Ultra-wide field imaging to assess the optic nerve and retina in Boston type I and II keratoprosthesis patients.

BACKGROUND: The ability to view the posterior segment in keratoprosthesis (Kpro) implanted patients is limited. The purpose of this retrospective, observational study was to investigate the use of ultra-wide field (UWF) scanning laser ophthalmoscopy imaging and its utility for serial evaluation of the retina and optic nerve in patients with either a Boston type I or II Kpro. METHODS: A retrospective chart review was performed for patients with a Boston type I or II Kpro seen at The Ohio State University Wexner Medical Center. Images were graded for quality by two masked observers on a defined four-point scale ("Poor", "Fair", "Good", or "Very good") and assessed for visible posterior segment anatomy. Interobserver agreement was described using the Kappa statistic coefficient (κ) with 95% confidence intervals. RESULTS: A total of 19 eyes from 17 patients were included in this study. Eighteen eyes had a type I Kpro, while one eye had a type II Kpro. UWF imaging from 41 patient visits were reviewed by two observers. Interobserver agreement between the two graders was fair for image quality (κ = 0.36), moderate for visibility of the macula with discernible details (κ = 0.59), moderate for visibility of the anterior retina with discernable details (κ = 0.60), and perfect agreement for visibility of the optic nerve with discernible details (κ = 1.0). In 6 eyes, UWF imaging was performed longitudinally (range 3-9 individual visits), allowing for long-term follow-up (range 3-46 months) of posterior segment clinical pathology. CONCLUSIONS: UWF imaging provides adequate and reliable visualization of the posterior segment in Kpro implanted patients. This imaging modality allowed for noninvasive longitudinal monitoring of retinal and optic nerve disease in this selected patient population.

Conjunctival Flaps: A Case Series and Review of the Literature.

PURPOSE: The purpose of this study was to examine the outcomes of conjunctival flaps performed at our institution. METHODS: The medical records of all patients who underwent a total or partial conjunctival flap surgery over the course of a 6-year period from 2010 to 2015 were reviewed to identify the ocular diagnoses and perform a retrospective review of the preoperative and postoperative course. RESULTS: Thirty-five patients received a conjunctival flap during the 6-year period, 20 partial flaps and 15 total flaps. Infectious keratitis or corneal ulceration accounted for 25 (71.4%) of the cases. The remaining flaps were performed for corneal perforation, graft rejection, bullous keratopathy, and secondary corneal edema. Seven patients had recession of their flap postoperatively, and 14 went on to have further surgical intervention. Conjunctival flap surgery successfully stabilized the patient's ocular surface in 97.1% of the cases. CONCLUSION: Conjunctival flap surgery is an important and useful surgical option in the treatment of ocular surface disease, especially recalcitrant infectious keratitis and corneal ulcers.

Keratoglobus with ARCL1B (EFEMP2 gene) cutis laxa.

PURPOSE: To report a case of keratoglobus in a patient with autosomal recessive (AR) cutis laxa. OBSERVATIONS: A 38 year old male presented with decreased vision in both eyes uncorrectable with spectacles and a history of corneal rupture in the left eye from incidental trauma a decade prior. His ocular exam was consistent with keratoglobus. His medical and family history indicated AR cutis laxa. CONCLUSIONS AND IMPORTANCE: We believe that this is the first reported case of keratoglobus associated with cutis laxa.

Cost effect of surgeon and patient discretion in regard to cataract surgery.

PURPOSE: The purpose of this study is to examine the cost effect of surgeon and patient discretion in regard to cataract surgery and how this affects population health care costs. METHODS: A model of cataract progression was created from preexisting published data and combined with mortality data and Medicare cataract statistics to estimate the effect of mortality on decreasing the rate of cataract surgery if surgery was delayed until any cataract progression occurred. RESULTS: Five-year cataract progression rates were determined for a given patient age, sex, and type of cataract. Combined with 5-year death rates, delaying surgery until progression occurred resulted in a 1.1% decrease in surgery for nuclear sclerosis at age 45 that increased to a 33.8% decrease by age 90; a 1.5% decrease in surgery for cortical cataract at age 45 that increased to a 51.1% decrease by age 90; and a 1.6% decrease in surgery for posterior subcapsular at age 45 that increased to a 59.7% decrease by age 90. The effect of this decrease in surgical volume on Medicare was estimated to result in a 13% overall decrease in cataract surgery annually at a cost of ~$660 million dollars per year. CONCLUSION: Overall, we conclude that surgeon and patient discretion in regard to cataract surgery has a substantial cost effect with the potential to reduce surgical volume by as much as 13% by the decision to delay surgery as a result of patient mortality.

Candida dubliniensis: A novel cause of fungal keratitis.

A 45-year-old female with history of contact lens wear presented with a persistent corneal ulcer that was unresponsive to topical moxifloxacin. The patient's exam was concerning for fungal keratitis. Cultures were obtained, and the patient was started on fortified amphotericin B drops and oral voriconazole. The cultures identified Candida dubliniensis as the causative organism. The patient's exam worsened despite treatment, and the decision was made for surgery. At the time of surgery, her cornea was found to have unexpectedly perforated. She underwent cryotherapy; tectonic penetrating keratoplasty; anterior chamber tap; intracameral voriconazole, amphotericin B, and cefuroxime; and a partial conjunctival flap. Pathology from the cornea showed GMS and PAS stains positive for fungal forms. C. dubliniensis is a yeast closely related to Candida albicans that was first described in 1995 as a cause of oral candidiasis in patients with AIDS. There are a few published cases of endophthalmitis due to C. dubliniensis in the ophthalmology literature, but to our knowledge, no cases of fungal keratitis due to this organism have been reported. C. dubliniensis is a novel cause of fungal keratitis that can be difficult to identify and treat but is felt to be less virulent than C. albicans and generally susceptible to available anti-fungal therapies.

Expression pattern of id proteins in medulloblastoma.

Inhibitor of DNA binding or inhibitor of differentiation (Id) proteins are up regulated in a variety of neoplasms, particularly in association with high-grade, poorly differentiated tumors, while differentiated tissues show little or no Id expression. The four Id genes are members of the helix-loop-helix (HLH) family of transcription factors and act as negative regulators of transcription by binding to and sequestering HLH complexes. We tested the hypothesis that Id proteins are overexpressed in medulloblastoma by performing immunohistochemistry using a medulloblastoma tissue microarray with 45 unique medulloblastoma and 11 normal control cerebella, and antibodies specific for Id1, Id2, Id3, and Id4. A semi-quantitative staining score that took staining intensity and the proportion of immunoreactive cells into account was used. Id1 was not detected in normal cerebella or in medulloblastoma cells, but 78 % of tumors showed strong Id1 expression in endothelial nuclei of tumor vessels. Id2 expression was scant in normal cerebella and increased in medulloblastoma (median staining score: 4). Id3 expression was noted in some neurons of the developing cerebellar cortex, but it was markedly up regulated in medulloblastoma (median staining score: 12) and in tumor endothelial cells. Id4 was not expressed in normal cerebella or in tumor cells. Id2 or Id3 overexpression drove proliferation in medulloblastoma cell lines by altering the expression of critical cell cycle regulatory proteins in favor of cell proliferation. This study shows that Id1 expression in endothelial cells may contribute to angiogenic processes and that increased expression of Id2 and Id3 in medulloblastoma is potentially involved in tumor cell proliferation and survival.