Cytomegalovirus as a cause of recurrent corneal endotheliitis in the Canadian population.

OBJECTIVE: To report the clinical manifestations, response to antiviral treatment, and long-term visual outcomes of cytomegalovirus endotheliitis in a Canadian population. DESIGN: Retrospective case series. PARTICIPANTS: A total of 9 eyes of 7 patients referred to a cornea subspecialty clinic in a major Canadian centre with corneal endotheliitis. METHODS: A retrospective review of all patients presenting with corneal endotheliitis to 1 corneal surgeon was completed. Patients underwent anterior chamber biopsy with positive cytomegalovirus polymerase chain reaction. All patients received systemic valganciclovir for a minimum of 3 months. Primary outcomes included visual acuity, intraocular pressure control, medication dependence, and corneal status. RESULTS: The average follow-up was 76.4 ± 11.8 months. Two patients had bilateral disease. Corneal manifestations included linear, disciform, and circinate patterns of endotheliitis. Best-corrected visual acuity improved from a mean of 0.48 ± 0.19 logMAR at presentation to 0.24 ± 0.11 logMAR at last follow-up. Intraocular pressure decreased from a peak of 35 ± 3.1 mm Hg to 14.2 ± 4.3 mm Hg. Antiglaucoma medications were reduced from 2.6 ± 0.45 to 0.89 ± 0.29 agents. Two eyes required endothelial transplantation. Valganciclovir therapy was well tolerated by all patients; at the time of last follow-up, all patients were stable on low-dose valganciclovir at an average dose of 1395 mg per week. CONCLUSIONS: Cytomegalovirus is an uncommon but clinically significant cause of corneal endotheliitis that must be considered in the differential diagnosis of corneal endotheliitis, even in the immunocompetent population. Our results support prior findings that this entity responds robustly to oral valganciclovir and demonstrate for the first time the efficacy of chronic low-dose antiviral maintenance therapy.

A MULTICENTER REVIEW EVALUATING THE RISK OF RHEGMATOGENOUS RETINAL DETACHMENT POST ENDOPHTHALMITIS: A Canadian Retina Research Network Investigation.

PURPOSE: To describe the characteristics of rhegmatogenous retinal detachments (RDs) that developed after endophthalmitis and to provide insight regarding incidence, management, and outcomes. METHODS: Retrospective chart review of consecutive patients diagnosed with endophthalmitis at two Canadian institutions and from one surgeon at a third center over a 10-year period. RESULTS: Three hundred sixteen eyes presented with endophthalmitis of which 32 (10%) were complicated by subsequent RD. The mean age (SD) of patients was 72.0 (13.77) years. Intravitreal injections (33%) and cataract surgery (31%) were the most common inciting events for the endophthalmitis. Cases were managed with tap and inject (16%), vitrectomy (30%), or both (47%). There were no differences in the rate of RD when comparing the inciting events, microbial growth, or management approaches. Baseline visual acuity was the only predictor of RD ( P = 0.009). There was an improvement in visual acuity following management of the RD (logarithm of the minimum angle of resolution 2.33 vs. 1.69 or Snellen hand motion vs. 20/980, P = 0.009), but final visual acuity in these patients was worse than in those who did not develop RD (logarithm of the minimum angle of resolution 1.69 vs. 1.27 or Snellen 20/980 vs. 20/372, P = 0.029). CONCLUSION: All patients with RD post endophthalmitis required additional surgery, and functional outcomes remained poor. Nevertheless, repair of these RDs can preserve some vision and be globe saving.

OUTCOMES OF VITRECTOMY IN TERSON SYNDROME: A Multicenter Canadian Perspective.

PURPOSE: To characterize the presentation of Terson syndrome, the occurrence of a vitreous hemorrhage in association with intracranial hemorrhage, and report on the outcomes of vitrectomy at two major centers in Canada. METHODS: Retrospective chart review of consecutive patients with Terson syndrome undergoing vitrectomy by retina specialists over the last 10 years. Primary outcome was the change in best-corrected visual acuity (BCVA) at 3 months from baseline. Secondary outcomes included the association between baseline BCVA and final BCVA, and the association between final BCVA and timing of surgery (early vs. later than 90 days). RESULTS: A total of 14 eyes of 11 patients were included. The mean time between observation of intraocular hemorrhage and vitrectomy was 160 days. Baseline preoperative BCVA was logarithm of the minimum angle of resolution 1.57 ± 1.03 (Snellen 20/740), which improved to logarithm of the minimum angle of resolution 0.53 ± 0.82 (Snellen 20/70) at the final postoperative follow-up, P = 0.01. Baseline BCVA was not significantly correlated with final BCVA, Spearman's rho = 0.016, P = 0.957. Final BCVA did not significantly differ between those who had surgery before 90 days compared with after 90 days, P = 0.087. CONCLUSION: Vitrectomy is safe and effective and should be considered for nonclearing vitreal bleeding due to Terson syndrome. Ocular hemorrhaging in Terson syndrome can be observed conservatively for spontaneous improvement without the risk of reduced visual potential. Ophthalmic evaluation should be considered promptly after intracranial hemorrhage.

Risk of Stroke in Patients With Ocular Arterial Occlusive Disorders: A Retrospective Canadian Study.

Background Monocular vision loss, attributed to either central retinal artery occlusion ( CRAO ), branch retinal artery occlusion ( BRAO ), or ocular ischemic syndrome ( OIS ), is thought to be associated with an increased prevalence of cerebral infarcts. However, there is a paucity of data substantiating this. We aimed to investigate this relationship in a Canadian center and further understand the importance of associated internal carotid artery stenosis in potential clinical decision making. Methods and Results We performed a retrospective cohort study at a comprehensive stroke center of patients presenting initially with CRAO , BRAO , or OIS to a centralized ophthalmology center over a 5-year period. Patients were followed for 3 years for the occurrence of a hemispheric stroke. We identified 83 affected eyes, with 31 CRAO , 35 BRAO , and 17 OIS patients. Before ocular diagnosis, 32.3%, 11.4%, and 41.2% of CRAO , BRAO , and OIS patients, respectively, experienced a symptomatic stroke. Of the remaining patients, 4.8%, 12.9%, and 40%, respectively, suffered a hemispheric stroke within 3 years of ocular diagnosis. Logistic regressions suggested that for CRAO and BRAO patients together, the degree of ipsilateral internal carotid artery stenosis is unable to predict the occurrence of a stroke ( P=0.18), whereas our model correctly predicted a stroke in 82.4% of OIS patients ( P=0.005). Conclusions CRAO , BRAO , and OIS are associated with significantly increased symptomatic stroke rates. Degree of ipsilateral internal carotid artery stenosis may not be useful in risk stratification for these patients, suggesting that they should be triaged appropriately for stroke risk-factor management, independent of internal carotid artery stenosis.

Success Rates for Retinal Detachment Repair in Alberta: A Physician Learning Program Initiative.

OBJECTIVE: To identify primary surgical success rates for retinal detachment repair in Alberta and compare functional outcomes of methods of repair. METHODS: Data was retrospectively extracted from the Alberta Health Services Discharge Abstract Database and the National Ambulatory Care Reporting System for all patients diagnosed with retinal detachment and vitreoretinal procedures during the 2008/09 to 2012/13 fiscal years. RESULTS: Of the 5,433 surgeries for retinal detachment identified, 279 were excluded due to invalid provincial health numbers, unidentified procedure location, and/or treating physician other than an Alberta retina surgeon. The final analysis included 4,336 detachments in 4,020 patients. The average primary retinal detachment success rate was 84.9% (3,680/4,336). Primary success rates varied between vitrectomy only (84.9%, 2,149/2,532), vitrectomy and scleral buckle (85.5%, 818/957), and scleral buckle (84.4%, 702/832). CONCLUSIONS: Alberta retina surgeons have an average primary success rate of 84.9% (3,680/4,336) for repair of retinal detachments. This result is in keeping with other published retinal detachment success rate studies.

Ocriplasmin versus vitrectomy for the treatment of macular holes.

OBJECTIVE: To compare the anatomical and visual outcomes of patients with bilateral macular holes (MH) who have been treated with pars plana vitrectomy in one eye and intravitreal ocriplasmin in the fellow eye. DESIGN: Multicentre, retrospective case series. PARTICIPANTS: Twenty-two eyes of 11 patients with bilateral MH treated with vitrectomy in one eye and ocriplasmin in the other were included. Patients were followed-up by 5 vitreoretinal surgeons from 3 retinal practices in Canada. METHODS: All charts were reviewed for data collection, and optical coherence tomography (OCT) scans pre- and posttreatment were evaluated. RESULTS: MH closed primarily in 36.4% (n = 4) of the ocriplasmin-treated eyes and in 90.9% (n = 10) of the vitrectomy-treated eyes (p = 0.031). The 4 successfully treated ocriplasmin MH were preceded by a vitreomacular traction (VMT) release. Three additional ocriplasmin-treated eyes achieved a VMT release without MH closure. All persistent MH (100%) closed with subsequent vitrectomy, with no significant difference in final best-corrected visual acuity (BCVA) between those who achieved MH closure with primary or secondary interventions (p = 0.073). Final BCVA improved from logMAR 0.85 ± 0.34 to 0.37 ± 0.22 (p = 0.005) in the vitrectomy eyes and from 0.56 ± 0.28 to 0.28 ± 0.16 (p = 0.009) in the ocriplasmin eyes, with no significant difference in final BCVA between treatments (p = 0.306). Postoperative ellipsoid zone disruption persisted more frequently in vitrectomy-treated eyes. CONCLUSION: Both procedures were associated with improved visual outcomes, but eyes initially treated with vitrectomy had a higher primary MH closure rate. On OCT, patients had more outer structural changes in vitrectomy eyes than in ocriplasmin eyes.

Lid splinting eyelid retraction technique: a minimised sterile approach for intravitreal injections.

BACKGROUND/AIMS: To describe an alternative technique for avoiding contact with the lids and lashes, without the use of a lid speculum, during intravitreal anti-vascular endothelial growth factor injections. METHODS: Retrospective case series of all patients undergoing intravitreal injections of bevacizumab and ranibizumab, with the lid splinting retraction technique from January 2010 to December 2015. Injections performed by six vitreoretinal specialists were included. The key preinjection ocular surface preparation includes topical anaesthetic, 5% povidone-iodine and a subconjunctival injection of 2% lidocaine with epinephrine. A second instillation of 5% povidone-iodine is given and the intravitreal injection is then performed. No lid speculum is used. A search of the electronic medical records identified patients diagnosed with postinjection endophthalmitis and charts were reviewed to ensure inclusion criteria were met. The main outcome measure was incidence of postinjection endophthalmitis. RESULTS: A total of 78 009 consecutive intravitreal injections were performed, of which 22 207 were bevacizumab and 55 802 were ranibizumab. In this cohort of patients (n=6320), 12 cases of endophthalmitis developed, corresponding to a rate of 0.015%. CONCLUSIONS: The technique of eyelid retraction for intravitreal injection has a low rate of endophthalmitis, similar to the reported rates using a metal lid speculum. This is beneficial for both the physician and the patient as it minimises patient discomfort as well as the duration of the procedure. To our knowledge, this is one of the largest studies performed to date evaluating intravitreal injection-related endophthalmitis.

TUBA1A Mutation Associated With Eye Abnormalities in Addition to Brain Malformation.

OBJECTIVE: We describe the case of a boy with a TUBA1A mutation presenting with microphthalmia and congenital cataracts in addition to microcephaly and severe brain malformation. METHODS: A boy presented in early infancy with microphthalmia, congenital cataracts, and microcephaly. His neurological course included severe hypotonia and drug-resistant epilepsy. Magnetic resonance imaging of the brain revealed a complex malformation that included agenesis of the corpus callosum, severely hypoplastic cerebellar vermis, mildly hypoplastic and dysplastic cerebellar hemispheres, mildly hypoplastic brainstem, mild posterior simplified cerebral gyral pattern, dysplastic basal ganglia and thalami, hypoplastic optic nerves, and absent olfactory bulbs. RESULTS: TUBA1A genetic testing was conducted and revealed a previously unreported heterozygous 808G>T missense mutation. Parental genetic testing was negative, indicating that the child's mutation was de novo. CONCLUSION: The TUBA1A gene encodes tubulin alpha-1A, a protein with an important role in microtubule function and stability. Human mutations can result in a wide spectrum of brain malformations including lissencephaly, microlissencephaly, cerebellar hypoplasia, agenesis of the corpus callosum, pachygyria and polymicrogyria. Although TUBA1A is expressed in both developing brain and retinal tissue, there are no reported cases of TUBA1A mutations in association with major developmental ophthalmologic abnormalities.

Real-world assessment of intravitreal dexamethasone implant (0.7 mg) in patients with macular edema: the CHROME study.

BACKGROUND: The purpose of this study was to evaluate the real-world use, efficacy, and safety of one or more dexamethasone intravitreal implant(s) 0.7 mg (DEX implant) in patients with macular edema (ME). METHODS: This was a retrospective cohort study of patients with ME secondary to retinal disease treated at ten Canadian retina practices, including one uveitis center. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), intraocular pressure (IOP), glaucoma and cataract surgery, and safety data were collected from the medical charts of patients with ≥3 months of follow-up after the initial DEX implant. RESULTS: One hundred and one patient charts yielded data on 120 study eyes, including diagnoses of diabetic ME (DME) (n=34), retinal vein occlusion (RVO, n=30; branch in 19 and central in 11), and uveitis (n=23). Patients had a mean age of 60.9 years, and 73.3% of the study eyes had ME for a duration of ≥12 months prior to DEX implant injection(s). Baseline mean (± standard error) BCVA was 0.63±0.03 logMAR (20/86 Snellen equivalents) and mean CRT was 474.4±18.2 µm. The mean number of DEX implant injections was 1.7±0.1 in all study eyes; 44.2% of eyes had repeat DEX implant injections (reinjection interval 2.3-4.9 months). The greatest mean peak changes in BCVA lines of vision occurred in study eyes with uveitis (3.3±0.6, P<0.0001), followed by RVO (1.3±0.5, P<0.01) and DME (0.7±0.5, P>0.05). Significant decreases in CRT were observed: -255.6±43.6 µm for uveitis, -190.9±23.5 µm for DME, and -160.7±39.6 µm for RVO (P<0.0001 for all cohorts). IOP increases of ≥10 mmHg occurred in 20.6%, 24.1%, and 22.7% of DME, RVO, and uveitis study eyes, respectively. IOP-lowering medication was initiated in 29.4%, 16.7%, and 8.7% of DME, RVO, and uveitis study eyes, respectively. Glaucoma surgery was performed in 1.7% of all study eyes and cataract surgery in 29.8% of all phakic study eyes receiving DEX implant(s). CONCLUSION: DEX implant(s) alone or combined with other treatments and/or procedures resulted in functional and anatomic improvements in long-standing ME associated with retinal disease.

Optimal Treatment of Retinal Vein Occlusion: Canadian Expert Consensus.

BACKGROUND: The availability of new therapeutic approaches, particularly intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapies, has prompted significant changes to the established treatment paradigms for retinal vein occlusion (RVO). Better visual outcomes and significantly lower rates of adverse events have been noted in multiple large randomized clinical trials and have led to a new standard of care for this sight-threatening condition. OBJECTIVE: To develop an expert consensus for the management of RVO and associated complications in the context of recent clinical evidence. METHODS: The development of a Canadian expert consensus for optimal treatment began with a review of clinical evidence, daily practice, and existing treatment guidelines and algorithms. The expert clinicians (11 Canadian retina specialists) met on February 1, 2014, in Toronto to discuss their findings and to propose strategies for consensus. RESULTS: The result of this expert panel is a consensus proposal for Canadian ophthalmologists and retina specialists treating patients presenting with RVO. Treatment algorithms specific to branch and central RVO (BRVO and CRVO) were also developed. CONCLUSIONS: The consensus provides guidelines to aid clinicians in managing RVO and associated complications in their daily practice. In summary, laser remains the therapy of choice when neovascularization secondary to RVO is detected. Adjunctive anti-VEGF could be considered in managing neovascularization secondary to RVO in cases of vitreous hemorrhage. Intravitreal anti-VEGF should be considered for symptomatic visual loss associated with center-involving macular edema on optical coherence tomography. Patients with BRVO and a suboptimal response to anti-VEGF could be treated with grid laser, and those with CRVO and an inadequate response to anti-VEGF may be candidates for intravitreal steroids.

LONG-TERM OUTCOMES FOR OPTIC DISK PIT MACULOPATHY AFTER VITRECTOMY.

PURPOSE: To evaluate the efficacy of pars plana vitrectomy for congenital optic disk pit maculopathy with various adjuvant techniques, including gas tamponade, internal limiting membrane peel, and temporal optic disk endolaser in a multicenter study with long-term follow-up. METHODS: A retrospective chart review was performed to identify eyes that underwent surgical repair for congenital optic disk pits and serous macular detachment with or without macular retinoschisis from four retinal centers across Canada from 2003 to 2013. Data collected included surgeries performed, preoperative and postoperative vision, central retinal thickness, and presence or absence of subretinal fluid. Optical coherence tomography was used to define anatomical success (i.e., foveal reattachment). RESULTS: Thirty-two eyes of 32 patients with optic disk pits and serous macular detachments were identified that had undergone surgical repair. All eyes underwent pars plana vitrectomy and induction of posterior vitreous detachment if one was not present. Additional procedures performed on occasion included internal limiting membrane peel (n = 8), temporal optic disk pits endolaser (n = 7), and gas tamponade (air, C3F8 or SF6; n = 31). After vitrectomy surgery, foveal attachment was achieved in 26 of 32 eyes (81.3%). The average number of surgeries required was 1.4 ± 0.6, with a maximum of 3 vitrectomies (n = 2). Mean change in best-corrected visual acuity was -0.47 ± 0.54 logMAR units, which corresponds to approximately 5 lines of visual improvement (P < 0.001). Median time to reattachment was 416 days. Preoperative vision, preoperative symptom days, and age were not associated with postoperative reattachment. Similarly, internal limiting membrane peel and temporal endolaser were not associated with postoperative reattachment, nor was there a difference between air and SF6 and C3F8 gas tamponade. Elevated preoperative central retinal thickness was associated with a lower chance of postoperative reattachment (P = 0.007) and was also the best prognostic indicator of success (P = 0.039). CONCLUSION: Vitrectomy for macular detachment due to optic disk pit has good long-term success and results in an improvement in visual acuity. However, adjuvant techniques such as internal limiting membrane peel and temporal endolaser may not improve outcomes, nor does there seem to be a difference between short- and long-acting gases. Patients should be made aware that it can take more than a year and multiple surgeries to achieve foveal reattachment and that increased baseline central retinal thickness is a poor prognostic sign.