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PurposeTo report logarithm of the minimal angle of resolution (logMAR) visual outcomes of the Boston keratoprosthesis type 1.DesignProspective cohort study.MethodsPreoperative, intraoperative, and postoperative parameters of 300 eyes of 300 patients who underwent implantation of a Boston keratoprosthesis type 1 device between January 2003 and July 2008 by 1 of 19 surgeons at 18 medical centers were collected.ResultsAfter an average of 17.1 ± 14.8 months, visual acuity improved significantly (P < .0001) to a mean final value of 0.89 ± 0.64 (20/150). There were also significantly fewer eyes with light perception (6.7%; n = 19; P < .0001), although 3.1% (n = 9) progressed to no light perception. There was no association between age (P = .08), sex (P = .959), operative side (P = .167), or failure (P = .494) and final visual acuity. The median time to achieve 20/200 visual acuity was 1 month (95% confidence interval 1.0-6.0) and it was retained for an average of 47.8 months. Multivariate analysis, controlling for preoperative visual acuity, demonstrated 2 factors associated with final visual outcome: chemical injury was associated with better final vision (P = .007), whereas age-related macular degeneration was associated with poorer vision (P < .0001).ConclusionsThe Boston keratoprosthesis type 1 is an effective device for rehabilitation in advanced ocular surface disease, resulting in a significant improvement in visual acuity. Eyes achieved a mean value of 20/150 (0.89 ± 0.64 logMAR units) after 6 months and this was relatively stable thereafter. The best visual prognosis is observed in chemical injury eyes, whereas the worst prognosis is in aniridia, although the latter has limited visual potential.

Frequency of somatic and germ-line mosaicism has been studied in retinoblastoma and implications for genetic counseling have been considered. An evaluation has been made of 156 families with retinoblastoma. In these families the initial oncogenic mutations in the retinoblastoma gene had been found. In about 10% of the families mosaicism for the initial mutation in the retinoblastoma gene was documented in the proband or in one of the parents of the proband. Germ-line DNA from two mosaic fathers was analyzed. Data suggest that mosaicism is more common that generally believed especially in disorders like retinoblastoma in which a high proportion of cases come from new mutations. The possibility of mosaicism should be considered in genetic counseling of newly identified retinoblastoma families. Genetic tests of germ-line DNA can give valuable information not found in analysis of somatic, leukocyte, DNA.

Purpose: To evaluate continuous wear of a fluid-ventilated, gas-permeable scleral PROSE device using a standardized protocol as treatment for refractory persistent corneal epithelial defects in patients with severe ocular surface disease. Methods: Retrospective review of eight eyes of seven consecutive patients with persistent epithelial defects refractory to traditional therapies. The standardized treatment regimen consisted of: (1) 24-hour-a-day PROSE wear until re-epithelialization was achieved, (2) brief daily device removal, cleaning, disinfection, and reservoir fluid replacement, (3) addition of a benzalkonium chloride (BAK)-free fourth-generation fluoroquinolone antibiotic drop to the reservoir, and (4) transition to long-term, daytime PROSE wear upon re-epithelialization . Results: All eight eyes exhibited resolution of the persistent epithelial defect. No eyes developed microbial keratitis. Four eyes exhibited recurrences; all recurrences promptly responded to reinstitution of continuous wear. Conclusions: Continuous wear of a PROSE device, using a strictly standardized regimen, constitutes an effective, safe treatment option for refractory persistent epithelial defects. [ABSTRACT FROM AUTHOR]

Background: Stevens-Johnson syndrome is often associated with blinding ocular surface cicatricial sequelae. Recent reports have described markedly improved clinical outcomes with the application of amniotic membrane to the ocular surface during the acute phase. Here we describe the clinical outcome of a patient with acute Stevens-Johnson syndrome and severe ocular surface involvement in whom the evolving medical condition and family consent resulted in amniotic membrane application to each eye at differing intervals from disease onset. Methods: We undertook a retrospective chart review of a woman with Stevens-Johnson syndrome who presented within hours of disease onset. She underwent application of amniotic membrane to the ocular surface of the left eye during the hyperacute phase (<72 hours after disease onset) and to the right eye at a later time point during the acute phase (six days after disease onset). The clinical outcomes of the two eyes, as well as associated ocular symptoms, were compared over a one-year postoperative period. Results: The right eye, treated later in the course of the disease, required additional surgical procedures and ultimately exhibited significantly more advanced ocular surface pathology than the left. Further, the patient reported more pronounced issues of chronic eye pain and visual difficulties in the right eye. Conclusion: Earlier intervention with application of amniotic membrane to the ocular surface in this patient with severe ocular involvement secondary to Stevens-Johnson syndrome proved superior. Application of amniotic membrane as soon as possible after disease onset, preferably in the hyperacute phase, appears to result in a significantly better clinical outcome than application later in the disease course. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study was to assess whether the resolution offered by two different, recently commercially available high-resolution, spectral-domain anterior segment optical coherence tomography (AS-OCT) instruments allows for detailed anatomic characterization of the critical device-donor cornea interface in eyes implanted with the Boston type I permanent keratoprosthesis. Methods: Eighteen eyes of 17 patients implanted with the Boston type I keratoprosthesis were included in this retrospective case series. All eyes were quantitatively evaluated using the Cirrus HD-OCT while a subset (five eyes) was also qualitatively imaged using the Spectralis Anterior Segment Module. Images from these instruments were analyzed for evidence of epithelial migration onto the anterior surface of the keratoprosthesis front plate, and presence of a vertical gap between the posterior surface of the front plate and the underlying carrier donor corneal tissue. Quantitative data was obtained utilizing the caliper function on the Cirrus HD-OCT. Results: The mean duration between AS-OCT imaging and keratoprosthesis placement was 29 months. As assessed by the Cirrus HD-OCT, 83% of eyes exhibited epithelial migration over the edge of the front plate. Fifty-six percent of the keratoprosthesis devices displayed good apposition of the device with the carrier corneal donor tissue. When a vertical gap was present (44% of eyes), the mean gap was 40 (range 8-104) microns. The Spectralis Anterior Segment Module also displayed sufficient resolution to allow for similar characterization of the device-donor cornea interface. Conclusion: Spectral-domain AS-OCT permits high resolution imaging of the keratoprosthesis device-donor cornea interface. Both the Cirrus HD-OCT and the Spectralis Anterior Segment module allowed for visualization of epithelial coverage of the device-donor cornea interface, as well as identification of physical gaps. These imaging modalities, by yielding information in regard to integration of the keratoprosthesis with surrounding corneal tissue, may help identify those at risk for keratoprosthesis-related complications, such as extrusion and endophthalmitis, and hence guide clinical management. [ABSTRACT FROM AUTHOR]