Angela C. Chen

Purpose: To report a case of delayed onset infectious crystalline keratopathy after corneal cross-linking (CXL). Methods: Slit-lamp examination, confocal microscopy, anterior segment optical coherence tomography imaging, and corneal culture were performed to characterize the corneal opacification. Results: A 49-year-old man presented with progressively worsening vision in the left eye 8 months after uncomplicated epithelium-off CXL for keratoconus. Examination demonstrated focal central corneal stromal thinning with surrounding stromal opacification. Confocal microscopy did not demonstrate evidence of parasitic or fungal infection. Two months later, gray-white crystalline stromal opacities were observed surrounding the area of central stromal thinning, consistent with infectious crystalline keratopathy. Corneal scrapings of the left eye were obtained, which were positive for Enterococcus faecalis. After cessation of topical loteprednol and initiation of topical vancomycin and linezolid, the stromal infiltrate increased in size and a hypopyon developed but had resolved before the discontinuation of 7 weeks of topical antibiotic therapy. A deep anterior lamellar keratoplasty was performed 6 weeks later, with no recurrence of infection 10 months after surgery. Conclusions: Infectious crystalline keratopathy can present more than 6 months after CXL. Progressive corneal stromal thinning associated with stromal opacification should raise suspicion of this rare late onset complication of CXL and prompt corneal scraping for stains and cultures to rule out an indolent infectious process.

Concomitant corneal ectasia and posterior lamellar corneal opacification is rare, and the genetic relationship between these two conditions is unclear. We report the genetic and clinical characteri...

We describe successful management of three cases of acquired monocular elevation deficiency (MED) with superior transposition of the lateral rectus augmented with a posterior fixation suture with or without simultaneous inferior rectus muscle weakening. In each case, the lateral rectus muscle was transposed superiorly to the superior rectus muscle along the spiral of Tillaux, with maintained distance between the original lateral rectus muscle poles and the limbus. Augmentation was achieved with a posterior fixation suture 8 mm posterior to the muscles’ insertion. At the time of lateral rectus transposition, simultaneous inferior rectus recession by 5.5 mm was performed in case 1 whereas simultaneous botulinum toxin injection was performed in case 3. With regards to all three cases, the mean age was 32 years [10–46 years] and the mean follow-up period was 10 months. The mean hypotropia was reduced from 35 prism diopters (PD) (range: 20 to 60 PD) to 4.67 PD (range: 0 to 14 PD) with a mean correction of 32.57 ± 9.34 PD after 9 months. In our experience, full-tendon-width transposition of the lateral rectus to the superior rectus with posterior fixation suture corrects primary position hypotropia in MED and does not always require simultaneous inferior rectus recession. When transposing the lateral rectus muscle along the spiral of Tillaux, the measured distance of the original muscle insertion point to the limbus must be maintained in order to prevent recession of the muscle.

Purpose: To compare uncorrected distance visual acuities (UDVAs) and induced higher-order aberrations (HOAs) in the early postoperative period between low-energy (LE) small-incision lenticule extraction (SMILE), high-energy (HE) SMILE, and femtosecond laser–assisted laser in situ keratomileusis (FS-LASIK) procedures. Setting: University based refractive surgery center. Study design: Retrospective cohort study. Methods: Records of patients who underwent SMILE or FS-LASIK were retrospectively reviewed. SMILE patients were separated into 2 groups: HE settings (125 nJ, 3.0 μm spot spacing) and LE settings (125-130 nJ, 4.5 μm spot spacing). UDVA was measured at postoperative day (POD) 1. Corneal HOAs and UDVA were measured at postoperative month (POM) 1. Induced spherical aberration, vertical coma, horizontal coma, total coma, and total HOAs were calculated. Results: The study included 147 eyes of 106 patients, 49 in each group. For SMILE patients, the difference in mean UDVA at POD1 was highly statistically significant in favor of the LE group (−0.003 vs 0.141, P < .0001). No significant difference in mean UDVA at POD1 was noted between the LE group and FS-LASIK group (−0.003 vs −0.011, P = .498). Induced change in spherical aberration was less in LE SMILE than that in FS-LASIK (0.136 vs 0.186 μm, P = .02) at POM1. No significant differences in POM1 mean UDVA (−0.033 vs −0.036) or induced change in all other HOAs were noted between LE SMILE and FS-LASIK. Conclusions: LE settings were associated with significantly improved POD1 UDVA. POD1 and POM1 UDVA were comparable with those of FS-LASIK. Spherical aberration induction was less with LE SMILE than that with FS-LASIK, whereas all other induced HOAs were comparable with FS-LASIK.

Purpose: To elucidate the molecular events in solute carrier family 4 member 11 (SLC4A11)-deficient corneal endothelium that lead to the endothelial dysfunction that characterizes the dystrophies associated with SLC4A11 mutations, congenital hereditary endothelial dystrophy (CHED) and Fuchs endothelial corneal dystrophy 4. Methods: Comparative transcriptomic analysis (CTA) was performed in primary human corneal endothelial cells (pHCEnC) and murine corneal endothelial cells (MCEnC) with normal and reduced levels of SLC4A11 (SLC4A11 KD pHCEnC) and Slc4a11 (Slc4a11^−/− MCEnC), respectively. Validation of differentially expressed genes was performed using immunofluorescence staining of CHED corneal endothelium, as well as western blot and quantitative PCR analysis of SLC4A11 KD pHCEnC and Slc4a11^−/− MCEnC. Functional analyses were performed to investigate potential functional changes associated with the observed transcriptomic alterations. Results: CTA revealed inhibition of cell metabolism and ion transport function as well as mitochondrial dysfunction, leading to reduced adenosine triphosphate (ATP) production, in SLC4A11 KD pHCEnC and Slc4a11^−/− MCEnC. Co-localization of SNARE protein STX17 with mitochondria marker COX4 was observed in CHED corneal endothelium, as was activation of AMPK–p53/ULK1 in both SLC4A11 KD pHCEnC and Slc4a11^−/− MCEnC, providing additional evidence of mitochondrial dysfunction and mitophagy. Reduced Na⁺-dependent HCO₃⁻ transport activity and altered NH₄Cl-induced membrane potential changes were observed in Slc4a11^−/− MCEnC. Conclusions: Reduced steady-state ATP levels and subsequent activation of the AMPK–p53 pathway provide a link between the metabolic functional deficit and transcriptome alterations, as well as evidence of insufficient ATP to maintain the Na⁺/K⁺-ATPase corneal endothelial pump as the cause of the edema that characterizes SLC4A11-associated corneal endothelial dystrophies.

Purpose: The purpose of this study was to evaluate the efficacy of intraocular pressure (IOP) control after combined CyPass Micro-Stent implantation and cataract surgery (combined surgery). Methods: All cases of combined surgery performed from February 2017 to July 2018 at Stein and Doheny Eye Institutes were reviewed. The primary outcome was a qualified success with stratified IOP targets based on criteria: final IOP (A) <= 18 mm Hg and reduction of 20%, (B) <= 15 mm Hg and reduction of 25%, (C) <= 12 mm Hg and reduction of 30%. Secondary outcome measures included postoperative IOP and number of medications, complications, additional glaucoma surgery, and postoperative refractive error. Predictive factors for failure were investigated. IOP spike was defined as a postoperative IOP >= 30 or >10 mm Hg increase from preoperative IOP. Results: One hundred forty-one eyes (107 patients) were included. Mean (+/- SD) preoperative IOP was 15.4 +/- 3.4 mm Hg on an average of 2.2 +/- 1.1 medications. A statistically significant reduction in IOP and number of medications was seen at 12 months (13.8 +/- 4.2 mm Hg, 1.3 +/- 1.3 medications,P<0.001). Fifteen eyes (10.6%) had a postoperative IOP spike. Thirteen eyes (9.2%) experienced 17 complications (12.1%). Additional glaucoma surgery was performed in 3 eyes (2 patients). Cumulative success rates at 1 year based on criteria A, B, and C were 42%, 33%, and 28%, respectively. Lower preoperative IOP, greater preoperative medication number, the occurrence of postoperative IOP spike, and non-Caucasian ethnicity were associated with failure. Conclusion: Combined CyPass Micro-Stent implantation and cataract surgery may reduce glaucoma medication burden with a success rate of 28% to 42% at 1 year.

Objectives: The literature suggests that after endoscopic endonasal transphenoidal surgery (EETS) without formal reconstruction of the sella, sinonasal quality of life (QOL) recovers by 3 to 6 months postoperatively. An option for sellar reconstruction, in the absence of a significant intraoperative cerebrospinal fluid (CSF) leak, is a free mucosal graft (FMG) taken from the posterior septum. In this study we analyze sinonasal quality of life (QOL) outcomes in patients undergoing EETS with FMG reconstruction. Design: Present study is a retrospective review. Setting: This study was conducted at a tertiary care academic center. Participants: Patients undergoing sellar reconstruction with a FMG after EETS from 2013 to 2018. Main Outcome Measures: Patient, tumor, and surgical factors were included, along with postoperative complications. Patients completed sinonasal outcome test 22 (SNOT-22) scores during the preoperative visit, as well as first two postoperative visits. SNOT-22 scores were further categorized into five domains, as previously described in the literature. Results: A total of 115 patients underwent sellar reconstruction with a FMG during EETS. Most surgeries were performed for pituitary adenomas (82%). Among the patients, 104 (90%) had no intraoperative CSF leak, while 11 patients (10%) had a low-grade intraoperative CSF leak. There was one patient (<1%) who developed a postoperative CSF leak. Three patients (3%) developed epistaxis, requiring an emergency room visit or operative intervention. The median time from surgery until the first postoperative visit was 13 days (95% CI: 6–22). The median time from surgery until the second postoperative visit was 57 days (95% CI: 15–126). The average preoperative SNOT-22 score was 17.5; first postoperative SNOT-22 score was 32.9; and second postoperative SNOT-22 score was 21.3 ([Fig. 1]), suggesting near normalization of sinonasal QOL by the second postoperative visit. After categorizing by SNOT-22 domains, there were significant improvements in SNOT-22 scores for almost all domains, including domain 1 (rhinologic symptoms), domain 2 (extra-nasal rhinologic symptoms), domain 3 (ear/facial Symptoms), and domain 4 (sleep dysfunction) symptoms ([Fig. 2]). Conclusion: During EETS, FMG reconstruction of sellar defects is an effective and well-tolerated option for patients without significant intraoperative CSF leaks. Patients demonstrated early recovery of baseline sinonasal QOL by 2 months postoperatively.

Acquired strabismus fixus is a rare form of severe esotropia described most frequently in patients with high axial myopia and infrequently associated with other conditions. Refractive errors, cataracts, and ocular motility disorders are common in patients with trisomy 21. We report two unusual cases of patients with trisomy 21 who developed severe acquired restrictive strabismus fixus. Both patients were successfully treated with medial rectus recession followed by lateral rectus and superior rectus myopexy combined with additional medial rectus weakening, including free tenotomy in one case.