pathophysiology of AMD. It is possible that applications of haplotype/genotype analysis in these genes will play a role in risk assessment and pharmacogenomic approaches to AMD diagnosis and management.