Background: Keratoconus is a disease characterized by progressive distortion of the corneal anatomy, coupled with a decrease in vision. Assessing quality of life (QoL) in keratoconus is essential. So far, no instrument in the world has been designed to evaluate both visual function and emotional distress in this population. The purpose of the following study is to develop and validate the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ) in a population of ectatic patients, the very first disease-specific scale to measure emotional latent traits in keratoconus. Methods: A last generation, Rasch analysis method was used for scale validation. First, a number of focus groups were carried out to create a pool of potential items. Then, a series of processes (such as "Content Validity Index") was carried out to develop a prior, 20question version of the KEPAQ. Then, a study including 150 keratoconus patients was performed, followed by a careful Rasch analysis to validate and optimize both sub-scales (Emotional Compromise, KEPAQ-E, and Functional Compromise, KEPAQ-F). Results: Initially, 86 items were considered as potential elements. After test optimization, 20 items were retained. A total of 150 patients with a confirmed diagnosis of keratoconus were included for the Rasch analysis. The mean age was 29.84 ± 9.96 years. In 150 patients, 12.6% had a history of keratoplasty, 46.0% had corneal rings, and 31.3% had crosslinking. For both sub-scales, misfitting items were removed until no misfitting was determined by repetitive Rasch runs. For the final version of the KEPAQ-E sub-scale, variance explained by the model was 62.4% with a dimensional scale. Person Separation Index and Person Number of Strata were 2.43 and 3.57, respectively. For the final version of the KEPAQ-F sub-scale, variance explained by the model was 61.3% with a unidimensional scale. Person Separation Index and Person Number of Strata were 3.19 and 4.59, respectively. Both sub-scales showed excellent Person Reliability. Conclusion: The KEPAQ is a robust scale, developed and validated through the latest theoretical models. It shows excellent psychometric properties, which render it extremely useful for both clinical and research use. To date, the KEPAQ is the only disease-specific scale worldwide to evaluate both functional and emotional compromise in keratoconus patients.

Background: Keratoconus is a disease characterized by progressive corneal distortion and quality of vision. So far, no study using disease-specific scales has evaluated whether different stages of the disease correlate with higher quality of life (QoL) compromise. Methods: A total of 114 patients with a confirmed diagnosis of Keratoconus were included in this retrospective study. All patients underwent a clinical and a Pentacam evaluation. They were also administered the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ). Belin ABCD criteria were used for Keratoconus classification. "Better eye" was defined as the eye with the lowest maximum keratometry value. Spearman Rank Order Correlation was used to determine the correlation between the different Belin criteria and the KEPAQ scores in both subscales. Results: Mean age was 28.13 ± 11.57 years, with 39.47% of patients being male. Mean score for the KEPAQ-E was 2.33 ± 3.40 Logit, while for the KEPAQ-F, it was 1.85 ± 3.61 Logit. Criteria A (anterior elevation), B (posterior elevation) and D (visual acuity) in the worse eye correlated significantly with a greater decrease in QoL (p < 0.05 for all correlations). No correlation could be found regarding the better eye. Conclusion: A greater corneal distortion in the worse eye, as determined by Belin ABCD, is associated with a greater decrease in patient's QoL. Surgical improvement of the worse eye should probably be performed before surgery of the better eye, as it may provide a better response regarding the quality of life improvement.

Background Refractive errors are widespread in the human population; nowadays, numerous surgical options allow for efficient and safe correction them. One of the main elements to ensure success in this kind of intervention will depend on the careful patient and surgical approach selection. Excimer laser corneal surgery is considered by most for low to moderate ametropias. Another option, which has been suggested may be safer, is to cut a small corneal lenticule with femtosecond laser, and then extracting it through a small incision. Nevertheless, in some specific cases, such as patients with high refractive error or those with some corneal abnormality, laser corneal ablations are considered unsafe from either a biomechanical or refractive standpoint. In this kind of particular cases, Phakic Intraocular Lens (P-IOL) implantation constitutes attractive, highly predictable and safe option. Objective The authors want to show the case of a young high-myopic woman, already pseudophakic in one eye, where the P-IOL implantation in the fellow eye yielded excellent short-term visual results, and high patient's satisfaction, is presented. Materials The authors present the case of a 32-years-old, highly myopic female patient underwent a Refractive Lens Exchange (RLE) with a monofocal Intraocular Lens (IOL) implantation in her left eye elsewhere, and developed severe visual issues, especially regarding near-work. Symptoms resolved through the implantation of a posterior chamber P-IOL in the contralateral eye. Results The postoperative course was unremarkable, inflammation was mild, and visual recovery was quick. There was no need to perform any procedure on her left eye or to use any reading glasses, as unilateral effective near vision through her right eye was enough for all her daily tasks. Conclusions RLE in young pre-presbyopic highly myopic patients may not be an advisable alternative in most cases, because of the high risks of retinal complications observed. In addition, eliminating accommodation will cause significant limitations, and multifocal IOLs currently available are far from the quality of vision that a young human crystalline lens yields. On the other hand, implantation of a P-IOL is a good option if eye conditions are optimal, as it preserves natural accommodation. In this case an EyeCryl Phakic Toric® IOL showed excellent short-term refractive predictability and safety.

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Background. Phakic Intraocular Lens (P-IOL) implantation is a safe, easy, predictable intervention designed to manage moderate to high refractive errors. Complications are relatively uncommon and include mainly cataract and intraocular pressure spikes. Toxic Anterior Segment Syndrome (TASS) is a rather unusual sterile anterior segment inflammation after uneventful intraocular surgery, extremely rarely reported after P-IOL implantation. Urrets-Zavalia Syndrome (UZS) is also very rarely described after P-IOL. To date, to the best of the authors’ knowledge, no article has ever described the simultaneous occurrence of TASS and UZS in a patient after P-IOL implantation. Objective. In this article, the authors present the case of a female patient with moderate myopic astigmatism, who underwent sequential P-IOL implantation at two different facilities. The postoperative course of the first eye was uneventful, but she developed complications associated to the intervention in the second eye. Materials. The article describes the case of a young patient who underwent a sequential Phakic Intraocular Lens (P-IOL) implantation at two different institutions. The postoperative course of the first eye (left eye) was uneventful; however, the second eye (right eye) initially developed Toxic Anterior Segment Syndrome (TASS). Although timely and correct management was instituted, upon resolution of TASS, the patient developed Urrets-Zavalia Syndrome, anterior subcapsular cataract, and significant endothelial damage in the same eye. Results. The patient was followed closely and managed accordingly; corneal edema and anterior segment inflammation of the right eye eventually resolved. Nevertheless, an anterior subcapsular cataract and a fixed dilated pupil remained; with normal intraocular pressure (IOP). Specular microscopy confirmed an endothelial cell loss in the TASS eye (right eye). Pupil size showed no reaction to repeated doses of Pilocarpine 2%. A month after surgery, refraction on her right eye was + 0.25 + 0.75 × 93 , which resulted in a 20/50 vision. Conclusions. TASS and UZS are both extremely rare complications after uneventful P-IOL implantation, with only a handful of cases having been reported of each of them. To date, this is the very first case where UZS ensued after and potentially as a consequence of TASS in a patient who had undergone P-IOL implantation. Although a direct causative element could not be pinpointed, the fact that the complication ensued after being operated in one surgical institution and not the other, could suggest some role of different sterilization and handling procedures, but no direct conclusion can be made on this case.