We report two cases who have undergone penetrating keratoplasty (three eyes total), and who were fitted with hydrogel lenses.In the first case, a 28-year-old male presented with an interest in contact lens fitting. He had undergone corneal transplantation in both eyes, about 5 years ago. After topographies and trial fitting were performed, it was decided to be fitted with reverse geometry hydrogel lenses, due to the globular geometry of the cornea, the resultant instability of RGPs, and personal preference. In the second case, a 26-year-old female who had also penetrating keratoplasty was fitted with a hydrogel toric lens of high cylinder in the right eye.The final hydrogel lenses for the first subject incorporated a custom tricurve design, in which the second curve was steeper than the base curve and the third curve flatter than the second but still steeper than the first. Visual acuity was 6/7.5 RE and a mediocre 6/15 LE (OU 6/7.5). The second subject achieved 6/4.5 acuity RE with the high cylinder hydrogel toric lens.In corneas exhibiting extreme protrusion, such as keratoglobus and some cases after penetrating keratoplasty, curvatures are so extreme and the cornea so globular leading to specific fitting options: sclerals, small diameter RGPs and reverse geometry hydrogel lenses, in order to improve lens and optical stability. In selected cases such as the above, large diameter inverse geometry RGP may be fitted only if the eyelid shape and tension permits so. The first case demonstrates that the option of hydrogel lenses is viable when the patient has no interest in RGPs and in certain cases can improve vision to satisfactory levels. In other cases, graft toricity might be so high that the practitioner will need to employ hydrogel torics with large amounts of cylinder in order to correct vision. In such cases, the patient should be closely monitored in order to avoid complications from hypoxia.

This report describes the occurrence of keratoglobus lesions in a rearing flock of broiler breeder grandparent stock (female line), and its ophthalmological and ocular pathomorphological features. Keratoglobus is known as a recessive sex-linked (Z chromosome) trait in some lines of chickens. The first cases were encountered at 5 weeks of age. At the end of the rearing period a total of 1.5% of the flock was affected. In the flock and affected birds, no signs of any other disease were detected. Compared to eyes of healthy flock mates, the most prominent symptom was ocular protrusion due to bulging and thinning of the cornea, and increase in anterior chamber depth. In addition, there were mononuclear, granulocytic and plasma cell infiltrates in the iris and corpus ciliare, and mild to minimal exudate in the anterior chamber in all cases examined. Although the corneal epithelium and collagen bundles appeared normal on electron microscopy, the corneal endothelium showed some loss of cells and the keratocytes some degenerative changes. The cornea had not lost its transparency and did not show any signs of inflammation, whereas the conjunctiva contained mononuclear infiltrations. Other eye structures did not show any clinical or pathological changes. The pathogenesis of keratoglobus lesions in chickens is unknown. Although a hereditary origin seems probable, it is not known whether the inflammatory changes found caused the presented pathology or were secondary to the extreme corneal bulging.

Evaluation of a new surgical technique for the management of corneal ectasia with peripheral corneal involvement.Twelve eyes of 12 patients with corneal ectasias and peripheral corneal thinning requiring surgical intervention, including eight patients with combined keratoconus and PMD and four patients of keratoglobus, were enrolled for the study in a tertiary care hospital. All patients were contact lens intolerant and had a best corrected visual acuity (BCVA) </=20/120 with nine patients (75%) having BCVA </=20/200. "Tuck In" Lamellar Keratoplasty (TILK) that included a central lamellar keratoplasty with intrastromal tucking of the peripheral flange was performed in these patients. The main outcome measures analysed were uncorrected visual acuity (UCVA), BCVA, keratometry, refractive status and time for epithelial healing.At the last follow-up (mean: 1.7 years (range 13-48 months)), six patients (50.0%) had BCVA >/=20/60, and all patients had BCVA >/=20/80. The mean keratometry decreased from 57.54 (SD 6.89) D preoperatively to 46.36 (2.39) D (p = 0.003), and the mean spherical equivalent (SEQ) refractive error decreased from -7.8 (4.6) D preoperatively to 1.23 (1.88) D (p = 0.007). A significant decrease was also seen in mean refractive astigmatism which decreased from 5.93 (3.06) D preoperatively to 3.23 (1.14) D (p = 0.037).Our technique of TILK is an effective surgical modality for the management of ectatic corneal dystrophies with peripheral corneal thinning.

To report a case of a 34-year-old woman presenting with keratoglobus and posterior polymorphous corneal dystrophy (PPMD).Observational case report including ophthalmologic examination, topographic findings, and specular microscopy findings.This patient presented with corneal steepening on topography consistent with keratoglobus, as well as large areas of irregular polymorphous changes of the corneal endothelium on specular microscopy consistent with PPMD.We report the first case with clinical features of both keratoglobus and PPMD. This report brings forth the description of keratoglobus findings on Orbscan topography.

We report the use of perfluoropropane (C(3)F(8)) gas for the treatment of acute hydrops with intrastromal cleft in a case of pellucid marginal degeneration with keratoglobus. Acute hydrops in a 36-year-old man with pellucid marginal degeneration with keratoglobus was treated with intracameral injection of 0.3 mL isoexpansile C(3)F(8) (14%). Reduction in corneal haze was seen beginning from the third postoperative day. Significant clearing had taken place by the end of the second week with the closure of intrastromal cleft. Best corrected visual acuity improved from finger counting at 1 foot preoperatively to 6/18 after 2 weeks. There was no postoperative rise in intraocular pressure or cataract formation. Intracameral injection of C(3)F(8) may possibly be a viable treatment option for acute hydrops in a case of corneal ectasia. Larger studies are required to clarify the role of intracameral gas injection in the treatment of acute hydrops in corneal ectasia.

Reduction in corneal transparence leading to the loss of sight may be caused by different factors, such as eye burns and injuries, keratitis, corneal ulcers, primary and secondary dystrophy, keratoconus, and keratoglobus. In many cases, keratoplasty presents an effective treatment. S. N. Fyodorov made a great contribution to the development of cornea replacement. This was him who placed the entire ophthalmology, including keratoplasty, at the microsurgical level, suggested the use of large-diameter grafts, and was the initiator of establishing the first Eye Tissue Bank. Perfection of microsurgical technique, selection and protection of the graft, as well as new approaches to pre- and postoperation therapy have provided transparent engrafting in 35 to 95%. Presently, Eye Microsurgery Complex is the only Russian institution where the problem of cornea replacement is being worked out as a scientific and practical issue. Three models of keratoprosthesis have been developed: Fyodorov-Zuyev model for implantation into a burn leukoma, Moroz-Glazko model for implantation into a dystrophic leukoma, and a "grid" for implantation into a leukoma of any etiology.

To evaluate the suggested role of the COL8A1 and COL8A2 genes in the pathogenesis of the corneal ectatic disorders keratoconus and keratoglobus through mutation screening in affected patients.DNA extraction, polymerase chain reaction amplification, and sequencing of COL8A1 and COL8A2 were performed in 50 unrelated keratoconus and 2 unrelated keratoglobus patients.No sequence variations were identified in COL8A1 and COL8A2 in the 2 patients with keratoglobus. Screening of COL8A1 in keratoconus patients revealed a previously identified single nucleotide polymorphism (SNP; c.1850C>T; Pro535Pro), in 1 patient. Screening of COL8A2 in keratoconus patients revealed 7 previously described SNPs: c.14G>A (Gly3Arg); c.112G>A (Ala35Ala); c.1012C>G (Leu335Leu); c.1308G>A (Arg434His); c.1492G>A (Gly495Gly); c.1512C>T (Thr502Met); and c.1765C>T (Pro586Pro). Four novel sequence variants were also identified, each in 1 affected patient: c.38_40dupCTG (Leu11dup), also identified in an unaffected relative of the affected proband, c.667G>A (Gly220Gly), c.1588G>A (Pro527Pro), and c.2026C>T (Val673Val). None of the 3 novel synonymous substitutions identified in COL8A2 was predicted to produce a splice acceptor site.The absence of pathogenic mutations in COL8A1 and COL8A2 in patients with keratoconus indicates that other genetic factors are involved in the pathogenesis of this corneal ectatic disorder.

The authors describe a patient with keratoglobus and a history of vernal keratoconjunctivitis and atopic dermatitis who acutely developed corneal hydrops and severe microbial keratitis. The infectious keratitis responded poorly to medical management and resulted in enucleation of the eye.A 25-year-old man presented with an acutely painful, red left eye. He had an ocular history of keratoglobus in association with vernal keratoconjunctivitis and atopic dermatitis. His visual acuity was light perception in the left eye and 20/40 in the right eye. Ocular examination showed a grossly edematous cornea with breaks in Descemet's membrane, a central infiltrative ulcer, and hypopyon.Corneal scrapings showed gram-positive beta-hemolytic streptococci, for which topical treatment of ceftazidime and benzylpenicillin every 30 minutes by day and night was commenced with an oral course of ciprofloxacin 750 mg twice a day. Despite aggressive therapy, the microbial keratitis progressed, and the hypopyon increased in size. The patient subsequently underwent enucleation of his left eye.This is the first reported case of microbial keratitis with corneal hydrops in a patient with keratoglobus, vernal keratoconjunctivitis, and atopic dermatitis. His ocular and dermatologic comorbidity may have impaired corneal integrity and allowed penetration of organisms, resulting in a severe case of keratitis that responded poorly to medical management.

To determine the visual and anatomic outcomes of epikeratoplasty in keratoglobus.In an interventional case series, 7 eyes of 6 patients with keratoglobus (KGB), 5 to 39 years of age, underwent epikeratoplasty. Three patients (3 eyes) had blue sclera, joint hypermobility, and consanguineous parents; 3 other patients (4 eyes) had only KGB. In the blue sclera group, all patients had lost the fellow eye because of minor trauma. We used a corneo-scleral button 1 mm larger than corneal diameter as an onlay graft. Orbscan pachymetry was performed before and 3 months after surgery.Mean visual acuity was 20/400 preoperatively and improved to 20/160 postoperatively. Mean central corneal thickness was 200 microm preoperatively, which increased to 800 microm after surgery. Epithelial inclusion cysts developed in 1 patient. Severe interface vascularization was observed in 1 eye of the blue sclera group, which had total Descemet membrane detachment before epikeratoplasty. Neurotrophic ulcer occurred in 1 case, which improved with tarsorrhaphy, punctal occlusion, and medical therapy.Epikeratoplasty is a safe and effective procedure in preserving ocular integrity and increasing visual acuity in patients with keratoglobus and should be considered before corneal perforation, which often results in loss of an eye.

To determine pro-inflammatory cytokine secretion from human corneas with different pathology and to establish whether cytokine profile influences corneal graft outcome.Secretion of both proinflammatory cytokine interleukin (IL)-1alpha and tumor necrosis factor (TNF)-alpha was measured after cultivation of 47 corneas collected from corneal graft recipients suffering from different corneal diseases. Non-inflammatory corneal diseases were keratoconus (n=8), keratoglobus (n=2), bullous keratopathy (n=11), and Groenouw stromal dystrophy type II (n=2), whereas inflammatory included vascularized corneal scar (n=14), rejected graft (n=6), and corneal ulcer (n=4). Corneas were cultivated at 37 degrees C for 24 hours and frozen until cytokine detection was measured by immunoassay. Donor corneas unsuitable for transplantation were used as controls (n=7). Corneal graft recipients were followed at least 18 months and rejection rate was calculated for each group.The median concentration of IL-1alpha secreted from corneas of recipients with non-inflammatory diseases was 2.47 pg/mm(3) (range, 0.13-9.95). In inflammatory corneal diseases, IL-1alpha concentration was significantly higher (median, 5.92 pg/mm(3); range, 0.48-12.68; P=0.005). IL-1alpha production in controls (median, 0.63 pg/mm3; range, 0.36-1.29 pg/mm(3)) was significantly lower than in inflammatory corneal diseases (P<0.001) and non-inflammatory diseases (P=0.008). Low level of TNF-alpha was detected only in 5 cases of vascularized corneal scars, 3 cases of bullous keratopathy, and 3 cases of graft rejection. Rejection rate was significantly higher in inflammatory than in non-inflammatory group (46% vs <10%, respectively, P=0.008). IL-1alpha and TNF-alpha were absent from all patient's sera, confirming its local intra-ocular production.Increased production of IL-1alpha in corneal recipients with inflammatory diseases suggests its role in corneal graft rejection in humans.