Leyla Yavuz Saricay

Purpose: To evaluate the effects of topical recombinant human nerve growth factor (rhNGF) in pediatric neurotrophic keratopathy (NK), with specific attention to corneal opacities, amblyopia, and visual outcomes. Methods: This retrospective study included 10 pediatric patients with NK refractory to conventional therapy who received topical rhNGF for 8 weeks. The cohort had complex multisystem comorbidities and frequently required bedside examinations with limited cooperation; therefore, standardized imaging, quantitative grading, and clinic-based photography were often not feasible. Results: Mean age was 5.1 ± 4.8 years, and 80% of patients were within the amblyogenic period. Mean follow-up was 71.7 ± 45.6 weeks. All patients presented with persistent epithelial defects and corneal opacities, central in 80% of cases. Baseline best-corrected visual acuity was 0.75 ± 0.23 logMAR. Complete epithelial healing was achieved in 9 of 10 patients (90%), with clinician-assessed qualitative improvement in corneal opacity appearance in 6 patients (60%) within 8 weeks. Baseline best-corrected visual acuity did not significantly improve at week 8 or at final follow-up. Six patients demonstrated asymmetric astigmatism greater than +1.50 diopters. One patient with advanced stage 3 NK did not achieve epithelial healing and had limited follow-up because of international referral. Conclusions: Topical rhNGF was effective and well tolerated in pediatric NK, producing rapid epithelial healing and qualitative improvement in corneal opacity appearance. Limited visual recovery likely reflects preexisting central stromal scarring and amblyopia, emphasizing the importance of early NK recognition and timely rhNGF initiation to preserve visual potential during childhood visual development and long-term clinical decision making globally care.

Introduction: Phakic intraocular lenses (pIOLs) are well recognized for effectively correcting high refractive errors; however, rare complications such as iris cysts may significantly compromise outcomes. To the authors' knowledge, this case is unique because it is the first documented instance of a recurrent iridociliary cyst associated with an iris-claw pIOL, adding important insights into the potential for such complications and emphasizing the need for meticulous postoperative monitoring. Patient and Clinical Findings: A 54-year-old woman with a longstanding iris-claw pIOL presented with fluctuating visual acuity. Clinical evaluation revealed a recurrent iridociliary cyst that had progressively displaced the implant, resulting in intermittent vision loss and mechanical distortion of the pIOL. Diagnosis, Intervention, and Outcomes: The primary diagnosis was a recurrent iridociliary cyst causing displacement of the iris-claw pIOL. Initial management with cyst drainage and ablation provided only temporary relief, as the lesion recurred. Definitive surgical intervention, including complete cyst excision, repositioning of the pIOL, and subsequent cataract extraction, was required. These interventions stabilized the patient's visual acuity, with no recurrence of the cyst at final follow-up. Conclusions: This case underscores the critical importance of early detection, vigilant postoperative assessment, and timely surgical intervention in managing iris cysts associated with iris-claw pIOLs to preserve long-term visual function.

Bardet-Biedl Syndrome (BBS) is a rare autosomal recessive ciliopathy. Within corneal development, primary cilia serve a critical role. We sought to investigate the association of BBS with corneal astigmatism among a cohort of patients with BBS. This was a cross-sectional, retrospective study performed at a pediatric ophthalmology department of a tertiary hospital. The study enrolled 45 patients with genetically confirmed Bardet-Biedl syndrome, encompassing a total of 90 eyes observed from February 2011 to August 2021. Spherical and cylindrical refractive errors and keratometry outcome measures, including diopter (D) values at the flattest and steepest axes, were recorded. Corneal astigmatism of greater than 3D is considered extreme corneal astigmatism based on previously published data. Among 45 patients (M:26; F:19), the mean age was 16.4 ± 8.2 years, and the mean best-corrected visual acuity was 20/60. The most common molecular diagnosis was BBS1, seen in 24 of 45 (53.3%). Among all the patients, the mean spherical refractive error was -2.9 ± 3.8D. The mean cylindrical refractive error was 2.6 ± 1.5D. The mean keratometry values at the flattest axis was 43.5 ± 5.3D (39.4-75.0) and at the steepest axis was 47.2 ± 7.3D(41.5-84.0). Among all the patients with BBS, the mean corneal astigmatism was 3.7 ± 1.0D(0.5-7.1), which is considered extreme. A cohort of individuals with BBS demonstrated high corneal astigmatism. These results suggest an association between corneal astigmatism and primary ciliary dysfunction and may assist in clinical management and future therapeutic targets among BBS and other corneal disorders.

Aicardi syndrome is a neurodevelopmental disorder characterized by a triad of partial or complete agenesis of the corpus callosum, infantile spasms, and pathognomonic chorioretinal lacunae.Examinat...