A mysterious myopic surprise.

A 73-year-old man with an ocular history of inactive age-related macular degeneration and chronic angle-closure glaucoma (CACG) in both eyes recently underwent femtosecond laser-assisted cataract surgery/phacoemulsification and intraocular lens (IOL) implantation with simultaneous Hydrus microstent (Ivantis, Inc.) implantation in the left eye. Although there was some reported subincisional iris prolapse due to intraoperative floppy iris, the case was otherwise uneventful according to the referring surgeon. Two months postoperatively, he was referred to our office for a myopic surprise of approximately 2.0 diopters (D) in the left eye (Figure 1JOURNAL/jcrs/04.03/02158034-202202000-00022/figure1/v/2022-01-26T192641Z/r/image-tiff). Of note, he has a distant history of acute ACG and complicated cataract surgery in the right eye with a failed trabeculectomy. He subsequently had laser peripheral iridoplasty to pull the iris away from the angle in the right eye (Figure 2JOURNAL/jcrs/04.03/02158034-202202000-00022/figure2/v/2022-01-26T192641Z/r/image-tiff). His topical intraocular pressure (IOP)-lowering medications at presentation included dorzolamide-timolol 1 drop twice daily in the left eye and 1 drop of timolol in the right eye once daily. His past medical history is significant for hypertension and benign prostatic hyperplasia, and his oral medications include Tamsulosin (Flomax), Irbasartan (Avapro), and Atenolol. On examination, he had an UCDVA of 20/20 in the right eye and 20/80 in the left eye, and a BCDVA of 20/20 in the right eye and 20/25 in the left eye. His manifest refraction was plano in the right eye and 1.50 -0.75 × 90 in the left eye. IOP measured 19 mm Hg in the right eye and 26 mm Hg in the left eye. Pupil examination revealed a nonreactive pupil in the right eye and a round sluggish pupil in the left eye without an obvious relative afferent pupillary defect. Extraocular motility and confrontational visual fields were full in both eyes. On slitlamp examination, pertinent findings included the following: 1+ corneal guttata without edema in both eyes; anterior chambers were shallow but adequate in both eyes with scattered peripheral anterior synechiae in the right eye and a uniformly shallow but adequate chamber in the left eye; there was no cell or flare in either eye. Iris findings included a surgical pupil with a fibrotic pupillary membrane, laser iridoplasty scars with scattered temporal transillumination defects (TIDs) in the right eye, and 2.5 clock hours of TIDs and a patent peripheral iridotomy at 1 o'clock in the left eye; lens examination revealed centered posterior chamber IOLs with open posterior capsules in both eyes and lens pitting in the left eye. Pertinent findings on dilated fundus examination included a cup-to-disc ratio of 0.3 in both eyes with good neuroretinal rims and macular examination revealed medium-sized drusen with pigment clumping in both eyes and no active choroidal neovascular membranes. The remainder of the examination was unremarkable. What is the etiology of this myopic surprise? What diagnostic testing will help confirm the diagnosis and what are the best management options for this patient?

Acute iris toxicity following bilateral gel stent implantation with mitomycin-C and intracameral moxifloxacin January consultation #1.

A 72-year-old woman with moderate primary open-angle glaucoma was referred for management of her glaucoma and photophobia. Her ocular history is significant for routine cataract surgery in both eyes 6 years prior to presentation. She was diagnosed with glaucoma 7 months prior to presentation at which time the patient underwent sequential, ab externo, open conjunctival, Xen Gel Stent (Allergan, Inc.) placement with mitomycin-C (MMC) in each eye, approximately 2 weeks apart. The history obtained directly from the surgeon revealed that MMC dosing was 0.2 mL in a concentration of 0.2 mg/mL delivered through subconjunctival injection after placement of the gel stent. Intracameral moxifloxacin was injected at the time of surgery, and moxifloxacin and Maxitrol eyedrops were used in the postoperative period. The patient noted that, approximately 1 month after each surgery, she developed significant photophobia. An outside examination noted bilateral tonic pupils and concern for early bleb failure in the left eye. 2 months after the initial gel stent placement in the left eye, she underwent a bleb revision with a McCannel suture iris cerclage in the left eye. The intraocular pressure (IOP) in both eyes remained well controlled off pressure-lowering medications; however, the tonic pupils and photophobia persisted. The patient was subsequently referred for further assessment. At presentation, the patient's corrected distance visual acuity was 20/20 in each eye. Applanation tonometry IOP was 17 mm Hg and 14 mm Hg for the right and left eyes, respectively. Pupils were tonic and irregular. The lack of pupillary response prevented testing for relative afferent pupillary defects directly or by reverse testing. Slitlamp examination of the right eye was notable for a minimally elevated superior bleb and severe iris stromal atrophy with diffuse transillumination defects. The intraocular lens (IOL) appeared well positioned in the capsular bag without signs of pseudophacodonesis (Figure 1JOURNAL/jcrs/04.03/02158034-202201000-00021/figure1/v/2021-12-20T152346Z/r/image-tiff). The left eye was notable for a diffuse superior bleb and similar iris and IOL findings to the right eye, except for 2 McCannel iris sutures in the iris stroma (Figure 2JOURNAL/jcrs/04.03/02158034-202201000-00021/figure2/v/2021-12-20T152346Z/r/image-tiff). In both eyes, the anterior chambers were deep and quiet, and there were no corneal endothelial abnormalities. Fundus examinations of both the right and left eyes were notable for glaucomatous-appearing optic nerves and otherwise healthy macula, vessels, and periphery. Gonioscopy revealed open angles in each eye with fairly marked, densely layered pigment throughout the inferior angle and trabecular meshwork. The gel stents were positioned just anteriorly to the trabecular meshwork superiorly, well away from iris tissue. Optical coherence tomography (OCT) of the nerve fiber layer (NFL) showed moderate to severe thinning inferiorly and superiorly in the right eye, with mild to moderate thinning inferiorly in the left eye (Supplemental Figure 1, http://links.lww.com/JRS/A510). Ganglion cell analysis correlated with the NFL findings (Supplemental Figure 2, http://links.lww.com/JRS/A510). Humphrey visual field testing revealed a dense superior arcuate in the right eye and moderate inferior arcuate in the left eye (Supplemental Figures 3 and 4, http://links.lww.com/JRS/A510). OCT of the macula in both eyes was unremarkable. Finally, specular microscopy showed normal endothelial density and configuration of each eye (Supplemental Figure 5, http://links.lww.com/JRS/A510). What part of the patient's ocular history would you consider relevant to the development of tonic, atrophic pupils? What additional information would you want to know to help refine your differential diagnosis? What is your preferred surgical technique when implanting the Xen Gel Stent? Please include whether ab interno or ab externo and open or closed conjunctival approach to be used and mention antifibrosis dosing and method of administration. Finally, what would be your approach for managing this patient's severe photophobia, in the setting of her moderate open-angle glaucoma?

Management of hypotony-related maculopathy after combined phacoemulsification and trabeculectomy: January consultation #1.

A 59-year-old man with mild to moderate pigmentary glaucoma was referred for management of hypotony-related maculopathy 3 years after combined phacoemulsification and trabeculectomy in his right eye. His ocular history is significant for retinal detachment in the right eye that was surgically treated with a pars plana vitrectomy and scleral buckle 5 years prior to the current presentation and 1 year prior to his combined phacoemulsification-trabeculectomy procedure. After trabeculectomy, he reportedly had a wound leak with hypotony and choroidal effusion. The patient was referred to a second surgeon who performed a bleb revision with a pericardial patch graft, but the patient had a severe intraocular pressure (IOP) spike in the immediate postoperative period requiring suture removal. This resulted in recurrent hypotony with maculopathy. A second bleb revision with pericardial patch graft was performed but was not successful in raising the IOP out of the single digits or in resolving the maculopathy. Accordingly, the patient was referred for further assessment. At presentation, the patient's corrected distance visual acuity was 20/100 in the right eye and 20/20 in the left eye. Applanation tonometry IOP was 4 mm Hg and 16 mm Hg for the right and left eyes, respectively. Central corneal thickness was 609 µm in the right eye and 574 µm in the left eye. The right pupil was noted to be slightly irregular with a relative afferent pupillary defect. Slitlamp examination of the right eye was notable for a moderately elevated, Seidel test-negative bleb, and deep anterior chamber. The left eye had a Krukenberg spindle and mild nuclear sclerotic cataract but was otherwise unremarkable. Fundus examination of the right eye was notable for significant macular folds with edematous nerve fiber layer (NFL) and optic nerve. The left optic nerve and fundus examination were unremarkable. Gonioscopy revealed open angles with dense trabecular meshwork pigment in both eyes. There was a nicely patent superior sclerostomy in the right eye. There was no cyclodialysis cleft. Optical coherence tomography (OCT) of the macula showed chorioretinal folds and NFL edema in the right eye and was normal in the left eye (Figure 1JOURNAL/jcrs/04.03/02158034-202101000-00022/figure1/v/2021-01-04T143903Z/r/image-tiff). The OCT of the optic nerve in the right eye was distorted secondary to the chorioretinal folds, whereas the left eye was notable for mild superior thinning of the NFL (Figure 2JOURNAL/jcrs/04.03/02158034-202101000-00022/figure2/v/2021-01-04T143903Z/r/image-tiff). Humphrey visual field revealed a superior nasal step in the right eye (Figure 3JOURNAL/jcrs/04.03/02158034-202101000-00022/figure3/v/2021-01-04T143903Z/r/image-tiff). Axial lengths were 25.33 mm and 26.53 mm in the right and left eye, respectively; it is notable that the right eye had a shorter axial length despite the scleral buckle, which demonstrates the degree of axial shortening from hypotony (Figure 4JOURNAL/jcrs/04.03/02158034-202101000-00022/figure4/v/2021-01-04T143903Z/r/image-tiff). What would be your approach for managing this patient's hypotony? Does the fact that the referring surgeon had performed 2 failed bleb revision procedures prior to referral influence your approach? Given that a previous attempt at revision resulted in an extreme IOP spike would you also recommend a glaucoma procedure? If so, what procedure would you perform? Would you do it coincident with the revision or in a staged procedure later on an as-needed basis?

Simultaneous Presentation of Uveitic Disc Edema and Papilledema in an Adult.

PURPOSE: To present a case of simultaneous uveitic disc edema and increased intracranial pressure (IICP) in an adult. METHODS: Retrospective case report. Patients. A 29-year-old woman affected by bilateral optic disc edema from bilateral posterior uveitis complicated by IICP with papilledema. RESULTS: Laboratory workup was negative for infectious and systemic inflammatory causes of uveitis. Computed Tomography scan of the chest was negative for Sarcoidosis. Magnetic Resonance Imaging of the brain and orbits revealed a partially empty sella, bilateral posterior globe flattening without optic nerve sheath enhancement, masses, white matter lesions, or meningeal enhancement. Cerebral Magnetic Resonance Venography showed narrowing of the right and left transverse sinuses without thromboses. Prednisone was initiated for the uveitis which improved the vision but caused weight gain. Neurology evaluation with a lumbar puncture in the lateral decubitus position revealed elevated opening pressure and otherwise normal cerebrospinal fluid. Ocular ultrasonography was considered but not available to measure optic nerve sheath diameter. Oral acetazolamide 1000 mg twice daily was started for papilledema as prednisone was tapered. Periocular steroid and intravitreal bevacizumab injections were used for sight threatening cystoid macular edema and choroidal neovascularization, respectively. Discussion. While previously described in children, we report the first known case of bilateral uveitic disc edema and papilledema in an adult. This report will discuss recommendations for evaluation of these rarely concurrent conditions and therapy for both uveitic disc edema and papilledema.

Intraorbital Graphite Foreign Body With a Delayed Presentation of Optic Neuropathy.

Traumatic penetrating injuries to the orbit from pencils, while uncommon, have a plethora of presentations, both acute and delayed. With the most incidents occurring in the pediatric population where obtaining a detailed history is difficult, the ability to effectively evaluate and diagnose these injuries is cumbersome, yet important. The authors report a patient who presented with optic neuropathy, blepharoptosis, and strabismus 10 months after an orbital injury with pencil graphite.

Assessing the Impact of Diabetes Mellitus on Donor Corneal Endothelial Cell Density.

PURPOSE: To quantify changes in endothelial cell density (ECD) of donor corneal tissue in relation to the presence or absence of a medical history of diabetes mellitus diagnosis, treatment, and complications. METHODS: A retrospective review was performed for all corneas collected at Iowa Lions Eye Bank between January 2012 and December 2015. For purposes of analysis, donor corneas were divided into 4 groups: nondiabetic, non-insulin-dependent diabetic, insulin-dependent diabetic without medical complications due to diabetes, and insulin-dependent diabetic with medical complications due to diabetes. ECD values (obtained through specular microscopy) and transplant suitability for endothelial transplantation (determined by the standard protocol of the eye bank) were compared among groups using linear mixed model analysis. RESULTS: In total, 4185 corneas from 2112 donors were included for analysis. Insulin-dependent diabetic samples with medical complications due to diabetes (N = 231 from 119 donors) showed lower ECD values compared with nondiabetic samples (-102 cells/mm, P = 0.049) and non-insulin-dependent diabetic samples (-117 cells/mm, P = 0.031). ECD values did not differ significantly among the remaining groups. The likelihood of suitability for endothelial transplantation did not differ among all 4 groups. CONCLUSIONS: Corneas from donors with insulin-dependent diabetes mellitus and medical complications resulting from the disease have lower mean ECD values compared with other donors. However, our analysis suggests that these corneas are equally likely to be included in the donor pool for corneal transplantation. Additional studies are needed to determine the mechanism(s) contributing to cell loss in donors with advanced diabetes and to assess associated endothelial cell functional impairment.

Regional assessment of energy-producing metabolic activity in the endothelium of donor corneas.

PURPOSE: We characterized mitochondrial respiration and glycolysis activity of human corneal endothelium, and compared metabolic activity between central and peripheral regions. METHODS: Endothelial keratoplasty-suitable corneas were obtained from donors aged 50 to 75 years. The endothelium-Descemet membrane complex (EDM) was isolated, and 3-mm punches were obtained from central and peripheral regions. Endothelium-Descemet membrane punches were assayed for mitochondrial respiration (oxygen consumption) and glycolysis (extracellular acidification) using an extracellular flux analyzer. Enzymatic (citrate synthase, glucose hexokinase) and mitochondrial density (MitoTracker) assays also were performed. RESULTS: Ten corneas were analyzed per assay. Metabolic activity for mitochondrial respiration and glycolysis showed expected changes to assay compounds (P < 0.01, all pairwise comparisons). Basal mitochondrial respiration and glycolysis activity did not differ between regions (P > 0.99). Similarly, central versus peripheral activity after assay compound treatment showed no significant differences (P > 0.99, all time points). The intracorneal coefficient of variation for basal readings between two and four peripheral punches was 18.5% of the mean. Although peripheral samples displayed greater enzymatic activity than central samples (P < 0.05), similar to extracellular flux results, mitochondrial density did not differ between regions (P = 0.78). CONCLUSIONS: Extracellular flux analysis of oxygen and pH is a valid technique for characterizing metabolic activity of human corneal endothelium. This technique demonstrates high reproducibility, allows quantification of metabolic parameters using small quantities of live cells, and permits estimation of overall metabolic output. Neither oxygen consumption nor extracellular acidification differed between central and peripheral regions of transplant suitable corneas in this series. Our results show that endothelial cell health can be quantified biochemically in transplant suitable corneas.

Graft survival of diabetic versus nondiabetic donor tissue after initial keratoplasty.

PURPOSE: To compare corneal graft survival using tissue from diabetic and nondiabetic donors in patients undergoing initial Descemet stripping automated endothelial keratoplasty (DSAEK) or penetrating keratoplasty (PKP). METHODS: A retrospective chart review of pseudophakic eyes that underwent DSAEK or PKP was performed. The primary outcome measure was graft failure. Cox proportional hazard regression and Kaplan-Meier survival analyses were used to compare diabetic versus nondiabetic donor tissue for all keratoplasty cases. RESULTS: A total of 183 eyes (136 DSAEK, 47 PKP) were included in the statistical analysis. Among 24 procedures performed using diabetic donor tissue, there were 4 cases (16.7%) of graft failure (3 DSAEK, 1 PKP), and among 159 procedures performed using nondiabetic donor tissue, there were 18 cases (11.3%) of graft failure (12 DSAEK, 6 PKP). Cox proportional hazard ratio of graft failure for all cases comparing diabetic with nondiabetic donor tissue was 1.69, but this difference was not statistically significant (95% confidence interval, 0.56-5.06; P = 0.348). There were no significant differences in Kaplan-Meier curves comparing diabetic with nondiabetic donor tissue for all cases (P = 0.380). Statistical analysis of graft failure by donor diabetes status within each procedure type was not possible because of the small number of graft failure events involving diabetic tissue. CONCLUSIONS: We found similar rates of graft failure in all keratoplasty cases when comparing tissue from diabetic and nondiabetic donors, but further investigation is needed to determine whether diabetic donor tissue results in different graft failure rates after DSAEK compared with PKP.