Keratoconus tomographic indices in osteogenesis imperfecta.

PURPOSE: Osteogenesis imperfecta (OI) is a rare inherited disease affecting collagen-rich tissues. Ocular complications have been reported such as thin corneas, low ocular rigidity, keratoconus, among others. The purpose of this study is to characterize corneal tomographic features in OI patients compared to unaffected patients, with particular focus on commonly studied keratoconus indices. METHODS: Cross-sectional case-control study including 37 OI patients and 37 age-matched controls. Patients and controls underwent comprehensive ophthalmological examination including corneal Scheimpflug tomography with a Pentacam HR device (Oculus Optikgeräte GmbH, Wetzlar, Germany) to analyse and compare topometric, tomographic, pachymetric and Belin-Ambrósio Enhanced Ectasia Display III (BAD-D) data of both eyes of each patient. RESULTS: Most OI patients had type I disease (n = 24; 65%) but type III-VII patients were also included. Two patients had clinically overt bilateral keratoconus. OI patients had significantly higher maximum keratometry (45.2 ± 2.1 vs. 43.7 ± 1.2; p = 0.0416), front and back elevation (3.0 ± 3.3 vs. 2.1 ± 1.3, p = 0.0201; 11.1 ± 8.2 vs. 5.0 ± 3.7, p < 0.0001), index of surface variance (25.5 ± 13 vs. 17.4 ± 8.3; p = 0.0016), index of vertical asymmetry (0.21 ± 0.14 vs. 0.15 ± 0.06; p = 0.0215), index of height asymmetry (9.2 ± 14 vs. 6.0 ± 4.5; p = 0.0421), index of height decentration (0.02 ± 0.01 vs. 0.01 ± 0.01; p < 0.0001) and average pachymetric progression (1.01 ± 0.19 vs. 0.88 ± 0.14; p < 0.0001) readings. Thinnest corneal thickness and maximum Ambrósio relational thickness were significantly lower (477 ± 52 vs. 543 ± 26; 387 ± 95 vs. 509 ± 49; p < 0.0001). Two-thirds of OI patients had corneas with a minimum thickness < 500 µm. BAD-D value was significantly higher in OI patients (2.1 ± 1.4 vs. 0.9 ± 0.2; p < 0.0001). CONCLUSION: OI patients showed significant changes in corneal profiles compared with healthy subjects. A high proportion of patients had tomographically suspect corneas when using keratoconus diagnostic indices. Further studies are warranted to assess the true risk of corneal ectasia in OI patients.

Topical corticosteroids with topical cyclosporine A versus topical corticosteroids alone for immunological corneal graft rejection.

PURPOSE: To assess the efficacy and safety of supplementing topical cyclosporine A (CsA) to topical corticosteroids (CS), in the prophylaxis and treatment of corneal graft rejection following penetrating keratoplasty (PK). METHODS: Meta-analysis. Search was performed in PubMed, CENTRAL, ClinicalTrials.gov, reference lists of articles and conference proceedings. Primary outcomes: 1-year rejection-free survival rate (prophylaxis); resolution rate of rejection episodes (treatment). Secondary outcomes: 6- and 24-month rejection-free graft survival rate, number of rejection episodes during follow-up, time-to-resolution of rejection episode, 12- and 24-months graft survival rate, adverse events. Subgroup analyses were planned for high-risk grafts; primary vs. secondary prophylaxis of graft rejection episodes; and CsA concentrations of 0.05%, 1%, and 2%. RESULTS: Five studies of moderate methodological quality were included (one retrospective, four RCT), assessing 459 eyes (CS + CsA 226, CS 233). In the prophylaxis setting, supplemental CsA was associated with a higher rejection-free survival rate at 12-months (RR 1.25, 95% CI: 1.00-1.56, p = 0.05) and 24-months post-PK (RR 1.56, 95% CI: 1.15-2.11, p < 0.01), though no differences were found at the 6-months timepoint (p = 0.93). This effect was mostly verified using CsA 2% in the high-risk subset of patients. In the treatment setting, no differences were found in the resolution rate of rejection episodes (p = 0.23). No differences existed on drug-related adverse events. CONCLUSION: In the prophylaxis of rejection episodes post-PK, the combined regimen of CS + CsA was associated with a higher 1- and 2-year rejection-free graft survival rate. Subgroup analysis mostly supported the use of CsA 2% for high-risk grafts. Further studies are needed to validate these results.

Characterization of posterior corneal astigmatism in a population with keratoconus.

Background: The curvature of the anterior corneal surface is traditionally used as a surrogate to estimate corneal astigmatism. In recent years, increasing importance has been attributed to posterior corneal astigmatism as an indicator. Our aim is to characterize the posterior corneal surface in a population with keratoconus and investigate its predictive value to keratoconus progression. Methods: Retrospective study from a tertiary care centre (Hospital de Santa Maria, Lisbon, Portugal). Eighty-five patients (85 eyes) with keratoconus were included. All patients had two tomographical examinations ≥12 months apart (Pentacam HR). Vector analysis was used to calculate anterior (ACA), posterior (PCA), and total corneal astigmatism (TCA). Multivariate logistic regression was used to assess the predictive value of PCA to keratoconus progression, adjusting for ACA, TCA and several tomographical indices. Results: Study participants had a mean age of 32 (SD = 12.5) years. Mean tomographical keratoconus classification was 2.16 (SD = 0.95), with a mean Kmax of 55.8D (SD = 7.8). Mean power of PCA, ACA and TCA was, respectively, -0.88D (SD = 0.84), 3.74D (SD = 2.36), and 3.06D (SD = 2.01) and its centroids were 0.44D x 15º, 1.65D x 112º, and 1.61D x 106º, respectively. The power of PCA was ≥0.50, 1.00 and 2.00D in 75.3%, 32.9%, and 3.5% of patients, respectively, inducing against-the-rule astigmatism in 60.0% of patients. On average, ACA overestimated TCA in 0.35D x 151º (p < .01). ACA and TCA were highly correlated but showed a lack of agreement for clinical purposes. A predictive role for PCA was excluded. Conclusions: In this population with keratoconus, PCA contributed substantially to TCA. However, PCA was not a valuable predictor for disease progression.

DMEK versus DSAEK for Fuchs' endothelial dystrophy: A meta-analysis.

PURPOSE:: To compare the safety and efficacy profiles of Descemet membrane endothelial keratoplasty (DMEK) and Descemet stripping automated endothelial keratoplasty (DSAEK) in adult patients with Fuchs' endothelial dystrophy. METHODS:: Electronic database search on MEDLINE and CENTRAL from inception to August 2017. We included all comparative studies of DMEK versus DSAEK in patients with diagnosed Fuchs' endothelial dystrophy. Studies assessing rescue procedures were excluded to minimize bias. Primary outcome: mean difference in best-corrected visual acuity (BCVA) at 3, 6, and 12 months postoperatively. Secondary outcomes: rates of graft primary failure, rejection, and rebubbling; other graft-related issues; mean difference in endothelial cell density; subjective visual outcomes; and patient satisfaction. RESULTS:: A total of 10 retrospective studies of moderate methodological quality were included (n = 947 eyes, 646 DMEK). BCVA was better with DMEK at all evaluated time points (0.16 logMAR at 12 months) comparing to DSAEK (0.30 logMAR; p < 0.001). DMEK had a 60% lower rate of rejection (risk ratio (RR) 0.4, 95% CI (0.24, 0.67), p = 0.0005), but required more rebubblings (RR = 2.48, 95% CI (1.32, 4.64), p = 0.005). DMEK had more primary graft failures and less endothelial cell density loss, but statistical difference was not reached. More patients were satisfied after DMEK (odds ratio = 10.29, 95% CI (3.55, 29.80), p < 0.0001). CONCLUSION:: DMEK showed better postoperative results regarding BCVA, patient satisfaction, and graft-related issues. However, the small number of studies with short follow-up times and other methodological issues prompt us to interpret these results carefully.

Sulfur Hexafluoride 20% Versus Air 100% for Anterior Chamber Tamponade in DMEK: A Meta-Analysis.

PURPOSE: To compare intracameral 20% sulfur hexafluoride (SF6) versus 100% air as tamponade for graft attachment in Descemet membrane endothelial keratoplasty (DMEK). METHODS: Using an electronic database search on MEDLINE and CENTRAL from inception to December 2017, we performed a literature review and meta-analysis including all comparative studies of SF6 at a 20% concentration (20% SF6) versus pure air (100% air) for anterior chamber tamponade in DMEK. The primary outcome was the rebubbling rate at the final observation. The secondary outcomes were 1) the graft detachment rate, 2) mean difference (MD) in best-corrected visual acuity (BCVA), 3) manifest refraction spherical equivalent, 4) central corneal thickness (CCT), 5) percentage of endothelial cell loss (ECL), and 6) rate of pupillary block by the final observation. Statistical analysis was performed using RevMan5.3 software. RESULTS: Five retrospective studies were included, assessing 1195 eyes (SF6 277; air 918). The main indication for surgery was Fuchs endothelial dystrophy (SF6 85.2%; air 86.2%) and bullous keratopathy (SF6 10.8%; air 10.0%). Overall, studies were of moderate to good methodological quality. Patients in the SF6 group required 58% less rebubbling procedures (risk ratio 0.42, 95% confidence interval (CI), 0.31-0.56, P < 0.0001). No differences were found regarding BCVA improvement (MD 0.03, 95% CI, -0.05 to 0.11, P = 0.49). SF6 was associated with a minor hyperopic shift (MD 0.37 D, 95% CI, -0.95 to -0.21, P = 0.21). No differences were found regarding CCT, ECL, and rate of pupillary block (P > 0.05). CONCLUSIONS: In DMEK, 20% SF6 tamponade and longer postoperative time supine were associated with 58% fewer rebubbling procedures, and an ECL not statistically different from using 100% air.

IgA nephropathy in a patient presenting with scleritis.

Scleritis is a very uncommon manifestation in patients with IgA nephropathy. Here, we report the case of a patient presenting with diffuse anterior scleritis in which the laboratory disclosed microscopic haematuria and nephrotic range proteinuria. Renal function was normal. Serology for lupus, vasculitis and cryoglobulinaemia was negative. Rheumatoid factor was negative, and serum C3 and serum C4 were on the normal range. Serology for human immunodeficiency virus types 1 and 2, hepatitis B, hepatitis C, syphilis, and Lyme disease was also negative. A renal biopsy was performed and revealed IgA nephropathy. Oral steroids were then started, and 6 months later, the patient was asymptomatic. Scleritis did not recur, and ophthalmologic examination was normal; however, proteinuria was still in non-nephrotic range. Renal function still remains normal.