Background/Objectives: Aniridia is a rare congenital disorder characterized by structural and functional abnormalities in ocular development due to PAX6 haploinsufficiency, leading to complications such as aniridia-associated keratopathy (AAK). Meibomian gland dysfunction (MGD), a prevalent yet underexplored condition in aniridia, exacerbates tear film instability and chronic ocular surface inflammation, contributing to AAK progression. This study investigates the relationship between MGD severity and AAK in individuals with aniridia. Methods: This prospective randomized study included 113 participants (53 with aniridia and 60 controls). Comprehensive ophthalmic evaluations, including noninvasive meibography, were performed. The MGD severity was assessed using a standardized meiboscore scale, while the AAK severity was classified according to established clinical grading criteria. Statistical analyses, including Spearman’s correlation and chi-squared tests, were used to evaluate the relationships among MGD, AAK, and visual acuity. Results: MGD was significantly more prevalent and severe in the aniridia group compared to controls (p < 0.00001). A strong positive correlation was observed between MGD severity and AAK grade (r = 0.72, p < 0.00001), with both conditions associated with reduced best-corrected visual acuity (BCVA; r = −0.80 and −0.86, respectively, p < 0.0001). Age was positively correlated with MGD (r = 0.47, p = 0.0004) and AAK (r = 0.34, p = 0.0123), with gender-specific trends observed in females. Conclusions: MGD significantly contributes to AAK progression and visual impairment in aniridia. Meibography offers valuable insights into MGD severity, supporting early diagnosis and targeted interventions. Addressing MGD through tailored therapies could mitigate AAK progression and improve visual outcomes in this challenging condition.

Background/Objectives: Aniridia-associated keratopathy (AAK) is a potentially vision-threatening pathology in congenital aniridia, for which both the underlying etiopathogenesis and effective treatment remain unclear. Methods:This prospective study was conducted to assess and compare the short-term outcome after superficial keratectomy (SK) alone or in a combination with an amniotic membrane transplantation (AMT). Here, 76 eyes were enrolled in 76 patients with grade 4 AAK. In all eyes, in order to assess preoperatively the efficiency of the limbal epithelial stem cells (LESC), the presence of corneal epithelial cells in confocal microscopy was established. The analyses included: best corrected visual acuity (BCVA), the stage of AAK and the number of corneal quadrants involved in corneal neovascularization (CNV). Results: Six months after surgery, the mean BCVA was 0.05 and ranged from 0.002 up to 0.1 in both groups. Improvement in BCVA occurred in 94.29% patients when *SK alone* was performed, and in 92.68% when in combination with AMT. There were no statistically significant differences in the effect of therapy depending on the type of surgery, regarding BCVA, stage of AAK and the number of quadrants with CNV. Conclusions: SK alone is an effective procedure in short outcomes limited to six months for advanced AAK in association with LESC partial efficiency.

Background: Aniridia is a rare panocular, bilateral, and congenital disease characterized by complete or partial iris hypoplasia and foveal hypoplasia, leading to decreased visual acuity and nystagmus. AAK, also referred to as aniridic keratopathy, manifests as corneal surface damage, epithelial thinning or loss, inflammation with immune cell infiltration, vascularization, and chronic progressive opacification. Methods: Twenty-one eyes in eighteen patients with aniridia underwent the triple procedure for visual rehabilitation. Subjects with stromal scarring with mild limbal deficiency were qualified for surgery. The majority of them developed stage II (15), and a few of them had third-degree (6) aniridic keratopathy. Results: The mean patient age was 38.4 ± 8.8. Visual acuity after one year of observation ranged from 0.4 in two eyes to 0.2 in nine eyes to below 0.1 in ten eyes. In the second year, VA remained at the same level in 13 patients (72.2%). In the third year, four patients (22.2%) experienced recurrence of AAK. Conclusions: A majority of the ARK cases (72.2%) had a graft providing useful vision for the patient 2 years after corneal transplantation, but the visual gain was modest at best. Longer follow-up time is required to evaluate functional graft outcomes.