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Penetrating keratoplasty (PK) in children is a complex and ultifaceted problem that has been experiencing pediatric ophthalmology for several decades With all its radicality and with all its therapeutic potential, most surgeons prefer, nevertheless, to refuse to transplant the cornea in childhood, and transfer it to a later date, especially in infants (up to a year). In the absence of alternative interest in this operation did not fade, the practice of corneal transplantation in children continued and improved. The desirability of PK in corneal opacities (CO) in children is no longer discussed as such. On the agenda is another question: how to make this operation a truly effective way of treatment. This is not an easy task in itself, with many problems. Among them – the complexity of communication with a young patient, a special plasticity of the tissues of the child’s eye, heavy, as a rule, the combined nature of the pathology, predisposition to violent inflammatory reactions, etc. In many years of practice, for the most part, by trial and error, these problems are gradually finding their solution. An empirical experience always precedes the understanding of the problem and the search for its solution, no matter how unsuccessful it may seem at the very beginning. This work is devoted to generalization of such experience. Objective . To Evaluate the immediate and long-term results of penetrating keratoplasty in children. Material and methods. Retrospectively and prospectively analyzed the medical history and hospital records of children operated in the Department of pathology of eyes in children, Moscow Helmholtz Research Institute of Eye Diseases in the period from 1997 to 2017. The total sample consisted of 208 cases of the PK, was performed in 185 children on 208 eyes. By the nature of the disease, all observations were divided into congenital and acquired CO. Among the latter, turbidity of traumatic and non-traumatic nature was distinguished. Biological and functional results of PK were evaluated. The biological result of the operation was evaluated in terms of graft survival (Kaplan-Mayer model). The functional result was estimated approximately: by tracking the child’s toys from a certain distance and by the method of pr eferred gaze. Results . The first 6 months after the surgery, the transplants, with rare exceptions, remained transparent. By the end of the 1st year, 72% of transplants remained transparent, by the end of the 2nd year not less than 65%, by the end of the 3rd year-not less than 55%, by the end of the 5th year not less than 45% of transplants. In search of a more rigorous determination of the results, PK assessed the impact of particular clinical circumstances on the engraftment of transplants. It turned out that regardless of the etiology of the disease graft survival is significantly lower if keratoplasty is carried out in a vascularized couch, if simultaneously with corneal transplantation are other optical-reconstructive surgery: cataract extraction, vitrectomy, plastic iris, if the diameter of the transplant >8 mm, in the eyes with glaucoma in history. Transparency of transplants is significantly reduced in complicated postoperative course: recurrent rejection crises, increased IOP or the appearance of synechiae. In at least 80% of patients, corneal transplants have resulted in improved visual aquity (VA). In most of these cases, VA increased from light perception to 0.1-0.3. Satisfactory results, when VA after surgery reached 0.6-0.8, were in patients with keratoconus and congenital hereditary corneal dystrophy. Among those who have measured hundredth of VA dominated children with severe congenital malformations of the cornea and anterior segment of the eye. Conclusion . PK in children today is a very successful surgical intervention, the therapeutic potential of which can be realized with proper consideration of risk factors, impeccable technique and careful postoperative monitoring.

Purpose We report our findings of a patient with a definitive diagnosis of familial lecithin-cholesterol acyltransferase deficiency (FLD), whose corneal thickness was measured using swept-source optical coherence tomography (OCT) and who underwent an ophthalmologic therapeutic intervention. Observations The patient was a 78-year-old Japanese man diagnosed with FLD at the age of 52 years. This was his first ever visit to an ophthalmology clinic. Slit lamp microscopy revealed bilateral diffuse corneal opacities and cataracts, angioid streaks of the retina, and macular atrophy in the fundus. The central corneal thickness, measured with swept-source OCT, was within the normal range in both eyes. Deep lamellar keratoplasty, phacoemulsification, and intraocular lens insertion were performed simultaneously for the left eye. The right eye was treated only using deep lamellar keratoplasty. Conclusions and importance As seen in patients with fish-eye disease, the central corneal thickness in this patient with FLD was within the normal range. Since FLD is a rare disease, it is essential to collect and examine further data on corneal thickness.

International audience; The cornea represents the external part of the eye and consists of an epithelium, a stroma and an endothelium. Due to its curvature and transparency this structure makes up approximately 70% of the total refractive power of the eye. This function is partly made possible by the particular organization of the collagen extracellular matrix contained in the corneal stroma that allows a constant refractive power. The maintenance of such an organization involves other molecules such as type V collagen, FACITs (fibril-associated collagens with interrupted triple helices) and SLRPs (small leucine-rich proteoglycans). These components play crucial roles in the preservation of the correct organization and function of the cornea since their absence or modification leads to abnormalities such as corneal opacities. Thus, the aim of this review is to describe the different corneal collagens and proteoglycans by highlighting their importance in corneal transparency as well as their implication in corneal visual disorders.

Background Investigating the efficacy and safety of phacoemulsification with intraocular lens (IOL) implantation in corneal opacities. Methods This retrospective study was conducted in a tertiary medical center. Twenty-three eyes of 19 patients with cataracts and corneal opacities obscuring the pupillary center having received phacoemulsification with IOL insertion without any ancillary techniques were enrolled. The primary study outcome measures were uncorrected and best corrected visual acuity (BCVA), and complications. Backscatters of corneal scar lesions were evaluated by slit lamp-based haze grading, Scheimpflug Pentacam and anterior segment optical coherence tomography (ASOCT). Visual outcomes after cataract surgeries and improvement range were used to determine the safety and efficacy of cataract surgery for our patients. Results All patients underwent uneventful capsulorhexis and phacoemulsification. The mean age was 72.22 ± 10.1 years, and the mean follow-up period was 18.57 ± 15.42 months. The mean BCVA significantly improved from 1.45 ± 0.65 preoperatively to 0.94 ± 0.55 logMAR postoperatively (p

The authors report on the occurrence of ocular abnormalities in X-linked ichthyosis (XLI) patients, in their carrier mothers and in healthy volunteers who served as controls. The diagnosis of XLI was based on: (1) demonstration of steroid sulfatase deficiency in cultured skin fibroblasts; (2) lack of hybridization of patient’s deoxyribonucleic acid (DNA) with specific steroid sulfatase complementary DNA probe; (3) electrophoretic mobility of plasma lipoproteins. Cholesterol sulfate plasma levels were also determined. The incidence of corneal opacities was the same in XLI patients and in their carrier mothers (23.7 and 24.3%, respectively). Neither other corneal nor ophthalmological alterations were found. Moreover, in XLI patients the plasma levels of cholesterol sulfate were about twenty times higher than in controls. Our findings demonstrate that ocular changes do not seem to be an absolute criterion for a definite diagnosis of XLI and the fact that the pathogenesis of corneal opacities is not due to an accumulation of cholesterol sulfate, but rather that this compound probably induces physicochemical changes of the corneal tissue properties.