PurposeTo report outcomes of phakic intraocular lens (IOL) implantation after deep anterior lamellar keratoplasty (DALK) to correct high ametropia.SettingCentro Hospitalar Universitário do Porto, Portugal.MethodsRetrospective case series with 11 eyes submitted to phakic IOL implantation after DALK. Main outcomes measured were uncorrected and corrected distance visual acuity (UDVA and CDVA), refractive error components, tomographic parameters and endothelial cell density (ECD). The minimum follow-up was 3 years for all cases.ResultsMean ECD loss was 8.7±6.7% at 1 year, 13.1±8.6% at 3 years (n=11; p=0.016, p=0.007, respectively) and 14.0±20.4% at 5 years (n=5, p=0.212). Mean logMAR UDVA increased from 1.27±0.90logMAR preoperatively to 0.16±0.15logMAR postoperatively (p≤0.001) and no statistically significant differences were registered during follow-up. All patients gained at least 5 lines of UDVA. 54.5% of the eyes gained 1 line in CDVA postoperative and only one eye lost one CDVA line through follow-up. Efficacy and safety indexes at 1 and 3 years were 1.01–0.97 and 1.24–1.21, respectively. Mean spherical equivalent was reduced from −7.84±4.63 D preoperatively to −1.05±1.07 D postoperatively (p=0.001). Mean percentage of reduction in refractive cylinder and spherical error was 83.8±15.8% and 73.1±31.5%, respectively, p≤0.001 for both. In one eye there was a significantly gradual ECD loss over 5 years follow-up and the patient will be submitted to IOL explant.ConclusionPhakic IOLs were effective for correction high ametropia after DALK, showing high efficacy and safety indexes with stability over time. However, it was registered a continuing endothelial cell loss postoperatively, which assumed to be higher than those reported in eyes without DALK.

PurposeTo analyze the long-term outcomes of intracorneal ring segments (ICRS) implantation for keratoconus management in pediatric patients.MethodsRetrospective case series review of the long-term (>5 years) outcomes of Intacs® ICRS implantation for keratoconus in pediatric patients (age <18 years old at the time of surgery) between January 2008 and December 2011 at Ophthalmology Department of Hospital de Santo António. Demographic data, follow-up time, preoperative and postoperative uncorrected visual acuity (UCVA) and best-corrected visual acuity (BCVA) in decimal scale, and corneal topography were evaluated. Statistical analysis was done using SPSS for windows (version 24). Significance was set at p<0.0125.ResultsFourteen eyes of 14 patients, with a mean age of 15.36 years (range 10–18 years), were included in this study. All patients had been diagnosed with keratoconus with reported progression in the 6 months prior to surgery. Follow-up time was 6.36±0.97 years. UCVA and BCVA improved after ICRS implantation (p<0.0125). Keratometry (K) minimum (Kmin) and K maximum (Kmax) decreased after surgery (p<0.0125). During follow-up, UCVA, BCVA, Kmin, and Kmax values ranged, showing a tendency to worsen at the end of follow-up. However, statistically significant differences were not observed.Conclusion: ICRS implantation showed good visual and topographic results in pediatric patients. Long-term follow-up suggests that, despite ICRS implantation, there is still progression of keratoconus. To the best of our knowledge, there are no reports regarding the long-term efficacy of ICRS implantation in pediatric patients.

Purpose To describe the anatomical and functional results of the implantation of asymmetric thickness intracorneal ring segments (AS-ICRS) in eyes with keratoconus and asymmetric/irregular astigmatism (type 2 – Duck – and type 3 – Snowman – phenotypes, Fernandez-Vega/Alfonso morphologic Keratoconus classification). Materials and Methods Retrospective observational study including 19 consecutive patients (21 eyes) with keratoconus who underwent manual implantation of the Keraring ® Asymmetric ICRS (AS). Analysis included demographic and clinical data and Pentacam (Oculus ® ) measurements: topographic astigmatism; topographic flat meridian (K1), steepest meridian (K2) and the maximum keratometric point (Kmax); total root mean square (RMS) and high order RMS (HOA) aberrations and comatic Zernike coefficients (Z3 1 ;Z3 −1 ) at 0º and 90º meridians. Results Overall mean age was 35.3±11.7 years and median follow-up was 7.1 months (range 6–12). At the end of follow-up, a significant improvement from baseline was achieved in both UDVA (0.24±0.22; p=0.017) and CDVA (0.21±0.17; p<0.001). Regarding topographic measurements, the greatest decrease was observed in K2 (2.76±1.9D; p<0.001) and astigmatism (1.97±1.5; p<0.001). Aberrometry analysis showed a reduction of 1.27±3.2µm in the total RMS (p=0.085), 0.24±0.9µm in HOA (p=0.227) and 0.78±0.5 (p<0.001) in the 90º component of comatic aberration. The procedure effects in the CDVA, topographic and aberrometric parameters were higher in type 2 cones without statistical significance (p>0.05 in all). Conclusion Implantation of the Keraring ® Asymmetric ICRS in keratoconus with asymmetric/irregular astigmatism allowed an improvement of several clinical, topographic and aberrometric parameters, with clinical efficacy and safety, with a tendency to a greater effect in the type 2 cones.

Fifty children with Ewing's sarcoma were consecutively treated from 1962 to 1987 and retrospectively analyzed at the I.P.O.F.G. of Lisbon. At first diagnosis, 10 cases had distant metastases. The remaining 40 patients had clinically localized disease, and different protocols were followed over the years. The best results were obtained with chemotherapy and radiotherapy with or without surgery; and for these children the two-year survival rate was 42.8% vs. 8.3% for the group of patients submitted to local treatment alone. Besides the treatment modality, other factors influenced the prognosis, such as inflammatory signs, sex, tumor volume, and tumor site as well as evidence of distant metastases.

Lattice corneal dystrophy gelsolin type was first described in 1969 by Jouko Meretoja, a Finnish ophthalmologist. It is caused by an autosomal dominant mutation in gelsolin gene resulting in unstable protein fragments and amyloid deposition in various organs. The age of onset is usually after the third decade of life and typical diagnostic triad includes progressive bilateral facial paralysis, loose skin, and lattice corneal dystrophy. We report a case of a 53-year-old female patient referred to our Department of Ophthalmology by severe dry eye and incomplete eyelid closure. She had severe bilateral facial paresis, significant orbicularis, and perioral sagging as well as hypoesthesia of extremities and was diagnosed with Meretoja's syndrome at the age of 50, confirmed by the presence of gelsolin mutation. At our observation she had bilateral diminished tear film break-up time and Schirmer test, diffuse keratitis, corneal opacification, and neovascularization in the left eye. She was treated with preservative-free lubricants and topical cyclosporine, associated with nocturnal complete occlusion of both eyes, and underwent placement of lacrimal punctal plugs. Ocular symptoms are the first to appear and our role as ophthalmologists is essential for the diagnosis, treatment, and monitoring of ocular alterations in these patients.