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.

Pronounced and extensive microtubule defects in a Saccharomyces cerevisiae DIS3 mutant.

Subunits of the RNA processing exosome assemble into structurally distinct protein complexes that function in disparate cellular compartments and RNA metabolic pathways. Here, in a genetic, cell biological and transcriptomic analysis, we examined the role of Dis3, an essential polypeptide with endo- and 3'→5' exo-ribonuclease activity, in cell cycle progression. We present several lines of evidence that perturbation of DIS3 affects microtubule (MT) localization and structure in Saccharomyces cerevisiae. Cells with a DIS3 mutant: (a) accumulate anaphase and pre-anaphase mitotic spindles; (b) exhibit spindles that are misorientated and displaced from the bud neck; (c) harbour elongated spindle-associated astral MTs; (d) have an increased G1 astral MT length and number; and (e) are hypersensitive to MT poisons. Mutations in the core exosome genes RRP4 and MTR3 and the exosome cofactor gene MTR4, but not other exosome subunit gene mutants, also elicit MT phenotypes. RNA deep sequencing analysis (RNA-seq) shows broad changes in the levels of cell cycle- and MT-related transcripts in mutant strains. Collectively, the data presented in this study suggest an evolutionarily conserved role for Dis3 in linking RNA metabolism, MTs and cell cycle progression.

Duane syndrome in the setting of chromosomal duplications.

PURPOSE: To describe the clinical findings in 3 patients with Duane syndrome and 3 different chromosomal duplications that may indicate the location of genes involved in the pathogenesis of this ocular motility disorder. DESIGN: Observational case series. METHODS: setting: Clinical practice. patient or study population: Three patients with Duane syndrome and chromosomal duplications from the clinical practice of 1 of the authors. observation procedures: Chart review and retrieval of clinical data and results of pertinent clinical tests, in this case chromosomal studies. main outcome measure: Reporting of details of clinical findings and duplicated chromosomal regions. RESULTS: Two patients had unilateral type I Duane syndrome and 1 had bilateral type I Duane syndrome. Two had cognitive delay, and all 3 had other systemic abnormalities, including a variety of congenital malformations. The chromosomal abnormalities that were detected using microarray analysis were 2q13(RP11-20G1,RP11-461N11) × 3, 10q24.2q26.3(101,532,585-135,284, 169) × 3, 20q13.12(44,796,613-44,945, 818) × 3, and 22q11.1q11.22(RP11-701M12, RP11-71G19) × 3. CONCLUSIONS: Patients with Duane syndrome and associated congenital malformations or developmental delay should be evaluated for the presence of underlying chromosomal duplications. The regions of chromosomes 2, 10, and 22 that we report may harbor genes involved in the pathogenesis of Duane syndrome.

Risk factors for ophthalmic complications in patients with burns.

A retrospective study of patients admitted to MetroHealth Medical Center was performed to identify the risk factors for short- and long-term ophthalmologic complications related to burn injury. From 2000 to 2007, the authors identified 293 patients with the inclusion criteria of facial burns, TBSA ≥20%, or smoke inhalation injury. Seventy (24%) developed ocular complications, and 16 (11%) developed long-term complications. Statistically significant risk factors identified for short-term complications were burn size, chemical burns, depth of facial burns, initial Glasgow Coma Scale, and need for mechanical ventilation/sedation. Risk factors for long-term complications included wound infection with Pseudomonas or Acinetobacter, third-degree burn size, hours to ophthalmology evaluation, LOS, time on mechanical ventilation, and need for STSG. In addition to facial burns, the requirement of mechanical ventilation, prolonged sedation, and presence of infection with Pseudomonas or Acinetobacter increase the risk of injury to the eye after burn injury, and these patients may benefit from serial eye examinations for early identification of ocular complications.